Module 3

 

The following reading list is recommended to prepare candidates for Module 3 of the

Postdoctoral Neurology Diplomate program.

Peripheral Neuropathy, 2nd edition, Dyck, Thomas, Lambert, Bunge: W.B. Saunders

Company

Structure and Function of the Peripheral Nervous System Pgs. 9-11

Gross Anatomy of the Peripheral Nervous System Pgs. 11-39

The Muscle Spindle Pgs. 171-203

The Golgi Tendon Organs Pgs. 203-210

Compression and Entrapment Neuropathies Pgs. 1435-1458

 

The following bibliography has been prepared by Professor F. R. Carrick to compliment his

lecture on Module 3.

 

Bibliography

 

1. Abramowicz, M. J.; Cochaux, P.; Cohen, L. H.; Vamos, E. Pernicious anaemia and

hypoparathyroidism in a patient with Kearns-Sayre syndrome with mitochondrial

DNA duplication. J-Inherit-Metab-Dis. 1996; 19(2): 109-11; ISSN: 0141-8955.

NETHERLANDS. 0.

2. Ahlgren, B. D.; Garfin, S. R. Cervical Radiculopathy. Orthop-Clin-North-Am. 1996

Apr; 27(2): 253-63; ISSN: 0030-5898.

UNITED-STATES. This article discusses the relevant anatomy, clinical

presentation, diagnosis and surgical treatment for cervical radiculopathy. The

etiology of cervical radiculopathy can play a role in the subsequent treatment of this

problem. Both anterior and posterior surgical management is discussed.

3. Akeyson, E. W.; McCutcheon, I. E.; Pershouse, M. A.; Steck, P. A.; Fuller, G. N.

Primitive neuroectodermal tumor of the median nerve. Case report with cytogenetic

analysis. J-Neurosurg. 1996 Jul; 85(1): 163-9; ISSN: 0022-3085.

UNITED-STATES. The authors describe a malignant peripheral primitive

neuroectodermal tumor (PNET) that originated in the median nerve in an elderly

adult. After the diagnosis was made by biopsy, the patient underwent radical local

resection with interpositional vein grafting of the brachial artery. The tumor had the

typical appearance of a primitive neural tumor with small, round cells forming

rosettes. It stained positively for both the Ewing's sarcoma/peripheral PNET

antigen (HBA-71) and neuron-specific enolase, confirming its neural origin.

Ultrastructural examination revealed dense core granules and suggested neural

differentiation of the neoplasm. Cytogenetic analysis suggested a chromosome

(11;22) translocation typical of peripheral PNET. Early reports consisted of tumors

arising solely in peripheral nerves, but recent series have focused mainly on tumors

arising in the soft tissues other than nerves. There are no other cases of true PNET

of peripheral nerve in the modern literature that have been fully characterized by

immunohistochemical, ultrastructural, and cytogenetic criteria. Although peripheral

PNETs occur more commonly in children, this unusual neoplasm should be

considered in the differential diagnosis of peripheral nerve neoplasms in adults.

Early diagnosis is desirable because of its aggressive nature and poor outcome.

4. Akhadov, T. A.; Grachev, I. u. V. [Magnetic resonance tomography of the brain in a

lesion of the trigeminal system]. Magnitno-rezonansnaia tomografiia golovnogo

mozga pri porazhenii trigeminal'noi sistemy. Zh-Nevropatol-Psikhiatr-Im-S-S-

Korsakova. 1996; 96(1): 70-4; ISSN: 0044-4588.

RUSSIA. 26 patients with trigeminal disorders were examined in terms of

magnetic resonance tomography. It was established that the trigeminal disorders

occurred to be secondary and symptomatic in all patients. They were caused by

various cranial and cerebral pathology mainly by voluminous changes of basal

localization. The results of investigation testified that the magnetic resonance

tomography application in atypical course of trigeminal pathology was quite useful

to avoid different diagnostic mistakes and to create the most effective treatment

strategy.

5. Akiya, S.; Nishio, Y.; Ibi, K.; Uozumi, H.; Takahashi, H.; Hamada, T.; Onishi, A.;

Ishiguchi, H.; Hoshii, Y.; Nakazato, M. Lattice corneal dystrophy type II

associated with familial amyloid polyneuropathy type IV. Ophthalmology. 1996

Jul; 103(7): 1106-10; ISSN: 0161-6420.

UNITED-STATES. BACKGROUND: Finnish-type familial amyloidosis (FAP-

IV) is an autosomal, dominantly inherited disorder characterized by progressive

polyneuropathy and lattice corneal dystrophy type II. The vast majority of families

with this disorder originated from Finland. Only two families, in neighboring

districts, have been reported in Japan previously. METHODS: The authors report

two additional Japanese patients with FAF-IV. The proband, a 70-year-old man,

had decreased perspiration and abnormal facial muscle movement. Results of

neurologic examination showed bilateral facial and hypoglossal nerve palsies, and

an autonomic disturbance, including orthostatic hypotension and dysfunction of

perspiration. Histochemical, immunohistological, and DNA studies confirmed the

diagnosis of FAP-IV. RESULTS: Results of ophthalmologic examination showed

asymptomatic lattice corneal dystrophy of both eyes, but the appearance of the

cornea was different from that described in the patients from Finland. Lattice lines

in the authors' patient were very fine, short, and glassy and could be observed with

indirect retroillumination, but might be missed with direct illumination by the slit-

lamp microscope. The proband's younger half-sister, a 68-year-old woman,

showed clinical findings and laboratory data similar to those of the proband.

CONCLUSION: The authors report two Japanese patients with lattice corneal

dystrophy type II related to FAP-IV. This is the third Japanese family with this

disorder, and there is no familial relationship to the two previously reported

families in Japan.. 0; 0.

6. Al Eissa, Y. A.; Al Herbish, A. S. Severe hypertension: an unusual presentation of

Guillain-Barre syndrome in a child with brucellosis. Eur-J-Pediatr. 1996 Jan;

155(1): 53-5; ISSN: 0340-6199.

GERMANY. A 9-year-old girl suffered from protracted paroxysms of severe

hypertension before she developed classical signs of Guillain-Barre syndrome.

Significant brucella antibody titres were found in the serum and CSF. Complete

recovery was observed after appropriate therapy. CONCLUSION: Brucella

organisms should be sought as a possible aetiological agent of Guillain-Barre

syndrome in patients living in or coming from endemic countries.

7. al Qattan, M. M.; Thomson, H. G.; Clarke, H. M. Carpal tunnel syndrome in children

and adolescents with no history of trauma. J-Hand-Surg-Br. 1996 Feb; 21(1): 108-

11; ISSN: 0266-7681.

SCOTLAND. Four cases of carpal tunnel syndrome in children and adolescents

with no history of trauma are discussed. The pertinent literature and a classification

of the different causes of carpal tunnel syndrome in this age group is presented.

8. Albeck, M. J. A critical assessment of clinical diagnosis of disc herniation in patients

with monoradicular sciatica. Acta-Neurochir-Wien. 1996; 138(1): 40-4; ISSN:

0001-6268.

AUSTRIA. The diagnostic power or clinical parameters in the diagnosis of lumbar

disc herniation in patients with monoradicular pain was evaluated in a prospective

study with a 100% verification of the diagnosis. Eighty patients with monoradicular

pain corresponding to the fifth lumbar or the first sacral nerve root were included.

Pre-operatively a number of clinical parameters were recorded and compared to the

intra-operative finding of a disc herniation. The parameters were analysed by

receiver operating characteristic (ROC) curves. Results from the available literature

were analysed by ROC curves for comparison. In 76% of the cases a disc

herniation was discovered. The level of the disc herniation was correctly predicted

in 93% of these cases by the location of the pain alone or supplemented by

neurological signs. Apart from radicularly distributed pain, all parameters in the

present study and in the literature had no or low diagnostic accuracy. Thus, in

patients with monoradicular sciatica further clinical parameters do not add to the

diagnosis of lumbar disc herniation.

9. Alevizon, S. J.; Finan, M. A. Sacrospinous colpopexy: management of postoperative

pudendal nerve entrapment. Obstet-Gynecol. 1996 Oct; 88(4 Pt 2): 713-5; ISSN:

0029-7844.

UNITED-STATES. BACKGROUND: Sacrospinous colpopexy requires the

placement of a suture through the sacrospinous ligament, under which lies the

pudendal nerve. Entrapment of this nerve may result in perineal or buttock pain.

This report analyzes a case of pudendal nerve entrapment and studies the

management of the resultant neuropathy. CASE: A 62-year-old patient experienced

a chronic pudendal neuropathy with perineal and buttock pain following a

sacrospinous colpopexy. Removal of the suture, 2 years after it was originally

placed, resulted in immediate relief of her pain. She has been asymptomatic for 1

year following surgery. CONCLUSION: Pudendal nerve entrapment should be

considered in the differential diagnosis of perineal or buttock pain after

sacrospinous colpopexy. Relief of pain can be achieved even 2 years after initial

surgery.

10. Alevizon, S. J.; Finan, M. A. Sacrospinous colpopexy: management of postoperative

pudendal nerve entrapment. Obstet-Gynecol. 1996 Oct; 88(4 Pt 2): 713-5; ISSN:

0029-7844.

UNITED-STATES. BACKGROUND: Sacrospinous colpopexy requires the

placement of a suture through the sacrospinous ligament, under which lies the

pudendal nerve. Entrapment of this nerve may result in perineal or buttock pain.

This report analyzes a case of pudendal nerve entrapment and studies the

management of the resultant neuropathy. CASE: A 62-year-old patient experienced

a chronic pudendal neuropathy with perineal and buttock pain following a

sacrospinous colpopexy. Removal of the suture, 2 years after it was originally

placed, resulted in immediate relief of her pain. She has been asymptomatic for 1

year following surgery. CONCLUSION: Pudendal nerve entrapment should be

considered in the differential diagnosis of perineal or buttock pain after

sacrospinous colpopexy. Relief of pain can be achieved even 2 years after initial

surgery.

11. Alhan, H. C.; Cakalagaoglu, C.; Hanci, M.; Toraman, F.; Idiz, M.; Kayacioglu, I.;

Tarcan, S. Critical-illness polyneuropathy complicating cardiac operation. Ann-

Thorac-Surg. 1996 Apr; 61(4): 1237-9; ISSN: 0003-4975.

UNITED-STATES. Critical-illness polyneuropathy is a complication of septic

syndrome. However, this complication has been largely unrecognized in cardiac

surgery units. Difficulty in weaning from the ventilator is an important early

manifestation. Electromyography should be routinely performed to establish the

diagnosis. Here we report a case of polyneuropathy complicating surgical repair of

acute aortic dissection.

12. Allmann, K. H.; Horch, R.; Gabelmann, A.; Laubenberger, J.; Stark, G. B.; Langer,

M. [Morphology of the carpal tunnel. Movement studies in patients with

constriction symptoms and healthy probands using MR tomography]. Zur

Morphologie des Karpaltunnels. Bewegungsstudien bei Patienten mit

Engpassbeschwerden und gesunden Probanden mittels MR-Tomographie.

Unfallchirurgie. 1996 Feb; 22(1): 5-11; ISSN: 0340-2649.

GERMANY. The morphological correlation of the phenomenon of increased

pressure in the carpal tunnel during wrist flexion and extension--as has been proved

though measurements using wick-catheters--was studied in healthy subjects (n =

15) and symptomatic patients with carpal tunnel syndrome (n = 15). Our own

measurements using magnetic resonance imaging (MRI) showed that there is a

significant reproducible decrease in carpal tunnel diameter when the wrist is held in

position of either flexion or extension. During flexion the diameter is decreased at

the pisiformes and hamate level as well as it is lowered during extension at the

pisiformes level. This might explain the rise in carpal tunnel pressure and thus the

consecutive negative influence on the median nerve. Proximal swelling, distal

flattening and increased signal intensity of the median nerve as well as the palmar

bulging of the flexor retinaculum at the level of the hook of the hamate and at the

level of the pisiformes were significantly higher in patients with carpal tunnel

syndrome than in normal volunteers (from p < 0.05 to p < 0.001). In post-

operative follow-up examinations of 13 patients with no clinic symptoms the distal

flattening of the median nerve normalized in 94% within 3 months. The increased

signal of the median nerve on T2-weighted images decreased postoperatively in 2/3

of the patients, whereas the motor latency of the median nerve recovered only in

39% of our patients who had 100% partial or complete clinical benefit. These

findings imply that postoperative imaging may be helpful for evaluating the success

or failure of surgical treatment.

13. Almadori, G.; Del Ninno, M.; Cadoni, G.; Di Mario, A.; Ottaviani, F. Facial nerve

paralysis in acute otomastoiditis as presenting symptom of FAB M2, T8;21

leukemic relapse. Case report and review of the literature. Int-J-Pediatr-

Otorhinolaryngol. 1996 Jun; 36(1): 45-52; ISSN: 0165-5876.

IRELAND. Granulocytic sarcoma (chloroma) is a rare solid, extramedullary

tumour composed of immature granulocytes, occurring during granulocytic

leukemia. Leukemic involvement of the temporal bone is not uncommon and may

present in a variety of ways. Symptomatic facial nerve paralysis is one of these.

The authors report a case of facial nerve paralysis as the presenting symptom of

leukemic relapse in a 16-year-old white male, affected by acute myelogenous

leukemia FAB M2, karyotype 46xy, T8;21.

14. Alusi, G. H.; Grant, W. E.; Quiney, R. E. Oculopharyngeal myopathy with

sensorineural hearing loss. J-Laryngol-Otol. 1996 Jun; 110(6): 567-9; ISSN:

0022-2151.

ENGLAND. A case is reported of a 59-year-old Caucasian male with

oculopharyngeal myopathy and sensorineural hearing loss. He presented with

progressive ptosis, sensorineural hearing loss over several years and symptoms of

mild dysphagia. Further enquiry into his family history revealed that every male

member in his family that lived beyond the age of 60 exhibited identical symptoms.

Symptoms of ptosis and dysphagia are consistent with the rare autosomal dominant

condition of oculopharyngeal myopathy, believed to be due to mitochondrial

disease. The combination of ptosis, dysphagia and sensorineural hearing loss with

normal distant muscle group biopsy has not been described before.

15. Alvarez, E.; Ferrer, T.; Perez Conde, C.; Lopez Terradas, J. M.; Perez Jimenez, A.;

Ramos, M. J. Evaluation of congenital dysautonomia other than Riley-Day

syndrome. Neuropediatrics. 1996 Feb; 27(1): 26-31; ISSN: 0174-304X.

GERMANY. We report on four children, from different families, who suffer from

a congenital autonomic disorder, presumably inherited. Three of them have a

sensory neuropathy but do not fit any described hereditary sensory and autonomic

neuropathy. All four were examined along with some of their immediate family

members. We assessed the cardiovagal, sympathetic adrenergic and sympathetic

cholinergic functions with a battery of non-invasive tests. Results demonstrated that

sudomotor and cardiovascular orthostatic regulation exhibited the greatest

abnormalities, pointing to a predominant impairment of sympathetic components,

both cholinergic and adrenergic. The overall examination showed a heterogeneous

group of congenital dysautonomia, exclusive of Riley-Day or other recognized

hereditary sensory and autonomic neuropathies. We emphasize the importance of

studying whole family groups to diagnose subclinical impairment and to provide

correct genetic counselling.

16. Amat Cecilia, M.; Romero Perez, P.; Sevilla Chica, F. I. [Lumbago-sciatica syndrome

as the first manifestation of metastatic renal adenocarcinoma]. Sindrome de

lumbociatica como primera manifestacion de un adenocarcinoma renal metastasico.

Actas-Urol-Esp. 1996 Jan; 20(1): 54-8; ISSN: 0210-4806.

SPAIN. Presentation of one case of metastatic renal adenocarcinoma with iliac

bone and soft parts involvement which presented as a lumbosciatic picture. A

comment is made on the different diseases which can result in painful lumbar

syndrome, reaching an agreement on the need to conduct more extensive studies

given the lack of response to standard medical treatment.

17. American Diabetes Association: clinical practice recommendations 1996. Diabetes-

Care. 1996 Jan; 19 Suppl 1: S1-118; ISSN: 0149-5992.

UNITED-STATES.

18. Ammann, D.; Weissert, M.; Gottlob, I. [Hallervorden-Spatz syndrome: a case

presentation]. Das Hallervorden-Spatz-Syndrom: eine Fallvorstellung. Klin-

Monatsbl-Augenheilkd. 1996 May; 208(5): 356-7; ISSN: 0023-2165.

GERMANY.

19. Anand, P. Neurotrophins and peripheral neuropathy. Philos-Trans-R-Soc-Lond-B-

Biol-Sci. 1996 Mar 29; 351(1338): 449-54; ISSN: 0962-8436.

ENGLAND. Endogenous nerve growth factor (NGF) levels were studied in

patients with nerve trauma, diabetes mellitus and leprosy, the most common causes

of human peripheral neuropathy. In diabetics, there was an early length-dependent

dysfunction of small-diameter sensory fibres, with depletion of skin NGF and the

sensory neuropeptide substance P. The NGF depletion correlated significantly with

decreased skin axon-reflex vasodilatation, which is mediated by small sensory

fibres at least partly via substance P release. Immunostaining showed depletion of

NGF in keratinocytes in diabetic skin. In injured nerves, NGF levels were reduced

when compared to intact nerve, except acutely distal to injury; NGF-

immunostaining was seen in Schwann cells in distal segments, including

neuromas. NGF levels were decreased in leprosy-affected skin and nerve. The role

of neurotrophins in the rational treatment of human neuropathies is discussed e.g.

loss of nociception and axon-reflex vasodilatation contribute to skin ulceration, a

major and serious complication, for which NGF may provide prophylaxis.. 0.

20. Andersen, H. Reliability of isokinetic measurements of ankle dorsal and plantar flexors

in normal subjects and in patients with peripheral neuropathy. Arch-Phys-Med-

Rehabil. 1996 Mar; 77(3): 265-8; ISSN: 0003-9993.

UNITED-STATES. OBJECTIVE: To establish a reliable method for evaluation of

motor performance of ankle dorsal and plantar flexors in healthy subjects and in

patients with peripheral neuropathy. DESIGN: Thirty-eight control subjects and 7

patients with peripheral neuropathy were studied. All patients and 25 control

subjects were test twice. PATIENTS AND CONTROL SUBJECTS: An outdoor

clinic sample of 7 patients with hereditary motor sensory neuropathy (HMSN) and

38 control subjects. MAIN OUTCOME MEASURES: The effect of a rest interval,

a displacement of the axis of rotation, examination by three investigators on the

peak torque and work of dorsal and plantar flexors, and the percentage difference at

test-retests. RESULTS: Control subjects had percentage differences of 5.6% and

8.0% for dorsal flexion and 3.8% and 8.7% for plantar flexion at 60 degrees/sec

and 180 degrees/sec, respectively. In neuropathic patients the percentage

differences were 0% and 8.6% for dorsal and 5.1% and 12.3% for plantar flexion

at 30 degrees/sec and 60 degrees/sec, respectively. No interindividual differences

between 3 investigators were found. A rest interval between trials resulted in an

increased plantar flexion peak torque (P < .05). Displacement of 1.5cm of the axis

of rotation resulted in a change of the peak torque of 8.3% for plantar flexion (P <

.01). CONCLUSIONS: A well-defined test protocol for isokinetic motor

performance of the ankle dorsal and plantar flexors provides a reliable procedure

for quantification of motor function in healthy subjects and in patients with

HMSN1.

21. Andres, E.; Courouau, F.; Kaltenbach, G.; Maloisel, F.; Imler, M. [POEMS

syndrome: role and value of interleukin-6]. Syndrome POEMS: roles et interets de

l'interleukine 6. Rev-Med-Interne. 1996; 17(2): 145-9; ISSN: 0248-8663.

FRANCE. POEMS syndrome is a systemic disorder with peripheral neuropathy,

organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The

association of POEMS syndrome with lympho-proliferative disorder is very

commun. The pathogenesis remains poorly understood but implication of cytokines

(interleukins 1 and 6) is suspected. We report a case of a classic POEMS syndrome

(with polyneuropathy, hepatomegaly, diabetes melitus, hyperpigmentation,

monoclonal IgG lambda, anasarca and solitary plasmocytoma), associated with

high serum levels of interleukin 6.. 0.

22. Annertz, M.; Wingstrand, H.; Stromqvist, B.; Holtas, S. MR imaging as the primary

modality for neuroradiologic evaluation of the lumbar spine. Effects on cost and

number of examinations [see comments]. Acta-Radiol. 1996 May; 37(3 Pt 1): 373-

80; ISSN: 0284-1851.

Note: Comment in: Acta Radiol 1996 May;37(3 Pt 1):381-2.

DENMARK. PURPOSE: To evaluate the effects on cost, and number of primary

and supplementary neuroradiologic examinations, after introducing MR imaging as

the primary modality in the evaluation of the lumbar spine. MATERIAL AND

METHODS: Two 5-month periods were compared: period 1--before MR; and

period 2--after introduction of a 2nd MR device. In period 1, patients were

examined with myelography and/or CT after referral from specialists only, whereas

in period 2 both specialists and general practitioners could refer patients for MR

imaging. The direct cost (neuroradiologic methods and hospitalization) and indirect

cost (sick-leave and estimated loss of production caused by the diagnostic

procedure) were estimated. RESULTS AND CONCLUSION: In period 1,

investigations were started in 75 patients (62 myelographies and 13 CT

examinations); in period 2, in 227 patients (198 MR, 21 CT, and 8 myelographies).

The estimated total cost increased from SEK 825,000 to 1,265,000 (53%), the cost

per investigated patient decreasing from 11,000 to 5565 (50%), and the cost of

preoperative investigation per operated patient decreasing from 8616 to 5563

(35%). The number of supplementary examinations was unchanged.

23. Anthony, D. C.; Crain, B. J. Peripheral nerve biopsies. Arch-Pathol-Lab-Med. 1996

Jan; 120(1): 26-34; ISSN: 0003-9985.

UNITED-STATES. Nerve biopsies require special handling procedures that may

not be familiar to many surgical pathologists. Most pathology laboratories handle

fewer than 10 nerve biopsies per year, often referring them to specialized

laboratories for evaluation. However, initial handling procedures may affect the

ability of the reference laboratory to evaluate the specimen, and the remote location

may impede communications and increase the time required for diagnostic

evaluation. In a recent needs assessment questionnaire conducted by the College of

American Pathologists, a need for understanding the handling of peripheral nerve

biopsies was identified. Reference laboratories reported that clinical history and

electrophysiologic data are very helpful, both in planning the handling of the biopsy

and in interpreting the findings. Understanding the clinical differential diagnosis

and the relationships between the differential diagnosis and the use of specialized

studies often helps in the initial handling of peripheral nerve biopsies, whether

evaluated locally or referred to a specialized laboratory. In this paper, we offer

some general guidelines for handling nerve biopsies, including the rationale for the

studies commonly used to evaluate them. With this background, decisions may be

made about handling specimens that are specific for the clinical situation and allow

for the highest diagnostic yield and fastest turnaround times.

24. Anto, C.; Aradhya, P. Clinical diagnosis of peripheral nerve compression in the upper

extremity. Orthop-Clin-North-Am. 1996 Apr; 27(2): 227-36; ISSN: 0030-5898.

UNITED-STATES. Compression neuropathies are common in clinical practice.

This article is a review of the clinical features of the common entrapment

neuropathies affecting the upper extremity. The frequently found entrapment

syndromes are discussed in detail. Uncommon syndromes are also briefly

discussed.

25. Antoine, J. C.; Mosnier, J. F.; Lapras, J.; Convers, P.; Absi, L.; Laurent, B.; Michel,

D. Chronic inflammatory demyelinating polyneuropathy associated with carcinoma.

J-Neurol-Neurosurg-Psychiatry. 1996 Feb; 60(2): 188-90; ISSN: 0022-3050.

ENGLAND. The association of chronic inflammatory demyelinating

polyneuropathy (CIDP) and carcinoma has rarely been reported and its relevance is

debated. Thirty three consecutive patients with probable or definite CIDP

(idiopathic or associated with M protein) were investigated. Three patients with

definite CIDP had a concomitant carcinoma. One had an IgM paraprotein. Steroids

and intravenous immunoglobulins were effective.

26. Apartis, E.; Leger, J. M.; Musset, L.; Gugenheim, M.; Cacoub, P.; Lyon Caen, O.;

Pierrot Deseilligny, C.; Hauw, J. J.; Bouche, P. Peripheral neuropathy associated

with essential mixed cryoglobulinaemia: a role for hepatitis C virus infection? J-

Neurol-Neurosurg-Psychiatry. 1996 Jun; 60(6): 661-6; ISSN: 0022-3050.

ENGLAND. BACKGROUND--The prevalence of hepatitis C virus (HCV)

infection has been estimated at 43 to 84% in patients with essential mixed

cryoglobulinaemia in recent large series. Some of these cases have been

successfully treated with interferon-alpha. The objective was to evaluate the

prevalence and the possible role of HCV infection in essential mixed

cryoglobulinaemia. METHODS--Fifteen patients (eight men and seven women;

mean age: 61.2 (SD 16.5) years) with peripheral neuropathy (10 polyneuropathies

and five multifocal mononeuropathies) and essential mixed cryoglobulinaemia were

tested for serum anti-HCV antibodies. RESULTS--Antibodies were found in 10 of

15 patients involving either polyneuropathies (seven patients) or multifocal

mononeuropathies (three patients). Electrophysiological studies and teased nerve

fibre studies (in seven patients) allowed neuropathies to be classified as

predominantly sensory axonopathies. Compared with HCV-negative (HCV -)

patients, HCV-positive (HCV +) patients had a more pronounced and more

widespread motor deficit; motor nerve conduction velocities in peroneal and median

nerves were more impaired in HCV + patients, although significance was not

reached except for the mean value of the amplitude of the compound muscle action

potentials of the median nerves (P < 0.05); necrotising vasculitis was found in two

of nine nerve biopsies from the HCV + patients studied and in none of the three

HCV - patients. In addition, HCV + patients had more frequent cryoglobulin

related cutaneous signs, higher aminotransferase and serum cryoglobulin

concentrations, lower total haemolytic complement concentrations, and more

frequent presence of rheumatoid factor. A liver biopsy performed in eight HCV +

patients disclosed a range of lesions, from chronic active hepatitis (six patients) to

persistent hepatitis (two patients). Lastly, treatment with interferon-alpha conducted

over six months in two patients seemed to improve the peripheral neuropathy.

CONCLUSIONS--Patients with peripheral neuropathy and essential mixed

cryoglobulinaemia should be tested for anti-HCV antibodies to determine the

appropriate treatment.. EC 2.6.1.; 0; 0; 0; 0.

27. Aprile, I.; Schiavo, F.; De Colle, M. C.; Fabris, G. [Sellar metastases simulating the

Tolosa-Hunt syndrome]. Metastasi sellare simulante la sindrome di Tolosa-Hunt.

Radiol-Med-Torino. 1996 Mar; 91(3): 311-3; ISSN: 0033-8362.

ITALY.

28. Arii, J.; Sugita, K.; Takanashi, J.; Niimi, H. [Two early-childhood cases of optic

neuritis]. No-To-Hattatsu. 1996 Jul; 28(4): 336-40; ISSN: 0029-0831.

JAPAN. We reported two early-childhood cases suffering from acute optic neuritis

(ON). Case 1 was a 3-year-old girl, who had a preceding upper respiratory

infection, headache, nausea and subsequent sudden visual disturbance. Cranial

MRI revealed multiple T2-elongated lesions in the white matter. She showed two

neurological relapses including ON, leading to the diagnosis of clinically probable

multiple sclerosis (MS). Case 2 was a 2-year-old boy, who had an acute onset of

visual disturbance without any other neurological deficits. MRI with Gd-DTPA

enhancement revealed not only a disorder of optic nerves but involvement of the

white matter in the acute phase. It has been suggested that there may be a broad

spectrum of demyelinating disorders between ON and MS even in early-childhood.

Therefore, we should bear in mind to the subsequent progression to MS in

childhood ON cases with silent brain lesions.

29. Arkkila, P. E.; Kantola, I. M.; Viikari, J. S.; Ronnemaa, T.; Vahatalo, M. A.

Dupuytren's disease in type 1 diabetic patients: a five-year prospective study. Clin-

Exp-Rheumatol. 1996 Jan; 14(1): 59-65; ISSN: 0392-856X.

ITALY. OBJECTIVE. To clarify which are the underlying factors in the

development of Dupuytren's disease (DD) in diabetic patients and to evaluate if the

presence of DD can predict the development of diabetic complications. METHODS.

A total of 207 type 1 diabetic patients [age (mean +/- SD): 29.9 +/- 9.5 years] was

studied at baseline. A follow-up study was performed five years later in 166

patients. The presence of DD was examined and the patients were assessed in terms

of the following diabetic complications: background and proliferative retinopathy,

peripheral symmetrical polyneuropathy, and clinical nephropathy. RESULTS. The

prevalence of DD was 4% at the baseline study. DD was significantly associated

with the age of the patient and the duration of diabetes, but not with the age at the

onset of diabetes, BMI or the control of diabetes. DD was associated with somatic

peripheral symmetrical polyneuropathy (p < 0.01), a history of myocardial

infarction (p < 0.01) and limited joint mobility (LJM) (p < 0.05), but all of these

associations could be exclusively explained by the age of the diabetic patients and

the duration of diabetes. DD developed in 17 new subjects (2% per year) during the

five years of the study. The subjects' age and the duration of diabetes were

associated with the development of DD. There was a predominance of the

development of DD in women (p < 0.05), and in subjects with retinopathy (p <

0.05), nephropathy (p < 0.05), neuropathy (p < 0.05) or hypertension (p < 0.01),

but these associations could also be exclusively explained by the time-related

variables. The presence of DD at the baseline study did not predict the development

of diabetic complications or hypertension when the confounding effects of age and

the duration of diabetes were controlled by logistic regression analysis.

CONCLUSION. This study shows that the patient's age and the duration of

diabetes are the most important factors predicting the development of DD in diabetic

patients. The associations between DD and diabetic complications were exclusively

explained by the age and the duration of diabetes. The presence of DD did not

predict the development of diabetic complications.

30. Arnold, A. C.; Badr, M. A.; Hepler, R. S. Fluorescein angiography in nonischemic

optic disc edema. Arch-Ophthalmol. 1996 Mar; 114(3): 293-8; ISSN: 0003-9950.

UNITED-STATES. OBJECTIVE: To determine whether nonischemic optic disc

edema is associated with significant delay in fluorescein angiographic optic disc

filling. METHODS: Fluorescein angiograms from 16 patients with acute papillitis,

five with papilledema, and one with optic disc edema from orbital cavernous

hemangioma were compared with those of age-matched controls. Early views of

the optic disc were evaluated for onset of central retinal artery dye filling and both

onset and completion of choroidal and prelaminar optic disc dye filling. Data were

compared with our previously published figures for patients with nonarteritic

anterior ischemic optic neuropathy (NAION) and a new group of patients aged 46

years and younger with NAION (NAIONy). Subgroup analysis was performed on

data from patients with papillitis. RESULTS: Mean onset or completion of filling

was not significantly delayed compared with controls for the central retinal artery,

choroid, or prelaminar optic disc in patients with nonischemic optic disc edema,

including the subgroup of patients with papillitis. In comparison, significant delay

had been detected for onset and filling of prelaminar disc in typical NAION; similar

significant delay was noted in this study for patients with NAIONy. No patients

with nonischemic optic disc edema (including those with papillitis) demonstrated

delay of disc filling by at least 5 seconds, while this feature was detected in 76% of

patients with typical NAION and 62% of those with NAIONy. CONCLUSIONS:

Optic disc filling delay is common in typical NAION and NAIONy; it is not a

feature of nonischemic optic disc edema. This characteristic may aid in the

differentiation of NAION from papillitis.

31. Arons, M. S.; Hasbani, M. Electrical studies as a prognostic factor in the surgical

treatment of carpal tunnel syndrome [letter]. J-Hand-Surg-Am. 1996 May; 21(3):

518-21; ISSN: 0363-5023.

UNITED-STATES.

32. Assessment: electronystagmography. Report of the Therapeutics and Technology

Assessment Subcommittee. Neurology. 1996 Jun; 46(6): 1763-6; ISSN: 0028-

3878.

UNITED-STATES.

33. Atasoy, E. Thoracic outlet compression syndrome. Orthop-Clin-North-Am. 1996 Apr;

27(2): 265-303; ISSN: 0030-5898.

UNITED-STATES. This article is concerned with thoracic outlet compression

syndrome (TOCS), one of the most controversial subjects in medicine. It may also

be the most underrated, overlooked, misdiagnosed, and probably the most

important and difficult to manage peripheral nerve compression in the upper

extremity. Contents of the chapter include the historical aspect, anatomy, etiology

and incidence, pathophysiology, symptomatology, diagnosis, conservative and

surgical treatment, other conditions associated with TOCS, and results of TOCS

surgical treatment.

34. Atlas, M. D.; Perez, de Tagle JR; Cook, J. A.; Sheehy, J. P.; Fagan, P. A. Evolution

of the management of hydrocephalus associated with acoustic neuroma.

Laryngoscope. 1996 Feb; 106(2 Pt 1): 204-6; ISSN: 0023-852X.

UNITED-STATES. The management of hydrocephalus in association with a

cerebellopontine angle tumor is controversial. There is a widely held belief that

initial therapy should always be directed toward treatment of hydrocephalus before

definitive surgery. The potential problems of cerebrospinal fluid (CSF) shunting

and drainage have to led to an evolution in the management of hydrocephalus at St.

Vincent's Hospital. There is growing evidence that complete removal of

cerebellopontine angle tumors will result in resolution of hydrocephalus without

requiring other methods of CSF decompression. The authors present their

experience of 14 patients with hydrocephalus found in a recent series of 104

consecutive cases of acoustic neuroma. This study has detected a significant

correlation between hydrocephalus and increasing tumor size (P = .0234). The

mean tumor size in this series was 3.8 cm. The series has also demonstrated that

successful. safe, and complete tumor removal can be achieved without CSF

drainage before surgery.

35. Auw Haedrich, C.; Mathieu, M.; Hansen, L. L. Complete circumvention of central

retinal artery and venous cilioretinal shunts in optic disc drusen [letter]. Arch-

Ophthalmol. 1996 Oct; 114(10): 1285-7; ISSN: 0003-9950.

UNITED-STATES.

36. Auzou, P.; Hannequin, D.; Patrux, B.; Proust, F.; Marie, J. P.; Augustin, P.

[Involvement of the major hypoglossal nerve disclosing dissection of internal

carotid artery]. Atteinte du grand hypoglosse revelant une dissection de l'artere

carotide interne. Ann-Otolaryngol-Chir-Cervicofac. 1996; 113(1): 45-7; ISSN:

0003-438X.

FRANCE. The authors report on a case of hypoglossal nerve palsy revealing an

homolateral dissection of the internal carotid artery in the retrostyloid space. MR

Imaging and MR angiography revealed the dissecting aneurysm and enabled to

study its evolution.

37. Averbuch Heller, L.; Stahl, J. S.; Remler, B. F.; Leigh, R. J. Bilateral ptosis and

upgaze palsy with right hemispheric lesions. Ann-Neurol. 1996 Sep; 40(3): 465-8;

ISSN: 0364-5134.

UNITED-STATES. Bilateral ptosis is reported with unilateral hemispheric lesions,

suggesting partial lateralization of the control of the levator palpebrae superioris.

There is a tight synkinesis between vertical eye and eyelid movements, but a

similar, lateralized control of vertical gaze has not been previously described. We

report 3 patients with right hemispheric infarctions, in whom bilateral ptosis was

accompanied by impaired upward gaze. We postulate that this lateralization of

ocular motor function reflects the special contribution that the nondominant

hemisphere makes to attention.

38. Baba, H.; Chen, Q.; Uchida, K.; Imura, S.; Morikawa, S.; Tomita, K. Laminoplasty

with foraminotomy for coexisting cervical myelopathy and unilateral radiculopathy:

a preliminary report. Spine. 1996 Jan 15; 21(2): 196-202; ISSN: 0362-2436.

UNITED-STATES. STUDY DESIGN: An assessment was made of the efficacy

of a combined laminoplasty and foraminotomy operation for patients with

coexisting myelopathy and unilateral radiculopathy. The procedure was done in 17

patients. OBJECTIVES: The patients were followed with lateral flexion and

extension radiographs, computed tomography scans, and an assessment system

specially designed to qualitatively evaluate the patients' neurologic status. Follow-

up period averaged 4 years (range, 2.1-9.3 years). SUMMARY OF

BACKGROUND DATA: Excellent-to-good results were obtained for 76% (13 of

17) of the patients without any significant functional compromise based on the

radiographs. Sixteen nerve roots were decompressed with a less than 25%

foraminotomy, whereas eight were decompressed by a 25%-50% foraminotomy

without serious neurologic damage, except for one patient. The neurologic results

appeared unrelated to the extent of foraminotomy. METHODS: A refined procedure

for combined laminoplasty and foraminotomy was reviewed retrospectively in

terms of neurologic outcome and radiographic data. RESULTS: The present series

is small, and results are not comparable directly with other methods. The procedure

appears effective for myelopathy and radiculopathy. This procedure is applicable to

patients with myelopathy and coexisting nerve root impingement anterolaterally or

in the neural foramen. CONCLUSION: The combined laminoplasty and

foraminotomy operation may provide greater neurologic improvement in patients

with coexisting myelopathy and unilateral radiculopathy, while maintaining cervical

spine stability after surgery.

39. Bain, P. G.; Britton, T. C.; Jenkins, I. H.; Thompson, P. D.; Rothwell, J. C.;

Thomas, P. K.; Brooks, D. J.; Marsden, C. D. Tremor associated with benign

IgM paraproteinaemic neuropathy. Brain. 1996 Jun; 119( Pt 3): 789-99; ISSN:

0006-8950.

ENGLAND. The clinical and neurophysiological features of six patients with

action tremor of the upper limbs associated with IgM paraproteinaemic neuropathy

are described. Symptomatic tremor was confined to the upper limbs and was

broadly symmetrical. The frequency of associated rhythmic muscle activity ranged

from 2.8 to 5.5 Hz in abductor pollicis brevis and from 3.7 to 5.5 Hz in the

forearm flexor muscles. Magnetic brain stimulation, somatosensory evoked

potentials (SEPs) and stretch reflex studies did not provide evidence for delayed

conduction within central pathways. There was marked slowing of the maximum

motor conduction velocities in peripheral nerves. Forearm stretch reflexes were

present but their latencies were prolonged. Somatosensory evoked potentials were

obtained in the majority of patients, but were delayed. Wrist tremor could be

modulated by mechanical perturbations or median nerve electrical shocks. Simple

voluntary wrist movements were of normal duration and peak velocity, but the

kinematic profile was asymmetric. Each movement was associated with a triphasic

EMG pattern in agonist-antagonist-agonist muscles but the durations of the bursts

were prolonged and the onset of the second agonist was delayed. These results

support the hypothesis that distorted, mistimed peripheral inputs reach a central

processor (probably the cerebellum) which although intact is misled into producing

tremor in certain parts of the body.. 0.

40. Bain, P. G.; Britton, T. C.; Jenkins, I. H.; Thompson, P. D.; Rothwell, J. C.;

Thomas, P. K.; Brooks, D. J.; Marsden, C. D. Tremor associated with benign

IgM paraproteinaemic neuropathy. Brain. 1996 Jun; 119( Pt 3): 789-99; ISSN:

0006-8950.

ENGLAND. The clinical and neurophysiological features of six patients with

action tremor of the upper limbs associated with IgM paraproteinaemic neuropathy

are described. Symptomatic tremor was confined to the upper limbs and was

broadly symmetrical. The frequency of associated rhythmic muscle activity ranged

from 2.8 to 5.5 Hz in abductor pollicis brevis and from 3.7 to 5.5 Hz in the

forearm flexor muscles. Magnetic brain stimulation, somatosensory evoked

potentials (SEPs) and stretch reflex studies did not provide evidence for delayed

conduction within central pathways. There was marked slowing of the maximum

motor conduction velocities in peripheral nerves. Forearm stretch reflexes were

present but their latencies were prolonged. Somatosensory evoked potentials were

obtained in the majority of patients, but were delayed. Wrist tremor could be

modulated by mechanical perturbations or median nerve electrical shocks. Simple

voluntary wrist movements were of normal duration and peak velocity, but the

kinematic profile was asymmetric. Each movement was associated with a triphasic

EMG pattern in agonist-antagonist-agonist muscles but the durations of the bursts

were prolonged and the onset of the second agonist was delayed. These results

support the hypothesis that distorted, mistimed peripheral inputs reach a central

processor (probably the cerebellum) which although intact is misled into producing

tremor in certain parts of the body.. 0.

41. Bak, J.; Olsson, Y.; Grimelius, L.; Spannare, B. Paraganglioma of the cauda equina.

A case report and review of the literature. APMIS. 1996 Mar; 104(3): 234-40;

ISSN: 0903-4641.

DENMARK. A 59-year-old man presented with clinical evidence of a primary

tumor of the cauda equina region. It was well circumscribed and was completely

removed by neurosurgery. Routine staining showed that it had structural

similarities to an ependymoma, but immunohistochemistry with antisera to

synaptophysin, NSE, chromogranin-A and PGP 9.5 proved it to be a

neuroendocrine tumor, i.e. a paraganglioma. We propose the use of endocrine

markers in cases with tumors of the cauda equina to differentiate a paraganglioma

from an ependymoma. Paragangliomas appear to have a better clinical outcome than

ependymomas. Recurrence after surgery for a paraganglioma in the cauda equina

region, especially if it is encapsulated, is rarely encountered.. 0; 0.

42. Bak, K.; Torholm, C. [Supinator syndrome. Entrapment of the posterior interosseous

nerve]. Supinatorlogesyndrom. Entrapment af ramus profundus n. radialis.

Ugeskr-Laeger. 1996 Feb 12; 158(7): 919-21; ISSN: 0041-5782.

DENMARK. Activity related pain on the lateral side of the elbow or proximal on

the forearm may be caused by compression of the posterior interosseous nerve in

the radial tunnel. A number of different specialties can be involved in this patient.

Often there is no effect of conservative treatment. Several investigations show that

the condition can be treated surgically with good to excellent results in the majority

of patients. Often the patients suffer a number of concomitant overuse syndromes.

The clinical characteristics and the surgical approach to the problem is described

and illustrated by two case stories.

43. Balcer, L. J.; Galetta, S. L.; Bagley, L. J.; Pakola, S. J. Localization of traumatic

oculomotor nerve palsy to the midbrain exit site by magnetic resonance imaging.

Am-J-Ophthalmol. 1996 Sep; 122(3): 437-9; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To present the magnetic resonance imaging

findings for a patient with traumatic oculomotor nerve injury. METHODS: We

examined a patient with a right pupil-involving oculomotor nerve palsy after severe

closed head trauma. RESULTS: Magnetic resonance imaging of the brain

demonstrated marked signal hypointensity on gradient-echo T2*-weighted images

consistent with hemorrhage at the midbrain exit site of the right oculomotor nerve.

CONCLUSIONS: Distal fascicular damage or partial rootlet avulsion is a

mechanism of injury in some traumatic oculomotor nerve palsies. Gradient-echo

T2*-weighted magnetic resonance imaging is the most sensitive method to detect

hemorrhagic changes associated with shearing injury.

44. Baloh, R. W.; Ishyama, A.; Wackym, P. A.; Honrubia, V. Vestibular neuritis: clinical-

pathologic correlation. Otolaryngol-Head-Neck-Surg. 1996 Apr; 114(4): 586-92;

ISSN: 0194-5998.

UNITED-STATES. Postmortem examination of the brain and temporal bones of a

patient with well-documented vestibular neuritis showed selective neuronal loss in

Scarpa's ganglia on the side with absent caloric response. There was loss of hair

cells and an "epithelialization" of the utricular macule and semicircular canal cristae

on the deafferented side, and synaptic density in the vestibular nuclei on the

deafferented side was decreased compared with that on the normal side. All

findings were consistent with an isolated viral infection of Scarpa's ganglia. This is

the first description of the effects of chronic deafferentation on the vestibular

sensory epithelia and the vestibular nuclei in a human being.

45. Barker, I. Home ventilation of a child with motor and sensory neuropathy. Central

funding and a central resource providing information are needed [letter]. BMJ.

1996 Oct 12; 313(7062): 940; ISSN: 0959-8138.

ENGLAND.

46. Barry, J. C.; Effert, R.; Hoffmann, N. [Detection and diagnosis of small ocular

misalignment with the Purkinje reflex pattern method]. Nachweis und Diagnose

von kleinen Augenfehlstellungen mit dem Purkinje-Reflexmusterverfahren. Klin-

Monatsbl-Augenheilkd. 1996 Mar; 208(3): 167-80; ISSN: 0023-2165.

GERMANY. BACKGROUND: Application test for an automatic classification

strategy for ocular alignment data for the detection of ocular misalignment in

strabismic patients. METHODS: Photographic Purkinje Reflection Pattern

Evaluation was used a) with a handheld device for the detection and measurement

of ocular misalignments in near fixation (group 1, n = 64 strabismic patients) and

b) with a stationary device for the detection and measurement of ocular

misalignments in near fixation (group 2, n = 38 patients) and in distance fixation

(group 3, n = 36 patients). The orthoptic diagnoses were mostly primary and

secondary microtropia with manifest angles of strabismus from naught or 0.25

degrees to 3-4 degrees, with maximum angles up to 6-9 degrees. The ocular

alignment data were classified using the computer based strabismus index

procedure. This strategy relies on thresholds derived from means and standard

deviations in orthotropic control populations. In this way the data sets were

classified automatically as "no referral" or "referral". In addition, an automatic

diagnosis of the type of misalignment was given and the results were compared to

the orthoptic gold standard. RESULTS: The sensitivity for the detection of a

manifest ocular misalignment was a ca. 80% in group 1 and 2, and 90% in group

3, with specificities from 90% to 100%. All manifest angles of strabismus larger

than 1 degree were correctly classified as "referral". There was good agreement

between the diagnoses of the type of misalignment in most cases. Discrepancies

were observed with very small ocular misalignments or with incomplete data sets,

or they could be explained by a switch of fixation. The amount of the misalignment

varied markedly as compared to the orthoptic measurement in a number of cases.

CONCLUSIONS: The examination allows for a detection of small manifest ocular

misalignments with a very high sensitivity. The deviated eye and the type of the

misalignment in the primary position are evaluated automatically by a data base

computer algorithm. The differences between the measured angles of strabismus

indicate that the photographic examination conditions and the orthoptic

simultaneous prism and cover test conditions are not exactly alike. Purkinje

Reflection Pattern Evaluation represents a step towards an examiner-independent

measurement of the angle of strabismus.

47. Bartlett, R. J.; Hill, C. A.; Devlin, R.; Gardiner, E. D. Two-dimensional MRI at 1.5

and 0.5 T versus CT myelography in the diagnosis of cervical radiculopathy.

Neuroradiology. 1996 Feb; 38(2): 142-7; ISSN: 0028-3940.

GERMANY. A prospective comparison was made of standard two-dimensional

MRI sequences, at both high and midfield strength, with CT myelography in 23

patients with cervical spondylosis. MRI is adequate for assessment of cord

compression, where high field strength is superior to midfield strength. MRI using

4-mm sections is inadequate for presurgical assessment of root compression. It

remains to be proven whether thin-section white-CSF volume sequences or

gadolinium-enhanced volume studies can replace CT myelography.

48. Bartose, V.; Zahumensky, E.; Jirkovska, A.; Rybka, J. [Standard care of the diabetic

foot]. Standardy pece o diabetickou nohu. Vnitr-Lek. 1996 Jan; 42(1): 54-6;

ISSN: 0042-773X.

CZECH-REPUBLIC.

49. Basinskii, S. N.; Sas'ko, V. I. [A method of direct electrophoresis of the optic nerve in

patients with far-advanced glaucoma]. Sposob priamogo elektroforeza zritel'nogo

nerva u bol'nykh s daleko zashedshei stadiei glaukomy. Vestn-Oftalmol. 1996 Jan;

112(1): 8-10; ISSN: 0042-465X.

RUSSIA. A new method for the treatment of diseases of the optic nerve and

posterior segment of the eyeball is proposed: direct electrophoresis. Surgical

treatment of 85 patients with far-advanced glaucoma in combination with the said

method helped attain a reliable improvement of the vision acuity, of the total visual

field, of critical frequency of flashing fusion, and of the linear velocity of the blood

flow in the ocular artery in the majority of patients. This method holds good

promise in the treatment of partial atrophies of the optic nerve of different origin,

including the descending ones, and of dystrophic conditions of the posterior

segment of the eye.

50. Baskin, E.; Turkay, S.; Icagasioglu, D.; Tanzer, F.; Cevit, O. High-dose intravenous

immune globulin in the management of severe Guillain-Barre syndrome. Turk-J-

Pediatr. 1996 Jan; 38(1): 119-23; ISSN: 0041-4301.

TURKEY. Guillain-Barre Syndrome (GBS) is the most common cause of acute

generalized paralysis. Although the cause and pathogenesis of GBS remain

unknown, there is increasing evidence to suggest that this syndrome has an

immunological basis. Two children suffering from GBS were treated with high-

dose intravenous immune globulin (IVIG) (1 g/kg/day over two consecutive days).

Both children showed marked clinical improvement within 48 hours of the onset of

treatment. It is suggested, on the basis of recent case reports, that immunoglobulins

may have an important role in the treatment of Guillain-Barre Syndrome.. 0.

51. Battistella, P. A.; Peserico, A. Central nervous system dysmyelination in PIBI(D)S

syndrome: a further case. Childs-Nerv-Syst. 1996 Feb; 12(2): 110-3; ISSN: 0256-

7040.

GERMANY. This is a report of new case of PIBI(D)S, a rare autosomal recessive

syndrome characterized by photosensitivity, ichthyosis, brittle sulfur-deficient hair

(trichothiodystrophy), impaired intelligence, decreased fertility, and short stature.

Bilateral cataract and axial osteosclerosis were also detected. Magnetic resonance

imaging (MRI) revealed diffuse central nervous system dysmyelination, a finding

also described in the only three other reported cases in which MRI was performed.

The paper also considers certain similarities in neurological signs and

neuroradiological findings between PIBI(D)S, Cockayne syndrome, and

xeroderma pigmentosum--all of which are inherited diseases characterized by

photosensitivity and DNA repair defect.

52. Bauer, C. A.; Coker, N. J. Update on facial nerve disorders. Otolaryngol-Clin-North-

Am. 1996 Jun; 29(3): 445-54; ISSN: 0030-6665.

UNITED-STATES. Many issues involving the diagnosis and treatment of facial

nerve disorders continue to engender controversy and debate. This article examines

the theory that Bell's palsy is a herpes simplex neuritis and proposes facial nerve

decompression for recurrent palsies. The contemporary management of herpes

zoster oticus, temporal bone fractures, otogenic facial paralysis, and hemifacial

spasm is reviewed.

53. Beard, J. P.; Wade, W. H.; Barber, D. B. Sacral insufficiency stress fracture as

etiology of positional autonomic dysreflexia: case report. Paraplegia. 1996 Mar;

34(3): 173-5; ISSN: 0031-1758.

ENGLAND. The medical literature is replete with case reports of the syndrome

known as autonomic dysreflexia. Although the majority of cases are known to be

induced by either bladder or bowel distention. there does exist a small number of

cases in which the inciting stimulus is more obscure. In such cases, a

comprehensive medical evaluation is necessary to ensure proper identification of the

source of irritation and the appropriate medical management of the patient. We

present a patient with a heretofore unreported suspected etiology of autonomic

dysreflexia, axial loading of a sacral stress fracture.

54. Belmin, J.; Valensi, P. Diabetic neuropathy in elderly patients. What can be done?

Drugs-Aging. 1996 Jun; 8(6): 416-29; ISSN: 1170-229X.

NEW-ZEALAND. The prevalence of diabetes mellitus increases markedly with

age. Furthermore, advancing age is a strong risk factor for diabetic neuropathy,

independent of the duration of diabetes mellitus and glycaemic control. Several

biological changes occurring during the aging process may account for the

facilitating effect of age on diabetic neuropathy. These include an increase in the

production of advanced glycosylation end-products (AGEs), a defect in the polyol

pathway, nerve vascular alterations and impaired resistance to oxidative stress. The

clinical diagnosis of diabetic neuropathy is often difficult in elderly patients. The

relationship between symptoms and neuropathy and that between neuropathy and

diabetes mellitus are more difficult to ascertain in elderly patients due to age-related

changes in the peripheral and autonomic nervous system and associated diseases

frequently encountered in this population. Diagnosis of diabetic neuropathy is

based on nerve conduction studies, vibratory perception threshold determination

and assessment of autonomic function. For most of these tests, reference values are

markedly influenced by age and their interpretation should use carefully age-

adjusted reference intervals. Identification of peripheral diabetic neuropathy

indicates a high risk of foot complications, such as ulcers and gangrene, often

resulting in amputation, whereas cardiovascular autonomic neuropathy is associated

with an increased risk of postural hypotension and coronary events. All these risks

increase markedly with aging. Therapeutic trials in elderly patients with diabetic

neuropathy are lacking. Treatment of diabetic neuropathy consists of achievement

of better glycaemic control and treatment of symptoms related to neuropathy.

Specific treatments capable of preventing or curing neuropathy are under

investigation. The interesting results obtained with aldose reductase inhibitors in

animal studies contrast with disappointing results in patients with diabetes mellitus.

Other metabolic approaches, like antioxidants and gamma-linolenic acid, seem

promising. Clinical complications of diabetic neuropathy in the elderly are often

severe. Early detection is required, since at the present time a preventive approach

is the most effective way to avoid or postpone debilitating complications. More

research is needed to make effective curative treatments of diabetic neuropathy

available.

55. Bendix, A. F.; Bendix, T.; Vaegter, K.; Lund, C.; Frolund, L.; Holm, L.

Multidisciplinary intensive treatment for chronic low back pain: a randomized,

prospective study. Cleve-Clin-J-Med. 1996 Jan; 63(1): 62-9; ISSN: 0891-1150.

UNITED-STATES. BACKGROUND: Americans with low back pain have been

helped to return to work by multidisciplinary intensive treatment programs.

Whether this treatment method will succeed in countries with a more generous

social welfare system, where the incentive to return to work might be less, is not

proven. OBJECTIVES: To evaluate a Danish program of functional restoration

combined with behavioral support. METHODS: Patients who had experienced at

least 6 months of disabling low back pain were randomly assigned to either a 3-

week intensive treatment program (n = 55) or an untreated control group (n = 51).

RESULTS: Of the 106 patients randomized, 94 (89%) returned for a 4-month

follow-up visit. At that time, 29 (64%) of the 45 treated patients were able to work,

compared with 14 of 49 (29%) in the control group. The treated patients had used

fewer days of sick leave (P < .02), had contacted health care. professionals fewer

times (P < .001), and had lower pain and disability scores. CONCLUSIONS:

Although such programs are expensive, they can reduce pension expenditures, sick

leave days, health care contacts, and pain.

56. Benecke, R. Magnetic stimulation in the assessment of peripheral nerve disorders.

Baillieres-Clin-Neurol. 1996 Mar; 5(1): 115-28; ISSN: 0961-0421.

ENGLAND. Relatively pain-free excitation of both superficial and deep nerves in

the assessment of nerve conduction velocity is the main advantage of magnetic

stimulation over conventional electrical stimulation. General utility of this technique

has often been called into question by a number of authors because of difficulties in

obtaining supramaximal responses or in determining the exact site of impulse

generation when stimulating a peripheral nerve distally. Meanwhile, magnetic

stimulation of the cervical and lumbar roots has become a routine procedure for the

assessment of peripheral conduction time and is combined with transcranial

magnetic stimulation of the motor cortex in the assessment of central conduction

time. Recent developments in magnetic coil and stimulator design have improved

the focality of the stimulus, so that selective supramaximal stimuli can be delivered

to commonly studied peripheral nerves in the upper and lower limbs, both at

proximal and distal segments. Furthermore, the introduction of small figure-8-

shaped coils enables safe diagnosis of chronic compression syndromes with exact

assessment of conduction velocities over short distances of peripheral nerves. The

ease of application of magnetic stimulation and the absence of pain for the patient

make magnetic stimulation a particularly attractive method for also investigating

patients with demyelinating polyneuropathies, and will certainly replace

conventional electrical stimulation in the near future.

57. Berkowitz, R. G. Laryngeal electromyography findings in idiopathic congenital

bilateral vocal cord paralysis. Ann-Otol-Rhinol-Laryngol. 1996 Mar; 105(3): 207-

12; ISSN: 0003-4894.

UNITED-STATES. Children with idiopathic congenital bilateral vocal cord

paralysis (BVCP) were investigated by electromyography (EMG) of the posterior

cricoarytenoid and thyroarytenoid muscles to determine whether laryngeal EMG

findings had diagnostic or prognostic significance. Four children between 3 weeks

and 33 months of age were studied. Three had abductor paralysis and were

tracheostomy-dependent, while the fourth had adductor paralysis requiring a

feeding gastrostomy. Two of these patients also had other anomalies. Motor unit

potentials showing phasic bursts with respiration were found in all four cases,

while three children developed a full interference pattern on lightening of the

anesthetic. Follow-up for between 37 and 52 months showed no significant clinical

improvement in any of the patients. While the diagnosis of idiopathic congenital

BVCP can represent a heterogeneous group of conditions, the findings suggest that

normal laryngeal EMG findings may be a feature of idiopathic congenital BVCP but

do not imply a favorable prognosis for early recovery. They may, however, have

implications to explain the likely site of lesion in idiopathic congenital BVCP.

58. Bertolotto, M.; Rosenberg, I.; Parodi, R. C.; Perrone, R.; Gentile, S.; Rollandi, G.

A.; Succi, S. Case report: Fibroma of tendon sheath in the distal forearm with

associated median nerve neuropathy: US, CT and MR appearances. Clin-Radiol.

1996 May; 51(5): 370-2; ISSN: 0009-9260.

ENGLAND.

59. Bharucha, E. P.; Sulaiman, R.; Bharucha, N. E. The neuropathy of Dejerine and

Sottas: report of an Indian family. J-Neurol-Sci. 1996 Jan; 135(1): 78-80; ISSN:

0022-510X.

NETHERLANDS. We report here a family in which 4 out of 11 sibs of unrelated

parents showed the typical clinical features of Dejerine-Sottas disease. Sensory

disturbance was present in only one case and age at onset was delayed to 4 years in

another. The others all conformed to the infantile form of the disease. This is the

first time in which mental retardation has been reported in this disorder.

60. Bimmler, D.; Meyer, V. E. Surgical treatment of the ulnar nerve entrapment

neuropathy: submuscular anterior transposition or simple decompression of the

ulnar nerve? Long-term results in 79 cases. Ann-Chir-Main-Memb-Super. 1996;

15(3): 148-57; ISSN: 1153-2424.

FRANCE. The surgical treatment of the ulnar nerve entrapment neuropathy at the

elbow is controversial. None of the presently advocated procedures (simple

decompression of the ulnar nerve, medial epicondylectomy, subcutaneous or

submuscular anterior transposition of the ulnar nerve) has proven optimal regarding

long-term results. We studied the outcome in 79 patients whose ulnar nerve had

been operated on for the first time, either by simple decompression (31 cases) or by

submuscular anterior transposition (48 cases). The mean follow-up was 76

months. Patients were classified according to McGowan pre- and postoperatively;

we also applied a more detailed scoring system of our own. Preoperatively, the

patients were distributed almost equally between the three McGowan classes.

Postoperatively, about one out of three patients in both treatment groups

experienced a distinct improvement, i.e. was upgraded to a better McGowan class.

Using our own scoring system, the overall rate of objective improvement was 73%

after transposition and 55% after simple decompression. Irrespective of the surgical

method, roughly 90% of the patients considered their postoperative condition to be

improved. However, one specific group of patients (people with habitual ulnar

luxation or subluxation of the ulnar nerve) experienced a distinctly better result

when treated by anterior transposition than by simple decompression. Our results

show that simple decompression of the ulnar nerve can be recommended in all

patients without cubital (sub)luxation of the nerve, whereas people with a tendency

of cubital (sub)luxation of the ulnar nerve should be treated by submuscular

anterior transposition.

61. Bimmler, D.; Meyer, V. E. Surgical treatment of the ulnar nerve entrapment

neuropathy: submuscular anterior transposition or simple decompression of the

ulnar nerve? Long-term results in 79 cases. Ann-Chir-Main-Memb-Super. 1996;

15(3): 148-57; ISSN: 1153-2424.

FRANCE. The surgical treatment of the ulnar nerve entrapment neuropathy at the

elbow is controversial. None of the presently advocated procedures (simple

decompression of the ulnar nerve, medial epicondylectomy, subcutaneous or

submuscular anterior transposition of the ulnar nerve) has proven optimal regarding

long-term results. We studied the outcome in 79 patients whose ulnar nerve had

been operated on for the first time, either by simple decompression (31 cases) or by

submuscular anterior transposition (48 cases). The mean follow-up was 76

months. Patients were classified according to McGowan pre- and postoperatively;

we also applied a more detailed scoring system of our own. Preoperatively, the

patients were distributed almost equally between the three McGowan classes.

Postoperatively, about one out of three patients in both treatment groups

experienced a distinct improvement, i.e. was upgraded to a better McGowan class.

Using our own scoring system, the overall rate of objective improvement was 73%

after transposition and 55% after simple decompression. Irrespective of the surgical

method, roughly 90% of the patients considered their postoperative condition to be

improved. However, one specific group of patients (people with habitual ulnar

luxation or subluxation of the ulnar nerve) experienced a distinctly better result

when treated by anterior transposition than by simple decompression. Our results

show that simple decompression of the ulnar nerve can be recommended in all

patients without cubital (sub)luxation of the nerve, whereas people with a tendency

of cubital (sub)luxation of the ulnar nerve should be treated by submuscular

anterior transposition.

62. Bird, S. J.; Brown, M. J.; Shy, M. E.; Scherer, S. S. Chronic inflammatory

demyelinating polyneuropathy associated with malignant melanoma. Neurology.

1996 Mar; 46(3): 822-4; ISSN: 0028-3878.

UNITED-STATES. We report three patients who developed chronic inflammatory

demyelinating polyneuropathy (CIDP) in association with malignant melanoma. In

two cases, melanoma was discovered during the initial evaluation for neuropathy.

Two patients also had vitiligo, an antibody-mediated disorder that may complicate

melanoma. Melanoma cells and Schwann cells are both of neuroectodermal cell

origin, with shared surface antigens. Shared immunoreactivity may account for the

association between melanoma and CIDP, as with vitiligo.

63. Birrer, R. B.; Dellacorte, M. P.; Grisafi, P. J. Prevention and care of diabetic foot

ulcers [see comments]. Am-Fam-Physician. 1996 Feb 1; 53(2): 601-11, 615-6;

ISSN: 0002-838X.

Note: Comment in: Am Fam Physician 1996 Jul;54(1):70. Comment in: Am Fam

Physician 1996 Jul;54(1):70, 72.

UNITED-STATES. The foot is frequently overlooked in the management of

diabetic patients. Failure to control diabetic foot ulcers at an early stage can lead to

life-threatening infection or amputation. Preventive care should emphasize patient

education, glycemic control, careful daily foot hygiene and appropriate footwear.

Early management of a diabetic foot ulcer should include culture-directed antibiotic

therapy when there is evidence of infection, moist dressings and adjustment of

footwear or casting to avoid pressure on the wound site. All patients with foot

ulcers should be evaluated for evidence of foot ischemia. Surgical intervention to

debride infected tissue and bone or to revascularize ischemic tissue can aid in ulcer

healing. Serious infection or severe ischemia, unfortunately, often necessitates

amputation.

64. Blackband, S. J.; Buckley, D. L.; Knowles, A. J.; Gibbs, P.; Turnbull, L. W.;

Horsman, A. Improved peripheral MR angiography with temperature regulation in

healthy patients. Radiology. 1996 Mar; 198(3): 899-902; ISSN: 0033-8419.

UNITED-STATES. Peripheral magnetic resonance (MR) angiography was

performed in eight volunteers, with a temperature-controlled water blanket. After

warming of the right foot, unobserved vessels became conspicuous in all patients:

Average increase in signal intensity was nearly twofold in the major artery and vein

depicted. A similar improvement was observed in an examination in one patient

with diabetes. Peripheral MR angiography performed after warming provided

additional diagnostic information.

65. Blair, R. C.; Troendle, J. F.; Beck, R. W. Control of familywise errors in multiple

endpoint assessments via stepwise permutation tests. Stat-Med. 1996 Jun 15;

15(11): 1107-21; ISSN: 0277-6715.

ENGLAND. We describe permutation based sequentially rejective multiple

comparison procedures useful in multiple endpoint assessments. We used Monte

Carlo methods to compare the power of these newly devised tests to that of tests

due to Holm and Rom as well as to the classical Bonferroni method. We illustrate

applications of the methods with analysis of visual field data collected from optic

neuritis patients. We conclude that the new methods are particularly useful when

there are many endpoints involved, the data are significantly correlated, and/or the

distributional assumptions are questionable.

66. Bode Lesniewska, B.; Dours Zimmermann, M. T.; Odermatt, B. F.; Briner, J.; Heitz,

P. U.; Zimmermann, D. R. Distribution of the large aggregating proteoglycan

versican in adult human tissues. J-Histochem-Cytochem. 1996 Apr; 44(4): 303-12;

ISSN: 0022-1554.

UNITED-STATES. We studied the distribution of the large hyaluronan-binding

proteoglycan versican (also known as PG-M) in human adult tissues using affinity-

purified polyclonal antibodies that recognize the core protein of the prominent

versican splice variants VO and V1. Versican was present in the loose connective

tissues of various organs and was often associated with the elastic fiber network.

Furthermore, it was localized in most smooth muscle tissues and in fibrous and

elastic cartilage. Versican staining was also noted in the central and peripheral

nervous system, in the basal layer of the epidermis, and on the luminal surface of

some glandular epithelia. In blood vessels, versican was present in all three wall

layers of veins and elastic arteries. In muscular arteries the immunoreactivity was

normally restricted to the tunica adventitia. However, it appeared in the media and

the split elastica interna of atherosclerotically transformed vessel walls. Our survey

of the distribution of versican in normal human tissues now forms the basis for

extended studies of potentially aberrant versican expression during pathogenic

processes.. 0; 126968-45-4.

67. Boncoeur Martel, M. P.; Lesort, A.; Moreau, J. J.; Labrousse, F.; Roche, I.; Bouillet,

P.; Pascaud, J. L.; Dupuy, J. P. MRI of paraganglioma of the filum terminale. J-

Comput-Assist-Tomogr. 1996 Jan; 20(1): 162-5; ISSN: 0363-8715.

UNITED-STATES.

68. Bonfiglioli, R.; Lodi, V.; Tabanelli, S.; Violante, F. S. [Entrapment of the ulnar nerve

at the elbow caused by repetitive movements: description of a clinical case].

Intrappolamento del nervo ulnare al gomito da movimenti ripetitivi: descrizione di

un caso clinico. Med-Lav. 1996 Mar; 87(2): 147-51; ISSN: 0025-7818.

ITALY. The paper describes a case of ulnar neuropathy in a man who made

household fittings and toys using bamboo. Several years after starting the job he

showed symptoms and physical signs of ulnar nerve entrapment at the elbow,

which were confirmed by electrophysiological findings. Job analysis revealed

biomechanical risk factors consisting of a high repetition of forceful flexion and

extension of the elbow, wrist and fingers without sufficient rest periods. Chronic

musculoskeletal overuse gradually leads to tendon and nerve disorders. The ulnar

neuropathy described can be classed under cumulative occupational trauma, which

is the most important cause of musculoskeletal disorders among the working

population.

69. Bonnard, C. [Thoracic outlet syndrome (TOS). Its limitations and indications for

surgical treatment]. Le thoracic outlet syndrome (TOS). Ses limites et les

indications a son traitement chirurgical. Rev-Med-Suisse-Romande. 1996 Mar;

116(3): 161-9; ISSN: 0035-3655.

SWITZERLAND.

70. Bonnard, C.; Egloff, D. V.; Favarger, N.; Heierli, P. [Carpal tunnel syndrome]. Le

syndrome du tunnel carpien. Rev-Med-Suisse-Romande. 1996 Mar; 116(3): 171-6;

ISSN: 0035-3655.

SWITZERLAND.

71. Bonnema, S. J. [Guillain-Barre syndrome. Cytomegalovirus infections and

pregnancy]. Guillain-Barre-syndromet. Cytomegalovirusinfektion og graviditet.

Ugeskr-Laeger. 1996 Oct 21; 158(43): 6112-3; ISSN: 0041-5782.

DENMARK. A case of Guillain-Barre syndrome during pregnancy is presented.

The patient was 11 weeks' pregnant and developed tetraparesis over a few weeks.

She did not require mechanical ventilation. Elevated liver enzymes and virocytosis

in the peripheral blood were noted. Based on a positive IgM-antibody titre, a

primary cytomegalovirus infection was diagnosed. The patient recovered

completely and gave birth to a child of 43 weeks' gestation. Cytomegalovirus IgM-

antibody was demonstrated in umbilical cord blood. The child appeared healthy

without abnormalities at nine weeks of age.. 0.

72. Bordet, R.; Benhadjali, J.; Destee, A.; Hurtevent, J. F.; Bourriez, J. L.; Guieu, J. D.

Sympathetic skin response and R-R interval variability in multiple system atrophy

and idiopathic Parkinson's disease. Mov-Disord. 1996 May; 11(3): 268-72; ISSN:

0885-3185.

UNITED-STATES. We compared autonomic function in patients with multiple

system atrophy (MSA) or with idiopathic Parkinson's disease (IPD) by measuring

sympathetic skin response (SSR) and R-R interval variability (RRIV). SSR was

investigated in 26 patients (13 with MSA and 13 patients with IPD). RRIV during

deep breathing, Valsalva maneuver, and on standing was investigated in 20 patients

(nine with MSA and 11 with IPD). MSA and IPD patients had similar age, illness

duration, and therapy. Abnormal SSR was more frequent in MSA (69%) than in

IPD (7.7%; x2, 10.4; p < 0.002). RRIV during deep breathing and the Valsalva

maneuver was lower in MSA than in IPD (p = 0.02). RRIV during standing up

was not significantly different in IPD and MSA. These differences between MSA

and IPD may be due to more severe and widespread autonomic disturbance in

MSA, related to more severe neuropathologic involvement of the autonomic

nervous system. SSR and RRIV may aid in the differential diagnosis of

parkinsonism and help to exclude from clinical trials MSA patients clinically

misdiagnosed as having IPD.

73. Borg, A. A. Reflex sympathetic dystrophy syndrome: diagnosis and treatment. Disabil-

Rehabil. 1996 Apr; 18(4): 174-80; ISSN: 0963-8288.

ENGLAND. The reflex sympathetic dystrophy syndrome is a very common,

poorly recognized syndrome which is associated with marked disability in some

cases. The historical aspects, current ideas about the pathogenesis and

pathophysiology, clinical features and staging are discussed. Early recognition and

appropriate intervention are the cornerstone of successful treatment and are also

discussed.

74. Borruat, F. X.; Sanders, M. D. [Vascular anomalies and complications of optic nerve

drusen]. Anomalies et complications vasculaires dans les drusen du nerf optique.

Klin-Monatsbl-Augenheilkd. 1996 May; 208(5): 294-6; ISSN: 0023-2165.

GERMANY. BACKGROUND: Drusen of the optic disc are associated with

slowly progressive optic neuropathy, characterized by accumulation of acellular

laminated concretions in the prelaminar portion of the optic nerve. Papillary

hemorrhages and vascular shunts have been reported with disc drusen but their

frequency and clinical significance is not well known. METHODS: Retrospective

study of fundus photographs of 116 patients with disc drusen referred to the

National Hospital for Neurology and Neurosurgery, London, between 1965 and

1991. RESULTS: Hemorrhages were found in 23 eyes from 16/116 (13.8%)

patients. Most cases (68.8%, 11/16 cases) occurred in patients with buried drusen,

and most hemorrhages were deeply located. Vascular shunts were present in 6.9%

(8/116 cases), most frequently in patients with exposed drusen (6/8 cases), most

being of the venous type (7/8 cases). DISCUSSION: Vascular anomalies are not

rare in disc drusen, as 20.7% (24/116 cases) of our patients presented either disc

hemorrhages or shunt vessels. Their presence supports the hypothesis of the

slowly progressive nature of disc drusen and the more advanced stage of optic

neuropathy in such eyes.

75. Borruat, F. X.; Schatz, N. J.; Glaser, J. S. [Post-actinic retrobulbar optic neuropathy].

Neuropathie optique retrobulbaire post-actinique. Klin-Monatsbl-Augenheilkd.

1996 May; 208(5): 381-4; ISSN: 0023-2165.

GERMANY. BACKGROUND: Radiation optic neuropathy (RON) is a rare,

unpredictable, late complication of radiotherapy secondary to obliterative

endarteritis. Tumor recurrence has to be ruled out by a clinical and

neuroradiological examination. METHODS: Five patients with RON were

investigated by magnetic resonance imaging (MRI) during 1992. RESULTS:

Radiation-induced lesions of the intracranial visual pathways were easily visible on

MRI. Without Gadolinium, a sectorial swelling was detectable, which markedly

enhanced with Gadolinium. Intracranial optic nerve was affected in 5/5 cases, optic

chiasm in 3/5 cases, and optic tract in 2/5 cases. CONCLUSIONS: MRI is the

examination of choice when RON is suspected: it will easily delineate the extent of

the lesion, and compression/infiltration by a recurrent tumor will be formally ruled

out. A segmental swelling of visual pathway with marked Gadolinium enhancement

on MRI is highly suggestive of radionecrosis.

76. Botelho, P. J.; Giangiacomo, J. G. Autosomal-dominant inheritance of congenital

superior oblique palsy. Ophthalmology. 1996 Sep; 103(9): 1508-11; ISSN: 0161-

6420.

UNITED-STATES. PURPOSE: A pedigree comprised of five affected members is

presented to demonstrate the genetic transmission of congenital superior oblique

palsy. METHODS: A 2-year-old boy referred for strabismus was found to have

bilateral congenital superior oblique palsy. The authors subsequently performed a

complete ophthalmologic examination on all available family members to determine

the inheritance pattern. The diagnosis of congenital superior oblique palsy was

based on results of prism cover testing, ductions, and the Bielschowsky head tilt

test, in addition to a history of early onset of symptoms and absence of preceding

head trauma. RESULTS: The father, paternal grandfather, and a brother of the 2-

year-old boy were found to have bilateral congenital superior oblique palsy.

Evaluation of the paternal aunt showed right congenital superior oblique palsy.

Bilateral absence of the superior oblique tendon was noted at the time of surgery in

the 2-year-old boy. CONCLUSION: The occurrence of genetic transmission by an

autosomal-dominant mode should be considered in patients with congenital

superior oblique palsy.

77. Bousquet, C. F.; Dufour, T. F.; Derome, P. C. Retrobulbar optic nerve cysticercosis.

Case report. J-Neurosurg. 1996 Feb; 84(2): 293-6; ISSN: 0022-3085.

UNITED-STATES. The authors report a first case of intraoptic neurocysticercosis

in a 12-year-old boy living on Reunion Island. Cysticercosis of the retrobulbar

portion of the optic nerve is rare. Because of the patient's age and disturbances in

both visual acuity and visual field, it was initially believed to be an optic nerve

tumor. Computerized tomography scans and surgical aspects were confirmed by

pathological findings. A conservative removal using en bloc orbitotomy showed

good functional and aesthetic results.

78. Bousquet, J. C.; Denjean, S.; Faure, C.; Venin, B.; Bochu, M. [Synovial cyst

involving isolated paralysis of the infraspinatus muscle. Ultrasonographic

diagnosis and MRI]. Kyste synovial entrainant une paralysie isolee du muscle

sous-epineux. Diagnostic echographique et IRM. J-Radiol. 1996 Apr; 77(4): 275-

7; ISSN: 0221-0363.

FRANCE. A case of entrapment neuropathy of the inferior branch of the

suprascapular nerve in the spinoglenoid notch is described. Ultrasonography and

MRI examination showed a ganglion cyst in the spinoglenoid notch with an

extension to the glenohumeral joint. The lesion was confirmed by surgical and

pathological examination.

79. Bousquet, O.; Basseville, M.; Vila Porcile, E.; Billette, de Villemeur T.; Hauw, J. J.;

Landrieu, P.; Portier, M. M. Aggregation of a subpopulation of vimentin filaments

in cultured human skin fibroblasts derived from patients with giant axonal

neuropathy. Cell-Motil-Cytoskeleton. 1996; 33(2): 115-29; ISSN: 0886-1544.

UNITED-STATES. Giant axonal neuropathy (GAN) is a generalized disorder of

intermediate filament networks which results in the formation of an ovoid aggregate

in a large variety of cell types. We investigated the cytoskeletal organization of

cultured skin fibroblasts derived from three GAN patients by indirect

immunofluorescence, confocal, and electron microscopy. Whereas the organization

of microfilaments seemed normal, the microtubule network appeared disorganized

and tangled. The organization of the intermediate filament network, composed of

vimentin, was probed with three antibodies directed against different epitopes: two

vimentin-specific antibodies, a monoclonal antibody (mAb V9) and a polyclonal

antibody, and a serum specific for all type III IFPs (PI serum). These experiments

showed that 20% of cultured skin fibroblasts from GAN patients have a vimentin

aggregate composed of densely packed filaments which coexists with a well-

organized vimentin network. After depolymerization of microtubules with

nocodazole, all fibroblasts from GAN patients contained a vimentin aggregate

which seemed to arise from a subpopulation of vimentin filaments normally

integrated in the vimentin network. Such aggregates were never observed in any

condition in control fibroblasts. Moreover, the ultrastructural analysis of GAN cells

revealed the presence of swollen mitochondria. We suggest that GAN may be due

to a defect in a factor which stabilizes cytoplasmic intermediate filament networks,

and we speculate on its identification and properties.. 0; 0; 31430-18-9.

80. Bower, S.; Moore, B. B.; Weiss, S. M. Neuralgia after inguinal hernia repair. Am-

Surg. 1996 Aug; 62(8): 664-7; ISSN: 0003-1348.

UNITED-STATES. Severe chronic pain after groin hernia repair is uncommon but

potentially debilitating. Fifteen patients with this condition were retrospectively

reviewed. All patients had severe pain, which prevented their working or normal

activity and was refractory to nonoperative treatment. Essentials of therapy included

1) a preoperative attempt to identify the involved nerve and 2) high ligation and

division of the involved nerve identified at exploration. Twelve patients obtained

excellent results and were able to return to normal activity with no requirement for

analgesia. Understanding of the typical nerve anatomy, as well as the individual

variation in nerve anatomy, can help prevent this complication and is essential for

correction if the complication does develop.

81. Bozzao, A.; Bonamini, M.; Gallucci, M. Neoplasms of the optic-chiasmatic region.

Rays. 1996 Jan; 21(1): 50-69; ISSN: 0390-7740.

ITALY. Diagnosis of neoplasms of the optic-chiasmatic region is facilitated by

assessment of onset symptoms. They represent the basis for a specific radiologic

study, allowing a differential diagnosis between radiologically similar forms. An

easy approach to these neoplasms is to separate malignant lesions from non-

neoplastic space-occupying lesions. Diagnostic imaging of neoplasms of the optic-

chiasmatic region is based on MRI, occasionally combined with CT. Because of its

peculiar embryology, the region is the site of numerous neoformations. However,

differential diagnosis is ready in most cases. To plan the most suitable surgical

management, the relations of the lesions with adjacent anatomical structures should

be carefully assessed. More complex syndromes (gliomas in NF1,

infundibulopeduncular histiocytosis in eosinophilic granulomatosis, etc.) should

also be considered.

82. Brandenburg, J. H.; Unger, J. M.; Koschkee, D. Vocal cord injection with autogenous

fat: a long-term magnetic resonance imaging evaluation. Laryngoscope. 1996 Feb;

106(2 Pt 1): 174-80; ISSN: 0023-852X.

UNITED-STATES. Since 1987, the senior author has injected autogenous fat into

paralyzed or atrophic vocal cords as an alternative to alloplastic substances for vocal

cord augmentation and medialization. To determine the fate of the injected

autogenous fat, the injected vocal cords of 10 patients were evaluated by laryngeal

magnetic resonance imaging (MRI) in the sagittal, coronal, and axial planes.

Imaging studies were performed as early as 17 hours after surgery to as long as 31

months after fat injection. In 9 patients, identification of a fat signal within the

previously injected vocal cords was observed (including the 31-month

postoperative follow-up). In 1 patient, no fat signal was identified 13 months after

surgery, but the vocal cord was noted to have a bulging, enlarged contour. The

results of this imaging study provide further evidence that autogenous fat, which

has not been damaged during harvesting or microinjection, can survive

transplantation into the vocal cord. The bulk of the vocal cord is maintained by

microlipocytes and fibrous connective tissue, both of which replace the damaged fat

cells that are gradually being reabsorbed.

83. Brandle, P.; Satoretti Schefer, S.; Bohmer, A.; Wichmann, W.; Fisch, U. Correlation

of MRI, clinical, and electroneuronographic findings in acute facial nerve palsy.

Am-J-Otol. 1996 Jan; 17(1): 154-61; ISSN: 0192-9763.

UNITED-STATES. Intratemporal enhancement of (Gd-DTPA) was investigated

by an interleaved-overlapping magnetic resonance imaging (MRI) technique in 35

cases of acute facial palsy. In a reference group (normal facial function),

enhancement was localized from the ganglion geniculi to the stylomastoid foramen.

In cases of acute palsy, the facial nerve enhanced in the meatal fundus independent

of etiology (idiopathic, herpetic, or traumatic). In 70% of those with Ramsay-Hunt

syndrome, the vestibular and cochlear nerves, the labyrinth, and the sheets of the

internal and external auditory canal additionally enhanced. No correlation was

found between intensity, extension, and duration of the enhancement and the

clinical, intraoperative, or electroneuronographic degree of the facial palsy. The

pathogenesis of the Gd-DTPA enhancement of the facial nerve appears to be closely

connected with the vascular supply of the fallopian canal and the permeability of the

neural sheets.

84. Brans, J. W.; Aramideh, M.; Schlingemann, R. O.; Oen, V. M.; Speelman, J. D.;

Ongerboer, de Visser BW. [Cornea protection in ptosis induced by botulinum

injection]. Corneabescherming door ptosis opgewekt door botuline-injectie. Ned-

Tijdschr-Geneeskd. 1996 May 11; 140(19): 1031-3; ISSN: 0028-2162.

NETHERLANDS. Two patients, men aged 82 and 53 years, with weakness of the

facial muscles after surgery in the posterior fossa had keratitis caused by the

inability to blink and to close the eye. Botulinum toxin type A was injected into the

levator palpebrae superioris muscle under electromyographic control. A selective,

reversible paralysis of this muscle was induced without weakness of the

anatomically related rectus superior muscle. This botulinum toxin-induced

protective ptosis appeared to be effective and safe in the treatment and prevention of

keratitis in patients with a temporary weakness of the facial muscles.. 0.

85. Braune, S.; Auer, A.; Schulte Monting, J.; Schwerbrock, S.; Lucking, C. H.

Cardiovascular parameters: sensitivity to detect autonomic dysfunction and

influence of age and sex in normal subjects. Clin-Auton-Res. 1996 Feb; 6(1): 3-15;

ISSN: 0959-9851.

ENGLAND. In 137 healthy volunteers between 18 and 85 years of age, blood

pressure (BP) and heart rate (HR) were measured continuously with the Finapres

device during active change of posture (ACP), i.e. standing upright, passive tilt

(PT, i.e. head-up tilt), Valsalva manoeuvre (VM), deep breathing (DB), isometric

muscle exercise (IME) and a mental arithmetic task (MA). Mean HR activation was

attenuated with increasing age in all manoeuvres, but was unrelated to sex. In non-

orthostatic challenge procedures like MA and IME mean BP increases were

independent of age and sex, despite lower increases in HR in the elderly. This

points to a preserved sympathetic efferent activity. Following a forced fall in BP

during ACP, PT and VM, the initial responses and maintenance values of BP

showed a significant age-related decrease. This finding was strongly related to

lower BP values in males compared with females, which became more pronounced

with increasing age. Further studies to investigate age-related changes in the

activation of the various components of the cardiovascular regulation need to

consider the mode of activation of the autonomic nervous system and sex as factors

of influence. Normal ranges, and also some new points in time not previously

measurable, were calculated for all standard autonomic tests based on the

continuous measurement of BP and HR. The minimum length of time necessary to

assess the cardiovascular responses during ACP and PT was found to be 60 s. The

upper time limits for reaching maximum activation during IME and MA were 3.5

min and 1 min, respectively. Age had a relevant influence on the lower limits of

normal of all HR parameters and of some BP measurements during PT, ACP and

VM. Sex was found to have no relevant impact on normal ranges. Over 65 years of

age the normal values for HR activation during VM and DB hardly exceeded

baseline values. The possibility of increasing the sensitivity of detection of

autonomic dysfunction by measuring BP continuously must be approached with

caution, as sufficient sensitivity was only reached at the lower limits of normal

during late phase II of the VM. The initial increase of HR after ACP and the BP

values after 60 s standing time proved to be the parameters with the best sensitivity

for detecting an affection of the regulation of HR and BP over the whole range of

age.

86. Brodsky, M. C. Congenital downbeat nystagmus. J-Pediatr-Ophthalmol-Strabismus.

1996 May; 33(3): 191-3; ISSN: 0191-3913.

UNITED-STATES.

87. Brower, A. C. Septic arthritis. Radiol-Clin-North-Am. 1996 Mar; 34(2): 293-309, x;

ISSN: 0033-8389.

UNITED-STATES. Diagnosis of septic joint can be a problem for both the

clinician and the imager. The longer the delay in diagnosis of a septic arthritis, the

greater the chance of significant complication. Many imaging modalities are

available to the imager and each plays a definite role. In determining the modality of

choice, one should choose what is most efficacious for the individual patient.

88. Brown, S. M.; Del Monte, M. A. Choroidal neovascular membrane associated with

optic nerve head drusen in a child. Am-J-Ophthalmol. 1996 Feb; 121(2): 215-7;

ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To illustrate the diagnosis, evaluation, and

complications of pseudopapilledema in children. METHODS: We examined a 9-

year-old boy who had suspected papilledema and a retinal mass. He had undergone

neuroradiologic imaging at an outside facility. RESULTS: Clinical examination of

the patient provided the diagnosis of optic nerve head drusen, pseudopapilledema,

and a cicatrized choroidal neovascular membrane. Examination of the boy's parents

disclosed optic nerve head drusen in the father. CONCLUSIONS: Choroidal

neovascular membranes caused by optic nerve head drusen are uncommon in

children. Clinical examination of the patient and family members, along with B-

scan ultrasonography, can establish this cause. Neuroradiologic testing is

unnecessary, and carries risk related to the need for sedation.

89. Budnick, L. D. Clinical strategies for work-related carpal tunnel syndrome. N-J-Med.

1996 Jun; 93(6): 27-31; ISSN: 0885-842X.

UNITED-STATES.

90. Bujia, J.; Kim, C.; Bruegel, F. Soluble interleukin 2 receptors in patients with Bell's

palsy. Allergol-Immunopathol-Madr. 1996 May; 24(3): 112-5; ISSN: 0301-0546.

SPAIN. The etiology and pathogenesis of Bell's palsy are still an enigma. Some

studies have reported about the presence of cellular and humoral immune

dysfunction in this disease. Recently, determination of soluble interleukin 2

receptor has proven to be a valuable clinical tool to detect dysregulation of T

lymphocyte function. The concentrations of soluble interleukin 2 receptor alpha

was determined in serum samples from 11 patients with Bell's palsy by an enzyme

linked immunosorbent assay (ELISA). Concomitantly 8 age- and sex-matched

healthy blood donor as well as six patients with dermatitis herpetiformis served as

negative and positive controls, respectively. The concentration of soluble

interleukin 2 receptor in serum samples from these patients was similar to that in

samples from normal subjects. In contrast to this, patients with dermatitis

herpetiformis showed higher values of interleukin 2 receptor. Our results showed

that Bell's palsy is not accompanied by a massive activation of T cells.. 0.

91. Burckhardt, B. [A case from practice (344). Neuralgic shoulder amyotrophy or plexus

neuritis]. Der Fall aus der Praxis (344).Neuralgische Schulteramyotrophie oder

Plexysneuritis. Schweiz-Rundsch-Med-Prax. 1996 Mar 12; 85(11): 348-50; ISSN:

0369-8394.

SWITZERLAND.

92. Burglen, L.; Amiel, J.; Viollet, L.; Lefebvre, S.; Burlet, P.; Clermont, O.; Raclin, V.;

Landrieu, P.; Verloes, A.; Munnich, A.; Melki, J. Survival motor neuron gene

deletion in the arthrogryposis multiplex congenita-spinal muscular atrophy

association. J-Clin-Invest. 1996 Sep 1; 98(5): 1130-2; ISSN: 0021-9738.

UNITED-STATES. The survival motor neuron (SMN) gene was lacking in 6/12

patients with arthrogryposis multiplex congenita (AMC) associated with spinal

muscular atrophy (SMA). Neither point mutation in the SMN gene nor evidence for

linkage to chromosome 5q13 were found in the other patients. Hitherto,

arthrogryposis was regarded as an exclusion criterion in SMA. Our data strongly

suggest that AMC of neurogenic origin is genetically heterogeneous, with a

subgroup being allelic to SMA. Absence or interruption of the SMN gene in the

AMC-SMA association will make the diagnosis easier and genetic counselling will

now become feasible.. 0; 0.

93. Burkey, J. M.; Rizer, F. M.; Schuring, A. G.; Fucci, M. J.; Lippy, W. H. Acoustic

reflexes, auditory brainstem response, and MRI in the evaluation of acoustic

neuromas. Laryngoscope. 1996 Jul; 106(7): 839-41; ISSN: 0023-852X.

UNITED-STATES. Patient records were reviewed to determine whether persons

with absent acoustic reflexes have a higher incidence of abnormal auditory

brainstem response (ABR) results in the absence of a cerebellopontine angle (CPA)

tumor than those with normal acoustic reflexes. Results showed patients with

absent reflexes to have borderline or abnormal ABR results in 45.2% of the cases.

Patients with normal reflexes had borderline or abnormal ABR results in 14.2% of

the cases. Results indicate that magnetic resonance imaging is a more appropriate

test for patients with absent reflexes, since ABR was often nondiagnostic for a

CPA tumor in this group.

94. Burns, S. Common foot problems. Prim-Care. 1996 Jun; 23(2): 203-14; ISSN: 0095-

4543.

UNITED-STATES. The foot is a complex region of the body that includes the

vascular, neurologic, dermatologic, and musculoskeletal organ systems. This

article addresses each of these systems and the common maladies that affect them,

with a view toward common presentations, evaluation, differential diagnoses,

treatment alternatives, and guidelines for proper referral. Finally, a section of the

article is devoted to the prevention and treatment of lower extremity problems

unique to patients with diabetes.

95. Cabezas Agricola, J. M.; Lado Abeal, J. J.; Otero Anton, E.; Sanchez Leira, J.;

Cabezas Cerrato, J. Hypoparathyroidism in POEMS syndrome [letter]. Lancet.

1996 Mar 9; 347(9002): 701-2; ISSN: 0140-6736.

ENGLAND.

96. Cairns, D. A.; Hansen, J. H.; Riski, J. E. A noninvasive technique for detecting

hypernasal speech using a nonlinear operator. IEEE-Trans-Biomed-Eng. 1996 Jan;

43(1): 35-45; ISSN: 0018-9294.

UNITED-STATES. Speakers with a defective velopharyngeal mechanism produce

speech with inappropriate nasal resonance (hypernasal speech). It is of clinical

interest to detect hypernasality as it is indicative of an anatomical, neurological, or

peripheral nervous system problem. There are various clinical techniques used to

determine hypernasality. The current techniques are physically invasive or intrusive

to some extent. A preferred approach for detecting hypernasality, would be

noninvasive to maximize patient comfort and naturalness of speaking. In this study,

a noninvasive technique based on the Teager Energy operator is proposed. Utilizing

a property of the Teager Energy operator and a model for normal and nasalized

speech, a significant difference between the Teager Energy profile for lowpass and

bandpass filtered nasalized speech is shown. This difference is shown to be

nonexistent for normal speech. A classification algorithm is formulated that detects

the presence of hypernasality using a measure of the difference in the Teager

Energy profiles. The classification algorithm was evaluated using a native English

speaker population producing front (/i/) and mid (/A/) vowels. Results show that

the presence of hypernasality in speech can be reliably detected using the proposed

classification algorithm.

97. Cameron, N. E.; Cotter, M. A. Rapid reversal by aminoguanidine of the neurovascular

effects of diabetes in rats: modulation by nitric oxide synthase inhibition.

Metabolism. 1996 Sep; 45(9): 1147-52; ISSN: 0026-0495.

UNITED-STATES. Aminoguanidine treatment prevents the development of nerve

conduction velocity (NCV) deficits and some renal and retinal complications in

diabetic rats. Pharmacological actions include inhibition of the formation of

advanced glycosylation end products (AGEs) and nitric oxide (NO) synthase. The

aims of the study were to determine the extent to which diabetic NCV and nerve

blood flow deficits could be corrected by aminoguanidine in an intervention study,

to assess the time course of drug action, and to examine the effects of cotreatment

with the NO synthase inhibitor, NG-nitro-L-arginine (NOLA). A 19.3% +/- 0.9%

reduction in sciatic motor NCV after 4 weeks of untreated diabetes was corrected

86.6% +/- 3.7% by aminoguanidine treatment for a further 4 weeks. Time-course

studies showed that 50% of the maximal effect was attained within 6 days. Sciatic

endoneurial capillary blood flow, reduced approximately 45% by diabetes, was

corrected 85.6% +/- 12.1% by aminoguanidine treatment. The NCV and blood

flow effects of aminoguanidine were completely blocked by cotreatment with

NOLA. Thus, the data support a neurovascular mechanism for aminoguanidine

involving improved NO action. The rapidity of aminoguanide's effect is consistent

with inhibition of free radical production by autoxidative glycosylation or

glycoxidation.. EC 1.14.13.39; 0; 0; 0; 0; 50903-99-6; 7004-12-8; 79-17-4.

98. Campbell, W. W. Electrical studies as a prognostic factor in the surgical treatment of

carpal tunnel syndrome [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 527-8; ISSN:

0363-5023.

UNITED-STATES.

99. Caprioli, J.; Prum, B.; Zeyen, T. Comparison of methods to evaluate the optic nerve

head and nerve fiber layer for glaucomatous change. Am-J-Ophthalmol. 1996 Jun;

121(6): 659-67; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To compare the rates of optic nerve damage in

early human glaucoma as measured by four methods to evaluate change in the optic

nerve and nerve fiber layer. METHODS: Four techniques were used to detect

progressive glaucomatous damage in a prospective, longitudinal study: (1)

qualitative evaluation of stereoscopic color optic disk photographs, (2) qualitative

evaluation of monochromatic nerve fiber layer photographs, (3) manual

stereoplanimetric measurements of disk rim area, and (4) computerized

measurement of peripapillary nerve fiber layer height. One eye of each patient with

glaucoma or ocular hypertension was evaluated at the beginning and end of a

follow-up period of not less than one year. The rates of structural change measured

by these techniques and the rate of visual field change measured with threshold

automated perimetry were determined. RESULTS: We followed up 193 patients for

a mean (+/- S.D.) of 3.3 +/- 1.0 years (range, one to six years). Twenty-nine

(15%) of 193 eyes progressed by qualitative optic disk evaluation, 14 (7.2%) of

193 eyes progressed by qualitative nerve fiber layer evaluation, seven (3.6%) of

193 eyes progressed by stereoplanimetry, and 24 (13.2%) of 182 eyes progressed

by measurement of nerve fiber layer height. Visual field deterioration was detected

in 12 (5.2%) of 193 patients and correlated best with qualitative optic disk and

nerve fiber layer evaluations. Evaluation by stereoplanimetry and nerve fiber layer

height measurement detected change in eyes with primarily diffuse structural

damage, a pattern not well detected by qualitative methods. CONCLUSION: Both

qualitative and quantitative methods of optic disk and nerve fiber layer evaluation

contribute to the identification of progressive damage, depending on the stage of

disease and the characteristics of optic nerve cupping.

100. Carlsson, M. [The man behind the syndrome: William John Adie. He won an

involuntary victory in an academic dispute]. Mannen bakom syndromet: William

John Adie. Vann ofrivillig seger i akademisk strid. Lakartidningen. 1996 May 15;

93(20): 1966-9; ISSN: 0023-7205.

SWEDEN.

101. Carter, G. T.; Kilmer, D. D.; Szabo, R. M.; McDonald, C. M. Focal posterior

interosseous neuropathy in the presence of hereditary motor and sensory

neuropathy, type I. Muscle-Nerve. 1996 May; 19(5): 644-8; ISSN: 0148-639X.

UNITED-STATES. A 30-year-old male with hereditary motor and sensory

neuropathy, type I (HMSN I), presented with asymmetric weakness of finger

extension and radial deviation with left wrist extension, previously felt to be a

manifestation of the peripheral neuropathy. Nerve conduction studies confirmed

HMSN I; however, needle EMG revealed marked, ongoing axonal loss in muscles

innervated by the left posterior interosseous nerve (PIN) only. At surgery there was

focal fusiform swelling in the PIN at exit from the supinator muscle, compatible

with localized hypertrophic neuropathy, which has not been reported before in

HMSN I. A concomitant focal mononeuropathy should be considered in cases of

hereditary neuropathy with marked asymmetry of weakness.

102. Casaubon, L. K.; Melanson, M.; Lopes Cendes, I.; Marineau, C.; Andermann, E.;

Andermann, F.; Weissenbach, J.; Prevost, C.; Bouchard, J. P.; Mathieu, J.;

Rouleau, G. A. The gene responsible for a severe form of peripheral neuropathy

and agenesis of the corpus callosum maps to chromosome 15q [see comments].

Am-J-Hum-Genet. 1996 Jan; 58(1): 28-34; ISSN: 0002-9297.

Note: Comment in: Am J Hum Genet 1996 Jan;58(1):7-16.

UNITED-STATES. Peripheral neuropathy with or without agenesis of the corpus

callosum (ACCPN) is a devastating neurodegenerative disorder that is transmitted

as an autosomal recessive trait. Genealogical studies in a large number of affected

French Canadian individuals suggest that ACCPN results from a single founder

mutation. A genomewide search using 120 microsatellite DNA markers in 14

French Canadian families allowed the mapping of the ACCPN gene to a 5-cM

region on chromosome 15q13-q15 that is flanked by markers D15S1040 and

D15S118. A maximum two-point LOD score of 11.1 was obtained with the marker

D15S971 at a recombination fraction of 0. Haplotype analysis and linkage

disequilibrium support a founder effect. These findings are the first step in the

identification of the gene responsible for ACCPN, which may shed some light on

the numerous conditions associated with the progressive peripheral neuropathy or

agenesis of the corpus callosum.. 0.

103. Case records of the Massachusetts General Hospital. Weekly clinicopathological

exercises. Case 8-1996. A 28-year-old woman with the rapid development of a

major personality change and global aphasia [clinical conference]. N-Engl-J-Med.

1996 Mar 14; 334(11): 715-20; ISSN: 0028-4793.

UNITED-STATES.

104. Castillo, M.; Mukherji, S. K. Magnetic resonance imaging of the olfactory apparatus.

Top-Magn-Reson-Imaging. 1996 Apr; 8(2): 80-6; ISSN: 0899-3459.

UNITED-STATES. We review the normal anatomy of the olfactory system with

special emphasis on its extraaxial portions. Pathological processes affecting this

cranial nerve are divided into those that do not require imaging and those that do.

Processes in which patients benefit from imaging, mostly with magnetic resonance,

include suspected tumors and congenital abnormalities.

105. Cedoz, M. E.; Larbre, J. P.; Lequin, C.; Fischer, G.; Llorca, G. Upper lumbar disk

herniations. Rev-Rhum-Engl-Ed. 1996 Jun; 63(6): 421-6; ISSN: 0035-2659.

FRANCE. Specific features of upper lumbar disk herniations are reviewed based

on data from the literature and from a retrospective study of 24 cases treated

surgically between 1982 and 1994 (seven at L1-L2 and 17 at L2-L3). Clinical

manifestations are polymorphic, misleading (abdominogenital pain suggestive of a

visceral or psychogenic condition, meralgia paresthetica, isolated sciatica; femoral

neuralgia is uncommon) and sometimes severe (five cases of cauda equina

syndrome in our study group). The diagnostic usefulness of imaging studies

(radiography, myelography, computed tomography, magnetic resonance imaging)

and results of surgery are discussed. The risk of misdiagnosis and the encouraging

results of surgery are emphasized.

106. Celiker, R.; Basgoze, O.; Bayraktar, M. Early detection of neurological involvement in

diabetes mellitus. Electromyogr-Clin-Neurophysiol. 1996 Jan; 36(1): 29-35; ISSN:

0301-150X.

BELGIUM. The aim of this study was to investigate diabetic patients to obtain

electrophysiological data of possible neurological abnormalities even in the absence

of neuropathy symptoms, taking into account metabolic control. Fifty five diabetic

patients and twenty healthy subjects were included in this study. Motor and sensory

nerve conduction velocities (CV), F wave and somatosensory evoked potentials

(SEP) were recorded. Metabolic data about glycemia and HbA1c were collected.

49.1% of the patients had peripheral nerve conduction slowing. 56.4% of the

patients had SEP abnormalities, and among them 38.7% did not have any

peripheral nerve alterations. 40% of diabetic patients had F wave abnormalities and

22.7% peripheral nerve conduction was within normal limits. 33.7% of our

patients had carpal tunnel syndrome and 38.8% of these were asymptomatic. There

was a significant association between electrophysiological parameters and metabolic

control. In diabetic patients it is essential to determine the presence and distribution

of neuropathy.

107. Chalmers, R. M.; Howard, R. S.; Wiles, C. M.; Hirsch, N. P.; Miller, D. H.;

Williams, A.; Spencer, G. T. Respiratory insufficiency in neuronopathic and

neuropathic disorders. QJM. 1996 Jun; 89(6): 469-76.

ENGLAND. Twenty-nine patients with a neuronopathic or neuropathic disorder

were referred for assessment of respiratory insufficiency between 1978 and 1994.

Diagnoses included spinal muscular atrophy (6), chronic idiopathic demyelinating

neuropathy (4), Vialetto-van Laere syndrome (3), hereditary motor and sensory

neuropathy (3) and a miscellaneous group (5). We also describe seven patients with

Guillain-Barre syndrome (GBS) who required long-term ventilatory support for

over 6 months to 7 years after the initial illness. Respiratory insufficiency occurred

as a consequence of respiratory muscle weakness, impaired bulbar function and

restrictive lung defects. In some groups presentation was with progressive

nocturnal hypoventilation culminating in acute respiratory failure. Five patients with

GBS or chronic idiopathic demyelinating neuropathy were weaned from ventilatory

support up to 18 months after the initial illness. The remaining 24 patients required

continuous or nocturnal ventilatory support using intermittent positive-pressure

ventilation (13), negative pressure ventilation (4), nasal-mask-delivered intermittent

positive-pressure ventilation (4), nasal-mask-delivered continuous positive-

pressure ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed

(1). None have been weaned from support after a period of ventilation ranging

from one month to 10 years. Eight patients have subsequently died.

108. Chancellor, M. B.; Shenot, P. J.; Rivas, D. A.; Mandel, S.; Schwartzman, R. J.

Urological symptomatology in patients with reflex sympathetic dystrophy. J-Urol.

1996 Feb; 155(2): 634-7; ISSN: 0022-5347.

UNITED-STATES. PURPOSE: We determined the effect of reflex sympathetic

dystrophy on lower urinary tract function. MATERIALS AND METHODS: A total

of 20 consecutive patients (16 women and 4 men) with neurologically verified

reflex sympathetic dystrophy was referred for voiding symptoms, including

urgency, frequency, incontinence and urinary retention. No patient had had voiding

symptoms before the initial trauma that induced reflex sympathetic dystrophy.

Evaluation included medical history, physical examination, video urodynamic

testing and cystoscopy. RESULTS: Mean patient age was 43.4 +/- 10.2 years

(range 28 to 58) and mean duration of urological symptoms was 4.9 +/- 3.6 years

(range 1 to 14). Urodynamic study demonstrated a mean cystometric bladder

capacity of 417 +/- 182 ml. (range 120 to 700). The urodynamic diagnoses

included detrusor hyperreflexia in 8 patients, detrusor areflexia in 8, sensory

urgency in 3 and detrusor hyperreflexia with detrusor-external sphincter

dyssynergia in 1. In 4 women genuine stress urinary incontinence was also

documented urodynamically. CONCLUSIONS: Reflex sympathetic dystrophy may

have a profound effect on detrusor and sphincter function.

109. Charness, M. E.; Ross, M. H.; Shefner, J. M. Ulnar neuropathy and dystonic flexion

of the fourth and fifth digits: clinical correlation in musicians. Muscle-Nerve. 1996

Apr; 19(4): 431-7; ISSN: 0148-639X.

UNITED-STATES. Peripheral nerve lesions are sometimes associated with focal

dystonia. We diagnosed ulnar neuropathy in 28 of 73 (40%) cases of occupational

cramp in musicians. Focal slowing of ulnar conduction across the elbow was

identified in 15 of 19 (79%) patients using the near nerve technique and in 5 of 17

(29%) patients using surface recording. Ulnar neuropathy was present in 24 of 31

(77%) cases with flexion dystonia of the fourth and fifth digits and only 4 of the

remaining 42 (10%) cases with other patterns of focal dystonia. Focal dystonia

improved in 13 of 14 patients whose ulnar neuropathy improved and appeared or

worsened in 2 patients following ulnar nerve injury. These data, together with our

recent observation of a dystonic pattern of antagonist bursting in patients with

isolated ulnar neuropathy (Muscle Nerve 1995, 18:606-611), suggest that ulnar

neuropathy may initiate or sustain a specific dystonia, flexion of the fourth and fifth

digits, by inducing a central disorder of motor control.

110. Chaudhry, V.; Moser, H. W.; Cornblath, D. R. Nerve conduction studies in

adrenomyeloneuropathy. J-Neurol-Neurosurg-Psychiatry. 1996 Aug; 61(2): 181-5;

ISSN: 0022-3050.

ENGLAND. OBJECTIVE--Adrenomyeloneuropathy (AMN) is an X linked

metabolic disorder presenting with progressive spastic paraparesis in the third to

fifth decade of life. Although peripheral neuropathy is also present in most patients,

prominent pyramidal signs may make its clinical recognition difficult. The objective

was to characterise the peripheral neuropathy in patients with AMN by nerve

conduction studies. METHODS--Nerve conduction studies were performed in 99

men known to have AMN and in 38 heterozygous women, all of whom had

neurological disabilities. RESULTS--Of the 13 variables obtained, at least one was

abnormal in 82% of patients. The abnormalities were more common in men than in

women (87% v 67%); in legs than in arms (77% v 38%); in motor than in sensory

conduction (80% v 39%); and in latency (distal and F wave) and velocity compared

with amplitude (80% v 29%). Twenty six patients had at least one nerve variable

value in the demyelinating range. Four variables (sural velocity, peroneal

amplitude, peroneal velocity, and peroneal F wave) were correlated with the

expanded disability status scale; five variables (peroneal velocity, tibial H reflex,

median distal latency, median conduction velocity, and median F wave latency)

were correlated with serum very long chain fatty acids (VLCFAs); and two

variables (sural amplitude and peroneal distal latency) were more likely to be

abnormal in patients with normal adrenal function than in patients with Addison's

disease. CONCLUSIONS--Nerve conduction studies in patients with AMN are

often abnormal and suggest a mixture of axonal loss and multifocal demyelination.

Their correlation with disability status and serum VLCFAs suggests that measures

from nerve conduction studies may be useful in evaluating future treatments.

111. Chelimsky, T. C.; Low, P. A.; Naessens, J. M.; Wilson, P. R.; Amadio, P. C.;

O'Brien, P. C. Reflex sympathetic dystrophy [letter; comment]. Mayo-Clin-Proc.

1996 May; 71(5): 524; discussion 525; ISSN: 0025-6196.

Note: Comment on: Mayo Clin Proc 1995 Nov;70(11):1124-6.

UNITED-STATES.

112. Chen, C. J.; Huang, C. C.; Ro, L. S. Evolution of pontine and extrapontine

myelinolysis: clinical correlation with serial CT and MRI studies. Eur-Neurol.

1996; 36(3): 179-80; ISSN: 0014-3022.

SWITZERLAND.

113. Chen, C. J.; Ro, L. S.; Chang, C. N.; Ho, Y. S.; Lu, C. S. Serial MRI studies in

pathologically verified Balo's concentric sclerosis. J-Comput-Assist-Tomogr. 1996

Sep; 20(5): 732-5; ISSN: 0363-8715.

UNITED-STATES. We report serial MRI studies in a patient with Balo's

concentric sclerosis, who presented with a progressive weakness in the right limbs

of 10 day duration. Serial MR images revealed several concentric lesions

characterized by layer by layer of demyelinated and myelinated appearance and

multiple plaques in the white matter. Pathological study of a biopsy of a concentric

lesion (which was performed to rule out neoplasm) further confirmed this

diagnosis. These MR findings resolved in correspondence with clinical

improvement. The patient was almost completely normal 1 year later. In

conclusion, in this case, we found that MRI could show the striking feature

reminiscent of the histopathologic findings of Balo's concentric sclerosis and

played an important role in antemortem diagnosis of this rare demyelinating

disease.

114. Chen, R. M.; Lupski, J. R.; Greenberg, F.; Lewis, R. A. Ophthalmic manifestations

of Smith-Magenis syndrome. Ophthalmology. 1996 Jul; 103(7): 1084-91; ISSN:

0161-6420.

UNITED-STATES. PURPOSE: The Smith-Magenis syndrome (SMS) is a

multiple-anomaly, mental retardation syndrome associated with deletions of a

contiguous region of chromosome 17p11.2. Prior reports have described

ophthalmic anomalies with SMS, including telecanthus, ptosis, strabismus,

myopia, iris anomalies, cataracts, optic nerve hypoplasia, and retinal detachment.

This report defines the ophthalmic spectrum in 28 individuals with SMS subjected

to a multidisciplinary clinical and molecular survey. METHODS: Individuals with

deletion of chromosome 17p11.2 detected by high-resolution cytogenetic analysis

underwent complete ophthalmologic evaluation comprised of ophthalmic history,

visual acuity, cycloplegic refraction, motility, and biomicroscopic and

ophthalmoscopic examination. RESULTS: Among the 28 subjects, ranging in age

from 0.8 to 29.3 years, the most frequent ocular findings were iris anomalies

(68%), microcornea (50%), myopia (42%), and strabismus (32%). Bilateral

microphthalmos with uveal and retinal coloboma was observed in one individual.

No subject had cataract or retinal detachment. CONCLUSIONS: This is the largest

single-center series of subjects with SMS that includes ophthalmic evaluation. As in

prior reports, iris anomalies and strabismus were observed, but microcornea had

not been noted previously. The absolute refractive error was hypermetropic in half

of these subjects. Cataract, ptosis, and retinal pathology, including detachment,

were not observed in any subject. All individuals with SMS should be evaluated by

an ophthalmologist, with special attention to strabismus, microcornea, iris

anomalies, and refractive errors.

115. Cheng, G.; Liu, X.; Wu, J. X.; Jones, B. Establishing a reliable visual function test

and applying it to screening optic nerve disease in onchocercal communities. Int-J-

Biomed-Comput. 1996 Mar; 41(1): 47-53; ISSN: 0020-7101.

IRELAND. The computer Controlled Video Perimetry (CCVP) is a computer

screening test for detecting visual function loss caused by onchocerciasis,

glaucoma, etc. Installed on portable computers, the CCVP has been shown to be

high acceptability in field community investigation. However, it is regarded to be

difficult in obtaining reliable results from portable computer screening tests because

of human behavioural variants and the lack of standard testing environment. In this

paper, we propose an architecture for implementing a more reliable CCVP system.

In particular, a self-organising neural network is applied to manage measurement

noise caused by behavioural factors. A control unit is introduced to manage the

overall behaviour of the system. The integrated test system has been used to screen

optic nerve disease in onchocercal communities of rural Nigeria and the

experimental results obtained from a large number of test records are very

encouraging: reliable results from volatile test environments may be obtained using

the proposed method.

116. Chernobel'skii, S. I. [Differential diagnosis of unilateral paresis and paralysis of the

larynx]. K differentsial'noi diagnostike odnostoronnikh parezov i paralichei

gortani. Vestn-Otorinolaringol. 1996 Mar; (2): 24-6; ISSN: 0042-4668.

RUSSIA. Differential diagnosis of laryngeal paresis and paralysis using electron

glottography (EGG) was tried in 57 patients and 30 healthy controls.

Measurements were made of speed quotient (SQ) and open quotient (OQ) at piano

and forte. It was found that in patients and controls SQ and OQ were not similar.

SQ and OQ differed also in paralysis and paresis. Tonicity of the inner laryngeal

muscles seems to decline more in paralysis than in paresis. Aerodynamic properties

of the larynx are damaged more in paralysis and remain unchanged in paresis.

Reduced SQ and high OQ at forte indicate unilateral laryngeal paralysis. OQ should

be measured both at piano and forte. Thus, EGG is an effective procedure for

differential diagnosis of laryngeal paralysis and paresis. It is especially convenient

when laryngostroboscopy is problematic.

117. Chiou, Tan FY; Vennix, M. J.; Dinh, T. l.; Robinson, L. R. Comparison of

techniques for detecting digital neuropathy. Am-J-Phys-Med-Rehabil. 1996 Jul;

75(4): 278-82; ISSN: 0894-9115.

UNITED-STATES. A complication of endoscopic carpal tunnel release (6/53

consecutive cases) is rupture of a digital branch. The objective of this study was to

find a method for detecting neuropathy of the digital branch innervating the radial

side of the fourth digit and the ulnar side of the third digit. This study examined

whether the following sensory nerve conduction techniques would enhance

diagnosis of this neuropathy: (1) recording with standard digital ring electrodes; (2)

recording with ring electrodes from two adjacent fingers; and (3) disc electrodes

placed between two fingers. Ten healthy individuals were studied before and after

lidocaine anesthesia of the digital branch between the ring and middle finger.

Statistical analysis was performed with a two-tailed, paired t test. Results show that

after lidocaine injection there was a decrease in antidromic amplitude of 94% for

ring electrodes on the fourth digit, 62% for rings on the third digit, 77% for disc

electrodes between the fourth and third digits, and 74% for rings on digits 4 and 3

(P < 0.005), with no significant change in peak latency (P > 0.3). In conclusion,

although all techniques used in this model yielded a significant change in amplitude,

rings on the third digit compared with the second and discs between digits 4/3

compared with 3/2 were most specific without false-positives from normal data. It

is hoped this study will aid the electromyographer in postoperative diagnosis.

118. Chitty, L. S.; Robb, S.; Berry, C.; Silver, D.; Baraitser, M. PEHO or PEHO-like

syndrome? Clin-Dysmorphol. 1996 Apr; 5(2): 143-52; ISSN: 0962-8827.

ENGLAND. PEHO syndrome is a rare progressive infantile encephalopathy, with

variable age of onset of hypotonia, convulsions, mental retardation, oedema, and

optic atrophy. Neuroimaging shows cerebellar and brainstem atrophy in most

instances. A PEHO-like syndrome has been described in which those affected do

not have the typical changes on neuroimaging. We report four new cases, two

isolated cases and two sisters, who might be part of the PEHO-like syndrome.

119. Chong, V. F. Trigeminal neuralgia in nasopharyngeal carcinoma. J-Laryngol-Otol.

1996 Apr; 110(4): 394-6; ISSN: 0022-2151.

ENGLAND. Tumours at the skull base may show perineural infiltration of the

mandibular nerve. Subsequent retrograde spread into the cavernous sinus,

Gasserian ganglion, the trigeminal nerve and the pons may be seen. The patient, a

known and treated case of nasopharyngeal carcinoma (NPC), complained of

trigeminal neuralgia and difficulty in chewing. Magnetic resonance imaging (MRI)

revealed a Gasserian ganglion and trigeminal infiltration with resultant atrophy of

the muscles innervated by the mandibular nerve. Proximal cranial nerve

involvement should be suspected in patients with skull base malignancy presenting

with trigeminal neuralgia. MRI is the modality of choice in delineating the

pathological process.

120. Chuang, T. Y.; Lin, S. W.; Chan, R. C. Guillain-Barre syndrome: an unusual

complication after snake bite. Arch-Phys-Med-Rehabil. 1996 Jul; 77(7): 729-31;

ISSN: 0003-9993.

UNITED-STATES. The mortality rate of the Formosan krait bite has been reported

to be 23%; death is from respiratory paralysis caused by neuromuscular junctions

being blocked by bungarotoxin. This article presents the first case report of

Guillain-Barre syndrome after snake envenomization. The patient presented with

symmetric paresis and sensory signs in the upper and lower limbs, autonomic

dysfunction, facial nerve involvement, and mild elevated cerebrospinal fluid protein

at about 4 weeks after the bite. Electrodiagnostic studies revealed profound sensory

and motor polyneuropathy. Repeated electrophysiologic findings confirmed nerve

regeneration. The patient reached satisfactory functional outcome after a short-term

intensive rehabilitation program despite severe axonal degeneration. This article

also discusses the possible mechanism of immunopathogenesis of Guillain-Barre

syndrome after krait bite.. 0.

121. Clinchot, D. M.; Lorch, F. Sympathetic skin response in patients with reflex

sympathetic dystrophy. Am-J-Phys-Med-Rehabil. 1996 Jul; 75(4): 252-6; ISSN:

0894-9115.

UNITED-STATES. The sympathetic skin response (SSR) was recorded in four

patients diagnosed with reflex sympathetic dystrophy (RSD) in one upper limb

using Kozin's clinical criteria in conjunction with a three-phase bone scan. All

patients had sustained cerebral vascular accidents and were classified as Stage I

RSD. The SSR was recorded in both hands after each of ten contralateral median

nerve surface stimulations and in both feet after each of ten contralateral peroneal

nerve surface stimulations. Amplitude, onset latency, and number of phases were

recorded for all responses in each limb. Mean amplitude, onset latency, and the

number of phases of the five largest potentials were then determined. In all patients,

there was a statistically significant difference in the amplitude and latency of the

SSR in the involved limb compared with the uninvolved limb; mean amplitude of

the involved limb was greater than the mean amplitude of the uninvolved limb (P <

0.001), and latency to onset of the SSR in the involved limb was shorter than that

of the uninvolved limb (P < 0.001). There was no statistically significant difference

in mean amplitude and latency between the involved side and uninvolved side

responses as measured at the feet.

122. Cobb, T. K.; Amadio, P. C.; Leatherwood, D. F.; Schleck, C. D.; Ilstrup, D. M.

Outcome of reoperation for carpal tunnel syndrome. J-Hand-Surg-Am. 1996 May;

21(3): 347-56; ISSN: 0363-5023.

UNITED-STATES. One hundred thirty-one patients with reoperation for carpal

tunnel syndrome were followed for a mean of 11 years. Reoperation failed in 15

patients, necessitating a third operation. Satisfaction, symptom severity, and

functional status scores were assessed with a standardized questionnaire in the

other 116 patients. Patients with normal findings on preoperative nerve conduction

studies, those who filed for compensation, and those who had pain in the

distribution of the ulnar nerve had significantly worse results. Those with abnormal

findings on nerve conduction studies who had not filed for compensation had the

best symptom and function scores and satisfaction at latest follow-up examination;

those with normal findings on nerve conduction studies who had filed for

compensation had the poorest outcome. Although most patients were satisfied with

the overall outcome, many reported residual symptoms; in addition to the 15

patients who required a third operation, 22 patients were dissatisfied with the final

result.

123. Cohen, G. S.; Braunstein, L.; Ball, D. S.; Domeracki, F. Effort thrombosis: effective

treatment with vascular stent after unrelieved venous stenosis following a surgical

release procedure. Cardiovasc-Intervent-Radiol. 1996 Jan; 19(1): 37-9; ISSN:

0174-1551.

UNITED-STATES. Acute symptomatic effort thrombosis in a 33-year-old male

necessitated an aggressive approach consisting of thrombolysis, angioplasty, and

surgical thoracic outlet release. The patient required postoperative placement of a

Wallstent and was placed on anticoagulation. He has remained symptom free for

the past 10 months, both clinically and sonographically.

124. Coker, S. B.; Ros, S. P. Ptosis associated with sinusitis. Pediatr-Neurol. 1996 Jan;

14(1): 62-3; ISSN: 0887-8994.

UNITED-STATES. An adolescent male developed eye pain and a drooping lid.

Imaging revealed adjacent pansinusitis and a swollen levator palpebrae and superior

rectus muscle. Compression of a branch of the oculomotor nerve is the postulated

cause because vertical eye movements were normal.

125. Consensus statement on the definition of orthostatic hypotension, pure autonomic

failure, and multiple system atrophy. The Consensus Committee of the American

Autonomic Society and the American Academy of Neurology. Neurology. 1996

May; 46(5): 1470; ISSN: 0028-3878.

UNITED-STATES.

126. Cook, M. W.; Levin, L. A.; Joseph, M. P.; Pinczower, E. F. Traumatic optic

neuropathy. A meta-analysis. Arch-Otolaryngol-Head-Neck-Surg. 1996 Apr;

122(4): 389-92; ISSN: 0886-4470.

UNITED-STATES. BACKGROUND: The management of traumatic optic

neuropathy remains controversial. Reports of improvement have been published

after observation alone, treatment with corticosteroids and surgical

decompressions. OBJECTIVE: To systematically review the published literature

about traumatic optic neuropathy using a meta-analysis. METHODS: We performed

a retrospective literature review of case series and case reports of traumatic optic

neuropathy. They include all English language cases and selected non-English

language cases for which patient data were available. The cases were organized into

four grades based on visual acuity and the locations and type of fracture. Grade 1

included patients with visual acuity greater than 20/200 in the affected eye and

without a posterior orbit fracture; grade 2, patients with visual acuity between

20/200 and light perception; grade 3, patients without light perception or with a

nondisplaced posterior orbital fracture and remaining vision; and grade 4, patients

with no light perception and a displaced posterior orbital fracture. A meta-analysis

was performed, analyzing for each case the recovery of visual acuity for treatment,

fracture pattern, and grade. RESULTS: The recovery of vision in treated patients

was significantly better than the recovery in patients receiving no treatment. No

significant difference in improvement was found among patients treated with

corticosteroids alone, with surgical decompression alone, or with corticosteroids

and surgical decompression. Recovery was related to the severity of initial injury,

as reflected in the grading system. A trend was noted for better improvement of

visual acuity in patients without orbital fractures than those with orbital fractures,

and also in patients with anterior orbital fractures than in patients with posterior

fractures. CONCLUSIONS: Treatment with corticosteroids, extracranial

decompression, or both, is better than no treatment of traumatic optic neuropathy.

Because the data are insufficient to determine whether corticosteroids, surgery, or

the use of both treatments is most effective, the findings of the ongoing

International Optic Nerve Trauma Study should prove valuable. The standardized

grading system we developed is a useful tool for comparing studies and treatment

protocols.. 0.

127. Cook, T. A.; Rahim, N.; Simpson, H. C.; Galland, R. B. Magnetic resonance imaging

in the management of diabetic foot infection. Br-J-Surg. 1996 Feb; 83(2): 245-8;

ISSN: 0007-1323.

ENGLAND. A prospective study was carried out of 22 patients admitted with 25

diabetic foot infections. All had cellulitis, 12 had discharging ulcers and eight had

digital gangrene. In one case magnetic resonance imaging (MRI) was unhelpful

owing to patient movement. Thirteen scans suggested deep-seated infection,

including abscess (ten), osteomyelitis (seven) and ankle effusion (one). Overall,

imaging provided a specificity of 77 per cent, a positive predictive value of 77 per

cent, a sensitivity of 91 per cent and a negative predictive value of 91 per cent. MRI

is valuable in determining the presence and extent of infection, which allows

appropriate planning of surgical intervention.

128. Cooke, E. Dealing with the diabetic foot. Practitioner. 1996 Feb; 240(1559): 112-4;

ISSN: 0032-6518.

ENGLAND.

129. Copley, L. A.; Dormans, J. P.; Davidson, R. S. Vascular injuries and their sequelae in

pediatric supracondylar humeral fractures: toward a goal of prevention. J-Pediatr-

Orthop. 1996 Jan; 16(1): 99-103; ISSN: 0271-6798.

UNITED-STATES. Between 1988 and 1994, 128 consecutive children with grade

III supracondylar humeral fractures presented for treatment at our hospital.

Seventeen had absent or diminished (detected with Doppler but not palpable) radial

pulses on initial examination. Fourteen of these 17 children recovered pulse

(palpable) after reduction and stabilization of their fractures. The remaining three

had persistent absence of radial pulse. Each of these three children was explored

immediately and found to have a significant vascular injury requiring repair. Two

of the 14 children who had initially regained their pulses had a progressive

postoperative deterioration in their circulatory status during the first 24-36 h,

including loss of the radial pulse. Both of these children had arteriograms that

identified vascular injuries. Both underwent exploration and bypass grafting. One

of these two children had been transferred 48 h after injury, resulting in delay of

management of his vascular impairment. Despite exploration, vascular repair, and

fasciotomy, he ultimately developed Volkmann's ischemic contracture. All five

children with significant vascular injuries had absent or diminished radial pulses on

presentation. Immediate reduction and fixation followed by careful evaluation and

treatment of ischemia were associated with excellent outcome in four of the five

children.

130. Cornblath, D. R. The electrophysiology of axonal and demyelinating polyneuropathies.

Baillieres-Clin-Neurol. 1996 Mar; 5(1): 107-13; ISSN: 0961-0421.

ENGLAND. Electrodiagnostic studies, which include nerve conduction studies,

electromyography, repetitive nerve stimulation, single fibre EMG, autonomic

function tests and quantitative sensory tests are a critical component of the

neuromuscular evaluation. These studies, which are an extension of the clinical

examination, help to classify further patients into the part of the peripheral nervous

system affected and provide significant insight into the pathophysiological

processes that underlie clinical illnesses. Traditionally, patients with peripheral

neuropathy are classified as having demyelinating or axonal neuropathies. The

methods of making this distinction are discussed. In addition, a number of

additional studies are now available which provide further, insights into selected

aspects of the peripheral nervous system.

131. Couper, J. Microvascular complications of insulin-dependent diabetes: risk factors,

screening and intervention. J-Paediatr-Child-Health. 1996 Feb; 32(1): 7-9; ISSN:

1034-4810.

AUSTRALIA. The ability to detect subclinical signs of the microvascular

complications of diabetes during adolescence and our increased understanding of

risk factors for their development provide an opportunity to prevent irreversible

organ damage. Glycaemic control makes a major contribution to the risk and

progression of microvascular complications. However, the unique psychological

and physiological changes of childhood and adolescence present a considerable

challenge for those attempting to reduce the burden of adult microvascular disease.

132. Cranovsky, C. [Neuralgic shoulder amyotrophy]. Die neuralgische

Schulteramyotrophie. Schweiz-Med-Wochenschr. 1996 Jan 27; 126(4): 111-9;

ISSN: 0036-7672.

SWITZERLAND. The syndrome of neuralgic myatrophy of the shoulder usually

presents with a classical clinical picture: sudden onset of intense pain in the

shoulder and/or upper arm, followed after hours, days or weeks by (often

myatrophic) paresis of the muscles of the shoulder region. Palsy and muscle

atrophy reach their maximum within 4 weeks. On the basis of this typical sequence,

the diagnosis was established in 22 patients during the period from 1983 to 1994.

In agreement with previous observations, males represented double the number of

female patients. The mean age at onset of the syndrome was 52.6 years (range 16-

81 years). Possible triggering factors were registered in 11/12 patients: infections,

abnormal physical activity or operative procedures 1-3 weeks ahead of the first

symptoms. No preferential lateralization was noted. Most frequently paresis

occurred in the deltoid and--in descending order--in the infraspinatus,

supraspinatus and biceps muscles. Scapula alata was observed 7 times in 5 patients

and concomitant unilateral paresis of the diaphragm in 3 cases. In 5/22 patients

NSA was bilateral, simultaneous or successive, usually asymmetrical and severe.

Symptoms of sensory deficit were rare and usually resolved rapidly.

Electromyographic examination was essential for diagnosis, pointing to

localization, follow-up and prognosis. Follow-up information was obtained in a

majority of patients and in 17/20 cases showed a favourable course with complete

recovery.

133. Croll, S. D.; Nicholas, G. G.; Osborne, M. A.; Wasser, T. E.; Jones, S. Role of

magnetic resonance imaging in the diagnosis of osteomyelitis in diabetic foot

infections. J-Vasc-Surg. 1996 Aug; 24(2): 266-70; ISSN: 0741-5214.

UNITED-STATES. PURPOSE: The role of magnetic resonance imaging (MRI) in

the diagnosis of osteomyelitis in foot infections in diabetics was investigated. The

accuracy, sensitivity, and specificity of MRI, plain radiography, and nuclear

scanning were determined for diagnosing osteomyelitis, and a cost comparison was

made. METHODS: Twenty-seven patients with diabetic foot infections were

studied prospectively. All patients underwent MRI and plain radiography. Twenty-

two patients had technetium bone scans, and 19 patients had Indium scans.

Nineteen patients had all four tests performed. Patients with obvious gangrene or a

fetid foot were excluded. RESULTS: The diagnosis of osteomyelitis was

established by pathologic specimen (n = 18), bone culture (n = 3), or successful

response to medical management (n = 6). Osteomyelitis was confirmed in nine of

the pathologic specimens. The diagnostic sensitivity, specificity, and accuracy for

MRI was 88%, 100%, and 95%, respectively, for plain radiography it was 22%,

94%, and 70%, respectively, for technetium bone scanning it was 50%, 50%, and

50%, respectively, and for Indium leukocyte scanning it was 33%, 69%, and 58%,

respectively. The data were analyzed statistically with the two-tailed Fisher's exact

test. MRI was the only test that was statistically significant (p < 0.01).

CONCLUSIONS: MRI appeared to be the single best test for the diagnosis of

osteomyelitis associated with diabetic foot infections. It had a better diagnostic

accuracy than conventional modalities and appeared to be more cost-effective than

the frequently used Indium scan.. 0; 0; 14514-42-2; 148-24-3; 63347-66-0.

134. Crooks, D. A.; Miles, J. B. Trigeminal neuralgia due to vascular compression in

multiple sclerosis--post-mortem findings. Br-J-Neurosurg. 1996 Feb; 10(1): 85-8;

ISSN: 0268-8697.

ENGLAND. A 71-year-old male with multiple sclerosis and trigeminal neuralgia

due to vascular compression underwent multiple radio-frequency nerve lesioning

bilaterally. He was anaesthetic on the left side and required right microvascular

decompression. He died after this procedure. The pathology of the pons and the

effects of treatment are described.

135. Cucuzzella, T. R.; Guille, J. T.; MacEwen, G. D. Charcot-Marie-Tooth disease

associated with hip dysplasia: a case report. Del-Med-J. 1996 Jun; 68(6): 305-7;

ISSN: 0011-7781.

UNITED-STATES. A 31-year-old woman with a known history of hip dysplasia

was found to have Charcot-Marie-Tooth disease following abnormal conduction

studies done at the time of surgery. Physical examination in this patient was

otherwise normal, and the diagnosis of Charcot-Marie-Tooth disease had not been

previously considered. This report demonstrates the importance of keeping in mind

the association between hip dysplasia and Charcot-Marie-Tooth disease.

136. Czech, A.; Luzniak, P. [Diabetic neuropathy: overview of clinical problems].

Neuropatia cukrzycowa: przeglad problemow klinicznych. Pol-Tyg-Lek. 1996 Jan;

51(1-5): 33-4; ISSN: 0032-3756.

POLAND.

137. Dalakas, M. C.; Cupler, E. J. Neuropathies in HIV infection. Baillieres-Clin-Neurol.

1996 Mar; 5(1): 199-218; ISSN: 0961-0421.

ENGLAND. Peripheral neuropathies represent the most common neurological

manifestation in patients infected with HIV infection occurring either early in the

infection or during the course of the illness. They present as acute or chronic

demyelinating neuropathies (Guillain-Barre syndrome or chronic inflammatory

demyelinating polyneuropathy), mononeuritis multiplex, ganglioneuronitis,

cytomegalovirus-related polyradiculoneuropathy, autonomic neuropathy or distal

painful sensory neuropathy. They are multifactorial in aetiology. Their putative

cause (viral, autoimmune, toxic, nutritional, co-infections) are often dictated by the

stage of the underlying HIV disease. The virus, which is not found within

ganglionic neurones or Schwann cells but only within the endoneurial

macrophages, may generate a tissue-specific autoimmune attack by secretion of

cytokines that promote trafficking of activated T cells and macrophages within the

endoneurial parenchyma. The wide use of the neurotoxic antiretroviral nucleoside

analogues ddC, ddI, d4T and 3TC, exacerbate or trigger subclinical neuropathy in

many of these patients.. 0.

138. Danielides, V.; Skevas, A.; van Cauwenberge, P.; Vinck, B.; Tsanades, G.;

Plachouras, N. Facial nerve palsy during pregnancy. Acta-Otorhinolaryngol-Belg.

1996; 50(2): 131-5; ISSN: 0001-6497.

BELGIUM. The aim of this prospective study was to investigate whether

pregnancy predisposes to facial nerve palsy and what the specific properties of

facial nerve palsy are during this period. Eight cases of idiopathic facial nerve palsy

occurring during the last trimester of pregnancy are presented. Out of 50 women

with idiopathic facial nerve palsy, 15 were in childbearing age; eight presented a

facial nerve palsy during pregnancy of which 6 during the last trimester of

pregnancy. The electrodiagnostic tests indicated nerve degeneration in two of the

eight cases; they were treated with corticosteroids. In the other six patients, no

nerve degeneration was observed, and therefore, no treatment was given. These

data suggest that pregnancy is probably associated with an increased predisposition

to facial nerve palsy.

139. Daniloova, E. I.; Grafova, V. N.; Ostrovskaia, R. U.; Reshetniak, V. K. [Action of

sodium oxybutyrate in pain syndromes]. Deistvie oksibutirata natriia pri bolevykh

sindromakh. Biull-Eksp-Biol-Med. 1996 Apr; 121(4): 395-8; ISSN: 0365-9615.

RUSSIA. 502-85-2.

140. Dario, A.; Scamoni, C.; Cerati, M.; Marra, A.; Dorizzi, A. Associated olfactory groove

meningioma and acoustic neurinoma. Tumori. 1996 Jul; 82(4): 405-7; ISSN: 0300-

8916.

ITALY. The authors describe the case of a 71-year-old man without signs of

phacomatosis with a history of progressive psychomotory lowering and two

simultaneous tumors of the olfactory groove and of the cerebellopontine angle, as

demonstrated by CT scans. Histologic examinations showed the lesions to be a

meningioma and a neurinoma. The rarity and the possible pathogenesis of this

association are discussed.

141. Darsow, U.; Lorenz, J.; Bromm, B.; Ring, J. Pruritus circumscriptus sine materia: a

sequel of postzosteric neuralgia. Evaluation by quantitative psychophysical

examination and laser-evoked potentials. Acta-Derm-Venereol. 1996 Jan; 76(1):

45-7; ISSN: 0001-5555.

NORWAY. A case of circumscribed pruritus existing since 1 year on clinically

uninvolved skin is reported, in which careful history revealed a 5-year previous

episode of herpes zoster in the same dermatome. Impairment of cutaneous

sensitivity was evaluated by use of a quantitative psychophysical examination and

laser-evoked cortical potentials (LEP).. 404-86-4.

142. Das, I.; Jayatunga, A. P.; Symonds, J. M. Pyomyositis: an unusual infection due to

staphylococcus aureus. J-R-Coll-Surg-Edinb. 1996 Jun; 41(3): 182-3; ISSN:

0035-8835.

ENGLAND. Pyomyositis is a primary pyogenic infection of skeletal muscle,

leading to the formation of intramuscular abscesses. Although common in tropical

climates, it is infrequent in temperate zones. We report a patient who developed the

condition without travelling to tropical areas.

143. Dasgupta, A. K.; Harrison, J. Effects of vibration on the hand-arm system of miners in

India. Occup-Med-Oxf. 1996 Feb; 46(1): 71-8; ISSN: 0962-7480.

ENGLAND. Sixty-six Jackhammer drillers and 35 blasters from two mines were

clinically screened for hand-arm vibration syndrome (HAVS). The screening

consisted of questionnaire-based information, clinical examination including

measurement of phalangeal circumferences of fingers (FCT) and motor nerve

conduction (MCV) studies. In a warm environment, the prevalence of symptom

complexes suggested the existence of peripheral neuropathy and musculoskeletal

abnormalities rather than any peripheral circulatory disorders. Clinical examination

revealed soft tissue wasting in the hands (26 cases), ulnar nerve impairment (23

cases), median nerve impairment (16 cases) and Dupuytren's contracture (4 cases).

Mean motor nerve conduction velocities of 59 out of 66 drillers who volunteered

for this assessment were not significantly different from those of the 35 blasters.

The results of their correlation coefficients varied considerably for MCVs when age

and years of vibration exposure underwent regression analysis. However,

comparison of MCV and FCT of 30 drillers with 30 matched blasters revealed that

the mean MCV of the right median nerve in the vibration exposed group was

significantly decreased (p < 0.01) and for the mean FCT, the proximal phalanxes of

the right index finger, left thumb and left ring fingers were thinner in the vibration

exposed group (p < 0.05).

144. Date, E. S.; Mar, E. Y.; Bugola, M. R.; Teraoka, J. K. The prevalence of lumbar

paraspinal spontaneous activity in asymptomatic subjects. Muscle-Nerve. 1996

Mar; 19(3): 350-4; ISSN: 0148-639X.

UNITED-STATES. Electrodiagnostic findings of fibrillations and positive sharp

waves in the lumbosacral paraspinals in patients without previous back surgery has

been generally considered to be abnormal, consistent with posterior rami

denervation. In some cases, it is the only abnormality on the electromyographic

examination. This study was undertaken to determine the prevalence of abnormal

spontaneous activity in lumbosacral paraspinals in asymptomatic individuals. Nine

(14.5%) of 62 subjects studied had positive sharp waves or fibrillations noted on

the needle examination of bilateral lumbosacral paraspinal muscles. There was a

significant increase in the prevalence of abnormal activity with increasing age. This

suggests that caution should be taken in attributing radiculopathy as the etiology of

low back pain when electromyographic lumbosacral paraspinal abnormalities are

the only positive findings in the middle-aged or older individual.

145. Davies, R. H. Home ventilation of a child with motor and sensory neuropathy. BMJ.

1996 Jul 20; 313(7050): 153-4; discussion 154-5; ISSN: 0959-8138.

ENGLAND.

146. De, la Meilleure G.; Dehaene, I.; Depondt, M.; Damman, W.; Crevits, L.; Vanhooren,

G. Benign paroxysmal positional vertigo of the horizontal canal. J-Neurol-

Neurosurg-Psychiatry. 1996 Jan; 60(1): 68-71; ISSN: 0022-3050.

ENGLAND. OBJECTIVES--To review the clinical features,

electronystagmography findings, the possible mechanism, and a possible

therapeutic approach to benign paroxysmal positional vertigo (BPPV). METHODS-

-Sixty-three cases of BPPV of the horizontal canal type have been reviewed. It is

characterised by horizontal nystagmus and an intense vertigo, provoked by rotation

of the head in a supine patient. The horizontal nystagmus beats towards the ground

on both sides, becomes more pronounced when lying on the pathological side, and

then the nystagmus often changes direction. RESULTS--Forty-eight patients

underwent electronystagmography. On the pathological side, the first phase

nystagmus had a mean latency of three seconds and a mean duration of 31.6

seconds. Nystagmus inversion occurred in 36 patients after a nystagmus free

interval. The mean second phase nystagmus duration lasted 33.4 seconds. On the

healthy side, the nystagmus had a mean latency of 3.4 seconds and a mean duration

of 39.5 seconds. Fatigue was seen in six patients. Simultaneous involvement of the

posterior canal was present in 16 patients. A liberatory manoeuvre was successful

in six patients. CONCLUSIONS--The liberatory manoeuvre should be tried in

patients with horizontal canal vertigo. It should not be performed in patients with

severe cervical arthrosis, vertebrobasilar insufficiency, or when the patient has neck

pain during the manoeuvre.

147. de Araujo, M. P. Electrodiagnosis in compression neuropathies of the upper

extremities. Orthop-Clin-North-Am. 1996 Apr; 27(2): 237-44; ISSN: 0030-5898.

UNITED-STATES. Compression neuropathies may occur at several points along

the course of a nerve. Electrodiagnostic studies are helpful in the evaluation of

nerve compression. Nerve conduction studies are the most useful of these

techniques in determining the site of compression. Compression neuropathies of

the upper extremities are common, and a well planned study is important to localize

the site of involvement and the severity of the nerve damage.

148. De Decker, V.; Pera, S. B.; Borenstein, S.; Tombroff, M. [A case of Guillain-Barre

syndrome associated with SIADH, treated by intravenous gammaglobulins]. A

case of Guillain-Barre syndrome associated with SIADH, treated by intravenous

gammaglobulins. Acta-Clin-Belg. 1996; 51(3): 170-4; ISSN: 0001-5512.

BELGIUM. The authors report a case of Guillain-Barre syndrome occurring after

a hip replacement associated with hyponatremia due to a syndrome of inappropriate

secretion of antidiuretic hormone (SIADH). They describe and review the literature

about this syndrome's etiology and its association, relatively rare, with SIADH.

The diagnostic reasoning and the therapeutic possibilities are described.. 0.

149. De Freitas, G. R.; De Freitas, M. R.; Nascimento, O. J. [Sural nerve biopsy in

myotonic muscular dystrophy]. Biopsia do nervo sural na distrofia muscular

miotonica. Arq-Neuropsiquiatr. 1996 Mar; 54(1): 19-24; ISSN: 0004-282X.

BRAZIL. Twelve patients with myotonic dystrophy were studied to look for the

involvement of the peripheral nervous system in this disease. All of them showed

the main signs and symptoms of the disease. They did not have another causes to

justify a polineuropathy. They were submitted to sural nerve biopsy with counting

of myelinated fibers and histogram. Patients showed a reduction in the number of

myelinated fibers and in two patients the histogram was unimodal. We concluded

that polineuropathy may be another multisystemic manifestation of myotonic

dystrophy.

150. de Heus, van Putten MA; Schaper, N. C.; Bakker, K. The clinical examination of the

diabetic foot in daily practise. Diabet-Med. 1996; 13 Suppl 1: S55-7; ISSN: 0742-

3071.

ENGLAND.

151. de Pedro, Cuesta J.; Abraira, V.; Jiang, G. X.; Solders, G.; Fredrikson, S. Guillain-

Barre syndrome in South-West Stockholm, 1973-1991, 3. Clinicoepidemiological

subgroups. Acta-Neurol-Scand. 1996 Feb; 93(2-3): 175-83; ISSN: 0001-6314.

DENMARK. Using hierarchical cluster analysis, applied to 47 cases of Guillain-

Barre Syndrome (GBS) incident in South-West Stockholm (SWS) during the

period from January 1973 to June 1992, we identified three major

clinicoepidemiological subgroups. The first subgroup, 25.5% of the cases (26.7

+/- 6.7 years), recorded a peak incidence at ages 20-29 years and presented

significant differences from other subgroups, a high proportion of cases with onset

at low age preceded by respiratory infection (83.3%) and with normal motor

conduction velocity (50.0%). Also found, were less affected biological parameters,

a rapidly progressive course and independence in gait at one month after onset. A

second subgroup, 27.7% of cases, was severely affected, clinically and

functionally. It consisted predominantly of young individuals (22.7 +/- 11.1

years), with a high incidence (69.2% of cases) in autumn. A third subgroup,

comprising 40.4% of cases, was older (61.1 +/- 11.0 years) and, in general, also

severely affected. The incidence of this form appeared to be invariant with time.

152. De Potter, P.; Shields, C. L.; Eagle, RC Jr; Shields, J. A.; Lipkowitz, J. L. Malignant

melanoma of the optic nerve. Arch-Ophthalmol. 1996 May; 114(5): 608-12; ISSN:

0003-9950.

UNITED-STATES. A 67-year-old man was diagnosed as having a melanocytoma

of the optic disc in the left eye. Observation during a 5-year period showed no

change in the lesion. At age 72 years, he had abrupt visual loss to no light

perception in the affected left eye. Clinical examination disclosed little enlargement

of the papillary tumor but ultrasonographic evidence of optic nerve infiltration.

Precontrast magnetic resonance imaging studies disclosed a hyperintense infiltrative

lesion in the enlarged left optic nerve. Enhancement features of the lesion excluded

a hemorrhagic process. The eye was removed with a 22.5-mm segment of optic

nerve. Histopathologic examination showed a large, necrotic, mixed-cell malignant

melanoma confined to the optic nerve. No choroidal involvement or viable

melanocytoma cells were documented. This case stresses that it may be difficult to

differentiate a melanocytoma from a primary malignant melanoma of the optic

nerve.

153. De Vries, D. D.; Went, L. N.; Bruyn, G. W.; Scholte, H. R.; Hofstra, R. M.;

Bolhuis, P. A.; van Oost, B. A. Genetic and biochemical impairment of

mitochondrial complex I activity in a family with Leber hereditary optic neuropathy

and hereditary spastic dystonia. Am-J-Hum-Genet. 1996 Apr; 58(4): 703-11;

ISSN: 0002-9297.

UNITED-STATES. A rare form of Leber hereditary optic neuropathy (LHON) that

is associated with hereditary spastic dystonia has been studied in a large Dutch

family. Neuropathy and ophthalmological lesions were present together in some

family members, whereas only one type of abnormality was found in others.

mtDNA mutations previously reported in LHON were not present. Sequence

analysis of the protein-coding mitochondrial genes revealed two previously

unreported mtDNA mutations. A heteroplasmic A-->G transition at nucleotide

position 11696 in the ND4 gene resulted in the substitution of an isoleucine for

valine at amino acid position 312. A second mutation, a homoplasmic T-->A

transition at nucleotide position 14596 in the ND6 gene, resulted in the substitution

of a methionine for the isoleucine at amino acid residue 26. Biochemical analysis of

a muscle biopsy revealed a severe complex I deficiency, providing a link between

these unique mtDNA mutations and this rare, complex phenotype including Leber

optic neuropathy.. EC 1.; EC 1.6.9.-; EC 1.6.99.2; EC 1.6.99.3; EC 4.1.3.7; 0.

154. Deffond, D.; Clavelou, P.; Colamarino, R.; Roche, C.; Dordain, G.; Tournilhac, M.

[Neurogenic muscle hypertrophy: 3 cases]. L'hypertrophie musculaire neurogene:

3 cas. Rev-Neurol-Paris. 1996 Apr; 152(4): 272-8; ISSN: 0035-3787.

FRANCE. Three cases of neurogenic muscular hypertrophy are reported. First

case presented a hypertrophy of one calf following S1 radiculopathy; the second

had a hypertrophy of tibialis anterior muscle, five years after a compressive injury

of the common peroneal nerve. The third case is a global hypertrophy of leg

muscles after intensive care or legionella neuropathy. Previous cases of the

literature are reviewed and the various pathophysiological hypothesis are

considered. Among these hypothesis, the role of abnormal electrophysiological

activities in most previous cases, as well as in ours (namely complex repetitive

discharges and fasciculations), seems the most consistent.

155. Delcourt, C.; Villatte Cathelineau, B.; Vauzelle Kervroedan, F.; Papoz, L. Clinical

correlates of advanced retinopathy in type II diabetic patients: implications for

screening. The CODIAB-INSERM-Zeneca Pharma Study Group. J-Clin-

Epidemiol. 1996 Jun; 49(6): 679-85; ISSN: 0895-4356.

ENGLAND. The clinical correlates of advanced retinopathy were determined in a

sample of 427 type II diabetic outpatients, aged 35 to 74 years, recruited in eight

centers from all parts of France. The presence of retinopathy was assessed by

fluorescein angiography with centralized interpretation. Advanced retinopathy

(proliferative and/or macular edema) was independently linked with nephropathy,

peripheral neuropathy, and insulin therapy. Prevalence of advanced retinopathy

was 1.6% in the absence of signs of nephropathy and/or peripheral neuropathy,

10.4% in patients with mild signs, and 17.5% in patients with moderate to severe

signs. Overall, 87% of the patients with advanced retinopathy had signs of

nephropathy and/or peripheral neuropathy. In conclusion, patients showing signs

of nephropathy and/or peripheral neuropathy should be sent in priority to an

ophthalmologist. Prospective data are necessary to determine if screening is

necessary in patients with no signs of nephropathy or peripheral neuropathy.

156. Delfini, R.; Missori, P.; Tarantino, R.; Ciapetta, P.; Cantore, G. Primary benign

tumors of the orbital cavity: comparative data in a series of patients with optic nerve

glioma, sheath meningioma, or neurinoma. Surg-Neurol. 1996 Feb; 45(2): 147-53;

discussion 153-4; ISSN: 0090-3019.

UNITED-STATES. BACKGROUND: Patients operated on for the most common

benign pathologies of the orbital cavity-optic nerve glioma, sheath meningioma and

neurinoma-should be surgically treated. However, postoperative visual impairment

is frequently inevitable. A wait-and-see policy due to a slow rate of growth of these

lesions is criticized. METHODS: Collecting data from three series of patients

operated, we compare the surgical procedures and long-term results. RESULTS:

"En bloc" removal in patients with optic nerve glioma led to complete visual deficit

but ensures excellent long-term prognosis. Because optic nerve meningiomas are

typically circumferential to the optic nerve and adhere tightly to the perineural pial

microvascular structures, it is impossible to avoid trauma to the optic nerve and

recurrences. Patients with neurinoma of the orbital cavity have the most favourable

prognosis both in terms of visual function as well as long-term quality of life. Due

to its slow rate of growth, a wait-and-see policy can be adopted for optic nerve

glioma before deciding on surgical removal, whereas surgical treatment of

meningioma may be postponed if symptoms are slight and steady. Removal of

orbital cavity neurinoma should not be postponed since surgical outcome is

excellent.

157. Delgado, M. R. Guillain-Barre syndrome: a pediatric challenge [editorial]. J-Child-

Neurol. 1996 Jan; 11(1): 1-3; ISSN: 0883-0738.

UNITED-STATES. 0.

158. Dematteis, J. A. Guillain-Barre syndrome: a team approach to diagnosis and treatment.

Am-Fam-Physician. 1996 Jul; 54(1): 197-200; ISSN: 0002-838X.

UNITED-STATES. Guillain-Barre syndrome is the most frequently acquired

demyelinating peripheral polyneuropathy. In approximately two-thirds of cases,

Guillain-Barre syndrome is preceded by a viral respiratory or gastrointestinal

infection. The mechanism of injury is unclear but is believed to be immunologic.

The cardinal clinical feature is symmetric and rapidly progressive weakness.

Aspiration and respiratory failure are the major concerns. Sensory symptoms, such

as paresthesias, are common. The most severe stage of the disease is reached two

to four weeks after onset. Dysautonomia has replaced respiratory failure as the

most common cause of death. Recovery is variable: 50 percent of patients recover

completely, about 35 percent experience permanent neurologic sequelae, and 15

percent are significantly and permanently damaged. About 10 percent relapse before

complete recovery, and 2 to 5 percent experience recurrence after full recovery.

Laboratory confirmation of Guillain-Barre syndrome includes the typical

cerebrospinal fluid cytoalbumin dissociation (elevated protein without white blood

cells). Treatment is primarily symptomatic and preventive. Convalescent patients

require intensive inpatient physical and occupational therapy to improve strength

and prevent disabling contractures.

159. Dengler, R.; Wohlfarth, K.; Zierz, S.; Jobges, M.; Schubert, M. Muscle fatigue,

lactate, and pyruvate in mitochondrial myopathy with progressive external

ophthalmoplegia. Muscle-Nerve. 1996 Apr; 19(4): 456-62; ISSN: 0148-639X.

UNITED-STATES. We studied muscle fatigue and serum lactate and pyruvate

levels in 20 patients with mitochondrial myopathy with progressive external

ophthalmoplegia (PEO). Fatigue was assessed in the adductor pollicis muscle (AP)

using a low-intensity exercise protocol (20 min). Forces (TFs) and relaxation times

of ulnar nerve evoked twitches, compound muscle action potentials (CMAPs), and

maximal voluntary contractions (MVCs) were monitored. Serum lactate and

pyruvate levels were independently measured at rest and after exercise on a bicycle

(15 min, 30 W). Most patients showed abnormal fatigue of the AP with a reduction

of TFs and MVCs and normal CMAPs. The reduced TFs were significantly

correlated with lactate levels at rest (r= - 0.60, P<0.05) and less so with those after

exercise (r=- 0.47,P<0.05). Pyruvate levels revealed a similar correlation although

they were widely scattered. We conclude that abnormal fatigue in PEO is metabolic,

is localized beyond the muscle fiber membrane, and involves the electrome-chanical

coupling and the contractile apparatus. Serum lactate levels at rest are good

predictors of fatigue in PEO.. 0; 0; 127-17-3; 50-21-5.

160. Dereymaeker, G.; Schroven, I.; Steenwerckx, A.; Stuer, P. Results of excision of the

interdigital nerve in the treatment of Morton's metatarsalgia. Acta-Orthop-Belg.

1996 Mar; 62(1): 22-5; ISSN: 0001-6462.

BELGIUM. Thirty-one patients (thirty-two feet) had excision of the interdigital

nerve as treatment of their Morton's metatarsalgia. A longitudinal dorsal incision

was used in all cases. Twenty-five out of 32 cases had a macroscopically visible

neuroma, and only two had no evidence of a neuroma on histological examination.

All thirty-two patients were available for follow-up at an average of 44.7 months

(range 14 to 71 months) postoperatively. Eighty-one per cent of the patients had a

good or excellent result, 12.5% had a fair result, with residual pain and some

restriction of activities, and 6.5% had no improvement after their operation. It is

noteworthy that 19 patients (60%) benefitted from wearing adapted shoes or inner

soles for a considerable time after the operation. Even at final follow-up, only 10

patients (30%) had no restrictions in the choice of their shoes.

161. Desikan, P. Leprous neuropathy as an autoimmune phenomenon. Indian-J-Lepr. 1996

Jan; 68(1): 15-21; ISSN: 0254-9395.

INDIA. 0.

162. Diaz Villoslada, P.; Nos, C.; Alvarez Sabin, J. [Dysphagia, dysphonia and Horner's

syndrome as a form of presentation of right subclavian artery aneurysm (letter)].

Disfagia, distonia y sindrome de Claudio Bernard-Horner como forma de

presentacion de un aneurisma de arteria subclavia derecha aberrante. Rev-Neurol.

1996 Jul; 24(131): 868; ISSN: 0210-0010.

SPAIN.

163. Dibenedetto, L. M.; Lei, Q.; Gilroy, A. M.; Hermey, D. C.; Marks, SC Jr; Page, D.

W. Variations in the inferior pelvic pathway of the lateral femoral cutaneous nerve:

implications for laparoscopic hernia repair. Clin-Anat. 1996; 9(4): 232-6; ISSN:

0897-3806.

UNITED-STATES. Laparoscopic repair of inguinal hernias is gaining acceptance

in the repertoire of the general surgeon. However, nerve entrapment sequelae have

been reported and appear to be higher with the laparoscopic approach. Contributing

factors include pelvic variations in nerve pathways and the use of staples. We

examined the pelvic relations of the lateral femoral cutaneous nerve (LFCN) to the

anterior superior iliac spine (ASIS) and the iliopubic tract (IPT) because of the high

morbidity of entrapment of this nerve, despite its low incidence. The LFCN, ASIS,

and IPT were identified and their relationships measured in 48 male and 24 female

cadavers ranging in age from 61 to 96 yr. The LFCN was located 1.7 (+/- 1.2) cm

medial to the ASIS along the IPT and 1.4 (+/- 0.7) cm posterior (deep) to the IPT at

this point, with no significant sex differences. The intrapelvic pathway of the

LFCN, including its branches, varied widely so that in 18% of these specimens the

LFCN was in either the vertical plane of the ASIS (13%) or in the plane of the IPT

(5%). In 11% this nerve was within 1 cm of the ASIS. These data indicate that

exclusive use of the ASIS as a guide for staple placement may result in entrapment

of this nerve or its branches.

164. Dickhaus, D. M.; Schuller, D. Desmoplastic malignant melanoma presenting as a lung

mass. Chest. 1996 Aug; 110(2): 570-1; ISSN: 0012-3692.

UNITED-STATES. A case of metastatic desmoplastic malignant melanoma is

reported. The patient presented initially with a lung mass and subsequently

developed facial swelling and numbness secondary to tumor involvement of the

maxillary division of the trigeminal nerve. The pleomorphism, histochemistry, and

schwannoid differentiation of these tumors is discussed.

165. Dobyns, W. B. Absence makes the search grow longer [editorial; comment]. Am-J-

Hum-Genet. 1996 Jan; 58(1): 7-16; ISSN: 0002-9297.

Note: Comment on: Am J Hum Genet 1996 Jan;58(1):28-34.

UNITED-STATES.

166. Dorta Contreras, A.; Martinez Torres, E.; Dotres Martinez, C. Local IgG synthesis in

three pediatric patients with Cuban epidemic neuropathy. Arq-Neuropsiquiatr. 1996

Mar; 54(1): 98-101; ISSN: 0004-282X.

BRAZIL. Three pediatric patients with Cuban epidemic neuropathy were studied.

Cerebrospinal fluid and sera were simultaneously obtained. Albumin and IgG were

quantified by immunodifusion. Albumin quotient and local synthesis of IgG were

calculated by Reiber/Felgenhauer formula. A patient with optic neuritis had a

dysfunction of the blood-cerebrospinal fluid barrier. All the group had local

synthesis of IgG.. 0; 0.

167. Drummond, P. D. The site of sympathetic deficit in cluster headache. Headache. 1996

Jan; 36(1): 3-9; ISSN: 0017-8748.

UNITED-STATES. The pattern of autonomic deficit in the face of cluster headache

patients resembles the deficit in patients with a postganglionic sympathetic lesion

from some other cause; however, the presence of abnormal cardiac rhythms and

bilateral pupillary reflex deficit in some patients with cluster headache suggests that

the lesion might compromise central sympathetic drive. To investigate this

possibility, the vasomotor and sudomotor startle reflex was investigated in the

hands of six cluster headache patients with ocular and thermoregulatory signs of

postganglionic sympathetic deficit in the face; for comparison, responses were also

investigated in 15 patients with a lesion in the cervical sympathetic pathway from

some other cause. The startle reflex was intact in the hands of the six cluster

headache patients, but was diminished ipsilaterally in patients with a central or

preganglionic sympathetic lesion and also, surprisingly, in patients with a

postganglionic lesion caused by an aneurysm of the internal carotid artery. Ocular

sympathetic deficit was greater in patients with an aneurysm of the internal carotid

artery than in cluster headache patients or in patients with a postganglionic

sympathetic lesion from some other cause; the aneurysm may have compromised

neurons with projections to the face and hand, or could have induced transsynaptic

degeneration of preganglionic fibers supplying both regions. The findings indicate

that central sympathetic drive is not impaired in cluster headache patients; thus, a

peripheral lesion probably induces sympathetic deficit on the symptomatic side of

the face.

168. Drummond, P. D.; Skipworth, S.; Finch, P. M. alpha 1-adrenoceptors in normal and

hyperalgesic human skin. Clin-Sci-Colch. 1996 Jul; 91(1): 73-7; ISSN: 0143-

5221.

ENGLAND. 1. Evidence of an adrenergic component of cutaneous hyperalgesia

has recently been obtained in animal models of painful peripheral neuropathy.

These findings have prompted speculation that an increased density or sensitivity of

peripheral alpha-adrenoceptors contributes to sensory abnormalities and chronic

neuropathic pain in conditions such as reflex sympathetic dystrophy. However, it is

not known whether alpha-adrenoceptors are present at the site of nociception, either

in hyperalgesic or normal skin. 2. We used the selective radioligand 125I-

hydroxyphenyl-ethyl-aminomethyl-tetralone (HEAT) to label alpha 1-

adrenoceptors, and quantitative autoradiography to assess the relative density of

these receptors in skin samples from seven normal individuals and from the

hyperalgesic and pain-free limbs of five patients with reflex sympathetic dystrophy.

The distribution of autoradiographic grains over the epidermis and dermis was

investigated in 10 microns serial transverse sections. 3. alpha 1-Adrenoceptors

were identified in the epidermis and dermal papillae of normal individuals, and in

the hyperalgesic and pain-free skin of patients with reflex sympathetic dystrophy.

The density of alpha 1-adrenoceptors was greater in the epidermis and dermal

papillae than further down in the dermis. 4. The mean density of alpha 1-

adrenoceptors was significantly greater in the hyperalgesic skin of patients than in

the skin of normal individuals (35.4 grains/1000 microns2 compared with 15.5

grains/ 1000 microns2, P < 0.01). The mean density of alpha 1-adrenoceptors in

the pain-free skin of patients (26.9 grains/1000 microns2) fell midway between

receptor density in hyperalgesic skin and in the skin of normal individuals, and did

not differ significantly from either. 5. Our findings indicate that alpha 1-

adrenoceptors are present in the epidermis, and suggest that their numbers may be

increased in the hyperalgesic skin of patients with reflex sympathetic dystrophy.

Further studies need to identify the dermal and epidermal cell types that express

high densities of alpha 1-adrenoceptors, and to investigate their normal function

and role in neuropathic pain.. 0.

169. Dubs, B.; Niparko, J. K. Diagnostic imaging quiz case 1 and 2. Recurrent acoustic

neuroma. Arch-Otolaryngol-Head-Neck-Surg. 1996 Mar; 122(3): 342-5; ISSN:

0886-4470.

UNITED-STATES. 7440-54-2.

170. Dubuisson, A. S.; Stevenaert, A. Recurrent ganglion cyst of the peroneal nerve:

radiological and operative observations. Case report. J-Neurosurg. 1996 Feb;

84(2): 280-3; ISSN: 0022-3085.

UNITED-STATES. This 34-year-old man presented with right leg pain and foot

drop of 1-month duration. The preoperative diagnosis of a 10-cm-long ganglion

cyst of the peroneal nerve was achieved using ultrasonography (US), computerized

tomography and, particularly magnetic resonance (MR) imaging. Surgical

exploration disclosed a lobulated cystic mass filled with gelatinous material, which

intermingled with the nerve substance of the deep peroneal nerve. The lesion was

completely resected, with the sacrifice of some electrically nonfunctioning fascicles.

No connection with the knee joint was found. A good postoperative recovery of

motor function was obtained. However, routine postoperative MR imaging

disclosed a recurrent ganglion cyst that was slightly less extensive than the original.

A careful radiological examination of the knee joint was performed, including

arthrography. A communication of the cyst with the tibiofibular joint was clearly

demonstrated and was meticulously closed at reoperation. The patient's

postoperative course was uneventful, and a third MR image, obtained 5 months

after reoperation, showed no sign of cyst recurrence. The patient remained free of

symptoms 11 months postoperatively. This case illustrates the value of US and MR

in diagnostic imaging. The diagnostic efficacy of US and MR imaging in

identifying and characterizing a ganglion cyst is described. Close contact between a

ganglion cyst and the tibiofibular joint should raise the possibility of an existing

cyst-joint communication and lead to an aggressive radiological workup and/or a

surgical search for such a communication.

171. Dukefoss, T. T.; Bovim, G.; Johnsen, H. J. [Glossopharyngeal neuralgia. Not just

pain]. Glossopharyngeusneuralgi. Ikke smerter alene. Tidsskr-Nor-Laegeforen.

1996 Apr 30; 116(11): 1325-7; ISSN: 0029-2001.

NORWAY. Glossopharyngeal neuralgia is a rare disease characterized by severe

paroxysmal attacks of pain in the distribution of the 9th cranial nerve. The most

important differential diagnosis is trigeminal neuralgia. Carbamazepin is the current

drug of choice in therapy, but modern neurosurgical treatment will probably

become more common in the future. Autonomic disturbances may occur during

pain attacks in some patients. We describe a patient suffering from

glossopharyngeal neuralgia with transitory unconsciousness due to cardiac asystole

and arterial hypotension accompanying the attack of pain.. 0; 298-46-4.

172. Dumitru, D.; Dreyfuss, P. Dermatomal/segmental somatosensory evoked potential

evaluation of L5/S1 unilateral/unilevel radiculopathies. Muscle-Nerve. 1996 Apr;

19(4): 442-9; ISSN: 0148-639X.

UNITED-STATES. Dermatomal and segmental somatosensory evoked potentials

(SEPs) have been reported to be of diagnostic utility in unilateral/unilevel L5 and

S1 radiculopathies. This investigation employs history, physical examination,

imaging studies, and electrodiagnostic medicine evaluations to clearly define

unilateral/unilevel L5 or S1 nerve root compromise. Inclusion criteria require all of

the preceding diagnostic methods to corroborate a specific nerve root lesion.

Regression equation analysis for cortical P1 latencies evaluating age and height

based on comparable patient and control reference populations reveals segmental

and dermatomal sensitivities for L5 radiculopathies to be 70% and 50%,

respectively, at 90% confidence intervals. Similar sensitivities are obtained for 2

standard deviation mean cortical P1 latencies. Side-to-side cortical P1 latency

difference data reveal segmental and dermatomal sensitivities for S1 radiculopathies

to be 50% and 10%, respectively, at two standard deviations. The clinical utility of

both segmental and dermatomal SEPs are questionable in patients with known

unilateral/unilevel L5 and S1 nerve root compromise.

173. Dursun, G.; Sataloff, R. T.; Spiegel, J. R.; Mandel, S.; Heuer, R. J.; Rosen, D. C.

Superior laryngeal nerve paresis and paralysis. J-Voice. 1996 Jun; 10(2): 206-11;

ISSN: 0892-1997.

UNITED-STATES. Superior laryngeal nerve paresis and paralysis are relatively

common but often difficult to diagnose with certainty. They are most commonly

caused by viral infections, though other etiologies must be considered. A thorough

history and physical examination, including strobovideolaryngoscopy and laryngeal

electromyography, are needed for definitive diagnosis. It is essential to establish the

diagnosis accurately to differentiate an apparent superior laryngeal nerve paresis

from other conditions, such as myasthenia gravis. Laryngeal electromyography is

used to confirm clinical impressions, as a guide for therapy, and as one measure of

recovery. In our experience, accurate and early diagnosis assure the best phonatory

outcome by directing therapy that will prevent or eliminate compensatory vocal

abuses, which may themselves lead to even more serious vocal injury.

174. Dyck, P. J.; Dyck, P. J.; Grant, I. A.; Fealey, R. D. Ten steps in characterizing and

diagnosing patients with peripheral neuropathy. Neurology. 1996 Jul; 47(1): 10-7;

ISSN: 0028-3878.

UNITED-STATES. Cost-effective approaches for arriving at correct diagnoses

should be used. Herein, we compare three approaches for the differential diagnosis

of a common neurologic syndrome, peripheral neuropathy. In the "shotgun"

approach, a standard battery of hematologic, biochemical, serologic, antibody,

enzyme, molecular genetic, and other tests are ordered after the presence of a

neuropathy is established, without a detailed characterization of the neuropathy. In

the "gestalt" approach, the specific variety of neuropathy is identified by

recognition of a clinical pattern of symptoms, course, or disease associations. The

"10-step" approach, encompassing components of the two other methods and

adding others, evolved from our assessment of many patients with peripheral

neuropathy. In the 10-step approach, the history and neurologic examination place

the patient's disorder into one of perhaps 21 anatomic-pathologic patterns. Next,

electrophysiologic and other tests confirm the correctness of this anatomic-

pathologic patterns. Finally, a series of evaluations exclude or include an

increasingly shorter list of diagnoses until only one likely one remains or the

disorder remains undiagnosed. We advocate the 10-step over the shotgun or gestalt

approach, because it emphasizes careful initial characterization of neuropathy and

emphasizes use of a logical step-by-step inclusion or exclusion to arrive at a short

list of diagnostic possibilities. The approach depends ultimately on the judgement

of adequately trained and experienced physicians, not on the results of single tests.

The 10-step approach is not a mindless algorithm, leading inevitably to the correct

diagnosis, but depends on judgment based on extensive background and

neurobiologic and clinical knowledge and training.

175. Ekbom, K. Idiopathic stabbing headache [editorial; comment]. Cephalalgia. 1996 Apr;

16(2): 77; ISSN: 0333-1024.

Note: Comment on: Cephalalgia 1996 Apr;16(2):93-6.

NORWAY.

176. Ekerot, L. A case of digital glomus tumour: misdiagnosis and verification by magnetic

resonance imaging. Case report. Scand-J-Plast-Reconstr-Surg-Hand-Surg. 1996

Jun; 30(2): 151-2; ISSN: 0284-4311.

SWEDEN. A patient with a digital glomus tumour had for years been

misdiagnosed as having ulnar nerve entrapment. The correct clinical diagnosis was

verified by magnetic resonance imaging (MRI) and excisional biopsy.

177. Ekerot, L. A case of digital glomus tumour: misdiagnosis and verification by magnetic

resonance imaging. Case report. Scand-J-Plast-Reconstr-Surg-Hand-Surg. 1996

Jun; 30(2): 151-2; ISSN: 0284-4311.

SWEDEN. A patient with a digital glomus tumour had for years been

misdiagnosed as having ulnar nerve entrapment. The correct clinical diagnosis was

verified by magnetic resonance imaging (MRI) and excisional biopsy.

178. El Salhy, M.; Suhr, O. Endocrine cells in rectal biopsy specimens from patients with

familial amyloidotic polyneuropathy. Scand-J-Gastroenterol. 1996 Jan; 31(1): 68-

73; ISSN: 0036-5521.

NORWAY. BACKGROUND: A decreased amount of duodenal endocrine cells

has recently been found in patients with familial amyloidotic polyneuropathy

(FAP), and it has been suggested that this may contribute to the development of the

gastrointestinal symptoms. The present study was performed to establish whether

the endocrine cells in the lower gastrointestinal tract are also affected. METHODS:

The endocrine cells in rectal biopsy specimens from 13 patients (7 women and 6

men) with FAP were investigated by means of immunocytochemistry and

computed image analysis. These specimens were taken early during the disease. As

controls, rectal specimens from 13 patients (7 women and 6 men) with rectal

bleeding caused by haemorrhoids or polyps were included. RESULTS: The

amount of both serotonin- and pancreatic polypeptide (PP)-immunoreactive cells

was significantly decreased compared with the controls. There was no significant

difference between patients and controls with regard to the amount of chromogranin

A-, polypeptide YY (PYY)-, enteroglucagon- and somatostatin-immunoreactive

cells. CONCLUSION: It has been suggested that serotonin depletion may

contribute to the development of the severe constipation encountered in FAP

patients in early stages of the disease. This constipation may promote bacterial

overgrowth in the small intestine, with diarrhoea and malabsorption as a result.. 0;

50-67-9; 51110-01-1; 59763-91-6; 62340-29-8.

179. Elliott, M. A.; Peroutka, S. J.; Welch, S.; May, E. F. Familial hemiplegic migraine,

nystagmus, and cerebellar atrophy. Ann-Neurol. 1996 Jan; 39(1): 100-6; ISSN:

0364-5134.

UNITED-STATES. Familial hemiplegic migraine (FHM) is an autosomal

dominant disorder characterized by transient hemiplegia during the aura phase of a

migraine attack. Nystagmus has been reported in individuals affected with this

disorder, but the origin of the ocular motility findings is unknown. A three-

generation family with FHM is described and clinical histories are outlined. Ocular

motility evaluations were performed on 7 family members, 5 with a history of

hemiplegic migraine and 2 without history of migraine. All affected family

members had abnormal eye movements consistent with vestibulocerebellar

dysfunction. Magnetic resonance imaging scans in affected family members

revealed cerebellar vermian atrophy. DNA linkage analysis revealed a common

marker in all the affected family members on chromosome 19. We suggest that the

hemiplegic migraine attacks and the cerebellar degeneration are linked genetically

and that the eye movements are not the ischemic sequelae of recurrent migraine.

Strikingly similar ocular motility findings and cerebellar degeneration are reported

in both FHM and a genetically related disorder, hereditary paroxysmal cerebellar

ataxia (HPCA). The significance of these similarities is discussed along with a

proposed pathophysiology for FHM.. 9007-49-2.

180. Enderle, M. D.; Haring, H. U.; Luft, D. [Diabetic foot syndrome. Etiology and

differential therapy]. Das diabetische Fusssyndrom. Atiologie und

Differentialtherapie. Dtsch-Med-Wochenschr. 1996 Oct 4; 121(40): 1236-42;

ISSN: 0012-0472.

GERMANY. 0.

181. Endres, M.; Heide, W.; Kompf, D. [See-saw nystagmus. Clinical aspects, diagnosis,

pathophysiology: observations in 2 patients]. See-saw-Nystagmus. Klinik,

Diagnose, Pathophysiologie: Beobachtung an 2 Patienten. Nervenarzt. 1996 Jun;

67(6): 484-9; ISSN: 0028-2804.

GERMANY. See-saw nystagmus is an uncommon but characteristic kind of

nystagmus. Typically there is intorsion and elevation of one eye and simultaneous

extorsion and depression of the other eye. The nystagmus can be of pendular-

waveform or jerk-waveform. The pendular-waveform see-saw nystagmus is

commonly due to a midline meso-diencephalic, bilaterally compressing mass. The

jerk-waveform see-saw nystagmus is mostly due to a unilateral lesion in the meso-

diencephalic junction. For explanation, a current theory assumes a unilateral lesion

of the interstitial nucleus of Cajal sparing the rostral interstitial nucleus of the medial

longitudinal fascicle. Another concept suggests a lesion of the vertical vestibulo-

ocular-reflex. We report two patients with jerk-waveform see-saw nystagmus. In

both patients an internuclear ophthalmoplegia was found additionally. The origin

was a unilateral brainstem infarction in both cases. We explain the symptomatology

of see-saw nystagmus and discuss the actual theories of its origin.

182. Ezra, E.; Spalton, D.; Sanders, M. D.; Graham, E. M.; Plant, G. T. Ocular

neuromyotonia. Br-J-Ophthalmol. 1996 Apr; 80(4): 350-5; ISSN: 0007-1161.

ENGLAND. AIMS/BACKGROUND: Ocular neuromyotonia is characterised by

spontaneous spasm of extraocular muscles and has been described in only 14

patients. Three further cases, two with unique features, are described, and the

underlying mechanism reviewed in the light of recent experimental evidence

implicating extracellular potassium concentration in causing spontaneous firing in

normal and demyelinated axons. METHODS: Two patients had third nerve

neuromyotonia, one due to compression by an internal carotid artery aneurysm,

which has not been reported previously, while the other followed irradiation of a

pituitary tumour, a common association in the published reports. Selective

activation occurred in both, where neuromyotonic activity was triggered by

prolonged voluntary activation of specific extraocular muscles with or without

spread of activity to other third nerve muscles. The other patient had fourth nerve

involvement, where spasms of the superior oblique muscle were induced only by

alcohol, a phenomenon which has not been described. RESULTS: The two patients

with third nerve involvement responded to carbamazepine and in one, an

improvement in a chronic partial third nerve paresis occurred. The other has not

required treatment and remains asymptomatic by refraining from alcohol.

CONCLUSIONS: A careful examination, including the effects of prolonged

voluntary muscle action is required to initiate episodes and to demonstrate selective

activation. Imaging is mandatory to exclude compressive intracranial lesions,

particularly where there is no history of pituitary fossa irradiation. A trial of

anticonvulsants should be considered in all patients. Extracellular potassium may

play a role in spontaneous firing and ephatic transmission in ocular

neuromyotonia.. 0; 298-46-4; 64-17-5.

183. Faber, C. E.; Pedersen, A. T. [Pain and sensory impairment following parotidectomy.

A descriptive study]. Smerter og sensibilitetsforstyrrelser efter parotidektomi. En

deskriptiv undersogelse. Ugeskr-Laeger. 1996 Jan 15; 158(3): 270-3; ISSN: 0041-

5782.

DENMARK. From 1985 to 1992 parotidectomy was performed at Vejle Hospital

in 114 patients with a parotid tumour. Ninety-five of the patients completed a

questionnaire. Twenty-four reported various degrees of pain at the time of the

investigation, and 57 reported numbness or uncomfortable sensations of the skin.

No association between symptoms and age, gender or follow-up time could be

traced. Twenty patients with clinically significant complaints were subsequently

interviewed and examined. Five patients had a neuroma in the scar region.

Hypoaesthesia was found in 18/20 of the patients. The results emphasize the

importance of avoiding unnecessary division of the great auricular nerve in order to

reduce the risk of pain and sensory impairment postoperatively. The authors

suggest that the posterior branch of the great auricular nerve be spared whenever

possible. Patients should be informed about the risk preoperatively.

184. Fahn, S.; Brin, M. F.; Dwork, A. J.; Weiner, W. J.; Goetz, C. G.; Rajput, A. H.

Case 1, 1996: rapidly progressive parkinsonism, incontinence, impotency, and

levodopa-induced moaning in a patient with multiple myeloma [clinical conference].

Mov-Disord. 1996 May; 11(3): 298-310; ISSN: 0885-3185.

UNITED-STATES. 0; 0.

185. Fantini, G. A. Reserving supraclavicular first rib resection for vascular complications

of thoracic outlet syndrome. Am-J-Surg. 1996 Aug; 172(2): 200-4; ISSN: 0002-

9610.

UNITED-STATES. BACKGROUND: The traditional approach to decompression

of the thoracic outlet has been by transaxillary resection of the first rib. Recently,

the trend has been toward a more selective and tailored surgical approach via the

supraclavicular route. METHODS: During a 51-month period, 14 consecutive

patients underwent decompressive surgery of the thoracic outlet via the

supraclavicular approach. There were ten women and four men; mean age was 44

years. Indications for operation were arterial (n = 3), venous (n = 2) and

neurogenic (n = 9). Mean follow-up was 31 months. Operation consisted of

resection of the anterior scalene and medial aspect of the middle scalene muscles

and brachial plexus neurolysis for neurogenic indication, with first rib resection

reserved for vascular complications. RESULTS: Operations performed for vascular

complication were successful and uncomplicated, with good clinical outcome.

Seven of nine operations (78%) performed for neurogenic indication produced

marked relief of symptoms, while two (22%) resulted in no clinical change.

Complications consisted of transient scapular winging (n = 1) and transient

diaphragmatic paralysis (n = 2). CONCLUSIONS: A selective approach to thoracic

outlet decompression, consisting of anterior scalenectomy and brachial plexus

neurolysis for neurogenic symptoms, and reserving first rib resection for arterial

and venous indications, is a safe procedure and yields satisfactory results in

appropriately selected patients.

186. Fasanaro, A. M.; Pizza, V.; Rossi, V. Immunoglobulins i.v.: a new approach to the

treatment of Guillain-Barre syndrome. Minerva-Med. 1996 Jan; 87(1-2): 17-20;

ISSN: 0026-4806.

ITALY. We report the results obtained with intravenous immunoglobulin (IVGG)

in 15 patients suffering from Guillain Barre syndrome (GBS) with substantial

motor damage. All of them met the NINCDS criteria for a diagnosis of certain GBS

and were graded using a specific scale on entry, after 2 weeks, one month, two

months, six months and one year. The clinical data were correlated to

neurophysiological results. The patients were treated within the seventh day of the

disease with IVGG (0.4 g/kg/day) for 5 days. Complete recovery was obtained in

all but 2 patients died. We measured the mean time taken to improve one grade of

the evaluation scale and the mean time taken to achieve walking unassisted. We

obtained 11 days and 14 days respectively, that is, times significantly shorter than

those reported in studies employing plasma exchange. No adverse effect was found

except in one case. No relapse was observed. Even the patients with more clinical

and neurophysiological damage had a rapid recovery. We advocate IVGG therapy

for GBS, as it is effective, safe and easy to use.. 0.

187. Fathers, E.; Fuller, G. N. Vasculitic neuropathy. Br-J-Hosp-Med. 1996 May 15;

55(10): 643-7; ISSN: 0007-1064.

ENGLAND. Vasculitic neuropathy usually receives only a footnote in neurology

textbooks. The condition is rare, but as it is potentially treatable it merits wider

discussion. This article discusses the pathological features, clinical features and

associations of vasculitic neuropathy. It recommends a scheme for investigating

patients and summarizes treatment and prognosis.

188. Feasby, T. E. Axonal CIDP: a premature concept? Muscle-Nerve. 1996 Mar; 19(3):

372-4; ISSN: 0148-639X.

UNITED-STATES.

189. Felsby, S.; Nielsen, J.; Arendt Nielsen, L.; Jensen, T. S. NMDA receptor blockade in

chronic neuropathic pain: a comparison of ketamine and magnesium chloride. Pain.

1996 Feb; 64(2): 283-91; ISSN: 0304-3959.

NETHERLANDS. Ten patients (4 female, 6 male) aged 34-67 years suffering

from peripheral neuropathic pain participated in a double-blind placebo-controlled

study where ketamine or magnesium chloride were administered by a 10 min bolus

infusion (ketamine: 0.84 mumol/kg = 0.2 mg/kg, magnesium: 0.16 mmol/kg)

followed by a continuous infusion (ketamine: 1.3 mumol/kg/h = 0.3 mg/kg/h,

magnesium: 0.16 mmol/kg/h). Ongoing pain determined by VAS score, area of

touch-evoked allodynia, detection and pain thresholds to mechanical and thermal

stimuli were measured before and during drug infusion. Ketamine produced a

significant reduction of spontaneous pain (57%) and of the area of allodynia (33%).

Magnesium chloride reduced pain (29%) and area of allodynia (18%)

insignificantly. Following ketamine there was a significant correlation between the

reduction in ongoing pain and reduction in area of touch-evoked allodynia.

Detection and pain thresholds to mechanical and thermal stimuli were not

significantly changed by the drugs. These findings suggest that both ongoing pain

and touch-evoked pain (allodynia) in neuropathic pain are inter-related phenomena,

which may be mediated by the same mechanism and involving a N-methyl-D-

aspartate receptor.. 0; 0; 6740-88-1; 7786-30-3.

190. Fenton, C. J.; Coddington, R.; Ramsay, A. D.; Garth, R. J. Primary neuroblastoma of

the facial nerve presenting as a recurrent facial paralysis. J-Laryngol-Otol. 1996

Feb; 110(2): 151-3; ISSN: 0022-2151.

ENGLAND. A case is described of a primary neuroblastoma of the facial nerve in

a 13-year-old boy presenting with a recurrent facial paralysis. This tumour was

excised preserving the nerve and followed with post-operative radiotherapy. The

pathology of the tumour is described and facial nerve tumours briefly discussed as

a cause of facial palsy. There have been no other cases of a primary neuroblastoma

of the facial nerve arising at this site reported in the last 20 years.. 0.

191. Fetter, M.; Dichgans, J. Vestibular neuritis spares the inferior division of the vestibular

nerve. Brain. 1996 Jun; 119( Pt 3): 755-63; ISSN: 0006-8950.

ENGLAND. Acute unilateral vestibulopathy, or vestibular neuritis, is the second

most common cause of vertigo. To quantify the involvement of the different

semicircular canal (SCC) afferents in this disease, we studied the three-dimensional

(3D) properties of the vestibuloocular reflex (VOR) in 16 patients 3-10 days after

onset of symptoms. Using 3D magnetic search coil eye movement recordings, we

measured the speed and axis of eye rotation during spontaneous nystagmus and

during rotation in the planes of the different SCCs. In all patients, spontaneous

nystagmus axes clustered between the direction expected with involvement of just

one horizontal SCC and the direction expected with combined involvement of the

horizontal and anterior SCC on one side. Likewise, dynamic asymmetries were

found only during rotations about axes which stimulated the ipsilesional horizontal

or ipsilesional anterior SCCs. No asymmetry was found when the ipsilesional

posterior SCC was stimulated. Thus, both measurements suggest that vestibular

neuritis is a partial and not a complete unilateral vestibular lesion and that this partial

lesion affects the superior division of the vestibular nerve which includes the

afferents from the horizontal and anterior SCCs.

192. Fibuch, E. E.; Mertz, J.; Geller, B. Postoperative onset of idiopathic brachial neuritis.

Anesthesiology. 1996 Feb; 84(2): 455-8; ISSN: 0003-3022.

UNITED-STATES. 0; 0.

193. Fidzianska, A. Spinal muscular atrophy in childhood. Semin-Pediatr-Neurol. 1996

Jun; 3(2): 53-8; ISSN: 1071-9091.

UNITED-STATES. Diagnosis and classification of spinal muscular atrophy

(SMA) in childhood are based on clinical, electrophysiological, and histological

studies. The concept of maturational arrest of motoneurons and their targets

(muscle cells in SMA type I) is documented by ultrastructural and

immunohistochemical data. The prolongated or markedly delayed process of

muscle cell and motoneuron elimination by apoptosis seen in SMA type I is

discussed according to the new finding of a gene for a neuronal apoptosis

inhibitory protein that is partially deleted in children with spinal muscular atrophy..

0.

194. Fink, J. K.; Heiman Patterson, T.; Bird, T.; Cambi, F.; Dube, M. P.; Figlewicz, D.

A.; Fink, J. K.; Haines, J. L.; Heiman Patterson, T.; Hentati, A.; Pericak Vance,

M. A.; Raskind, W.; Rouleau, G. A.; Siddique, T. Hereditary spastic paraplegia:

advances in genetic research. Hereditary Spastic Paraplegia Working group.

Neurology. 1996 Jun; 46(6): 1507-14; ISSN: 0028-3878.

UNITED-STATES. Hereditary spastic paraplegia (HSP) is a diverse group of

inherited disorders characterized by progressive lower-extremity spasticity and

weakness. Insight into the genetic basis of these disorders is expanding rapidly.

Uncomplicated autosomal dominant, autosomal recessive, and X-linked HSP are

genetically heterogeneous: different genes cause clinically indistinguishable

disorders. A locus for autosomal recessive HSP is on chromosome 8q. Loci for

autosomal dominant HSP have been identified on chromosomes 2p, 14q, and 15q.

One locus (Xq22) has been identified for X-linked, uncomplicated HSP and shown

to be due to a proteolipoprotein gene mutation in one family. The existence of HSP

families for whom these loci are excluded indicates the existence of additional, as

yet unidentified HSP loci. There is marked clinical similarity among HSP families

linked to each of these loci, suggesting that gene products from HSP loci may

participate in a common biochemical cascade, which, if disturbed, results in axonal

degeneration that is maximal at the ends of the longest CNS axons. Identifying the

single gene defects that cause HSPs distal axonopathy may provide insight into

factors responsible for development and maintenance of axonal integrity. We

review clinical, genetic, and pathologic features of HSP and present differential

diagnosis and diagnostic criteria of this important group of disorders. We discuss

polymorphic microsatellite markers useful for genetic linkage analysis and genetic

counseling in HSP.

195. FitzGibbon, E. J.; Calvert, P. C.; Dieterich, M.; Brandt, T.; Zee, D. S. Torsional

nystagmus during vertical pursuit. J-Neuroophthalmol. 1996 Jun; 16(2): 79-90;

ISSN: 1070-8022.

UNITED-STATES. We examined three patients with cavernous angioma within

the middle cerebellar peduncle. Each patient had an unusual ocular motor finding:

the appearance of a strong torsional nystagmus during vertical pursuit. The

uncalled-for torsion changed direction when vertical pursuit changed direction. In

one patient, we recorded eye movements with the magnetic field technique using a

combined direction and torsion eye coil. The slow-phase velocity of the

inappropriate torsional nystagmus was linearly related to the slow-phase velocity of

vertical smooth pursuit, and changed direction when vertical pursuit changed

direction. This torsional nystagmus also appeared during fixation suppression of

the vertical vestibulo-ocular reflex (VOR), but was minimal during vertical head

rotation when fixing a stationary target in the light. We suggest that inappropriately

directed eye movements during pursuit might be another ocular motor sign of

cerebellar dysfunction. Furthermore, we speculate that the signals used for vertical

smooth pursuit are, at some stage, encoded in a semicircular canal VOR coordinate

framework. To illustrate, for the vertical semicircular canals, vertical and torsional

motion are combined on the same cells, with the anterior semicircular canals

mediating upward movements and the posterior semicircular canals mediating

downward movements. For the right labyrinth, however, both vertical semicircular

canals produce clockwise slow phases (ipsilateral eye intorts, contralateral eye

extorts). The opposite is true for the vertical semicircular canals in the left labyrinth;

counterclockwise slow phases are produced. Hence, to generate a pure vertical

VOR, the anterior or posterior semicircular canals on both sides of the head must be

excited so that opposite-directed torsional components cancel. Thus, if pursuit were

organized in a way similar to the VOR, pure vertical pursuit would require that

oppositely-directed torsional components cancel in normals. If this did not happen,

a residual torsional nystagmus could appear during attempted vertical pursuit.

196. Flachenecker, P.; Hartung, H. P. [Course of illness and prognosis of multiple

sclerosis. 2: Predictive value of clinical and paraclinical factors]. Krankheitsverlauf

und Prognose der multiplen Sklerose. 2. Teil: Pradiktiver Wert klinischer und

paraklinischer Faktoren. Nervenarzt. 1996 Jun; 67(6): 444-51; ISSN: 0028-2804.

GERMANY. The second part of this review summarizes the predictive value of

demographic factors, the early clinical course and paraclinical methods in the

prognosis of multiple sclerosis (MS). The chronic progressive course is generally

thought to be associated with a worse outcome compared to relapsing-remitting

MS. Moderate disability within 5 years, residual pyramidal and cerebellar deficits 6

months following an acute attack, motor, cerebellar and possibly brain stem

exacerbations as well as frequent relapses were found to indicate an increased risk

for developing severe disability or increased mortality. Magnetic resonance imaging

(MRI), evoked potentials and cerebrospinal fluid findings were not found to be

predictive in clinically definite MS, although there was a weak association of MRI

findings and disability. However, these paraclinical modalities were important

methods to predict the further development of clinically isolated demyelinating

syndromes. In this regard, MRI was identified as the strongest predictive factor of

the conversion to definite MS.

197. Flammer, F.; Messerli, J.; Haefliger, I. [Vision disorders to vascular dysregulation].

Sehstorungen durch vaskulare Dysregulationen. Ther-Umsch. 1996 Jan; 53(1): 37-

42; ISSN: 0040-5930.

SWITZERLAND. As in the case in other organs, there are infarctions in the eye

due to arterial sclerosis. Here, the classic risk factors apply. In addition to such

infarctions, there are reversible perfusion disturbances caused by vascular

dysregulation. These very frequently lead to slight, transient functional failures

which are scarcely noticed by the patient. In more seldom cases, such dysregulation

can contribute to the pathogenesis of various disease entities. Of these, the ocular

vasospastic syndrome, migraine, glaucoma, apoplexy of the optic nerve, and vein

thrombosis were examined.

198. Flanigan, K.; Gardner, K.; Alderson, K.; Galster, B.; Otterud, B.; Leppert, M. F.;

Kaplan, C.; Ptacek, L. J. Autosomal dominant spinocerebellar ataxia with sensory

axonal neuropathy (SCA4): clinical description and genetic localization to

chromosome 16q22.1. Am-J-Hum-Genet. 1996 Aug; 59(2): 392-9; ISSN: 0002-

9297.

UNITED-STATES. The hereditary ataxias represent a clinically and genetically

heterogeneous group of neurodegenerative disorders. Various classification

schemes based on clinical criteria are being replaced as molecular characterization of

the ataxias proceeds; so far, seven distinct autosomal dominant hereditary ataxias

have been genetically mapped in the human genome. We report linkage to

chromosome 16q22.1 for one of these genes (SCA4) in a five-generation family

with an autosomal dominant, late-onset spinocerebellar ataxia; the gene is tightly

linked to the microsatellite marker D16S397 (LOD score = 5.93 at theta = .00). In

addition, we present clinical and electrophysiological data regarding the distinct and

previously unreported phenotype consisting of ataxia with the invariant presence of

a prominent axonal sensory neuropathy.. 0.

199. Flueler, U. R.; Elhatton, K. M.; Guyton, D. L. [A combination of horizontal/vertical

prism bar]. Eine Kombination einer horizontalen/vertikalen Prismenleiste. Klin-

Monatsbl-Augenheilkd. 1996 May; 208(5): 343-7; ISSN: 0023-2165.

GERMANY. BACKGROUND: Calibration and positioning of prism bars is not

well standardized. When both the horizontal and vertical prism bars are used, they

are frequently held back-to-back in front of one eye; the prism bar then reversed

from its usual position will give an erroneous measurement. METHODS: We used

an optical bench to investigate calibration and errors from improper positioning of

prism bars manufactured by the companies R. O. Gulden and Luneau. We also

urged Gulden to redesign its prism bars so that they can be held back-to-back and

slid vertically with respect to one another, held in alignment by grooves along the

edges of the flat surfaces of the bars. The combination horizontal/vertical prism bar

thus created demands that the horizontal prism bar be calibrated precisely in the

frontal plane position, and the new vertical prism bar be calibrated in the Prentice

position. RESULTS: Gulden's new combination horizontal/vertical prism bar can

be used without significant error (measured deviations within +/- 0.5 delta of

labelled values). Gulden's old vertical prism bar has always been calibrated in the

Prentice position (a surprise to the company). Luneau's horizontal and vertical

prism bars are both calibrated close to the frontal plane position (within +/- 0.4

delta). Improper positioning demonstrated an increasing error with larger prisms.

Luneau's 25 delta segment measured in the Prentice position 27.8 delta and the 40

delta segment 67.5 delta; the 25 delta segment of Gulden's old and new vertical

prism bars measured 23.2 delta in the frontal plane position. CONCLUSIONS:

Gulden's vertical prism bars, calibrated in the Prentice position, whether used in

combination with the horizontal prism bar or by themselves, should always be held

with the flat surface toward the examiner. Luneau's horizontal and vertical prism

bars should be held one in front of each eye when used simultaneously. As a

consequence, neither eye is looking directly at the fixation object, and strictly

defining primary and secondary deviations is not possible.

200. Foresman, B. H.; Friess, G.; Brown, A.; McIntosh, W. Acute diplopia and a solitary

lung mass: a unique presentation of light-chain myeloma. J-Am-Osteopath-Assoc.

1996 Jun; 96(6): 371-2; ISSN: 0003-0287.

UNITED-STATES. The patient described, a 51-year-old woman, had diplopia and

cephalgia of two weeks' duration. On admission, the radiologic evaluation revealed

a mass in the sphenoid sinus, multiple lesions in the calvarium and a solitary lung

mass. Biopsy of the lung mass revealed an atypical plasmacytic infiltration.

Laboratory findings confirmed the diagnosis of light-chain myeloma presenting

with a pulmonary plasmacytoma and cranial nerve involvement.

201. Fournier, L.; Musard, D.; Lecorsier, A. Lymphocyte esterases and hydroxylases in

neurotoxicology. Vet-Hum-Toxicol. 1996 Jun; 38(3): 190-5; ISSN: 0145-6296.

UNITED-STATES. Certain human lymphocytic enzymes, such as neuropathy

target esterase (NTE), have become useful markers in clinical toxicology. NTE has

been proposed as a predictive marker in organophosphate poisoning for the

subsequent development of organophosphate-induced delayed neuropathy. We

studied lymphocyte aryl hydrocarbon hydroxylases, and using a differential method

based on that of NTE employing a phenyl-alkanoic substrate, developed an enzyme

assay for use in toxic neuropathies. The assayed enzyme, which we refer to as

neuropathy target hydroxylase (NTH), performed similarly to NTE in the

evaluation of coherent clinical data obtained in patients with toxic neuropathies.

This study indicates good correlation between the severity of clinical illness and

abnormally low levels of NTH in neuropathies of varied etiology. A simplified

protocol for NTH assay is presented.. EC 1.14.14.-; EC 3.1.1; EC 3.1.1.-; 0; 0;

0; 311-45-5.

202. Franek, E. [Erectile dysfunction in patients with diabetes]. Zaburzenia erekcji u

chorych na cukrzyce. Przegl-Lek. 1996; 53(2): 88-93; ISSN: 0033-2240.

POLAND. Erectile dysfunction is present in approximately 50% of diabetic men. It

is most often caused by diabetic neuropathy and angiopathy but psychogenic

factors are also of importance. Treatment includes: elimination of risk factors,

psychotherapy, pharmacologic treatment and implantation of penile prosthesis.

203. Frank, P. W.; Bakkum, B. W.; Darby, S. A. The communicating branch of the lateral

plantar nerve: a descriptive anatomic study. Clin-Anat. 1996; 9(4): 237-43; ISSN:

0897-3806.

UNITED-STATES. Since the communicating branch of the lateral plantar nerve

has been implicated as a factor in the etiology of Morton's neuroma, a painful

perineurofibrosis of a common plantar digital nerve, this project was designed to

investigate the anatomy of this communicating branch. Both feet of 40 embalmed

human cadavers were dissected to show the frequency of occurrence and

anatomical variation of the communicating branch. The communicating branch was

present in 66.2% of the feet we studied with no large gender-based differences.

Branches occurred bilaterally in 52.5% of cadavers, while 27.5% had branches

unilaterally. The occurrence of this branch does not correlate well with the

likelihood of development of Morton's neuroma. Differences in diameter of the

communicating branch ranged from less than 0.5 mm to as large as the common

plantar digital nerves themselves, about 2 mm. The presence or absence of the

communicating branch made no qualitative difference in the diameters of the

common plantar digital nerves. There were 60.4% of the communicating branches

in this study that had a typically-described orientation, arising more proximally in

the foot from the fourth common plantar digital nerve, while 39.6% of the branches

had a reversed orientation, arising more proximally from the third common plantar

digital nerve. These reversed branches had a more oblique orientation when

compared to the classic branches. Other anatomical variations were noted, including

accessory branches that attached to deeper structures in the foot. These data form a

basis for further research into the etiology of Morton's neuroma and improved

surgical techniques for correcting this condition.

204. Franzen, A.; Koegel, K. [Neurinoma of the neck area]. Neurinome im Halsbereich.

Laryngorhinootologie. 1996 Apr; 75(4): 250-3; ISSN: 0935-8943.

GERMANY. BACKGROUND: Neurinomas of the cranial nerves and the

sympathetic trunk are rare benign tumors, and as such are rarely considered in

differential diagnostics. Neither clinical criteria nor diagnostic imaging studies are

usually conclusive. PATIENTS: We present a case report in which the problems of

diagnosis, differential diagnosis, and surgical therapy are discussed. RESULTS:

Preoperative diagnosis include clinical examination, B-mode sonography, CT, and

MRI. A biopsy does not usually help verify the diagnosis. A Glomus tumor has to

be excluded preoperatively. The therapy of choice is complete surgical removal of

the tumor, with a good postoperative cure rate. The nerve of origin can be

preserved in many cases. However, there are some cases in which temporary or

permanent damage of the nerve cannot be avoided. Especially where parts of the

vagus nerve require resection, autologous nerve grafts are indicated.

CONCLUSIONS: A neurinoma should be considered in cases of an uncertain neck

tumor. Problems for the surgeon may result from failure to inform patients about

possible nerve lesions before therapy.

205. Frederiksen, J. L.; Petrera, J.; Larsson, H. B.; Stigsby, B.; Olesen, J. Serial MRI,

VEP, SEP and biotesiometry in acute optic neuritis: value of baseline results to

predict the development of new lesions at one year follow up. Acta-Neurol-Scand.

1996 Apr; 93(4): 246-52; ISSN: 0001-6314.

DENMARK. INTRODUCTION--In an attempt to establish the value of MRI,

VEP, SEP, and biotesiometry in monitoring disease evolution we undertook a one

year follow up study of 70 untreated patients with acute optic neuritis (ON).

MATERIAL & METHODS--ON was monosymptomatic in 48 patients (bilateral in

10) and part of clinically definite multiple sclerosis (CDMS) in 22 patients,

examined as mentioned below. RESULTS--Results are given at onset and at follow

up (in brackets). In monosymptomatic ON, brain MRI was abnormal in 53%

(53%), VEP in the eye with acute ON in 79% (71%), VEP in the clinically

unaffected eye in 34% (47%), SEP in 25% (23%), and biotesiometry in 29%

(17%). In CDMS, brain MRI was abnormal in 95% (95%), VEP in the eye with

acute ON in 86% (77%), VEP in the clinically unaffected eye in 50% (64%), SEP

in 55% (50%), and biotesiometry in 63% (53%). Only minor changes in test scores

were observed after one year except for significant improvement of VEP in eyes

with acute ON. Eight of 32 patients, characterized by at least one abnormal

paraclinical test at onset of monosymptomatic ON, had developed CDMS versus

none of 16 patients with normal paraclinical results (p = 0.03; Fisher).

CONCLUSION--Patients with monosymptomatic ON with paraclinical signs of

multifocal involvement at onset had an increased risk of developing CDMS. No

single test predicted the evolution of CDMS, perhaps due to the relatively short

follow up time.

206. Freeman, A. W.; Nguyen, V. A.; Jolly, N. Components of visual acuity loss in

strabismus. Vision-Res. 1996 Mar; 36(5): 765-74; ISSN: 0042-6989.

ENGLAND. Strabismus, the misalignment of the visual axis of one eye relative to

that of the other eye, reduces visual acuity in the affected eye. Several processes

contributing to that loss are: amblyopia, which results in a chronic acuity loss

whether or not the fellow eye is viewing; strabismic deviation, which shifts the

image of an acuity target onto more peripheral, and therefore less acute, retina when

the fellow eye fixates; interocular suppression and binocular masking, which

reduce visibility in the strabismic eye due to neural influences from the other eye.

We measured the losses due to these processes in nine small-angle strabismic

subjects. Amblyopia reduced acuity by a median of 34% relative to its value in

subjects with normal binocular vision, and strabismic deviation produced a loss of

44%. Suppression and masking together reduced acuity by 20%, and therefore had

substantially less effect than the other factors.

207. Freije, J. E.; Harvey, S. A.; Haberkamp, T. J. False-negative magnetic resonance

imaging in the evaluation of facial nerve paralysis. Laryngoscope. 1996 Feb; 106(2

Pt 1): 239-42; ISSN: 0023-852X.

UNITED-STATES.

208. Freund, K. B.; Yannuzzi, L. A.; Barile, G. R.; Spaide, R. F.; Milewski, S. A.;

Guyer, D. R. The expanding clinical spectrum of unilateral acute idiopathic

maculopathy. Arch-Ophthalmol. 1996 May; 114(5): 555-9; ISSN: 0003-9950.

UNITED-STATES. OBJECTIVE: To report on new features of unilateral acute

idiopathic maculopathy (UAIM). PATIENTS: We have evaluated an additional 17

patients with UAIM since 1991. This is a report of new features of the maculopathy

noted in seven patients from this new series. RESULTS: New clinical findings in

UAIM included eccentric macular lesions, subretinal exudation, papillitis, and

bilaterality. The occurrence of UAIM in association with pregnancy and human

immunodeficiency virus was also observed. CONCLUSIONS: The description of

these newly reported features broadens our understanding of the nature of UAIM.

With recognition of the expanded clinical spectrum of this disorder, a more

confident approach to diagnosis and management may be achieved.. 3599-32-4.

209. Fricker, R.; Fuhr, P.; Pippert, H.; Troeger, H. Acute median nerve compression at the

distal forearm caused by a thrombosed aneurysm of an epineural vessel: case

report. Neurosurgery. 1996 Jan; 38(1): 194-6; ISSN: 0148-396X.

UNITED-STATES. The case of a patient with a 2-day history of symptoms

suggesting acute carpal tunnel syndrome is presented. However, an urgent

electroneurographic examination revealed median nerve compression at the forearm

and magnetic resonance imaging confirmed compression by a mass proximal to the

carpal tunnel. Surgical exploration showed a recently thrombosed aneurysm of an

epineural vessel. Histological and, later, general and angiological investigations

could not reveal the underlying cause of this aneurysm. Preoperative

electrodiagnostic examination is recommended in acute peripheral nerve

compression to prevent decompression at an incorrect site. If atypical nerve

compression is suspected, magnetic resonance imaging may be indicated to detect

localized nerve compression and its underlying cause.

210. Fritzell, B. [Work-related voice problems. Teachers, social workers, lawyers and

priests should receive preventive voice training]. Rostproblem foljer yrket. Larare,

socialarbetare, jurister och praster bor tidigt fa skadeforebyggande rostvard.

Lakartidningen. 1996 Apr 3; 93(14): 1325-8; ISSN: 0023-7205.

SWEDEN.

211. Frohman, E. M.; Tusa, R.; Mark, A. S.; Cornblath, D. R. Vestibular dysfunction in

chronic inflammatory demyelinating polyneuropathy. Ann-Neurol. 1996 Apr;

39(4): 529-35; ISSN: 0364-5134.

UNITED-STATES. Chronic inflammatory demyelinating polyneuropathy (CIDP)

has occasionally been associated with clinical or laboratory evidence (magnetic

resonance imaging,[MRI], visual evoked response, and brainstem auditory evoked

response [BAER] of cranial neuropathy. In most cases, the relationship of cranial

nerve involvement to CIDP remains unclear. A 45-year-old woman noted foot

numbness, limb weakness, gait and postural instability, and oscillopsia. An IgG

kappa monoclonal gammopathy of undetermined significance was found. Bilateral

vestibulopathy was documented by clinical examination, bithermal calorics, rotary

chair testing, BAERs, and dynamic posturography. MRI with gadolinium

demonstrated enhancement of cranial nerve VIII bilaterally. Over the next 6 years,

the patients's relapsing and remitting course of CIDP and vestibulopathy was

assessed by quantitative muscle and vestibular function testing (clinically and

neurophysiologically), and dynamic visual acuity. There was a striking

synchronization between her CIDP and vestibulopathy with respect to clinical

course including relapses and responses to immune therapy. The response to

therapy, and evidence derived from clinical and laboratory investigations, suggest

that the vestibular dysfunction was immune mediated.

212. Frontoni, M.; Fiorini, M.; Strano, S.; Cerutti, S.; Giubilei, F.; Urani, C.; Bastianello,

S.; Pozzilli, C. Power spectrum analysis contribution to the detection of

cardiovascular dysautonomia in multiple sclerosis. Acta-Neurol-Scand. 1996 Apr;

93(4): 241-5; ISSN: 0001-6314.

DENMARK. In multiple sclerosis (MS) autonomic cardiovascular dysfunction is

an uncommon, but potentially dangerous event, to which studies of spectral

analysis of heart rate variability have not been applied, yet. MATERIAL AND

METHODS--We studied 16 patients with definite MS (11 women and 5 men, mean

age 30.3 +/- 7.4 yrs., mean EDSS 2.06 +/- 1.42) and 16 sex- and age-matched

healthy controls. Besides cardiovascular reflex tests (valsalva manoeuvre, deep

breathing, lying to standing, Blood Pressure response to standing and sustained

handgrip), each underwent spectral analysis of the R-R interval short-term

variability at rest and after tilting, to detect three components: very low frequency

(VLF), low frequency (LF) and high frequency (HF). A recent brain MRI was

obtained from patients, to compare plaque characteristics with spectral parameters.

RESULTS--At cardiovascular reflexes, only four patients (25%) showed an

impairment, mostly of a mild degree. VLF and LF at rest were lower in MS

subjects than in controls (p < 0.01). No significant correlation was found between

spectral parameters and lesion area or localization as detected on MRI.

CONCLUSIONS--Spectral analysis could usefully flank reflex tests to detect

autonomic subclinical cardiovascular abnormalities.

213. Fukutake, T.; Kita, K.; Sakakibara, R.; Takagi, K.; Tokumaru, Y.; Kojima, S.;

Hattori, T.; Hirayama, K. Late-onset hereditary ataxia with global thermoanalgesia

and absence of fungiform papillae on the tongue in a Japanese family. Brain. 1996

Jun; 119( Pt 3): 1011-21; ISSN: 0006-8950.

ENGLAND. Two Japanese male siblings, aged 68 and 59 years, affected by late-

onset progressive ataxia distinguished by extensive sensory and mild autonomic

disturbances are described. They had global thermoanalgesia, positive Romberg

signs, sensorineural deafness, canal paresis and ageusia. Their autonomic

disturbances consisted of absence of overflow tears with usual stimuli, dysphagia,

blood pressure and vasomotor instability, diarrhoea/constipation, and urinary

frequency. Sensory nerve action potentials were completely absent, whereas motor

conduction velocity was slightly reduced only in the lower extremities. Sural nerve

biopsy on the younger brother demonstrated a marked loss of myelinated fibres and

a reduction in the number of unmyelinated axons. Tongue histology revealed

absence of fungiform papillae and taste buds. Autonomic function tests showed

widespread but mild sympathetic and parasympathetic failures. Neuro-imaging

studies revealed atrophy of the spinal cord, cerebellum, brainstem and corpus

callosum, and enlargement of the lateral, third and fourth ventricles. These siblings

represent a previously unrecognized variant of late-onset hereditary spinocerebellar

degeneration with global thermoanalgesia and absence of fungiform papillae on the

tongue.. 0.

214. Gacon, P. H.; Cabre, P.; Smadja, D.; Merle, H.; Vernant, J. C. [Myocardiopathy in

POEMS syndrome (letter)]. Myocardiopathie dans le cadre d'un POEMS

syndrome. Ann-Med-Interne-Paris. 1996; 147(2): 124-6; ISSN: 0003-410X.

FRANCE.

215. Galetta, S. L.; Liu, G. T.; Volpe, N. J. Diagnostic tests in neuro-ophthalmology.

Neurol-Clin. 1996 Feb; 14(1): 201-22; ISSN: 0733-8619.

UNITED-STATES. This article reviews the diagnostic testing used in the

evaluation of several common neuro-ophthalmologic entities including optic nerve

disease, pseudotumor cerebri, anisocoria, ptosis, and ocular motor palsies.

Emphasis is placed on these bedside tests that help to establish the diagnosis of

these common clinical problems. The utility of the cocaine and Tensilon

(edrophonium chloride) tests as well as the role of neuroimaging in these conditions

are reviewed.

216. Garbalosa, J. C.; Cavanagh, P. R.; Wu, G.; Ulbrecht, J. S.; Becker, M. B.;

Alexander, I. J.; Campbell, J. H. Foot function in diabetic patients after partial

amputation. Foot-Ankle-Int. 1996 Jan; 17(1): 43-8; ISSN: 1071-1007.

UNITED-STATES. The function of partially amputated feet in 10 patients with

diabetes mellitus was studied. First-step bilateral barefoot plantar pressure

distribution and three-dimensional kinematic data were collected using a Novel

EMED platform and three video cameras. Analysis of the plantar pressure data

revealed a significantly greater mean peak plantar pressure in the feet with

transmetatarsal amputation (TMA) than in the intact feet of the same patients. The

heels of the amputated feet had significantly lower mean peak plantar pressures than

all the forefoot regions. A significantly greater maximum dynamic dorsiflexion

range of motion was seen in the intact compared with the TMA feet. However, no

difference was noted in the static dorsiflexion range of motion between the two feet

and there was, therefore, a trend for the TMA feet to use less of the available range

of motion. Given the altered kinematics and elevated plantar pressures noted in this

study, careful postsurgical footwear management of feet with TMA would appear

to be essential if ulceration is to be prevented.

217. Gass, A.; Moseley, I. F.; Barker, G. J.; Jones, S.; MacManus, D.; McDonald, W. I.;

Miller, D. H. Lesion discrimination in optic neuritis using high-resolution fat-

suppressed fast spin-echo MRI. Neuroradiology. 1996 May; 38(4): 317-21; ISSN:

0028-3940.

GERMANY. Fast spin-echo (FSE) is a new sequence with acquisition times

currently down to one-sixteenth of those obtained with conventional spin-echo

sequences, which allows high-resolution (512 x 512 matrix) images to be acquired

in an acceptable time. We compared the higher resolution of FSE with the medium

resolution of a short inversion-time inversion-recovery (STIR) sequence in

depicting the optic nerves of healthy controls and patients with optic neuritis. Optic

nerve MRI examinations were performed in 18 patients with optic neuritis and 10

normal controls. Two sequences were obtained coronally: fat-suppressed FSE

(FSE TR 3250 ms/TEef 68 ms, echo-train length 16, 4 excitations, 24 cm

rectangular field of view, 3 mm interleaved contiguous slices, in-plane resolution

0.5 x 0.5 mm) and STIR (TR 2000 ms/TE 50 ms/TI 175 ms, in-plane resolution

0.8 x 0.8 mm, slice thickness 5 mm). FSE demonstrated much more anatomical

detail than STIR, e.g. distinction of optic nerve and sheath. Lesions were seen in

20 of 21 symptomatic nerves using FSE and in 18 of 21 using STIR. Nerve

swelling or partial cross-sectional lesions of the optic nerve were each seen only on

FSE in 3 cases. Fat-suppressed FSE imaging of the optic nerve improves

anatomical definition and increases lesion detection in optic neuritis.

218. Gauthier, P.; Decherchi, P.; Rega, P.; Lammari Barreault, N. [Nerve transplantation in

the central nervous system: a strategy for inducing nerve fiber regeneration in

lesions of the brain and spinal cord]. Transplantation de nerf dans le systeme

nerveux central: une strategie pour provoquer la repousse des fibres nerveuses lors

de lesions du cerveau et de la moelle epiniere. Rev-Neurol-Paris. 1996 Feb; 152(2):

106-15; ISSN: 0035-3787.

FRANCE. Nerve grafts have long been used as a reconstructive strategy in the

human peripheral nervous system. In the nervous system (brain and spinal cord),

peripheral nerve grafts have been used experimentally for promoting axonal

regeneration in lesioned pathways. Peripheral nerve grafts play the role of physical

and trophic supports that are favourable for the regeneration of central fibres that

normally present only abortive regeneration in adult mammals. On the basis of

several experimental models (axonal regeneration of retinal ganglion cells,

respiratory neurones, ...) it has now been established that central fibres that have

regenerated within the nerve grafts retain their ability to transmit normal nervous

information and can make functional synaptic contact with a target. If such a

strategy is not yet used in man, it nevertheless constitutes a promising approach for

the investigation of the central nervous system plasticity, and could be useful in the

treatment of spinal cord injuries.

219. Gellman, R.; Burns, S. Walking aches and running pains. Injuries of the foot and

ankle. Prim-Care. 1996 Jun; 23(2): 263-80; ISSN: 0095-4543.

UNITED-STATES. Running is enjoyed by approximately 30 million people in the

United States, 10 million on a regular basis. It is common to encounter a patient

who runs and expects his or her primary physician to have a degree of expertise in

injuries caused by running. The primary care clinician also may experience the

frustration of motivating a patient to exercise, only to have him or her return with

complaints of foot or ankle pain. Running injuries occur from an overload on the

muscles, tendons, bones, or joints. The knee, foot, and ankle are the most common

sites of injury.

220. Genis, D.; Busquets, C. Comments on Pentland and Donald [letter]. Pain. 1996 Feb;

64(2): 402-3; ISSN: 0304-3959.

NETHERLANDS. 0; 57-27-2.

221. Ghanem, I.; Zeller, R.; Seringe, R. [The foot in hereditary motor and sensory

neuropathies in children]. Le pied dans les neuropathies peripheriques hereditaires

sensitivo-motrices chez l'enfant. Rev-Chir-Orthop-Reparatrice-Appar-Mot. 1996;

82(2): 152-60; ISSN: 0035-1040.

FRANCE. PURPOSE: Although Charcot-Marie-Tooth disease (CMT) is known to

be the most common neuromuscular cause of pes cavovarus, other paralytic

deformities of the foot may be present with hereditary motor and sensory

neuropathies (HMSN). The purpose of our review is to analyze these foot

deformities and to assess the results of the different therapeutic methods used.

MATERIAL AND METHODS: We evaluated 66 patients who had HMSN and had

a total of 127 foot deformities. Fifty three patients had CMT, 6 patients Dejerine

Sottas disease (DS), and 7 patients had an unclassified HMSN. The average age at

diagnosis was of 9 years and 11 months. There were 35 males and 31 females. The

deformity was unilateral in 5 cases. In 50 bilateral cases, the severity of the

deformity was not similar in both feet. In three bilateral cases, the deformity was

completely asymmetrical. The chief complaint was mainly deformity in all cases,

followed by subtalar or ankle instability in 57 cases. There were 105 cases of cavus

or cavovarus, and 22 cases or valgus or planovalgus deformity (8 of which

changed spontaneously to cavovarus). A non surgical treatment was undertaken in

57 cases for minor deformity. Soft tissue release with or without osteotomies was

done in 39 cases, and triple arthrodesis in the remaining 31 cases. A clawtoe

deformity was treated operatively in 14 cases. RESULTS: The mean follow-up

period was 6 years and 9 months for non operated feet and 7 years and 10 months

for operated feet (all of the surgically treated feet were reviewed after the end of

growth). Three patients of the non operated group and 8 patients of the surgically

treated feet underwent triple arthrodesis for a recurrence of the deformity. Thirty

nine per cent of the feet which underwent triple arthrodesis (a total of 42 feet) were

considered to have fair or bad result at 6 years and 3 months follow-up period.

DISCUSSION AND CONCLUSION: The foot deformity in HMSN is of a wide

variety. A valgus flat foot is not uncommon, especially in DS and unclassified

neuropathies. The young age of the patient is not a contrindication to surgical

management. Even if minor previous surgeries do not always succeed in avoiding

recurrence of the deformity, they nevertheless prepare the foot fort a possible triple

arthrodesis, that will be done in better anatomical conditions.

222. Ghavanini, M. R.; Kazemi, B.; Jazayeri, M.; Khosrawi, S. Median-radial sensory

latencies comparison as a new test in carpal tunnel syndrome. Electromyogr-Clin-

Neurophysiol. 1996 Apr; 36(3): 171-3; ISSN: 0301-150X.

BELGIUM. Median and radial distal sensory latencies (DSL) were compared in 50

patients with carpal tunnel syndrome (CTS) and 50 healthy subjects by stimulating

each nerve separately and recording sensory nerve action potential (SNAP) from

standard anatomical locations for stimulation and recording sites. The range of

difference between median DSL and radial DSL was 0.18 -1.18 msec in control

group and 1.12-4.46 msec in CTS patients with a mean of 0.69 msec and 1.99

msec respectively (P < or = 0.0001). We found the value of 1 msec as a good cut-

off point for diagnosis of CTS. The test described here seems to be an effective and

simple criteria for increasing the sensitivity of nerve conduction studies in CTS.

223. Gheorghiu, M. [The pathogenesis of diabetic dyschromatopsia]. Patogenia

discromatopsiei diabetice. Oftalmologia. 1996 Jan; 40(1): 11-5; ISSN: 1120-0875.

ROMANIA. Essential data about the physiology of colour vision are reminded,

with a stress layed on the possible contribution of the rodes in perception of blue

and green colours. The main features of the diabetic dyschromatopsia are reviewed,

together with their pathomorphological support and the two existing theories

regarding its pathogenesis: vascular origin versus neuronal origin of the defect. The

contribution of the photocoagulation to these alterations is also discussed.

224. Gilbert, R. J.; Daftary, S.; Woo, P.; Seltzer, S.; Shapshay, S. M.; Weisskoff, R. M.

Echo-planar magnetic resonance imaging of deglutitive vocal fold closure: normal

and pathologic patterns of displacement. Laryngoscope. 1996 May; 106(5 Pt 1):

568-72; ISSN: 0023-852X.

UNITED-STATES. Abnormalities of vocal fold closure during deglutition

predispose to aspiration due to impairment of airway protection. Conventional

assessment of deglutitive vocal fold motion with laryngoscopy does not permit

visualization through a complete adduction-abduction cycle. We determined

spatiotemporal patterns of deglutitive vocal fold adduction through echo-planar

magnetic resonance imaging in 15 normal volunteers and 6 patients with vocal fold

paralysis. In normal volunteers, deglutitive vocal fold adduction was synchronized

with laryngeal elevation, with complete vocal fold closure at the apex. Patients with

unilateral vocal fold paralysis demonstrated reduced elevation and medial movement

of the involved vocal fold. At maximal laryngeal elevation the uninvolved vocal

fold attained a position superior to the paralyzed fold, resulting in level differences

and an interglottic gap. Patients with bilateral vocal fold paralysis demonstrated

reduced elevation and medial movement of both vocal folds. These findings

indicate that normal and abnormal patterns of vocal fold displacement can be

distinguished noninvasively through the use of echo-planar imaging.

225. Gimse, R.; Tjell, C.; Bjorgen, I. A.; Saunte, C. Disturbed eye movements after

whiplash due to injuries to the posture control system. J-Clin-Exp-Neuropsychol.

1996 Apr; 18(2): 178-86; ISSN: 0168-8634.

NETHERLANDS. Self-reports after whiplash often indicate associations with

vertigo and reading problems. Neuropsychological and otoneurological tests were

applied to a group of whiplash patients (n = 26) and to a carefully matched control

group. The whiplash group deviated from the control group on measures of eye

movements during reading, on smooth pursuit eye movements with the head in

normal position, and with the body turned to the left or to the right. Clinical,

caloric, and neurophysiological tests showed no injury to the vestibular system or

to the CNS. Test results suggest that injuries to the neck due to whiplash can cause

distortion of the posture control system as a result of disorganized neck

proprioceptive activity.

226. Gines, M. A.; Jaber, I.; Perez, J.; Estrada, R. V. [Papilledema as the first

manifestation of Behcet's disease (letter)]. Edema de papila como primera

manifestacion de la enfermedad de Behcet. An-Med-Interna. 1996 May; 13(5): 255-

6; ISSN: 0212-7199.

SPAIN.

227. Gioannini, P.; Cariti, G. [Atypical hepatitis]. L'epatite atipica. Minerva-Gastroenterol-

Dietol. 1996 Sep; 42(3): 153-9; ISSN: 0026-4776.

ITALY. Acute viral hepatitis has a rather homogeneous clinical pattern, excepting a

few features which are related to the specific viral agent. However, a few cases

may be defined "atypical", as fas as clinical features, transmission pattern, serology

are concerned. These include cholestatic hepatitis syndrome, fulminant HEV

hepatitis during pregnancy, neonatal hepatitis. Non-typical aspects may be

observed when extrahepatic manifestations are present (cryoglobulinemia, bone

marrow aplasia, guillain-Barre syndrome, peripheral neuropathy, skin

involvement, etc.). Finally, viral hepatitis may be "atypical" when associated with

other infections (malaria, typhoid fever, etc.), mostly in patients from tropical

Countries.

228. Glatt, H. J. Optic nerve dysfunction in thyroid eye disease: a clinician's perspective

[editorial; comment]. Radiology. 1996 Jul; 200(1): 26-7; ISSN: 0033-8419.

Note: Comment on: Radiology 1996 Jul;200(1):123-7.

UNITED-STATES.

229. Gockel, M. Operative treatment of thoracic outlet syndrome in Finland. Ann-Chir-

Gynaecol. 1996; 85(1): 59-61; ISSN: 0355-9521.

FINLAND. The data from the nationwide hospital discharge register was used for

collecting the diagnoses of the thoracic outlet syndrome (TOS) which were

combined with the procedure numbers of first rib resection and scalenotomy.

During the years 1987-1993 the total number of operative operative periods for

patients with a TOS diagnosis was 483 for 464 patients. Each year first rib

resections were done significantly (P = 0.001) more often (55.7 SE 7.1;

1.11/100,000) than scalenotomies (13.4 SE 1.8; 0.27/100,000). The operation for

TOS was most commonly combined with the diagnosis of TOS with brachial

plexus lesion in 53%, TOS NUD (not classified) in 21%, TOS with subclavian

artery compression in 19%, with a cervical rib in 4% and TOS with venous

compression in 3%. The large proportion of the diagnosis TOS NUD clearly shows

the need for a better definition for the TOS diagnosis. As long as clear diagnostic

criteria are lacking, the division of TOS into subgroups is arbitrary. Diagnostic

division into true neurogenic, major arterial and venous TOS, and classifying the

rest of the TOS diagnoses under TOS NUD or cervicobrachiale diffusum is

recommended.

230. Goksu, N.; Kemaloglu, Y. K.; Ataoglu, O.; Ileri, F.; Hicyilmaz, C. Incidental

tumours during middle ear surgery. J-Otolaryngol. 1996 Jun; 25(3): 195-9; ISSN:

0381-6605.

CANADA.

231. Goldberg, D. S.; Ludwig, I. H. Congenital central hypoventilation syndrome: ocular

findings in 37 children. J-Pediatr-Ophthalmol-Strabismus. 1996 May; 33(3): 175-

80; ISSN: 0191-3913.

UNITED-STATES. BACKGROUND: Congenital central hypoventilation

syndrome (CCHS) is a rare cause of central sleep apnea. Although ophthalmic

abnormalities have been reported, the ocular findings have not been discussed in

detail. METHODS: We examined or obtained the records of 37 children with

CCHS. RESULTS: Twenty-seven patients were found to have abnormal pupils,

most of which were miotic and reacted poorly to light. In 18 cases, the anterior

surface of the iris was unusually smooth. Ten of the children with abnormal pupils

also demonstrated light-near dissociation. Twenty had strabismus of various types,

and 18 showed evidence of convergence insufficiency. CONCLUSIONS: The high

incidence of strabismus, pupillary abnormalities, and convergence insufficiency

may be a result of neurologic defects in the midbrain.

232. Goldstein, D. S.; McRae, A.; Holmes, C.; Dalakas, M. C. Autoimmune autonomic

failure in a patient with myeloma-associated Shy-Drager syndrome. Clin-Auton-

Res. 1996 Feb; 6(1): 17-21; ISSN: 0959-9851.

ENGLAND. We report here the case of a patient with the Shy-Drager syndrome

and multiple myeloma who had evidence consistent with a central neural

autoimmune basis for sympathetic autonomic failure. Autonomic function testing

showed no recordable peroneal skeletal muscle sympathoneural traffic, normal

arterial norepinephrine (NE) spillover during supine rest and no increment in NE

spillover during exposure to lower body negative pressure. The patient's

cerebrospinal fluid and serum contained an immunoglobulin G that bound to rat

locus ceruleus (LC) in an in vitro test system. The myeloma protein was of the

lambda subtype and bound in the rat LC, without binding in the substantia nigra, as

demonstrated with anti-lambda antiserum. Since in this case the monoclonal

antibody produced by the myeloma bound specifically to LC cells, the results are

consistent with the hypothesis that in this patient the Shy-Drager syndrome may

have had an immune-mediated basis.. 0; 0; 0.

233. Golnik, K. C.; Hund, PW 3rd; Stroman, G. A.; Stewart, W. C. Magnetic resonance

imaging in patients with unexplained optic neuropathy. Ophthalmology. 1996 Mar;

103(3): 515-20; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: To investigate the anatomic relation between the

optic nerve and the adjacent intracranial internal carotid artery (ICA) in patients with

unexplained optic neuropathy. METHODS: Coronal magnetic resonance images

(MRIs) of 20 patients with unexplained optic neuropathy and 20 age-matched

controls were evaluated with digitizing software. RESULTS: The distance between

the optic nerve and ICA was significantly less on the side ipsilateral to the atrophic

optic nerve than either the study group's contralateral optic nerve-ICA distance

(P<0.001) or the control group's optic nerve-ICA distance (P<0.001). The

diameter of the atrophic optic nerves was significantly less than either the diameter

of the study group's contralateral normal nerves (P=0.008) or the control group's

nerves (P<0.001). There was no significant difference between the diameters of the

ICA flow voids adjacent to the atrophic optic nerves versus either the study group's

contralateral ICA flow voids (P=0.91) or the control group's ICA flow voids

(P=0.74). CONCLUSIONS: The authors speculate that the intimate relation

between the optic nerve and adjacent ICA may be important in the development of

unexplained optic neuropathy. The ICA need not appear abnormal in MRIs.

234. Goonetilleke, A.; Yuill, G. M. Neurological picture. Aberrant regeneration of the third

cranial nerve. J-Neurol-Neurosurg-Psychiatry. 1996 Mar; 60(3): 281; ISSN: 0022-

3050.

ENGLAND.

235. Gordon, N. Benign paroxysmal positional vertigo. Br-J-Clin-Pract. 1996 Jun; 50(4):

208-10; ISSN: 0007-0947.

ENGLAND. Benign paroxysmal positional vertigo should be considered when a

patient complains of attacks of dizziness of brief duration, that seem to occur under

particular circumstances related to the position of the head. The true nature of the

disorder may be missed unless examination is carried out in a certain way: the head

must be positioned below the rest of the body and turned to one or other side.

Diagnosis then depends on the occurrence of vertigo and on noting the transient

nystagmus. There are a number of causes of benign paroxysmal positional vertigo-

head injury is a relatively frequent one. It has been suggested that this disorder is

due to deposits on the cupula of the precipitating posterior semicircular canal.

Indeed, exercises designed to dislodge such deposits can be successful. If not, and

the vertigo lasts for more than a few months, surgery can be considered.

236. Gorizontova, M. P.; Mironova, I. V.; Reshetniak, V. K. [Effect of etimizol on

development of a neuropathic pain syndrome]. Vliianie etimizola na razvitie

neiropaticheskogo bolevogo sindroma. Biull-Eksp-Biol-Med. 1996 Mar; 121(3):

258-61; ISSN: 0365-9615.

RUSSIA. 0; 64-99-3.

237. Gorsche, R. G.; Verstraten, K. L. A butcher with sharp pains in his arms. Lancet.

1996 Sep 28; 348(9031): 862; ISSN: 0140-6736.

ENGLAND.

238. Gottesman, M. H.; Ibrahim, B.; Elfenbein, A. S.; Mechanic, A.; Hertz, S. Cardiac

arrest caused by trigeminal neuralgia. Headache. 1996 Jun; 36(6): 392-4; ISSN:

0017-8748.

UNITED-STATES. A 67-year-old man with a 12-year history of trigeminal

neuralgia experienced multiple fainting episodes preceded by right facial pain. One

episode resulted in cardiac arrest with successful resuscitation. Pacemaker insertion

prevented further episodes of syncope despite the occurrence of pain. The fainting

episodes and cardiac arrest are believed to be unusual manifestations of trigeminal

neuralgia.

239. Grand, C.; Monier, P.; Andre, L.; Dubreuil, C. [Recurrent peripheral facial paralysis.

An unusual case (letter)]. Paralysie faciale peripherique recidivante. Une

observation inhabituelle. Presse-Med. 1996 Feb 10; 25(5): 215; ISSN: 0755-4982.

FRANCE.

240. Griffin, A. C.; Wood, W. G. Brachial plexitis: a rare and often misdiagnosed

postoperative complication. Aesthetic-Plast-Surg. 1996 May; 20(3): 263-5; ISSN:

0364-216X.

UNITED-STATES. Surgeons should be aware of the brachial plexitis syndrome in

order to properly make an early diagnosis and educate the patient and his family on

the etiology of this syndrome. It is characterized by the acute onset of shoulder

pain, weakness, and paralysis in patients following a variety of surgical

procedures.

241. Griffin, J. W.; Hsieh, S. T.; McArthur, J. C.; Cornblath, D. R. Laboratory testing in

peripheral nerve disease. Neurol-Clin. 1996 Feb; 14(1): 119-33; ISSN: 0733-

8619.

UNITED-STATES. Selecting appropriate laboratory tests in diagnosing peripheral

neuropathies is important because it increases the yield of correct diagnoses and is

cost effective. A large number of tests are available. This article provides a guide to

selecting appropriate tests and reviews the clinical situations that suggest specific

tests. Electrodiagnostic testing is valuable in almost all patients with peripheral

neuropathy. Quantitative sensory testing adds additional information and is

especially useful in patients with small fiber neuropathy. On occasion, routine

blood tests may discover metabolic disorders causing a patient's neurologic

disorder. A number of antibody assays for neuropathies are available commercially,

with the most useful being anti-MAG, anti-GM1, anti-GQ1b, anti-Hu, and

anticalcium channel antibodies, but only in very select situations and not as

"screening studies". The role of cutaneous nerve and skin biopsies in selected

disorders is discussed.

242. Grigor'ev, G. M. [Positioning nystagmus]. O nistagme ukladyvaniia. Vestn-

Otorinolaringol. 1996 Jan; (1): 20-3; ISSN: 0042-4668.

RUSSIA. 256 patients with postural vertigo were examined clinically and

neurootologically. Positioning nystagmus (PN) was detected in 209 (81.6%) of

them. Postural vertigo arose as a result of inner ear diseases or traumas (n = 52),

affection of cervical spine (n = 24), cardiovascular diseases (n = 97), organic

impairment of the posterior cranial fossa (n = 20), endocrine disorders (n = 5),

intoxication from chronic infection foci (n = 24), neuroses with vegetative

dysfunction (n-34). PN emerged, as a rule, in patients having 2-3 of the above

conditions. A genesis scheme of PN is outlined with regard to interaction of the

main pathogenic factors influencing the vestibular system. Horizontal-rotatory or

rotatory, vertical, diagonal, bilateral horizontal or multiple PN are characteristic for

peripheral vestibular dysfunctions and central vestibular dysfunction, respectively.

It is reasonable to perform position tests after assessment of the patients' nervous

and cardiovascular systems, the condition of cervical spine.

243. Guiral, H.; Risco, J.; Garcia, B.; Mayayo, E. Functioning glomus vagale tumor: report

of case. J-Oral-Maxillofac-Surg. 1996 Feb; 54(2): 227-30; ISSN: 0278-2391.

UNITED-STATES.

244. Gunn, C. C. Chronic pain: time for epidemiology [letter]. J-R-Soc-Med. 1996 Aug;

89(8): 479-80; ISSN: 0141-0768.

ENGLAND.

245. Gupta, V. K. Ocular palsy and CIPD [letter; comment]. Neurology. 1996 Jul; 47(1):

306; ISSN: 0028-3878.

Note: Comment on: Neurology 1995 Apr;45(4):846-7.

UNITED-STATES.

246. Gupta, V. K. Painless Horner's syndrome in cluster headache [letter]. J-Neurol-

Neurosurg-Psychiatry. 1996 Apr; 60(4): 462-3; ISSN: 0022-3050.

ENGLAND.

247. Hadden, F. M. Home ventilation of a child with motor and sensory neuropathy. Life

does have a price, and the rest of us have to pay it [letter]. BMJ. 1996 Oct 12;

313(7062): 940; ISSN: 0959-8138.

ENGLAND.

248. Hagay, Z.; Weissman, A. Management of diabetic pregnancy complicated by coronary

artery disease and neuropathy. Obstet-Gynecol-Clin-North-Am. 1996 Mar; 23(1):

205-20; ISSN: 0889-8545.

UNITED-STATES. Various manifestations of diabetic neuropathy may complicate

pregnancies of young diabetic patients. Of all forms of diabetic neuropathy,

autonomic neuropathy, and, in particular, gastropathy, may cause the most

devastating complications. Because neuropathy is a common abnormality in young

asymptomatic diabetic women, screening for this disorder may be advisable and

can be accomplished by relatively simple and noninvasive tests. Screening is best

performed before conception or early in pregnancy, because pregnancy itself and its

possible complications later modify the autonomic nervous function tests and make

testing unreliable. Practitioners and obstetricians who provide care and counseling

to young diabetic patients should be familiar with the risks and consequences to

maternal and fetal health that may be imposed by the different forms of neuropathy.

Moderate-to-severe autonomic dysfunction may be considered a relative

contraindication to pregnancy, especially if gastropathy is part of the clinical

presentation. The management dilemmas and high mortality and morbidity

associated with symptomatic diabetic neuropathy may justify the addition of a new

independent class, class N (neuropathy), to the current classification systems for

diabetes in pregnancy.

249. Hagen, P.; Lyons, G. D.; Nuss, D. W. Dysphonia in the elderly: diagnosis and

management of age-related voice changes. South-Med-J. 1996 Feb; 89(2): 204-7;

ISSN: 0038-4348.

UNITED-STATES. In our laryngology practice, we have noted an increasing

number of elderly patients referred to us for problematic dysphonia. We present our

findings of the most common disorder affecting this age group. A sample of 47

consecutive patients over age 60 with dysphonia revealed presbylaryngis, ie, age-

related anatomic and physiologic changes, as the most common etiology found in

this tertiary referral practice, accounting for 30% (14 patients) of new diagnoses.

None of the patients with presbylaryngis received this diagnosis from the referral

source. Understanding the anatomic and physiologic changes of the aging vocal

tract, along with the clinical correlation of each change, is crucial in evaluating this

group of patients. Managing this disorder includes specific goal-oriented speech

therapy, with surgery as an adjunct should conservative therapy prove

unsuccessful. Earlier recognition of this disorder and prompt intervention are key

factors in reversing vocal decompensation, with a primary effect of improving the

quality of life for the patient with age-related dysphonia.

250. Haig, A. J.; Ho, K. C.; Ludwig, G. Clinical, physiologic, and pathologic evidence for

vagus dysfunction in a case of traumatic brain injury. J-Trauma. 1996 Mar; 40(3):

441-4; ISSN: 0022-5282.

UNITED-STATES. A trauma victim with locked-in syndrome demonstrated

severely decreased bowel sounds, intact response to suppository, and elevated, but

unchanging pulse. Absent cardiac response to tracheal suctioning, high gastric

residual volumes, and pulmonary edema in response to a urecholine challenge

demonstrated dysfunction in the automatic system. Symptoms persisted for 2 1/2

years until death. At autopsy, asymmetric bilateral involvement of the dorsal motor

nucleus of the vagus and of the nerve tract in the medulla were demonstrated. In

contrast, a control subject with locked-in syndrome caused by a stroke did not

demonstrate these phenomena. In trauma patients with delayed gastric emptying,

measurement of the heart rate response to deep suctioning may lead to the diagnosis

of this vagus dysfunction syndrome.

251. Hainfellner, J. A.; Kristoferitsch, W.; Lassmann, H.; Bernheimer, H.; Neisser, A.;

Drlicek, M.; Beer, F.; Budka, H. T-cell-mediated ganglionitis associated with acute

sensory neuronopathy. Ann-Neurol. 1996 Apr; 39(4): 543-7; ISSN: 0364-5134.

UNITED-STATES. A 67-year-old man presented with acute painful sensory loss,

areflexia, ataxia, urinary retention, and severe constipation and became unable to

walk within 2 weeks. He died suddenly 5 weeks after the onset of symptoms.

Autopsy revealed widespread inflammation of sensory and autonomic ganglia with

immunocytochemical evidence of a CD8+ T cell-mediated cytotoxic attack against

ganglion neurons. This observation suggests a novel pathogenetic mechanism of

immune-mediated human ganglion cell damage comparable to mechanisms

operating in polymyositis.

252. Haney, C.; Snipes, G. J.; Shooter, E. M.; Suter, U.; Garcia, C.; Griffin, J. W.;

Trapp, B. D. Ultrastructural distribution of PMP22 in Charcot-Marie-Tooth disease

type 1A. J-Neuropathol-Exp-Neurol. 1996 Mar; 55(3): 290-9; ISSN: 0022-3069.

UNITED-STATES. Peripheral Myelin Protein-22 (PMP22) is a membrane

glycoprotein which represents up to 5% of total protein in myelin of peripheral

nerves. Mutations affecting the PMP22 gene have been linked to the inherited

peripheral neuropathies Charcot-Marie-Tooth disease type 1A (CMT1A;

duplications and point mutations), Dejerine-Sottas syndrome (DSS; point

mutations), and hereditary neuropathy with liability to pressure palsies (HNPP;

deletions). In this study, we determined the ultrastructural distribution of PMP22

and other myelin proteins in normal human peripheral nervous system (PNS)

nerves and in CMT1 patients with or without the CMT1A duplication on

chromosome 17. Our results demonstrate that PMP22, P0 protein, and myelin

basic protein are present in compact myelin of all patients examined. PMP22 was

also present in the plasma membrane of Schwann cells of unmyelinated fibers and

onion bulbs. Although the precise biological role of PMP22 remains to be

discovered, our results support the hypothesis that this protein serves multiple

functions in Schwann cells.. 0; 0.

253. Harkness, K.; Howell, S. J.; Davies Jones, G. A. Encephalopathy associated with

intravenous immunoglobulin treatment for Guillain-Barre syndrome [letter]. J-

Neurol-Neurosurg-Psychiatry. 1996 May; 60(5): 586; ISSN: 0022-3050.

ENGLAND. 0.

254. Harrad, R.; Sengpiel, F.; Blakemore, C. Physiology of suppression in strabismic

amblyopia. Br-J-Ophthalmol. 1996 Apr; 80(4): 373-7; ISSN: 0007-1161.

ENGLAND.

255. Harris, C. M.; Shawkat, F.; Russell Eggitt, I.; Wilson, J.; Taylor, D. Intermittent

horizontal saccade failure ('ocular motor apraxia') in children. Br-J-Ophthalmol.

1996 Feb; 80(2): 151-8; ISSN: 0007-1161.

ENGLAND. BACKGROUND: Ocular motor apraxia (OMA) in childhood is a

poorly understood condition involving a failure of horizontal saccades. OMA is

thought to be rare but the literature indicates wide clinical associations. OMA is

often identified by abnormal head movements, but failure of reflexive quick phases

has been reported in all but a few patients. The extent of this oculomotor disorder

was examined in a large group of children with diverse clinical backgrounds.

METHODS: The degree of quick phase failure during horizontal vestibular and

optokinetic nystagmus was measured using DC electro-oculography and video in

74 affected children, aged 17 days to 14 years. RESULTS: All children showed an

intermittent failure of nystagmic quick phases, except for total failure in one case.

Other visuomotor abnormalities were common including saccadic hypometria

(85%), low gain smooth pursuit (70%), neurological nystagmus (28%), strabismus

(22%), and vertical abnormalities (11%). Non-ocular abnormalities were common

including infantile hypotonia (61%), motor delay (77%), and speech delay (87%).

There was a wide range of clinical associations including agenesis of the corpus

callosum, Joubert syndrome, Dandy-Walker malformation, microcephaly,

hydrocephalus, vermis hypoplasia, porencephalic cyst, megalocephaly, Krabbe

leucodystrophy, Pelizaeus Merzbacher disease, infantile Gaucher disease, GM1

gangliosidosis, infantile Refsum's disease, propionic acidaemia, ataxia

telangiectasia, Bardet-Biedl syndrome, vermis astrocytoma, vermis cyst, carotid

fibromuscular hypoplasia, Cornelia de Lange syndrome, and microphthalmos.

Perinatal and postnatal problems were found in 15% including perinatal hypoxia,

meningitis, periventricular leucomalacia, athetoid cerebral palsy, perinatal

septicaemia and anaemia, herpes encephalitis, and epilepsy. Only 27% were

idiopathic. CONCLUSION: Quick phase failure is a constant feature of OMA,

whereas abnormal head movements were detected in only about half, depending on

the underlying diagnosis. This oculomotor sign is better described as an intermittent

saccade failure rather than as a true apraxia. It indicates central nervous system

involvement, has wide clinical associations, but it is not a diagnosis.

256. Hartl, D. L. The most unkindest cut of all [news; comment]. Nat-Genet. 1996 Mar;

12(3): 227-9; ISSN: 1061-4036.

Note: Comment on: Nat Genet 1996 Mar;12(3):288-97.

UNITED-STATES. 0; 0; 0.

257. Harvey, S. Management of painful diabetic neuropathy. J-Am-Acad-Nurse-Pract. 1996

Mar; 8(3): 127-9; ISSN: 1041-2972.

UNITED-STATES. 0.

258. Hashizume, H.; Nishida, K.; Nanba, Y.; Shigeyama, Y.; Inoue, H.; Morito, Y. Non-

traumatic paralysis of the posterior interosseous nerve. J-Bone-Joint-Surg-Br.

1996 Sep; 78(5): 771-6; ISSN: 0301-620X.

ENGLAND. We treated 31 patients with non-traumatic paralysis of the posterior

interosseous nerve over 15 years. There were 10 men and 21 women of mean age

40.3 years (17 to 71). Six were managed conservatively, and 25 by operation. In

14 patients entrapment occurred at the supinator, including three who had double

compression at both the entrance and exit from the muscle. In four it was caused by

a ganglion, in one by a lipoma, in one by a dislocated radial head and in two by a

marked constriction in the nerve of unknown cause. The remaining three patients

were retrospectively diagnosed as having neuralgic amyotrophy, the only

observable change at operation being slight oedema of the nerve. Paralysis

recovered in 24 out of the 25 patients at between 2 to 18 months (mean 5.6) after

operation, and the one failure was treated later by tendon transfer.

259. Haussmann, P.; Patel, M. R. Intraepineurial constriction of nerve fascicles in pronator

syndrome and anterior interosseous nerve syndrome. Orthop-Clin-North-Am. 1996

Apr; 27(2): 339-44; ISSN: 0030-5898.

UNITED-STATES. In PS or AINS, if obvious epineurial compression deformity

of the median nerve or the AIN is not found at known sites of compression, IeCNF

should be considered. IeCNF may occur in one or more nerve fascicles of the

median nerve at one of multiple levels in the distal upper arm and proximal forearm.

Decompression of the nerve fascicles is achieved by epineurotomy, microsurgical

interfascicular dissection, and removal of the constricting outer layer of the

perineurium above and below the elbow. Resection of the constricted segments of

the nerve fascicles is not necessary. Intraepineurial exploration of the nerve trunk

also may be considered if, after surgical decompression of PS and AINS, expected

recovery has not occurred and there is no evidence of axonal degeneration.

260. Heafield, M. T.; Gammage, M. D.; Nightingale, S.; Williams, A. C. Idiopathic

dysautonomia treated with intravenous gammaglobulin. Lancet. 1996 Jan 6;

347(8993): 28-9; ISSN: 0140-6736.

ENGLAND. BACKGROUND: A previously healthy 23-year-old man presented

with a short history of abdominal pain and diarrhoea followed by blurred vision,

severe postural hypotension, reduced sweating and unremitting fever. METHODS:

Examination revealed fixed dilated pupils, impaired sweating and postural

hypotension. Clinical and neurophysiological examination showed no motor or

sensory deficit. A diagnosis of idiopathic autonomic neuropathy was made. He

became gravely ill with profound life-threatening hypotension and a prolonged

ileus. FINDINGS: Within 36 h of receiving intravenous gammaglobulin (IVGG)

his pupillary areflexia and severe hypotension resolved. 2 weeks later the

autonomic failure recurred but again responded to treatment with IVGG. IVGG is a

recognised treatment for Guillain-Barre syndrome. INTERPRETATION: This case

report demonstrates that IVGG is also effective in the rare pure dysautonomic

variant.. 0.

261. Heart rate variability. Standards of measurement, physiological interpretation, and

clinical use. Task Force of the European Society of Cardiology and the North

American Society of Pacing and Electrophysiology. Eur-Heart-J. 1996 Mar; 17(3):

354-81; ISSN: 0195-668X.

ENGLAND.

262. Heart rate variability: standards of measurement, physiological interpretation and

clinical use. Task Force of the European Society of Cardiology and the North

American Society of Pacing and Electrophysiology. Circulation. 1996 Mar 1;

93(5): 1043-65; ISSN: 0009-7322.

UNITED-STATES.

263. Heierli, P.; Bonnard, C.; Favarger, N.; Egloff, D. V. [Reflex sympathetic

algodystrophy]. L'algodystrophie sympathique reflexe. Rev-Med-Suisse-

Romande. 1996 Mar; 116(3): 197-203; ISSN: 0035-3655.

SWITZERLAND.

264. Heinemann, F.; Assion, H. J. [Therapy of coinciding trigeminal neuralgia and

schizophrenia]. Therapie bei Koinzidenz von Trigeminusneuralgie und

Schizophrenie. Psychiatr-Prax. 1996 Jul; 23(4): 197; ISSN: 0303-4259.

GERMANY. 0; 2062-78-4.

265. Herdman, S. J.; Tusa, R. J. Complications of the canalith repositioning procedure.

Arch-Otolaryngol-Head-Neck-Surg. 1996 Mar; 122(3): 281-6; ISSN: 0886-4470.

UNITED-STATES. OBJECTIVE: To describe the conversion of benign

paroxysmal positional vertigo involving the posterior canal into benign paroxysmal

positional vertigo involving the anterior or horizontal canals after treatment using

the canalith repositioning maneuver. DESIGN: Retrospective study of outcome.

SETTING: Outpatient clinic. PATIENTS: Consecutive sample of 85 patients

diagnosed as having benign paroxysmal positional vertigo affecting the posterior

canal. Identification of posterior canal involvement was based on the observation of

the direction of the vertical component of nystagmus after the Hallpike-Dix

maneuver. INTERVENTION: Canalith repositioning maneuver. MAIN

OUTCOME MEASURE: Eye movements were observed about 1 week after the

treatment. The direction of nystagmus elicited after movement of the patient into the

Hallpike-Dix position indicated which canal was involved in the patients who had

not responded to treatment. RESULTS: Of the 85 patients studied who originally

had posterior canal benign paroxysmal positional vertigo, five (6%) had anterior

canal (n=2) or horizontal canal (n=3) positional vertigo after undergoing this

maneuver. CONCLUSION: Careful observation of the direction of the nystagmus

is necessary for correct identification of which canal is involved in patients who do

not respond to the initial treatment using the canalith repositioning maneuver.

266. Hernando, I.; Ara, J. R.; Monzon, M. J.; Lopez, A. [Sensory-motor polyneuropathy

associated with lung tuberculosis: presentation of two new cases]. Polineuropatia

sensitivomotora asociada a tuberculosis pulmonar: presentacion de dos casos. Rev-

Neurol. 1996 Jul; 24(131): 841-2; ISSN: 0210-0010.

SPAIN. The association of active tuberculosis with changes in the peripheral

nervous system is not well defined, although it is included amongst the neuro-

muscular disorders of the peripheral nerve diseases due to infective agents. We

describe the case of two patients with sensory-motor polyneuropathy and coexistant

active pulmonary tuberculosis which was untreated prior to diagnosis of the

peripheral nervous system disorder. In both cases the condition was sub-acute and

did not improve until treatment was begun. Anti-tuberculosis treatment was

followed by marked improvement of both the pulmonary tuberculosis and of the

polyneuropathy. The close chronological relationship of both clinical pictures with

the therapeutic response supports the hypothesis of the pathogenesis of the

peripheral nervous system disorder being mediated by an anomolous immune

response, initiated by the infection with Koch's bacillus.

267. High, M. E. Acute canine polyradiculoneuritis. Can-Vet-J. 1996 May; 37(5): 305;

ISSN: 0008-5286.

CANADA.

268. Hilburn, J. W. General principles and use of electrodiagnostic studies in carpal and

cubital tunnel syndromes. With special, attention to pitfalls and interpretation.

Hand-Clin. 1996 May; 12(2): 205-21; ISSN: 0749-0712.

UNITED-STATES. Electrodiagnostic studies can help confirm the clinical

impression of CTS and cubital tunnel syndrome with a high degree of sensitivity

and specificity. These tests can provide valuable information regarding the exact

point of nerve compression as well as the severity of damage. Differentiating these

nerve entrapment syndromes from other conditions with similar clinical

presentations can lead to effective therapy and can help predict response to

treatment. The electrodiagnostic laboratory should employ the most current and

sensitive techniques for obtaining optimal results, and the ordering physician

should have a working knowledge of the principle involved in the tests to make

best use of the resulting information.

269. Hilburn, J. W. General principles and use of electrodiagnostic studies in carpal and

cubital tunnel syndromes. With special, attention to pitfalls and interpretation.

Hand-Clin. 1996 May; 12(2): 205-21; ISSN: 0749-0712.

UNITED-STATES. Electrodiagnostic studies can help confirm the clinical

impression of CTS and cubital tunnel syndrome with a high degree of sensitivity

and specificity. These tests can provide valuable information regarding the exact

point of nerve compression as well as the severity of damage. Differentiating these

nerve entrapment syndromes from other conditions with similar clinical

presentations can lead to effective therapy and can help predict response to

treatment. The electrodiagnostic laboratory should employ the most current and

sensitive techniques for obtaining optimal results, and the ordering physician

should have a working knowledge of the principle involved in the tests to make

best use of the resulting information.

270. Hirsch, I. B. Surveillance for complications of diabetes: don't wait for symptoms

before intervening. Postgrad-Med. 1996 Mar; 99(3): 147-55, 159-62; ISSN: 0032-

5481.

UNITED-STATES. Surveillance and treatment of diabetes-related complications

should be part of routine care of all patients with diabetes. The natural history and

screening recommendations for diabetic retinopathy, nephropathy, and neuropathy

must be understood, since even advanced disease can be asymptomatic. Most

adults require yearly ophthalmologic evaluations and determinations of

albuminuria. Regular foot examinations by the patient and physician are required,

with special attention to identifying patients with increased susceptibility to

neuropathic ulcer and lower extremity amputation (ie, the "high-risk foot"). Cardiac

autonomic neuropathy has become easier to diagnose, and its presence has several

implications. Measurement of lipid levels and glycosylated hemoglobin and

assessment of nutritional health should also be included in evaluation. Although

understanding and prevention of diabetes complications are improving, the impact

of end-organ damage remains a major problem. Early diagnosis and treatment often

improve outcome and should dramatically decrease the burden of diabetes in our

society.

271. Holder, L. E. Diagnosis of reflex sympathetic dystrophy [letter; comment]. J-Hand-

Surg-Am. 1996 Mar; 21(2): 325; ISSN: 0363-5023.

Note: Comment on: J Hand Surg [Am] 1995 May;20(3):458-63.

UNITED-STATES.

272. Howlett, W. P.; Vedeler, C. A.; Nyland, H.; Aarli, J. A. Guillain-Barre syndrome in

northern Tanzania: a comparison of epidemiological and clinical findings with

western Norway. Acta-Neurol-Scand. 1996 Jan; 93(1): 44-9; ISSN: 0001-6314.

DENMARK. To study Guillain-Barre syndrome (GBS), the clinical files of GBS

patients, 59 in northern Tanzania (1984-1992) and 56 in western Norway (1980-

1992), were retrospectively reviewed and compared. The average annual incidence

rate for GBS in the Kilimanjaro region was 0.83/100,000 population as compared

to 1.2/100,000 reported in western Norway (25). GBS patients in the Tanzanian

series were younger, had less antecedent infection and were a longer time interval

from onset to admission and in hospital. On examination arm and sensory

involvement were less common and urinary sphincteric involvement more common

in the Tanzanian series. The overall mortality rate was higher in the Tanzanian

series, 15.3% (9/59) versus 1.8% (1/56). HIV infection was found in 30.5%

(11/36) of GBS patients in the Tanzanian series as compared to 3.4% (161/4687) in

corresponding blood donors. There was no evidence of HIV infection in the

Norwegian series. The HIV-seropositive GBS patients in comparison with HIV

seronegatives had a shorter duration of onset, greater neurological involvement and

a higher mortality rate, 45.5% (5/11) versus 16% (4/21). This study shows that

apart from minor clinical differences and an increased mortality rate in the

Tanzanian series GBS was similar in both series. GBS in the Tanzanian series was

strongly associated with HIV infection, and seropositives by comparison with

seronegatives appeared to have more severe disease.

273. Huang, J. J.; Jong, Y. J.; Huang, M. Y.; Chiang, C. H.; Huang, T. Y.

Electrocardiographic findings in children with spinal muscular atrophy. Jpn-Heart-

J. 1996 Mar; 37(2): 239-42; ISSN: 0021-4868.

JAPAN. Most of the patients in this study with spinal muscular atrophy were

found to have tremors of the isoelectric line in the electrocardiogram (ECG)

tracings. There were a total of 47 cases of SMA (mean age 40.8 months). All three

types of SMA had a similar incidence (about 80%) of tremors in the tracings (p =

0.885). In 7 cases the ECG tremors had an intermittent pattern. ECG tremors were

commonly found in the majority of SMA patients and this finding, though non-

specific, may suggest a possible SMA diagnosis.

274. Huber, A. [Vision disorders in retrochiasmatic lesions of the visual pathways].

Visuelle Storungen bei retrochiasmalen Lasionen der Sehbahn. Ther-Umsch. 1996

Jan; 53(1): 31-6; ISSN: 0040-5930.

SWITZERLAND. Anatomic elements of the retrochiasmatic pathway. Synopsis of

homonymous pathway. Synopsis of homonymous hemianopia: unilateral forms

[quadrant, total], bilateral forms [tunnel field, cerebral blindness], homonymous

scotomas, horizontal hemianopsias, checkerboard hemianopsias, sparing of

temporal crescent. Additional disorders: hemi-neglect, color agnosia, hemi-

achromatopsia, alexia, abnormal optokinetic nystagmus, cog-wheel pursuit

movement, hemianopic pupillary defect, statokinetic dissociation [Riddoch

phenomenon], hallucinations, illusions, visual agnosia, prosopagnosia.

275. Hughes, R. A. Intravenous IgG in Guillain-Barre syndrome [editorial]. BMJ. 1996

Aug 17; 313(7054): 376-7; ISSN: 0959-8138.

ENGLAND. 0; 0.

276. Hughes, R.; Sharrack, B.; Rubens, R. Carcinoma and the peripheral nervous system.

J-Neurol. 1996 May; 243(5): 371-6; ISSN: 0340-5354.

GERMANY. An underlying carcinoma is an important differential diagnosis in

peripheral neuropathy. While direct compression and infiltration of spinal roots or

peripheral or cranial nerve trunks or branches can be identified easily when they

occur in association with established malignancy, their diagnosis when they are

presenting features may be difficult. In paraneoplastic sensory neuronopathy

autoantibodies to neuronal antigens have become useful diagnostic markers for an

underlying carcinoma, especially anti-Hu antibodies. Strong circumstantial

evidence suggests that these antibodies form part of an autoimmune response which

is responsible for the pathogenesis of some of these syndromes. Neuropathy

appearing during the course of treatment of carcinoma may be due to radiation-

induced damage or the neurotoxic effects of some chemotherapeutic agents.

Neurotrophic factors are being investigated as a strategy for reducing the neurotoxic

effects of these agents.

277. Hund, E. F.; Fogel, W.; Krieger, D.; DeGeorgia, M.; Hacke, W. Critical illness

polyneuropathy: clinical findings and outcomes of a frequent cause of

neuromuscular weaning failure [see comments]. Crit-Care-Med. 1996 Aug; 24(8):

1328-33; ISSN: 0090-3493.

Note: Comment in: Crit Care Med 1996 Aug;24(8):1282-3.

UNITED-STATES. OBJECTIVE: To describe clinical and electrophysiologic

features and outcomes of critically ill patients with neuromuscular causes of failure

to wean from mechanical ventilator support. DESIGN: A prospective, consecutive,

case series. SETTING: Neurological, neurosurgical, and medical intensive care

units in a university hospital. PATIENTS: Seven patients during a 3-yr period with

failure to wean from mechanical ventilation not explained by pulmonary

complications. INTERVENTIONS: Muscle and nerve biopsy in three patients.

MEASUREMENTS AND MAIN RESULTS: Detailed electrodiagnostic studies

were done in all patients 3 to 6 wks (median 4.5) after the onset of the acute illness

and were repeated 3 months to 3.5 yrs later in those patients who survived.

Primary illnesses included various intracranial and medical conditions. All patients

had moderate-to-severe limb weakness with marked muscle atrophy. Tendon

reflexes were decreased in three patients, exaggerated in two patients with

intracranial lesions, and absent in two patients. Electromyography demonstrated

severe acute denervation, with striking involvement of proximal muscles. Muscle

and nerve biopsies showed severe neurogenic atrophy and axonal degeneration

without inflammation. There was no evidence of primary myopathy. Two patients

died of complications of sepsis. Of the survivors, three patients had no further

weakness at the time of reexamination, except for peroneal nerve palsy in one

patient. Two patients, still in the recovery period, showed markedly improved

conditions but still showed slight weakness of the proximal muscles. By

electrophysiology, signs of chronic neurogenic damage were demonstrable in all

survivors at follow-up. CONCLUSIONS: Critical illness polyneuropathy is a

frequent cause of neuromuscular weaning failure in critically ill patients, regardless

of the type of primary illness. Involvement of proximal (including facial and

paraspinal) muscles is striking. Tendon reflexes are often preserved. Patients with

central nervous system injury may likewise develop critical illness polyneuropathy.

In these latter patients, tendon reflexes may even be exaggerated. Recovery from

critical illness polyneuropathy is usually rapid and clinically complete, although

incomplete on electrodiagnostic study. Residual peripheral nerve lesion, generally

of the peroneal nerve, is the most frequent feature of incomplete recovery. The need

for careful electrophysiologic testing is emphasized to clarify the nature and extent

of neuromuscular disturbances in critically ill patients. Failure to recognize the

development of neuropathy in these patients may lead to erroneous conclusions

about the ability to wean them from the ventilator.

278. Hunter, J. M. Reconstruction of the transverse carpal ligament to restore median nerve

gliding. The rationale of a new technique for revision of recurrent median nerve

neuropathy. Hand-Clin. 1996 May; 12(2): 365-78; ISSN: 0749-0712.

UNITED-STATES. Two clinical and four surgical steps can be taken to return

gliding and nutritional balance to the median nerve after revision surgery for a

recurrent median nerve neuropathy: Step One (Clinical): An accurate diagnosis of

the median nerve traction neuropathy. Consider brachial plexopathy and terminal

neuropathies. Step Two (Surgical): Comprehensive exploration and meso-

epineurolysis of the median nerve from the radial remnants of the divided TCL and

fibrosed radial and ulnar bursae by resection of the bursae and flexor

tenosynovectomy. Step Three (Surgical): Fasciotomy of the volar carpal ligament to

lengthen the ulnar leaf of the TCL; permits visualization of the ulnar artery and

neurolysis of the ulnar motor nerve. Step Four (Surgical): Mobilize the previously

released radical leaf TCL and motor median nerve from the origin of the thenar

muscles. Step Five (Surgical): Restore the inner gliding surface and strength of the

flexor retinaculum of the hand and wrist by reconstruction of the TCL and the

forearm antebrachial fascia. Step Six (Clinical): An immediate postoperative hand

therapy program to restore gliding to the median nerve and flexor tendons.

Protocols for upper extremity nerve gliding should be carried out under supervision

at selected daily intervals. A prevailing brachial plexopathy requires positive

supervised therapy for nerve gliding and posture control.

279. Husby, G. [Classification of amyloidosis]. Klassifisering av amyloidose. Tidsskr-

Nor-Laegeforen. 1996 Jan 20; 116(2): 234-7; ISSN: 0029-2001.

NORWAY. Amyloidosis is a collective term for a heterogeneous group of

disorders characterized by deposition of a fibrillar, proteinaceous material, amyloid,

in various tissues and organs. Increasing knowledge about the different proteins

that constitute the amyloid fibrils has made it possible to classify amyloidosis by the

fibril protein, which appears more rational than the traditional classification by its

clinical symptoms. A group of experts on amyloidosis met in Oslo in 1990 and

agreed upon a nomenclature and classification based on the chemical properties of

the amyloid fibrils.. 0.

280. Iani, C.; Attanasio, A.; Manfredi, M. Paroxysmal staring and masticatory automatisms

during postural hypotension in a patient with multiple system atrophy. Epilepsia.

1996 Jul; 37(7): 690-3; ISSN: 0013-9580.

UNITED-STATES. PURPOSE: We studied a 51-year-old man with multiple

system atrophy and autonomic insufficiency. He had repeated episodes of loss of

contact, staring, and masticatory automatisms. METHODS: Blood pressure during

these events documented a systolic pressure of 60 mm Hg. Cardiovascular reflex

tests provided evidence of autonomic failure. Head computed tomography (CT)

revealed moderate, diffuse cortical and cerebellar atrophy. RESULTS: These events

were strictly related to blood pressure decreases and could be reproduced

consistently by having the patient sit up after a meal. Ictal polygraphic recordings

showed EEG changes consistent with anoxia, preceded by sudden hypotension

with fixed heart rate. CONCLUSIONS: Cerebral anoxia during a syncopal attack

may therefore precipitate transient, sudden neurologic dysfunction that closely

mimics complex partial seizures. Masticatory automatisms may represent a release

phenomenon resulting from inactivation of neocortical structures by cerebral anoxia

or reticular disconnection.

281. Idler, R. S. General principles of patient evaluation and nonoperative management of

cubital syndrome. Hand-Clin. 1996 May; 12(2): 397-403; ISSN: 0749-0712.

UNITED-STATES. Cubital tunnel syndrome is reported to be the second most

common entrapment neuropathy in the upper extremity. We can now easily

appreciate how ulnar nerve pathology can occur at the elbow in the absence of

trauma. We can also appreciate the role of conservative management in the

treatment of a condition that previously was thought to be a surgical disease.

282. Idler, R. S. Persistence of symptoms after surgical release of compressive neuropathies

and subsequent management. Orthop-Clin-North-Am. 1996 Apr; 27(2): 409-16;

ISSN: 0030-5898.

UNITED-STATES. Persistent symptoms following surgical release for entrapment

neuropathy are a source of great frustration for both the surgeon and patient.

Fundamentals of management require review of the patient's history and

examination in an attempt to confirm the initial diagnosis and to rule out elements of

the differential diagnosis. Persistent symptomatology may be a reflection of an

incorrect diagnosis or double crush syndrome. If the initial diagnosis can be

reconfirmed, then it is reasonable to reinstitute conservative management and to

objectively re-evaluate the entrapment neuropathy by electrodiagnostic testing. This

electrodiagnostic testing must be comprehensive in order to evaluate all potential

entrapment neuropathies within the differential diagnosis. Individuals who

demonstrate a transient response to conservative management or evidence of further

deterioration on electrodiagnostic testing may be considered candidates for revision

surgery. These individuals may be found to have had an incomplete release, error

of technique, or iatrogenic compression. Persistence of symptoms on the basis of

end-stage disease must be recognized to avoid further surgery that is unlikely to be

of benefit to the patient. If surgical intervention is chosen, the procedure must

address issues of residual compression, preservation of nerve vascularity,

prevention of neurodesis, and protective padding in the presence of nerve

hypersensitivity.

283. Iester, A.; Vignola, S.; Callegarini, L.; Gimelli, G.; Alpigiani, M. G. [18q syndrome

with deficiency of myelin basic protein (MBP)]. Sindrome 18q- con carenza di

myelin basic protein (MBP). Pediatr-Med-Chir. 1996 Mar; 18(2): 201-5; ISSN:

0391-5387.

ITALY. The Authors present a patient with 18q- Syndrome in which lymphatic cell

karyotype could resume development of extrapyramidal degeneration signs before

they appeared. Severity range of phenotypic manifestations in the 18q- syndrome is

correlated with chromosomic breakpoint and with genetic background. Many

chromosome 18's distal arm genes have been mapped Myelin Basic Protein gene

(MBP) has been located in 22-23 position; it forms about 30-40% of myelinic

sheath proteins. Failure in MBP gene expression would be correlated in the central

white matter with extrapyramidal system degeneration signs: in 18q- patients with

involuntary movements studied by MRI or by post-mortem autopsy unmyelinated

areas in central white matter tracts have been put in evidence. As MBP absence in

peripheral nervous system does not appear to have a functional effect, it has been

suggested that some specific component of peripheral myelin is functionally

equivalent to MBP and capable to substitute this protein in its absence.. 0.

284. Igram, C. M.; Harris, M. B.; Dehne, R. Charcot spinal arthropathy in congenital

insensitivity to pain. Orthopedics. 1996 Mar; 19(3): 251-55; ISSN: 0147-7447.

UNITED-STATES.

285. Illarioshkin, S. N.; Tanaka, H.; Markova, E. D.; Nikolskaya, N. N.; Ivanova

Smolenskaya, I. A.; Tsuji, S. X-linked nonprogressive congenital cerebellar

hypoplasia: clinical description and mapping to chromosome Xq. Ann-Neurol.

1996 Jul; 40(1): 75-83; ISSN: 0364-5134.

UNITED-STATES. We examined a large family in which an X-linked recessive

congenital ataxia manifested in 7 males from three generations. The affected boys

first exhibited a marked delay of early developmental motor milestones. A

neurological syndrome became evident by 5 to 7 years of age and included

cerebellar ataxia, dysarthria, and external ophthalmoplegia; there were no

symptoms of mental retardation, spastic paraparesis, or sensory loss.

Neuroimaging studies revealed hypoplasia of cerebellar hemispheres and vermis.

The disease showed no progression beyond early childhood. The unique heredity

and clinical features clearly distinguish this new entity from a variety of previously

described familial ataxias. Pairwise linkage analysis and haplotype reconstruction

allowed us to map the gene responsible for this disorder to a 38-cM interval on

chromosome Xp11.21-q24 flanked by the loci DXS991 and DXS1001. Upon

multipoint linkage analysis, the disease gene was determined to be located most

likely in the proximal part of chromosome Xq, with the maximal lod score of 4.66

at the locus DXS1059 (Xq23). This is the first example of the genetic mapping of a

pure congenital cerebellar hypoplasia syndrome.

286. Incesu, L.; Bekir Selcuk, M.; Akan, H.; Senel, A.; Seyfullahi Mokaddem, V. Pineal

germinoma with cranial nerve metastasis. Radiol-Med-Torino. 1996 Apr; 91(4):

471-2; ISSN: 0033-8362.

ITALY.

287. Ing, E. B.; Leavitt, J. A.; Younge, B. R. Papilledema following bowtie optic atrophy.

Arch-Ophthalmol. 1996 Mar; 114(3): 356-7; ISSN: 0003-9950.

UNITED-STATES.

288. Inoue, K.; Hukuda, S.; Katsuura, A.; Saruhashi, Y. Alternating sciatica while jogging:

an early symptom of cauda equina tumor. Clin-Orthop. 1996 Jul; (328): 102-7;

ISSN: 0009-921X.

UNITED-STATES. Three athletic patients with cauda equina or lumbosacral cord

tumor noticed, as an early symptom of the disease, alternating bilateral sciatica

synchronized with each stride while jogging. Comparison with athletic patients

who developed lumbar disc hernia suggested that this symptom was significant.

The authors speculated that the mechanism producing this symptom is the inertial

force induced while jogging, which acts on the tumor in its early stage, when it is

still quite mobile in the intradural space. The diagnostic role of this symptom in

cauda equina and lumbosacral cord tumor should be recognized.

289. Irie, S.; Saito, T.; Nakamura, K.; Kanazawa, N.; Ogino, M.; Nukazawa, T.; Ito, H.;

Tamai, Y.; Kowa, H. Association of anti-GM2 antibodies in Guillain-Barre

syndrome with acute cytomegalovirus infection. J-Neuroimmunol. 1996 Aug;

68(1-2): 19-26; ISSN: 0165-5728.

NETHERLANDS. We examined serum anti-cytomegalovirus (CMV) and anti-

ganglioside antibodies by ELISA in 51 patients with Guillain-Barre syndrome

(GBS), and titers were compared with those from 47 normal and 74 disease

controls. Three GBS patients with IgM anti-CMV antibodies had high titers of IgM

and IgG anti-GM2 antibodies. The other GBS patients without IgM anti-CMV

antibodies, and the normal and disease controls except one of 6 non-GBS patients

with acute CMV infections had no anti-GM2 antibodies. The titers of anti-GM2

antibodies decreased on absorption with CMV-infected cells. These findings

suggest that anti-GM2 antibodies are associated with acute CMV infections in GBS

patients.. 0; 0; 0; 19600-01-2.

290. Isakov, A. P.; Broome, J. R.; Dutka, A. J. Acute carpal tunnel syndrome in a diver:

evidence of peripheral nervous system involvement in decompression illness. Ann-

Emerg-Med. 1996 Jul; 28(1): 90-3; ISSN: 0196-0644.

UNITED-STATES. Conclusive evidence for involvement of the peripheral

nervous system in decompression illness is lacking. We report a case of

decompression illness associated with shoulder pain and the clinical features of

median nerve injury at the wrist. Initial recompression and hyperbaric oxygen

treatment produced prompt relief of all symptoms and signs, but carpal tunnel

syndrome subsequently recurred. Nerve conduction studies confirmed median

nerve conduction delay at the wrist. Repeat measurements after treatment with

hyperbaric oxygen showed electrophysiologic improvement that was consistent

with improvement in symptoms. We believe this is the first objectively

substantiated case of injury to the peripheral nervous system caused by

decompression illness.

291. Ishii, K.; Takahashi, S.; Matsumoto, K.; Ishibashi, T.; Sakamoto, K.; Hashimoto, S.;

Katakura, R.; Ogawa, A.; Yuasa, R. Hemorrhage and abnormal veins in acoustic

neurinoma: MR findings. Radiat-Med. 1996 Mar; 14(2): 65-9; ISSN: 0288-2043.

JAPAN. We reviewed the MR imaging findings of 57 acoustic neurinomas which

were verified at surgery or diagnosed on the basis of neuroradiological and

neurootological data. Two uncommon MR findings of acoustic neurinoma were

found. First, hypointense areas were observed on T2-weighted images in five of

the 12 tumors larger than 25 mm in diameter. These hypointense areas represented

hemosiderin deposition secondary to occult intratumoral hemorrhage. Second,

curvilinear or round signal voids were noted at the periphery of 11 large or

medium-sized tumors, and these corresponded to "abnormal veins" seen on

angiographic studies.

292. Ishikawa, M.; Ohira, T.; Namiki, J.; Gotoh, K.; Takase, M.; Toya, S.

Electrophysiological investigation of hemifacial spasm: F-waves of the facial

muscles. Acta-Neurochir-Wien. 1996; 138(1): 24-32; ISSN: 0001-6268.

AUSTRIA. In patients with hemifacial spasm (HFS), the spasm is due to cross-

compression of the facial nerve by a blood vessel. There are currently two

hypotheses for the mechanism of HFS: 1) the spasm is caused by ephaptic

transmission and an increase in excitability at the site of compression; and 2) the

spasm is caused by hyperexcitability in the facial nerve nucleus. In peripheral

nerves, F-waves, which result from the backfiring of antidromically activated

anterior horn cells, have been proposed as indices of proximal motoneuron

conduction and anterior horn cell excitability. Enhancement of the F-waves

indicates increased anterior horn cell excitability. We have therefore measured F-

waves in the facial muscle of HFS patients in order to investigate the excitability of

the facial nerve nucleus. The authors obtained facial nerve evoked responses from

20 HFS patients before microvascular decompression (MVD), 10 HFS patients

after MVD and 10 healthy controls. The F-waves, obtained with surface electrodes

from the mentalis muscle, were the second response after the M-wave. On the

patient's spasm side, the F-wave duration, F/M amplitude ratio and frequency of F-

wave appearance significantly increased compared with those of the normal side or

healthy controls; minimum latency and chronodispersion did not significantly differ

between these groups. In patients whose spasm disappeared completely following

MVD, the abnormal muscle response (lateral spread), which is a characteristic sign

of HFS, and the enhancement of the F-wave eventually also disappeared. Because

of the correlation between HFS and F-waves, the authors' study supports the

hypothesis that the cause of HFS is hyperexcitability of the facial motonucleus.

293. Ishiura, Y.; Tsuji, H.; Takeda, S.; Takazakura, E.; Fujimura, M.; Matsuda, T.;

Kitagawa, M. Acute eosinophilic pneumonia accompanied by Guillain-Barre

syndrome. Br-J-Clin-Pract. 1996 Jul; 50(5): 283-5; ISSN: 0007-0947.

ENGLAND. A 74-year-old man presented with chest pain, dry cough, progressive

respiratory distress and infiltrative lung shadow. Diagnosis of acute eosinophilic

pneumonia was confirmed by histological examination as well as clinical features.

On the 8th day post-admission, he developed progressive generalised muscle

weakness that required mechanical ventilation. Clinical investigations revealed

features concurring with the accepted diagnostic criteria for Guillain-Barre

syndrome. Although precise aetiologies for the disorders suffered by this case were

unknown, a common cause of allergic nature was speculated. This is the first report

of acute eosinophilic pneumonia accompanied by Guillain-Barre syndrome.

294. Isono, M.; Murata, K.; Tanaka, H.; Kawamoto, M.; Azuma, H. An objective

evaluation method for facial mimic motion. Otolaryngol-Head-Neck-Surg. 1996

Jan; 114(1): 27-31; ISSN: 0194-5998.

UNITED-STATES. This technique was designed to establish a simple, objective

evaluation system for facial paralysis through the use of a personal computer. A

total of 24 marks were placed on the faces of subjects for the following procedures.

Movements of the face were photographed with a video-camera and fed

continuously into the computer. Ten frames per movement representing facial

movement from rest to maximum movement were selected for analysis. By means

of a digital image-processing technique, only the marks placed on the face were

extracted, and the movement of these marks was quantitatively analyzed. A total of

44 healthy subjects with no history of facial paralysis were used as a normal control

group. The patients with facial paralysis consisted of nine subjects with Bell's

palsy and three with Ramsay Hunt syndrome. In the eye-closing motions, no

significant differences were found between the sum of the movement distances on

the left and right sides in each normal subject. However, the patients with facial

paralysis showed distinct differences from those obtained in the normal subjects.

The improvement process was also evaluated with a ratio of affected- and normal-

side facial movements.

295. Isozaki, E.; Naito, A.; Horiguchi, S.; Kawamura, R.; Hayashida, T.; Tanabe, H.

Early diagnosis and stage classification of vocal cord abductor paralysis in patients

with multiple system atrophy. J-Neurol-Neurosurg-Psychiatry. 1996 Apr; 60(4):

399-402; ISSN: 0022-3050.

ENGLAND. OBJECTIVES: Vocal cord abductor paralysis (VCAP) is a life

threatening complication which may cause nocturnal sudden death in patients with

multiple system atrophy. However, the early diagnosis of VCAP is often difficult

to make on routine laryngoscopy performed during wakefulness, as stridor, which

is the sole symptom of VCAP in the early stage, develops only during sleep. The

aim was to investigate laryngeal dysfunction in patients with multiple system

atrophy while awake and asleep. METHODS: Seven patients with multiple system

atrophy with nocturnal stridor and five control patients were studied. Vocal cord

movement was analysed by laryngoscopy while the patients were awake and also

during sleep induced by intravenous diazepam. RESULTS: When awake, for the

seven patients with multiple system atrophy normal movement of the vocal cords

occurred in three, mild abduction restriction in three, and paradoxical movement in

one. When asleep, however, all showed obvious paradoxical movement with high

pitched inspiratory stridor. In controls, there were no differences in the vocal cord

movement between wakefulness and sleep. From these findings, VCAP could be

divided into four stages: stage 0 (normal) with normal vocal cord movement during

both wakefulness and sleep, stage 1 (mild VCAP) with normal movement during

wakefulness and paradoxical movement during sleep, stage 2 (moderately severe

VCAP) with abduction restriction during wakefulness and paradoxical movement

during sleep, and stage 3 (severe VCAP) with an almost midline position for the

vocal cords during both wakefulness and sleep. CONCLUSIONS: Laryngoscopy

during sleep can disclose subclinical VCAP, making an early diagnosis of VCAP in

patients with multiple system atrophy. Stage 2 of VCAP seems to be a suitable

stage for tracheostomy in patients with multiple system atrophy.

296. Ito, M.; Sonokawa, T.; Mishina, H.; Iizuka, Y.; Sato, K. Dural arteriovenous

malformation manifesting as tic douloureux. Surg-Neurol. 1996 Apr; 45(4): 370-5;

ISSN: 0090-3019.

UNITED-STATES. BACKGROUND: Although cerebral arteriovenous

malformation (AVM) is an established cause of trigeminal neuralgia, dural AVM

has rarely been reported to cause tic douloureux. Treatment of dural AVM in the

tentorial and torcular regions by transarterial embolization is rarely curative. It has

recently been proposed that many cranial dural AVMs with leptomeningeal venous

drainage require only interruption of the draining vein as it enters the subarachnoid

space for successful, lasting elimination. CASE REPORT: We present a case of 65-

year-old man with typical trigeminal neuralgia caused by a dural AVM. Carotid

angiography revealed a dural AVM in the petrotentorial region with enlarged and

serpiginous draining veins, which compressed the trigeminal nerve. Facial pain had

been completely relieved for 2 months after successful transarterial embolization,

but the symptom recurred. The patient subsequently underwent surgical

interruption of the draining veins just beneath the cerebellar tentorium. The dural

AVM disappeared, and the trigeminal neuralgia was completely relieved.

CONCLUSION: The literature concerning the etiology of tic douloureux is

reviewed and the selection of treatment modality of this case is discussed. The

striking clinical and radiologic improvement in this case emphasizes the pivotal role

of simple interruption of the arterialized vein for petrotentorial dural AVMs, which

are not amenable to cure by endovascular procedures.

297. Iyer, R. B.; Fenstermacher, M. J.; Libshitz, H. I. MR imaging of the treated brachial

plexus. AJR-Am-J-Roentgenol. 1996 Jul; 167(1): 225-9; ISSN: 0361-803X.

UNITED-STATES.

298. Jackson, D. L.; Hynninen, B. C.; Caborn, D. N.; McLean, J. Electrodiagnostic study

of carpal tunnel syndrome in wheelchair basketball players. Clin-J-Sport-Med.

1996 Jan; 6(1): 27-31; ISSN: 1050-642X.

UNITED-STATES. OBJECTIVE-DESIGN-SUBJECTS: Compression

neuropathies are common injuries about the wrist in wheelchair athletes. Thirty-

three world-class wheelchair basketball players were studied electrodiagnostically

to determine the prevalence and severity of median neuropathy at the wrist in these

athletes. RESULTS: Thirty percent of these athletes had symptoms consistent with

carpal tunnel syndrome (CTS), and 70% of these had electrodiagnostic

confirmation of this injury. Overall, 52% of the 33 athletes had electrodiagnostic

findings of median neuropathy at the wrist with nine athletes (27%) exhibiting

bilateral abnormalities. Four athletes (12%) had abnormal electrodiagnostic findings

involving the ulnar nerve at the wrist. CONCLUSIONS: This prevalence of CTS in

wheelchair basketball players appears to be similar to that found in the general

paraplegic population. Early recognition and treatment of CTS in these athletes are

recommended to avoid chronic problems.

299. Jacobs, B. C.; van Doorn, P. A.; Schmitz, P. I.; Tio Gillen, A. P.; Herbrink, P.;

Visser, L. H.; Hooijkass, H.; van, der Meche FG. Campylobacter jejuni infections

and anti-GM1 antibodies in Guillain-Barre syndrome. Ann-Neurol. 1996 Aug;

40(2): 181-7; ISSN: 0364-5134.

UNITED-STATES. The group of patients with Guillain-Barr'e syndrome (GBS)

is very heterogenous with regard to antecedent infections, immunological

parameters, clinical manifestations, and response to treatment. In this study, the

presumed pathogenic factors anti-GM1 antibodies and Campylobacter jejuni

infections were related to the clinical characteristics. Serum from 154 patients with

GBS, 63 patients with other neurological diseases (OND), and 50 normal controls

(NC) were tested for the presence of antibodies against GM1 and C. jejuni. Anti-

GM1 antibodies were detected in 31 (20%) GBS patients, 5 (8%) OND patients,

and in none of the NC. Evidence for a recent C. jejuni infection was found in 49

(32%) GBS patients and less often in OND patients (11%) or NC (8%). In GBS

patients, the presence of anti-GM1 antibodies was significantly associated with C.

jejuni infections. The subgroup of GBS patients with anti-GM1 antibodies suffered

more often from a rapidly progressive and more severe neuropathy with

predominantly distal distribution of weakness, without deficits of cranial nerves or

sensory disturbances. The subgroup with C. jejuni infection also more often had a

severe pure motor variant of GBS. Recovery of the patients with anti-GM1

antibodies and C. jejuni infections was not as good after plasma exchange

compared with intravenous immunoglobulins.. 0; 0; 0; 0; 37758-47-7.

300. Jacobson, D. M. Progressive ophthalmoplegia with acute ischemic abducens nerve

palsies. Am-J-Ophthalmol. 1996 Aug; 122(2): 278-9; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To determine the frequency of early progression

of opthalmoplegia in patients with acute ischemic abducens nerve palsies.

METHODS: Patients with ischemic abducens nerve palsy examined within one

week from the onset of symptoms were identified using predetermined entry criteria

and serially followed up using a standardized ophthalmoplegia grading scheme.

RESULTS: Only two of the 35 patients initially had a complete abduction deficit.

Of the 33 patients who initially had an incomplete abduction deficit, 18 (54%)

showed progression of their ophthalmoplegia. CONCLUSIONS: Patients with

acute incomplete ischemic abducens nerve palsies frequently demonstrate early

progression of their ophthalmoplegia.

301. James, D. G. All that palsies is not Bell's. J-R-Soc-Med. 1996 Apr; 89(4): 184-7;

ISSN: 0141-0768.

ENGLAND.

302. Jamieson, W. G.; Chinnick, B. Thoracic outlet syndrome: fact or fancy? A review of

409 consecutive patients who underwent operation. Can-J-Surg. 1996 Aug; 39(4):

321-6; ISSN: 0008-428X.

CANADA. OBJECTIVES: From an analysis of patients operated on by a single

surgeon between 1973 and 1994 for thoracic outlet syndrome (TOS), to determine

what findings are helpful in substantiating this diagnosis and what are the results of

decompressive thoracic outlet surgery in the management of TOS. DESIGN: A

chart review with emphasis on signs, symptoms and results. A preoperative

questionnaire was presented to all patients operated on since 1989. Postoperative

follow-up was by interview or telephone. SETTING: A 560-bed university-

affiliated tertiary-care teaching hospital. PATIENTS: There were 409 adults; 83%

were women and the average age was 36 years. In 95% of patients physiotherapy

had been unsuccessful. Patients presented with neurologic type (368 [(90%]),

arterial impingement (29 [7%]) and venous obstructive symptoms (12 [3%]). The

series included 44 patients with bilateral symptoms and 26 patients with cervical

ribs. Some form of litigation or compensation was associated with 177 patients.

INTERVENTIONS: Transaxillary first rib resection in 380 (93%) patients and

supraclavicular thoracic outlet decompression in 29 (7%). In 244 patients there was

a follow-up of at least 2 years. These patients constituted the follow-up group.

RESULTS: Preoperatively, in the 368 patients who had neurologic TOS, 99%

displayed supraclavicular brachial plexus tenderness and 98% exacerbation of

symptoms with arms in the abduction external rotated position. There were eight

complications of surgical intervention (1.9%). In the follow-up group, there were

no deaths, no subclavian/axillary artery or vein damage and no brachial plexus

injury. Seventy-eight percent of the patients with neurologic TOS in this group

improved postoperatively; 21% had complete relief, 32% had good relief and 25%

had fair relief. Twenty-two percent showed no improvement. CONCLUSIONS:

Signs and symptoms helpful in making the diagnosis of neurologic TOS are

supraclavicular tenderness on palpation and exacerbation of symptoms with the

arms in the abducted external rotated position. The surgical procedures are safe.

Patients with TOS refractory to medical management can benefit from thoracic

outlet decompression.

303. Jan, J. E.; Good, W. V.; Lyons, C. J.; Hertle, R. W. Visually impaired children with

sensory defect nystagmus, normal appearing fundi and normal ERGS. Dev-Med-

Child-Neurol. 1996 Jan; 38(1): 74-80; ISSN: 0012-1622.

ENGLAND. The differentiation of congenital motor nystagmus (CMN) from

sensory defect nystagmus (SDN) in early life is a common diagnostic problem,

since their wave forms are indistinguishable. A diagnosis of SDN is made when

fundal or ERG abnormalities are present. CMN is generally diagnosed by

exclusion. This approach can lead to late or incorrect diagnosis, and to the

overlooking of important genetic implications. In addition, the classification of

congenital nystagmus into pure motor and sensory types is inadequate. The authors

present case reports illustrating this problem and suggest strict criteria for a

diagnosis of CMN. They also point out that a miscellaneous group of rare disorders

of the macula tend to be overlooked and misdiagnosed.

304. Jander, S.; Pohl, J.; Gillen, C.; Stoll, G. Differential expression of interleukin-10

mRNA in Wallerian degeneration and immune-mediated inflammation of the rat

peripheral nervous system. J-Neurosci-Res. 1996 Jan 15; 43(2): 254-9; ISSN:

0360-4012.

UNITED-STATES. Interleukin-10 (IL-10) is a potent immunosuppressant

cytokine which downregulates MHC class II antigen expression and inflammatory

cytokine production. In this study we localized mRNA for IL-10 in the rat

peripheral nervous system (PNS) by nonradioactive in situ hybridization using a

digoxygenin-labeled riboprobe specific for rat IL-10. IL-10 mRNA was expressed

by some Schwann cells (SCs) in the normal sciatic nerve. During Wallerian

degeneration, SCs strongly expressed IL-10 mRNA between days 2 and 4 after

transection. By day 14 only occasional cells were positive for IL-10 mRNA. The

vast majority of ED1-positive macrophages were IL-10 negative after axotomy.

Contrastingly, infiltrating macrophages expressed IL-10 mRNA conincident with

beginning clinical recovery in experimental autoimmune neuritis (EAN), the rat

model of human Guillain-Barre syndrome. Our data suggest that SCs provide a

constitutive immunosuppressant system in the PNS. In EAN additional

macrophage-derived IL-10 may be important for the resolution of the T cell-

mediated immune response.. 0; 0; 130068-27-8.

305. Jardine, E.; Wallis, C. Home ventilation of a child with motor and sensory neuropathy.

Study aims to set up national database of children receiving ventilatory support

[letter]. BMJ. 1996 Oct 12; 313(7062): 940-1; ISSN: 0959-8138.

ENGLAND.

306. Jaster, J. H.; Bertorini, T. E.; Dohan, FC Jr; O'Brien, T. F.; Wang, H.; Becske, T.;

Menke, P. G.; Handorf, C. R.; Horner, L. H.; Monkemuller, K. E. Solitary focal

demyelination in the brain as a paraneoplastic disorder. Med-Pediatr-Oncol. 1996

Feb; 26(2): 111-5; ISSN: 0098-1532.

UNITED-STATES. Solitary focal demyelination (SFD) in the brain is an

uncommon and poorly understood disorder of uncertain etiology that may represent

an intermediate entity between multiple sclerosis and acute disseminated

encephalomyelitis. In a few reported cases of SFD, the patient was briefly noted to

have a nonneurological malignancy. We studied two patients who had solitary focal

lesions in the brain. Utilizing magnetic resonance imaging and tissue biopsy, we

found the characteristics of the brain lesions in these two patients to be those of

SFD. In our combined experience over the past 10 years, we have encountered no

similar brain lesions at our medical center. We found it remarkable that both of

these patients also had malignancy outside of the nervous system. One had a

seminoma, and the other a lymphoma. We conclude that some cases of SFD in the

brain may occur as a paraneoplastic disorder associated with nonneurological

malignancies.

307. Jepsen, J. R. [Supinator syndrome (letter)]. Supinatorlogesyndrom. Ugeskr-Laeger.

1996 Apr 15; 158(16): 2275; ISSN: 0041-5782.

DENMARK.

308. Jespersen, J. H.; Dupont, E.; Gelineck, J.; Lundorf, E. Hemifacial spasm: magnetic

resonance angiography. Acta-Neurol-Scand. 1996 Jan; 93(1): 35-8; ISSN: 0001-

6314.

DENMARK. Twenty-three consecutive patients with hemifacial spasm were

studied. Magnetic resonance imaging angiography of the brain was performed in 20

patients and 15 controls. The angiograms were evaluated by two independent

observers and blinded for side-location of the spasm. Contact between an artery

from the vertebrobasilar circulation and the intracranial part of the facial nerve was

observed ipsilaterally to the spasm in 17 patients (85%) and in two of 30 control

half-brains (7%), respectively. Treatment is discussed. The study confirms that

arterial relation to the facial nerve root is the most frequent cause of hemifacial

spasm. Magnetic resonance imaging is recommended to exclude mass lesions in the

posterior cranial fossa, and magnetic resonance angiography is recommended in

preoperative evaluation and in research.

309. Jinkins, J. R.; Athale, S.; Xiong, L.; Yuh, W. T.; Rothman, M. I.; Nguyen, P. T. MR

of optic papilla protrusion in patients with high intracranial pressure. AJNR-Am-J-

Neuroradiol. 1996 Apr; 17(4): 665-8; ISSN: 0195-6108.

UNITED-STATES. PURPOSE: To evaluate the signal characteristics of the optic

papilla (optic nerve head) on routine cranial MR images in patients with clinical

evidence of optic papilla elevation caused by high intracranial pressure, and to

compare these findings with findings in healthy adult volunteers. METHODS: We

reviewed retropectively the MR imaging examinations of 15 patients who were

referred with objectively decreased visual acuity and funduscopic findings of optic

papilla elevation. T1-weighted and T2-weighted axial MR images were obtained by

using conventional spinecho acquisitions on 1.5-T MR imagers. In addition, the

MR imaging studies in 10 healthy adult volunteers without visual impairment were

reviewed as controls. RESULTS: In 10 (67%) of the 15 patients, visual elevation

of the optic papilla was shown by MR imaging. In all 15 patients, the MR signal

intensity of the optic papilla was hypointense relative to the vitreous of the globe on

T2-weighted images. In the healthy volunteer group, the optic papillae were all

similarly hypointense relative to the vitreous of the globe on T2-weighted images;

however, these optic papillae were flat. CONCLUSION: Clinical examination and

MR imaging may show elevation of the optic papilla in patients with high

intracranial pressure. When chronic, optic papilla elevation has been shown to

correlate well with severe loss of vision. Actual edema of the optic papilla seems to

play little role in the physical elevation observed clinically in the chronic stages of

this pathologic process.

310. Johnson, J. E.; Kennedy, E. J.; Shereff, M. J.; Patel, N. C.; Collier, B. D.

Prospective study of bone, indium-111-labeled white blood cell, and gallium-67

scanning for the evaluation of osteomyelitis in the diabetic foot. Foot-Ankle-Int.

1996 Jan; 17(1): 10-6; ISSN: 1071-1007.

UNITED-STATES. Twenty-two adult diabetic patients with clinical suspicion of

foot and/or ankle infection were prospectively evaluated using radiography,

technetium-99m methylene diphosphonate bone scanning (99mTc), indium-111-

labeled leukocyte scanning (111In), and gallium-67 scanning (67Ga) to determine

the presence of clinically suspected osteomyelitis. Biopsy for culture and histology

was performed in 16 patients. The diagnosis of osteomyelitis was confirmed by

biopsy in 12 patients. The remaining 10 patients had no evidence of osteomyelitis

with long-term follow-up. 99mTc was shown to be of limited valued when used

alone in these patients with peripheral neuropathy. 67Ga, either alone or in

combination with 99mTc bone scanning, was of little diagnostic value and gave no

additional information that was not available from 111In. The combination of three-

phase 99mTc and 111In had the highest diagnostic efficacy (100% sensitivity, 80%

specificity, and 91% accuracy), followed closely by 111In alone (100% sensitivity,

70% specificity, and 86% accuracy). We conclude that for adult diabetic patients

with clinical suspicion of osteomyelitis but no radiographic findings of that disease,

111In alone is an appropriate nuclear medicine evaluation for ruling out infection if

it is negative. However, if an area of 111In white blood cell uptake is present, a

"simultaneous" 99mTc is often helpful in providing the anatomic correlation to

differentiate osteomyelitis from infection that is limited to soft tissue.. 0; 0.

311. Johnson, L. N.; Gould, T. J.; Krohel, G. B. Effect of levodopa and carbidopa on

recovery of visual function in patients with nonarteritic anterior ischemic optic

neuropathy of longer than six months' duration. Am-J-Ophthalmol. 1996 Jan;

121(1): 77-83; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: We conducted a pilot clinical trial to determine the

efficacy of levodopa in promoting visual recovery in eyes with nonarteritic anterior

ischemic optic neuropathy of greater than six months' duration. METHODS: This

prospective, randomized, double-masked, placebo-controlled clinical trial involved

20 subjects with nonarteritic anterior ischemic optic neuropathy of 30 months'

mean duration. Subjects were randomly assigned to receive either low-dose

levodopa and carbidopa or a placebo for three weeks. At 12 weeks after the

baseline visit, the levodopa group then was provided a higher, conventional dose of

levodopa and carbidopa for three more weeks. Change in visual function was

monitored at four, 12, 16, and 24 weeks after the baseline visit. RESULTS: At 12

weeks after the baseline visit, the levodopa group experienced a significant (P =

.016) mean difference in improvement of visual acuity of 5.9 letters from the

placebo group. At 24 weeks after the baseline visit, a significant treatment effect (P

= .036) for visual acuity was still evident; the levodopa group had a mean gain in

improvement of 7.5 letters difference from baseline from the placebo group. Three

subjects in the levodopa group experienced a doubling of the visual angle as

denoted by a gain of at least 15 letters. Significant improvement was not observed

for color vision (P = .82) or mean deviation of visual field loss (P = .82).

CONCLUSION: The study found significant improvement of visual acuity among

subjects receiving levodopa and carbidopa despite long-standing visual loss from

nonarteritic anterior ischemic neuropathy. Confirmation of our results is awaited

from larger population studies and with a longer follow-up time interval regarding

the efficacy of levodopa in reversing visual loss in this disease.. 0; 0; 38821-49-7.

312. Jonas, J. B.; Dichtl, A. Evaluation of the retinal nerve fiber layer. Surv-Ophthalmol.

1996 Mar; 40(5): 369-78; ISSN: 0039-6257.

UNITED-STATES. In normal eyes, the retinal nerve fiber layer (RNFL) is usually

best visible in the inferior temporal part of the fundus, followed by the superior

temporal region, the nasal superior region and the nasal inferior region. This

distribution correlates with the configuration of the neuroretinal rim, the diameter of

the retinal arterioles, the location of the foveola, and the lamina cribrosa

morphology. With increasing age, the RNFL visibility decreases diffusely without

preferring special fundus regions and without the development of localized defects.

With all optic nerve diseases, the visibility of the RNFL is decreased in addition to

the age-related loss, in a diffuse and/or a localized manner. The localized defects are

wedge-shaped and not spindle-like defects, running toward or touching the optic

disk border. Typically occurring in about 20% of all glaucoma eyes, they can be

found also in other ocular diseases, such as optic disk drusen, toxoplasmotic

retinochoroidal scars, longstanding papilledema or optic neuritis due to multiple

sclerosis. Since they are not present in normal eyes, they almost always signify an

abnormality. RNFL evaluation is especially helpful for early glaucoma diagnosis

and in glaucoma eyes with small optic disks. In advanced optic nerve atrophy,

other examination techniques, such as perimetry, may be more helpful for

following optic nerve damage. Considering its great importance in the assessment

of optic nerve anomalies and diseases and taking into account the feasibility of its

ophthalmoscopic evaluation using green light, the retinal nerve fiber layer should be

examined during any routine ophthalmoscopy.

313. Jonas, J. B.; Dichtl, A. [Ophthalmoscopic study of the retinal nerve fiber layer].

Ophthalmoskopische Untersuchung der retinalen Nervenfaserschicht. Klin-

Monatsbl-Augenheilkd. 1996 Feb; 208(2): aA3-9; ISSN: 0023-2165.

GERMANY.

314. Jones, C. M. ABC of work related disorders. Occupational hearing loss and vibration

induced disorders. BMJ. 1996 Jul 27; 313(7051): 223-6; ISSN: 0959-8138.

ENGLAND.

315. Jones, H. R. Childhood Guillain-Barre syndrome: clinical presentation, diagnosis, and

therapy. J-Child-Neurol. 1996 Jan; 11(1): 4-12; ISSN: 0883-0738.

UNITED-STATES. A rapidly progressive, generally symmetric, ascending flaccid

paraparesis or quadriparesis that develops in an infant or child constitutes an

uncommon but important pediatric neurologic emergency that requires immediate

evaluation and treatment. The differential diagnosis primarily includes acute

neuropathies, most commonly the childhood Guillain-Barre syndrome and, rarely,

acute transverse myelitis or infantile poliomyelitis. A clinical distinction may be

difficult in the younger child in whom detailed sensory examination is not possible.

Although most children with Guillain-Barre syndrome usually have a benign and

relatively limited clinical illness, some become severely ill, requiring intubation and

careful intensive monitoring. To date, no well-controlled multi-institutional studies

of treatment with either plasmapheresis or intravenously administered

immunoglobulin have been developed in children despite the success of these

modalities in adults. A review of the data available using these therapies is included

in this study.

316. Joos, U. [Therapy of orbital fractures]. Therapie der Orbitafrakturen. Fortschr-Kiefer-

Gesichtschir. 1996; 41: 32-8; ISSN: 0071-7916.

GERMANY.

317. Jung, S.; Toyka, K.; Hartung, H. P. T cell directed immunotherapy of inflammatory

demyelination in the peripheral nervous system. Potent suppression of the effector

phase of experimental autoimmune neuritis by anti-CD2 antibodies. Brain. 1996

Aug; 119( Pt 4): 1079-90; ISSN: 0006-8950.

ENGLAND. Experimental autoimmune neuritis (EAN) of Lewis rats, an

inflammatory demyelinating neuropathy and model of the human Guillain-Barre

syndrome (GBS), was used to evaluate the novel T cell directed immunotherapy

with the anti-CD2 monoclonal antibody (mAb) OX34. Clinical signs of EAN

actively induced by immunization with bovine peripheral nerve myelin in complete

Freund's adjuvant (CFA) were totally prevented or markedly suppressed by

preventative injections of OX34 starting 8 days post-immunization (p.i.).

Moreover, therapeutic application of the mAb beginning on the day of first clinical

signs of EAN markedly inhibited progression of disease. Electrophysiological and

histological investigation of sciatic nerves 17 and 18 days p.i. respectively, also

revealed an inhibitory effect of OX34 on EAN-associated functional and

morphological nerve damage. Similarly, therapeutic injections of OX34 after onset

of EAN actively induced by immunization with a neuritogenic peptide of the P2

protein completely halted further deterioration of clinical disease. Finally, clinical,

electrophysiological and histological signs of adoptive transfer EAN mediated by

injection of neuritogenic T helper line cells were prevented or strongly suppressed

by OX34-application on the day of cell transfer and 4 days later, underlying the

impact of the mAb on the effector phase of the disease. Since the anti-CD2 mAb did

not exert its effect by inhibition of T cell activation, induction of anergy,

modulation of CD2 antigens, or by T cell depletion, we assume that it may affect

migration of T lymphocytes across the blood-nerve barrier. The immediate and

marked suppression of ongoing EAN by the mAb lead to the recommendation of

anti-CD2 mAbs as candidates for T cell directed immunotherapy of the GBS.

318. Kahn, C. B. Expanding zoster's differential diagnosis [letter]. Hosp-Pract-Off-Ed.

1996 Oct 15; 31(10): 42; ISSN: 8750-2836.

UNITED-STATES.

319. Kaiser, H. J. [Assessment of vision disorders using color duplex ultrasonography].

Abklarung von Sehstorungen mit der Farbduplexsonographie. Ther-Umsch. 1996

Jan; 53(1): 43-8; ISSN: 0040-5930.

SWITZERLAND. Disturbed circulation is a common cause of visual impairment

which time course and severity can vary. Prompt diagnosis and therapy are

mandatory to restore visual function in cases of acute drop of vision. Color Doppler

imaging is a noninvasive method to investigate blood-flow velocity. During the past

years this method has been introduced into ophthalmology. For the first time it is

possible to measure the blood-flow velocity in the ophthalmic artery, the central

retinal artery and vein as well as the ciliary arteries to quantify the extent of

impaired ocular circulation.

320. Kamal, A. Assessment of autonomic function using complex demodulation and posture

entrainment techniques: an application to normal subjects and diabetic patients.

Front-Med-Biol-Eng. 1996; 7(1): 1-10; ISSN: 0921-3775.

NETHERLANDS. The assessment of autonomic nervous systems (sympathetic

and parasympathetic) using the method of complex demodulation and posture

entrainment are presented. The heart rate variability signals were derived from the

ECG of normal subjects and diabetic patients in both supine and standing positions.

The method of complex demodulation is employed on the heart rate variability data

for both groups. Two significant bands in the heart rate variability spectrum were

investigated by this technique. The amplitude of the high frequency band (0.181-

0.4 Hz) showed the parasympathetic changes in normal subjects and, to a lesser

extent, in the diabetic patients. The amplitude of the low frequency band (0.03-0.15

Hz) showed the influence of sympathetic changes in both groups. The complex

demodulation method indicated the effect of high frequency and low frequency

bands on both divisions of autonomic function in regulating the heart rate variability

in both normal subjects and diabetic patients. In the supine position, the

parasympathetic and sympathetic were balanced. On standing (posture

entrainment), the high frequency band showed a decrease in amplitude due to

decreased parasympathetic activity, while the low frequency band showed an

increase in amplitude, which indicated an increase in sympathetic activity. These

patterns were repeated in both normal subjects and diabetic patients. It seems that

the use of posture entrainment combined with the complex demodulation method is

significant to characterize, assess and quantify the autonomic function in health and

disease.

321. Kameyama, S.; Tanaka, R.; Kawaguchi, T.; Honda, Y.; Yamazaki, H.; Hasegawa, A.

Long-term follow-up of the residual intracanalicular tumours after subtotal removal

of acoustic neurinomas. Acta-Neurochir-Wien. 1996; 138(2): 206-9; ISSN: 0001-

6268.

AUSTRIA. We examined growth potential of residual intracanalicular tumours left

from subtotal removal of large acoustic neurinomas. Eleven patients were followed-

up by magnetic resonance (MR) imaging. The interval between surgery and MR

study ranged from 12 to 29 years (median, 16 years). MR images of two patients

showed no evidence of tumour remnant, and in six a small tumour was localized in

the internal auditory canal. The other three showed an intracanalicular tumour

protruding slightly towards the intracranial portion. This result suggests that the

intracanalicular residual tumours have less risk of regrowth after subtotal removal

of acoustic neurinomas. It is advisable to choose intracapsular, subtotal removal

without opening the internal auditory canal in the treatment of acoustic neurinoma,

if it is large in size and there is a high risk of nerve injury.

322. Kani, K. [Optic nerve diseases in children]. No-To-Hattatsu. 1996 May; 28(3): 191-8;

ISSN: 0029-0831.

JAPAN. The optic nerve is a part of the white matter in the central nervous system

and can easily be observed with a funduscope. Neuro-ophthalmologic studies of

the optic nerve is useful in monitoring disorders in the central nervous system. In

this paper we presented four cases of optic nerve diseases and discussed them from

the standpoint of neuro-ophthalmology. Case 1, a 9-year-old girl, had left

retrobulbar optic neuritis and right papillitis which occurred one year after the onset

of left optic neuritis. She was treated with a three day course of methylprednisolone

(500 mg). Her left optic disc became atrophic, but her right optic disc became

normal. Her visual acuity recovered to 1.0 in each eye. Relative afferent pupillary

defect was seen in her left eye. Steroid therapy for optic neuritis and pupillary light

reaction were discussed. Case 2 was a 7-year-old girl with teratoma in the chiasmal

region, and case 3 was a 10-year-old boy with craniopharyngioma. Visual field

changes caused by tumors in the chiasmal region were discussed. Case 4 was a 7-

year-old boy with right optic atrophy and left papilledema, an example of Foster

Kennedy syndrome. The effects of papilledema on visual function was discussed.

323. Kanzaki, J. [Imaging and functional diagnosis of acoustic neuroma (CT, MRI, ABR)].

Nippon-Jibiinkoka-Gakkai-Kaiho. 1996 May; 99(5): 706-9; ISSN: 0030-6622.

JAPAN.

324. Karlberg, M.; Johansson, R.; Magnusson, M.; Fransson, P. A. Dizziness of suspected

cervical origin distinguished by posturographic assessment of human postural

dynamics. J-Vestib-Res. 1996 Jan; 6(1): 37-47; ISSN: 0957-4271.

UNITED-STATES. Useful clinical tests are lacking for the controversial entity

"cervical vertigo". In earlier studies patients assumed to suffer from cervical vertigo

or dizziness manifested disturbed postural control as compared to healthy subjects,

but were hard to distinguish from patients with other balance disorders. Using

posturography in which stance was perturbed by a vibratory stimulus applied

towards the calf muscles, we studied 16 consecutive patients with recent onset of

neck pain and concomitant complaints of vertigo or dizziness, but normal findings

at otoneurological examination and electronystagmography; 18 patients with recent

vestibular neuritis; and 17 healthy subjects. We performed system identification of

a model of the control of upright human stance, using the vibratory stimulus as

input and the recorded body sway as output. According to values for the three

normalized parameters of the transfer function of the model (i.e., swiftness,

stiffness, and damping), cervical vertigo patients were distinguished both from

healthy subjects (P < 0.001), and from vestibular neuritis patients (P < 0.001). It

was also possible to distinguish the vestibular neuritis group from the group of

healthy subjects (P < 0.01). The results show disturbed postural control in patients

with cervical vertigo to differ from that in patients with recent vestibular neuritis,

and indicate posturographic assessment of human posture dynamics to be a

possible future tool for use in diagnosing cervical vertigo.

325. Karlsen, B.; Vedeler, C. [Guillain-Barre syndrome. Variation on the theme]. Guillain-

Barres syndrom. Tema med variasjoner. Tidsskr-Nor-Laegeforen. 1996 Jan 20;

116(2): 242-5; ISSN: 0029-2001.

NORWAY. Guillain-Barre syndrome, or acute inflammatory demyelinating

polyneuropathy, is a frequent cause of acute onset of flaccid paresis and areflexia.

Electrophysiological studies show demyelination, sometimes with varying degrees

of axonal degeneration. In some cases axonal degeneration apparently develops

rapidly, without signs of primary demyelination. However, it is still a matter of

discussion whether a pure axonal form of Guillain-Barre syndrome exists, or

whether the axonal degeneration is secondary to demyelination. We report on

clinical and electrophysiological findings in three patients with variants of Guillain-

Barre syndrome. These include pure demyelination, combined demyelination and

axonal degeneration and possible primary axonal degeneration.

Electrophysiological studies can differentiate between the variants of Guillain-Barre

syndrome, and thus give indications of pathogenesis and prognosis.

326. Kartush, J. M.; Brackmann, D. E. Acoustic neuroma update. Otolaryngol-Clin-North-

Am. 1996 Jun; 29(3): 377-92; ISSN: 0030-6665.

UNITED-STATES. We have described refinements and alternative techniques in

the management of acoustic neuromas. The outcome for patients with both

unilateral and bilateral tumors continues to improve.

327. Kasdan, M. L.; Vender, M. I.; Lewis, K.; Stallings, S. P.; Melhorn, J. M. Carpal

tunnel syndrome. Effects of litigation on utilization of health care and physician

workload. J-Ky-Med-Assoc. 1996 Jul; 94(7): 287-90; ISSN: 0023-0294.

UNITED-STATES. We performed a study consisting of two parts to investigate

the impact of litigation on patient recovery and physician workload. We received

556 replies from a questionnaire sent to hand surgeons and discovered that 98.20%

of them felt that litigation increased the subjective complaints of patients. Most of

these physicians (89.75%) also felt that litigation led to a worse result from

treatment. Second, we undertook a retrospective chart review of 447 patients to see

if there was a correlation between litigation, patient utilization of health care and

physician workload. We found that workers' compensation patients with pending

litigation went to the doctor's office more. They also had more letters, phone calls,

and forms associated with their care, had more nerve conduction studies

performed, and took longer to be discharged from care than patients with non-

work-related carpal tunnel syndrome as well as workers' compensation patients

who did not have pending litigation. These results indicated that litigation does

affect patient utilization of health care and increases the workload on the physician.

328. Katoulis, E. C.; Boulton, A. J.; Raptis, S. A. The role of diabetic neuropathy and high

plantar pressures in the pathogenesis of foot ulceration. Horm-Metab-Res. 1996

Apr; 28(4): 159-64; ISSN: 0018-5043.

GERMANY. Diabetic foot ulceration is currently a serious medical problem and

has, therefore, attracted much research attention during the last two decades.

Previous foot ulceration, diabetic neuropathy, limited joint mobility, high plantar

pressures, microangiopathy, macroangiopathy and diabetic nephropathy have

already been identified as risk factors for future foot ulceration. Neuropathy has

clearly been shown to be an essential permissive factor in the development of

ulceration in the non-ischaemic foot. Moreover, the pathogenetic role of high

plantar pressures is crucial in the presence of established clinical neuropathy.

Nowadays, our therapeutic efforts clearly aim to prevent than treat foot ulcers. This

demands specialist and team work in the setting up of a diabetic foot clinic in an

attempt to identify and educate the diabetic patients at risk and, where possible to

use suitable plantar pressure-reducing systems (footwear, hosiery etc.). Then only

would it be reasonable to postulate that a significant reduction in amputations of

diabetic aetiology could be achieved in the near future.

329. Katz, J. N.; Punnett, L.; Simmons, B. P.; Fossel, A. H.; Mooney, N.; Keller, R. B.

Workers' compensation recipients with carpal tunnel syndrome: the validity of self-

reported health measures. Am-J-Public-Health. 1996 Jan; 86(1): 52-6; ISSN:

0090-0036.

UNITED-STATES. OBJECTIVES. This study compared the reliability, validity,

and responsiveness of self-reported measures of health related quality of life in

recipients and non-recipients of workers' compensation who have carpal tunnel

syndrome. METHODS. Patients with carpal tunnel syndrome complete

questionnaires at study enrollment and 6 months later scales measuring symptom

severity functional status, and satisfaction were included. The scales internal

consistency, validity, and responsiveness were assessed. RESULTS. The internal

consistencies for each scale were high (Cronbach's alpha .88 to .96) and virtually

identical in recipients and nonrecipients of workers' compensation. The correlations

between self-reported and objectively measured grip strength were .32 in recipients

and .30 in nonrecipients; these correlations were not influenced by whether

workers' compensation recipients were out of work. Correlations between changes

in scale scores and three indicators of perceived improvement were higher in

recipients (.48 to .69) than in nonrecipients (.19 to .41) CONCLUSIONS. The

reliability, validity, and responsiveness of these measures were comparable in

nonrecipients and recipients of workers compensation, these data support the use of

self-report measures in studies of workers.

330. Kaufman, M. A. Differential diagnosis and pitfalls in electrodiagnostic studies and

special tests for diagnosing compressive neuropathies. Orthop-Clin-North-Am.

1996 Apr; 27(2): 245-52; ISSN: 0030-5898.

UNITED-STATES. The differential diagnosis of compressive neuropathies in the

arms includes syndromes involving the nerve roots and brachial plexus, as well as

the peripheral nerves. Often these conditions coexist. Nerve conduction velocity

studies as well as electromyography have a role along with the clinical evaluation in

differentiating these conditions. Limitations in routine electrodiagnostic testing are

present, which necessitate several specialized techniques for identifying

compressive neuropathies.

331. Kawasaki, S.; Hashikura, Y.; Matsunami, H.; Ikegami, T.; Nakazawa, Y.; Watanabe,

M.; Iijima, S.; Makuuchi, M. Temporary shunt between right portal vein and vena

cava in living related liver transplantation. J-Am-Coll-Surg. 1996 Jul; 183(1): 74-6;

ISSN: 1072-7515.

UNITED-STATES. 372-75-8.

332. Keane, J. R. Twelfth-nerve palsy. Analysis of 100 cases. Arch-Neurol. 1996 Jun;

53(6): 561-6; ISSN: 0003-9942.

UNITED-STATES. OBJECTIVE: To describe the causes and characteristics of

hypoglossal nerve palsy. DESIGN: A review of 26 years of personal experience in

a large public hospital. RESULTS: Twelfth-nerve palsies usually appear as signs

rather than symptoms. Tumors, predominantly malignant, produced nearly half of

the palsies (49 cases), while gunshot wounds made trauma (12) the second most

common cause. Stroke (6), hysteria (6), multiple sclerosis (6), surgery (5),

Guillain-Barre neuropathy (4), and infection (4) together accounted for about one

third of the patients. CONCLUSION: Twelfth-nerve palsy proved to be an

ominous sign, with only 15% of patients experiencing complete or nearly complete

recovery.

333. Kelkar, P.; Ross, M. A.; Murray, J. Mononeuropathy multiplex associated with celiac

sprue. Muscle-Nerve. 1996 Feb; 19(2): 234-6; ISSN: 0148-639X.

UNITED-STATES.

334. Kelly, P.; Staunton, H.; Lawler, M.; Brennan, P.; Jennings, S.; Unger, E. R.; Sung,

J. H.; Farrell, M. A. Multifocal remitting-relapsing cerebral demyelination twenty

years following allogeneic bone marrow transplantation. J-Neuropathol-Exp-

Neurol. 1996 Sep; 55(9): 992-8; ISSN: 0022-3069.

UNITED-STATES. We report a case study of a female who received an allogeneic

bone marrow transplantation (BMT) from a sex-mismatched related donor and

who, after a twenty-year interval, developed an acute fulminant biopsy-proven

demyelinating disorder of cerebral white matter which followed a remitting-

relapsing chronic course. In situ hybridization studies using Y-chromosome-

specific markers revealed Y-chromosome-positive mononuclear cells in biopsy

samples of white matter. Magnetic resonance imaging (MRI) studies of the

asymptomatic healthy male donor showed multiple white matter lesions. These

observations suggest that donor lymphocytes were sensitized to central nervous

system (CNS) antigens prior to or at the time of transplantation but remained

dormant for 20 years before becoming activated to cause widespread

demyelination.. 9007-49-2.

335. Khoo, D.; Carmichael, S. W.; Spinner, R. J. Ulnar nerve anatomy and compression.

Orthop-Clin-North-Am. 1996 Apr; 27(2): 317-38; ISSN: 0030-5898.

UNITED-STATES. Compression of the ulnar nerve can be understood in terms of

the anatomic and dynamic factors. Although the ulnar nerve may be compressed at

any point along its course, it is particularly susceptible at the elbow and the wrist.

Clinically relevant anatomy will be reviewed in an attempt to provide the reader

with a logical framework for successfully diagnosing and managing typical and

atypical ulnar nerve compression lesions.

336. Killer, H. E.; Job, O.; Forrer, A. [Retrobulbar neuritis--diagnosis and differential

diagnosis]. Retrobulbarneuritis--Diagnostik und Differentialdiagnose. Schweiz-

Rundsch-Med-Prax. 1996 Apr 23; 85(17): 554-8; ISSN: 0369-8394.

SWITZERLAND. The American multicenter study 'A randomized, controlled trial

of corticosteroids in the treatment of acute optic neuritis' (5) showed how a

retrobulbar neuritis should not be treated, Oral steroids (1 mg per kilogram of body

weight per day) are not only ineffective but also associated with a higher rate of

recurrences compared to high dose i.v. methylprednisolone. In the light of this

study, 'low-dose' steroid therapy for retrobulbar neuritis is contraindicated. High-

dose methylprednisolone speeds up recovery of the visual function and lowers the

recurrence rate two years after treatment; however, this protective effect could not

be demonstrated after three years. These recommendations are valid only for

primary demyelinating retrobulbar neuritis. Other less common optic neuropathies,

such as these of microvascular origin, respond to 'low-dose' steroids; therefore,

the diagnosis of primary demyelinating retrobulbar neuritis must be made with

caution as a diagnosis of exclusion. This paper discusses a number of important

optic neuropathies and gives recommendations for investigations. Compressive

optic neuropathies and chiasmal disease will not be covered here.. 0; 83-43-2.

337. Killian, J. M.; Tiwari, P. S.; Jacobson, S.; Jackson, R. D.; Lupski, J. R. Longitudinal

studies of the duplication form of Charcot-Marie-Tooth polyneuropathy. Muscle-

Nerve. 1996 Jan; 19(1): 74-8; ISSN: 0148-639X.

UNITED-STATES. This study presents a longitudinal comparison of motor nerve

conduction velocities (MCVs) in patients with Charcot-Marie-Tooth type 1A with

proven duplication of a segment of chromosome 17p11.2p12. Results were

compared for 8 CMT1A duplication patients from one family whose MCV

measurements were taken 22 years apart (1967 and 1989). Measurements from a

total of seven median motor and five peroneal motor MCVs were compared.

Median MCVs showed a slight reduction that averaged 2.2 m/s, and peroneal

MCVs showed an average decrease of 3.0 m/s. In addition, mild objective increase

in limb weakness was seen in only 1 of 8 patients and subjective symptoms of

gradual worsening of leg strength were noted in half the patients over the same

period. In this study of a small group of CMT1A patients with proven segmental

duplication of chromosome 17p11.2p12, the motor conduction velocities and

clinical motor exam did not change significantly over 22 years.

338. Kimura, H.; Masai, H.; Kashii, S. Optic neuropathy following elcatonin therapy. J-

Neuroophthalmol. 1996 Jun; 16(2): 134-6; ISSN: 1070-8022.

UNITED-STATES. A 34-year-old man, who had a 14-year history of

hemodialysis, presented with a sudden onset of blurring of vision in both eyes after

elcatonin therapy for hypercalcemia. Fundus examination showed well-colored

discs without swelling and normal retina in both eyes. Goldmann kinetic perimetry

demonstrated central scotomas in both eyes. One month after discontinuance of

elcatonin, his best visual acuity returned to 20/20 in both eyes. Although a cause-

and-effect relationship between elcatonin and optic neuropathy is not definitive,

optic neuropathy is presumably linked to elcatonin therapy.. 60731-46-6; 9007-12-

9.

339. Kimura, J. [Electrophysiological diagnosis and therapy of peripheral nerve

demyelinating diseases]. Nippon-Naika-Gakkai-Zasshi. 1996 Mar 10; 85(3): 389-

92; ISSN: 0021-5384.

JAPAN. 0; 0; 83-43-2.

340. Kinjo, T.; Mukawa, J.; Tomiyama, N. Modified petrosal approach using cosmetic

osteoplastic craniotomy with NEURO-SAT guidance. Neurol-Med-Chir-Tokyo.

1996 May; 36(5): 310-3; ISSN: 0470-8105.

JAPAN. The petrosal approach has become a routine procedure for petroclival

lesions, but there are some disadvantages such as the time-consuming craniotomy

during surgery and depressed deformity of the mastoid area after surgery. To solve

these disadvantages, we modified the petrosal approach. Before surgery, the three-

dimensional points of the sigmoid sinus and semicircular canals are calculated on

computed tomography scan and are input to a computer. A single temporooccipital

bone flap is made, and the outer table of bone overlying the mastoid is preserved by

forming a narrow groove with a small air drill and cutting the outer table with a

chisel. After removing these two free bone flaps, mastoidectomy is performed

guided by NEURO-SAT (neuronavigation by computer-assisted frameless

stereotaxy). The mastoid bone can be drilled out safely and quickly. The two bone

flaps are connected and replaced at the end of the procedure. Osteoplastic petrosal

craniotomy guided by NEURO-SAT can achieve a quick craniotomy and

satisfactory cosmetic result.

341. Kinoshita, A.; Hayashi, M.; Miyamoto, K.; Oda, M.; Tanabe, H. Inflammatory

demyelinating polyradiculitis in a patient with acute disseminated encephalomyelitis

(ADEM). J-Neurol-Neurosurg-Psychiatry. 1996 Jan; 60(1): 87-90; ISSN: 0022-

3050.

ENGLAND. A patient with severe acute disseminated encephalomyelitis died 12

days after the first symptom. Necropsy showed widespread severe demyelination

in the CNS and some foci of demyelination in the spinal roots. The lesions in the

peripheral nervous system were characterised by myelin stripping and the presence

of macrophages, being severest in the spinal nerve roots. Some axons were

completely demyelinated, whereas the axons themselves were preserved.

Pathologically established ongoing demyelination in both CNS and peripheral

nervous systems raises the possibility of a shared pathological epitope.

342. Kiroglu, M. M.; Zorludemir, S.; Suleymanova, D. Bilateral acoustic neurofibromatosis

with bilateral multicentric facial schwannomas. Eur-Arch-Otorhinolaryngol. 1996;

253(4-5): 305-8; ISSN: 0937-4477.

GERMANY. A case report of a 20-year-old female with bilateral acoustic

neurofibromatosis (NF-2) and bilateral facial schwannomas is presented. Multiple

segmental schwannomas were found with clinically intact tissue between each

tumoral enlargement in the right parotid region. Translocation (2;8) (p2.4:q2.1)

was detected in this patient, and has not been reported in a schwannoma until now.

The patient's family would not allow any major surgery to be performed.

343. Kishikawa, M.; Nakanishi, T.; Miyazaki, A.; Shimizu, A.; Nakazato, M.; Kangawa,

K.; Matsuo, H. Simple detection of abnormal serum transthyretin from patients

with familial amyloidotic polyneuropathy by high-performance liquid

chromatography/electrospray ionization mass spectrometry using material

precipitated with specific antiserum [letter]. J-Mass-Spectrom. 1996 Jan; 31(1):

112-4; ISSN: 1076-5174.

ENGLAND. 0; 0.

344. Kleindienst, A.; Hamm, B.; Hildebrandt, G.; Klug, N. Diagnosis and staging of carpal

tunnel syndrome: comparison of magnetic resonance imaging and intra-operative

findings. Acta-Neurochir-Wien. 1996; 138(2): 228-33; ISSN: 0001-6268.

AUSTRIA. PURPOSE: In order to determine the reliability of magnetic resonance

imaging (MRI) in the diagnosis and staging of carpal tunnel syndrome (CTS), the

most common entrapment neuropathy, the following prospective study has been

performed. METHODS: We compared clinical and electrophysiological studies in

58 cases of CTS with MRI investigations and confirmed the reliability by exact

correspondence with intra-operative findings. RESULTS: Typical MRI

characteristics of the median nerve in CTS have been established. There is a

significant difference in flattening (p < 0.05), swelling (p < 0.01) and signal

intensity (p < 0.05) of the median nerve between early and advanced CTS.

Comparison of MRI and intra-operative findings revealed that median nerve

compression was diagnosed correctly in 91% of cases. Additional lesions in the

carpal tunnel, which are a primary cause of nerve compression, were established by

MRI in 25 cases and confirmed by surgery. CONCLUSION: MRI is a reliable

diagnostic tool for assessing as well as staging of CTS. Morphological changes

following chronic nerve compression can be visualized. It is particularly useful in

cases of suspected lesions within the carpal tunnel as a cause of CTS. The

information provided may support the choice of adequate treatment modality.

345. Kleinert, J. M.; Mehta, S. Radial nerve entrapment. Orthop-Clin-North-Am. 1996 Apr;

27(2): 305-15; ISSN: 0030-5898.

UNITED-STATES. The radial nerve is frequently more involved in entrapment

syndromes than the ulnar and median nerves. Common sites of compression are the

juncture of the middle and distal third of the arm (especially with fractures of the

humerus), just distal to the elbow (radial tunnel), and proximal to the wrist between

the brachioradialis and extensor carpi radialis longus. Often in entrapment

syndromes involving the radial nerve, the true diagnosis is not evident and is

arrived at only by exclusion, which sometimes delays initiation of effective

treatment. Radial tunnel syndrome is rare, but decompression when indicated, can

provide relief. Radial sensory nerve entrapment in the forearm (distal third) does

occur, but patients often respond to temporary thumb spica splinting.

346. Kleinert, J. M.; Mehta, S. Radial nerve entrapment. Orthop-Clin-North-Am. 1996 Apr;

27(2): 305-15; ISSN: 0030-5898.

UNITED-STATES. The radial nerve is frequently more involved in entrapment

syndromes than the ulnar and median nerves. Common sites of compression are the

juncture of the middle and distal third of the arm (especially with fractures of the

humerus), just distal to the elbow (radial tunnel), and proximal to the wrist between

the brachioradialis and extensor carpi radialis longus. Often in entrapment

syndromes involving the radial nerve, the true diagnosis is not evident and is

arrived at only by exclusion, which sometimes delays initiation of effective

treatment. Radial tunnel syndrome is rare, but decompression when indicated, can

provide relief. Radial sensory nerve entrapment in the forearm (distal third) does

occur, but patients often respond to temporary thumb spica splinting.

347. Kleinschmidt DeMasters, B. K.; Newell, J. Multiple sclerosis with hydromyelia

demonstrated at autopsy. J-Neurol-Sci. 1996 Mar; 136(1-2): 185-8; ISSN: 0022-

510X.

NETHERLANDS. Multiple sclerosis (MS) associated with intramedullary cysts

has been recently documented radiographically in approximately a dozen cases, but

histological confirmation in these cases has been lacking. These cysts have been

noted to occur centrally in the spinal cord and have been previously diagnosed as

syringomyelia. We report a patient with extensive hydromyelia, but no dissecting

syringomyelic cavity, of the thoraco-lumbar spinal cord, which was co-existent

with centrally located, non-necrotic, chronic demyelinative plaques. A few well

demarcated non-necrotic plaques were seen in the cerebrum and cerebellum with

sparing of optic chiasm, confirming the diagnosis of chronic, non-Devic type MS.

Although a cause and effect between the hydromyelia and MS cannot be proven in

this or any of the previously reported radiographic cases our report provides

pathological confirmation of the association between these two entities and further

delineates the type of cyst.

348. Kluin, K. J.; Gilman, S.; Lohman, M.; Junck, L. Characteristics of the dysarthria of

multiple system atrophy. Arch-Neurol. 1996 Jun; 53(6): 545-8; ISSN: 0003-9942.

UNITED-STATES. OBJECTIVE: To characterize the dysarthria in patients with

multiple system atrophy (MSA). DESIGN: Motor speech examinations, consisting

of oral motor, oral agility, and perceptual speech analysis, were performed on 46

patients with MSA. SETTING: University department of neurology referral center.

RESULTS: All patients had dysarthria with combinations of hypokinesia, ataxia, or

spasticity. Thirty-two patients had all 3 components, 13 had 2 components, and 1

had only 1 component. In most patients the hypokinetic components were the most

severe. Hypokinetic components predominated in 22 patients (48%), whereas

ataxic components predominated in 16 (35%), and spastic components in 5 (11%).

In 1 patient (2%) the hypokinetic and spastic components were equal and greater

than the ataxic components, and in 1 patient (2%) the hypokinetic and ataxic

components were equal and greater than the spastic components. One patient (2%)

had only ataxic dysarthria. The predominant type of dysarthria corresponded well

to the subtype of MSA. CONCLUSIONS: The finding of a mixed dysarthria with

combinations of hypokinetic, ataxic, and spastic components is consistent with

both the overall clinical and the neuropathologic changes in MSA. Motor speech

examination can provide helpful information in evaluating patients who might have

MSA.

349. Knockaert, D. C.; Boonen, A. L.; Bruyninckx, F. L.; Bobbaers, H. J.

Electromyographic findings in ilioinguinal-iliohypogastric nerve entrapment

syndrome. Acta-Clin-Belg. 1996; 51(3): 156-60; ISSN: 0001-5512.

BELGIUM. The ilioinguinal-iliohypogastric nerve entrapment syndrome is a

recognised cause of, usually chronic, lower abdominal pain. Diagnosis is based

upon a typical clinical triad and relief of pain by injection of a local anaesthetic. In

the present study we assessed the value of abdominal muscle electromyography in

41 patients with a clinical syndrome suggestive of ilioinguinal-iliohypogastric nerve

entrapment. Electromyographic abnormalities were detected in 15 of 25 cases

(60%) with definite diagnosis and in 6 of 16 (37%) of those with probable

diagnosis of ilioinguinal-iliohypogastric nerve entrapment syndrome. The rather

low sensitivity and the clinical value of this technique are discussed.. 0.

350. Ko, C. Y.; Jones, N. F.; Steen, V. D. Compression of the median nerve proximal to

the carpal tunnel in scleroderma. J-Hand-Surg-Am. 1996 May; 21(3): 363-5;

ISSN: 0363-5023.

UNITED-STATES.

351. Koch, F.; Augustin, A. J.; Boker. Neuroborreliosis with retinal pigment epithelium

detachments. Ger-J-Ophthalmol. 1996 Jan; 5(1): 12-5; ISSN: 0941-2921.

GERMANY. Borreliosis or Lyme disease, a tick-borne infection with the

spirochete Borrelia burgdorferi, can cause various ocular and neurological

symptoms. A 41-year-old man had been repeatedly bitten by ticks in June 1992; 6

months later, the patient complained of blurred vision in both eyes of 1-week

duration, bifrontal headache that was more pronounced on the right side, and neck

pain that had appeared months earlier and was becoming more severe. On

ophthalmoscopy, clover-shaped retinal pigment epithelium detachments around the

optic disc were observed in both eyes. The patient's visual acuity was reduced to

0.5 in his left eye. Liquor cells and total protein were significantly increased;

however, a hemagglutination inhibition test revealed only moderately increased

immunoglobulin values. After 2 weeks of daily application of 4 g ceftriaxone

disodium, ophthalmological and neurological symptoms disappeared. Even though

the immunoglobulin values remained unchanged, neuroborreliosis with

involvement of the retinal pigment epithelium was the most probable diagnosis,

considering the history of tick bites and headache. The authors assume that the

tissue around the optic nerve head, which does not have an effective blood-brain

barrier, allowed the spirochetes to spread from the central nervous system into the

subpigment-epithelium space, thus causing the observed parapapillary pigment

epithelium detachments.

352. Kohut, G. N.; Della Santa, D. R.; Chamay, A. [Ulnar nerve compression syndrome of

the elbow. Analysis of 50 operated cases]. Le syndrome de compression du nerf

cubital au coude. Analyse de 50 cas operes. Ann-Chir-Main-Memb-Super. 1996;

15(3): 138-47; ISSN: 1153-2424.

FRANCE. This is a retrospective review of 50 elbows (44 patients) treated

operatively by simple decompression (seven cases), decompression with

epitrochlectomy (13 cases), and anterior transposition (30 cases) for compression

neuropathy of the ulnar nerve at the elbow. We performed a superficial

transposition in five cases, an intramuscular transposition in 13 cases, and a deep

submuscular transposition in 12 cases. The mean follow-up was three years and

two months. The overall results show cure or improvement in 78% of cases, no

changes in 20% of cases, and one case with subjective worsening. The

postoperative result was inversely proportional to the initial degree of the

neuropathy. The cases related to trauma obtained a better result.

353. Kommerell, G. The relationship between infantile strabismus and latent nystagmus.

Eye. 1996; 10( Pt 2): 274-81; ISSN: 0950-222X.

ENGLAND. The so-called infantile strabismus syndrome consists, among other

signs, of (1) strabismus, (2) a defect of pursuit and optokinetic tracking with

particular involvement of temporally directed responses on monocular viewing, (3)

latent nystagmus and (4) adduction preference of the fixating eye. The following

causal relationship between these three phenomena is suggested. (1) Binocularity in

the visual cortex is impaired, either as a primary defect or as a consequence of

misalignment of the eyes. (2) The reduced binocularity prevents maturation of

signal transmission from the visual cortex to the brainstem such that slip control is

evident in poor pursuit and optokinetic responses, particularly to monocular,

temporally directed stimuli. (3) The asymmetry of the pursuit and optokinetic

systems is also evident in latent nystagmus which reflects a tonic preponderance,

directed nasally with reference to the fixating eye. The directional preponderance

drives the slow phases of latent nystagmus if the visual input is unbalanced in

favour of one eye. Because of the maldeveloped slip control latent nystagmus is not

inhibited by visual contours. When both eyes are open the better-functioning

nasally directed pursuit and optokinetic control systems of the two eyes

complement each other and largely prevent drifting of the eyes. The defect

responsible for the abnormal motor control cannot be located between the retina and

the visual cortex because perception of motion is only slightly impaired and a nasal-

temporal asymmetry of the motion VEP, typically encountered in infantile

strabismus, does not correlate quantitatively with the asymmetry of the motor

control. Rather, the defect is located between the cortex and the brainstem. (4)

Adduction preference of the fixating eye with a compensatory headturn is due to a

gaze-evoked component added to the latent component of the nystagmus. The gaze-

evoked component is a purposeful reaction that allows dampening of the nystagmus

in adduction at the expense of an increase in abduction.

354. Komori, H.; Shinomiya, K.; Nakai, O.; Yamaura, I.; Takeda, S.; Furuya, K. The

natural history of herniated nucleus pulposus with radiculopathy. Spine. 1996 Jan

15; 21(2): 225-9; ISSN: 0362-2436.

UNITED-STATES. STUDY DESIGN: The present study retrospectively

investigated the morphologic changes that occurred during conservative treatment

of patients with unilateral leg pain resulting from herniated nucleus pulposus

without significant lumbar canal stenosis. OBJECTIVES: The results were

correlated with clinical outcomes and extruding forms to determine which type of

herniated nucleus pulposus had the greatest capacity for spontaneous regression

and how rapidly such regression might occur. SUMMARY OF BACKGROUND

DATA: The study population consisted of 77 patients with radiculopathy.

METHODS: All patients complained primarily of unilateral leg pain, and 94% had

positive tension signs. Additionally, 32% exhibited muscle weakness

corresponding to the symptomatic nerve root. All patients were studied more than

twice using magnetic resonance imaging during conservative therapy at a mean

interval of 150 days. Morphologic changes on magnetic resonance imaging fell into

four categories, with herniated nucleus pulposus classified into three types using

T1-weighted sagittal views. Each patient was reexamined on the same scanner; 53

patients were examined twice, and 24 patients were examined more than three

times. RESULTS: Morphologic changes, with the exception of 13 false-negative

cases, basically corresponded to clinical outcome. In half of the cases that showed

some improvement at follow-up evaluation, improvement of clinical findings were

seen before those observed on magnetic resonance imaging. Migrating herniated

nucleus pulposus frequently presented an obvious decrease in size, and even

disappearance in seven cases. The further the herniated nucleus pulposus migrated,

the more decrease in size could be observed. The cases apparently corresponding to

"protrusion" showed little or no change on follow-up magnetic resonance imaging.

Regarding the mechanism of herniated nucleus pulposus disappearance, exposure

to the vascular supply undoubtedly took a part, although many factors were

suspected to have some influence. CONCLUSION: Morphologic changes on

magnetic resonance imaging mainly corresponded to clinical outcomes but tended to

lag behind improvement of leg pain. Disappearance of herniate nucleus pulposus

was seen frequently in the cases of migrating disc herniation, and it was presumed

that exposure to the vascular supply had a lot to do with this phenomenon.

355. Korenke, G. C.; Fuchs, S.; Krasemann, E.; Doerr, H. G.; Wilichowski, E.;

Hunneman, D. H.; Hanefeld, F. Cerebral adrenoleukodystrophy (ALD) in only

one of monozygotic twins with an identical ALD genotype. Ann-Neurol. 1996

Aug; 40(2): 254-7; ISSN: 0364-5134.

UNITED-STATES. We report on monozygotic twins with different clinical

phenotypes of X-linked adrenoleukodystrophy. At the age of 10 years both boys

were neurologically asymptomatic. The first cranial magnetic resonance

examination showed normal findings in the first twin and parietooccipital

demyelination in the second. The latter developed behavioral problems 9 months

later, followed by visual impairment and gait ataxia. His cranial magnetic resonance

image at the age of 11 years showed progressive demyelination. In contrast,

neurological status and magnetic resonance images remained normal in the first

twin. The same point mutation in exon 8 of the adrenoleukodystrophy gene

(C2203T) was detected in both boys. All genotype examinations were consistent

with the diagnosis of monozygotic twins, suggesting that some nongenetic factors

may be important for different adrenoleukodystrophy phenotypes.

356. Korinthenberg, R.; Monting, J. S. Natural history and treatment effects in Guillain-

Barre syndrome: a multicentre study. Arch-Dis-Child. 1996 Apr; 74(4): 281-7;

ISSN: 0003-9888.

ENGLAND. A retrospective multicentre study was performed to investigate the

natural history and treatment effects in childhood Guillain-Barre syndrome in a

large number of patients. Structured questionnaires were sent to 155 paediatric

hospitals for details of patients who conformed to internationally accepted

diagnostic criteria and who were treated from spring 1989 to summer 1994. Sixty

nine hospitals reported data of 175 patients aged 11 months to 17.7 years. At the

height of the disease 26% of the patients remained able to walk, but 16% had to be

artificially ventilated. The median time from onset of symptoms to first recovery

was 17 days, to walk unaided 37 days, and to be free of symptoms 66 days. There

was a large group with a benign and a smaller one with a more protracted course.

At long term follow up, 98/106 patients were free of symptoms and the remainder

were able to walk unaided. Maximum disability grade was the most powerful

prognostic factor. In children unable to walk but not yet tetraplegic,

immunoglobulins were able to accelerate recovery. Corticosteroids were less

potent. Plasmapheresis could not be evaluated because it was administered only in

the most severe cases. The natural history of Guillain-Barre syndrome in children is

extremely variable and more benign than in adults. Treatment with

immunoglobulins should be considered in patients unable to walk. Corticosteroids

are not as effective and should be withheld except when, in protracted courses,

suspicion of chronic inflammatory demyelinating polyneuropathy arises.. 0; 0.

357. Kostic, N.; Secen, S. [Response of pancreatic polypeptide to a protein test meal in the

evaluation of diabetic autonomic neuropathy]. Odgovor pankreaticnog polipeptida

na proteinski test obrok u proceni dijabetesne autonomne neuropatije. Med-Pregl.

1996; 49(5-6): 177-9; ISSN: 0025-8105.

YUGOSLAVIA. In normal subjects, the early human pancreatic polypeptide

increase induced by food is mainly dependent on vagal activity. Parasympathetic

function and plasma human pancreatic polypeptide response to a protein rich meal

were evaluated in 105 insulin nondependent diabetic patients: 20 only with

autonomic neuropathy (group A), diagnosed by clonidin test and tests of

cardiovascular reflexes, 35 patients with neurophysiological evidence of

polyneuropathy (group B), 30 patients with autonomic neuropathy and

polyneuropathy (group C) and 20 patients without any sign of neuropathy (group

D). Plasma human pancreatic polypeptide levels were determined by

radioimmunoassay using an anti-human pancreatic polypeptide antiserum. Blood

was taken at 0, 45 and 60 minutes after the beginning of the meal. In groups A and

C, the meal induced human pancreatic polypeptide increase was significantly lower

than in group D (45. min:, 65.8 + 16.2(A), 54.0 + 19.2 pg/ml (C) in regard to

130.0 + 27.6 pg/ml (D); 60, min: 55.2 + 15.6 (A), 37.0 + 11.2 pg/ml (C) in regard

to 121.7 + 15.4 pg/ml (D). In group B patients had a marked increase of peptide,

similar to that in diabetics without neuropathy. These results suggest that diabetic

autonomic neuropathy is associated with the dysfunction of human pancreatic

polypeptide secretion and that evaluation of his response to test meal may be a

sensitive and simple method for the assessment of parasympathetic impairment in

diabetics.. 0; 0; 59763-91-6.

358. Kothari, M. J.; Preston, D. C.; Logigian, E. L. Lumbrical-interossei motor studies

localize ulnar neuropathy at the wrist. Muscle-Nerve. 1996 Feb; 19(2): 170-4;

ISSN: 0148-639X.

UNITED-STATES. Ulnar nerve entrapment at the wrist (UNW) is uncommon and

often difficult to localize electrophysiologically. The difference between the motor

latencies to the median-innervated second lumbrical (2L) and ulnar-innervated

palmar interosseous (Pl) (Diff 2L-Pl) has been shown to be of localizing value in

patients with median neuropathy at the wrist. In the last year, we evaluated 2

patients with clinically definite ulnar neuropathy at the wrist. We performed motor

studies to the 2L-Pl on the 2 patients and 12 disease controls with ulnar neuropathy

at the elbow as follows: Using the same electrodes to record both the 2L and Pl, the

median and ulnar nerves were each stimulated supramaximally above the wrist

using identical distances. In the disease control subjects, the Diff 2L-Pl was

essentially the same as normal controls (mean [0.13], range [(-0.3)-0.4]). In both

patients with UNW, the Diff 2L-Pl clearly supported the routine

electrophysiological studies in localizing the lesion (ulnar latencies were 1.1 and

1.8 ms longer than the median latencies). We conclude that the lumbrical-

interosseous latency difference is useful in localizing ulnar nerve entrapment to the

wrist.

359. Kramer, P. W. Microsurgical carpal tunnel release [letter]. Neurosurgery. 1996 Jun;

38(6): 1261; ISSN: 0148-396X.

UNITED-STATES.

360. Kril, J. J. Neuropathology of thiamine deficiency disorders. Metab-Brain-Dis. 1996

Mar; 11(1): 9-17; ISSN: 0885-7490.

UNITED-STATES. The Wernicke-Korsakoff syndrome (WKS) is the most

frequently encountered manifestation of thiamine deficiency in Western society. It

is commonly seen in alcoholic patients, but may also occur in patients with

impaired nutrition from other causes, such as those with gastrointestinal disease or

AIDS. The pathology is restricted to the central nervous system and is characterised

by neuronal loss, gliosis and vascular damage in regions surrounding the third and

fourth ventricles and the cerebral aqueduct. In addition to WKS, thiamine

deficiency may also result in beriberi, a cardiac and peripheral nervous system

disease, and it has been implicated in the pathogenesis of cerebellar degeneration

and peripheral neuropathy. Thus thiamine deficiency results in significant nervous

system pathology and vigilance should be maintained in the diagnosis and treatment

of this readily preventable cause of disease.

361. Kudo, A.; Suzuki, M.; Kubo, N.; Kuroda, K.; Ogawa, A.; Iwasaki, Y. Schwannoma

arising from the intermediate nerve and manifesting as hemifacial spasm. Case

report. J-Neurosurg. 1996 Feb; 84(2): 277-9; ISSN: 0022-3085.

UNITED-STATES. This 35-year-old man presented with left facial spasm that had

persisted for 10 months. Microvascular decompression was performed to relieve

the symptom. No responsible vessel could be identified during surgery, but a small

mass seeming to arise from the intermediate nerve and compressing the seventh

cranial nerve was removed. The histological diagnosis was Antoni-B type

schwannoma. This unique case of schwannoma arising from the intermediate nerve

was recognized by means of an operative microscope, and supports the idea that

portions of the seventh nerve schwannoma originate from the components of the

intermediate nerve.

362. Kulick, R. G. Carpal tunnel syndrome. Orthop-Clin-North-Am. 1996 Apr; 27(2): 345-

54; ISSN: 0030-5898.

UNITED-STATES. Patients with carpal tunnel syndrome should be told that it is a

progressive condition that, if not treated, probably will worsen as time goes on.

When release is performed properly, they have an excellent chance for substantial

improvement, although some always may have a degree of residual numbness at

the fingertips. Initial relief of pain is rapid, with subsequent improvement in

numbness and weakness occurring more slowly. Carpal tunnel syndrome is a very

common problem. Although there may be a distinct cause in some patients, the

underlying reason for the increased bulk of synovium is not known in most.

Conservative treatment gives temporary relief, but surgical release remains the most

effective treatment. Complications are not common, and proper attention to details

minimizes them. The results generally are excellent.

363. Kumar, A.; Chaudhary, D.; Gupta, S. K. Wildervanck syndrome. Australas-Radiol.

1996 May; 40(2): 160-1; ISSN: 0004-8461.

AUSTRALIA. We report a case of the Wildervanck (cervico-oculo-acoustic)

syndrome exhibiting Klippel-Feil anomaly, congenital sensorineural deafness and

bilateral sixth nerve palsy. Associated anomalies included short stature,

microcephaly, mental retardation, and cleft palate.

364. Kurokawa, H.; Nakagawa, I.; Kubota, M.; Niinai, H.; Takezaki, T.; Yamada, K.

[Electrophysiological examinations in Bell's palsy using electroneuronography and

strength-duration curve]. Masui. 1996 Jul; 45(7): 842-5; ISSN: 0021-4892.

JAPAN. In 30 patients with Bell's palsy electroneuronography (ENoG) and

strength-duration curve (S-D curve) were used to evaluate the recovery from the

palsy at the orbicularis oris and the oculi muscles. At the orbicularis oris muscle,

the final outcome was poor in the patients in whom minimum ENoG < 10%, and

all the patients with 30% <or= minENoG showed good recovery. In the patients

with 10 <or= minENoG < 30, when S-D curve was without scale out, all the

patients showed complete recovery. The recovery in the orbicularis oculi muscle of

the patients with minENoG < 10% was all poor, but two patients with 30% <or=

minENoG, remained in the impaired situation, and one patient with 10 <or=

minENoG < 30, without scale out in S-D curve showed poor recovery. These

findings suggests that electrophysiological examinations using ENoG and S-D

curve in the patients with Bell's palsy can provide valuable information at the

orbicularis oris muscle, but at the orbicularis oculi muscle, these are less reliable.

365. Kurvers, H. A.; Hofstra, L.; Jacobs, M. J.; Daemen, M. A.; van, den Wildenberg FA;

Kitslaar, P. J.; Slaaf, D. W.; Reneman, R. S. Reflex sympathetic dystrophy: does

sympathetic dysfunction originate from peripheral neuropathy? Surgery. 1996 Mar;

119(3): 288-96; ISSN: 0039-6060.

UNITED-STATES. BACKGROUND: Sympathetic dysfunction in reflex

sympathetic dystrophy (RSD) has been purported to consist of an afferently-

induced increase in efferent sympathetic nerve impulses (somato-sympathetic

reflex) and/or denervation-induced supersensitivity to catecholamines. In addition,

both the central and peripheral nervous systems have been claimed to be involved.

It was the aim of this study to obtain more insights into these underlying

mechanisms. METHODS: In the affected extremeties of 42 patients with RSD we

investigated as indirect measures of sympathetic (dys)function: (1) skin blood flow

and the vasoconstrictive response to dependency of skin microvessels by means of

laser Doppler flowmetry (distal to the site of trauma), (2) relative distention of the

brachial artery and changes in relative distention consequent to a cold pressor test

by means of ultrasonic vessel wall tracking (proximal to the site of trauma), and (3)

arterial blood pressures by means of the Finapres technique. Both provocation tests

induce a sympathetically mediated response. Patients were divided into three

categories according to their perception of skin temperature in their injured limb

(stage I, stationary warmth sensation; stage II, intermittent warmth and cold

sensation; or stage III, stationary cold sensation). RESULTS: Distal to the site of

trauma, when compared with controls, skin blood flow was increased at stage I and

decreased at stages II and III, whereas the vasoconstrictive response to dependency

was impaired at all three stages. Proximally, when compared with controls, relative

distention of the brachial artery and its response to the cold pressor test were

decreased at all three stages. No differences were observed in pulse pressure

between patient groups and controls. CONCLUSIONS: These results suggest that

sympathetic dysfunction in extremities of patients with RSD distal to the site of

trauma consists of hypersensitivity to catecholamines at stages II and III as a result

of autonomic denervation at stage I, whereas proximal to the site of trauma

sympathetic nerve impulses may be increased at all three stages.

366. Lacoste, L.; Karayan, J.; Lehuede, M. S.; Thomas, D.; Goudou Sinha, M.; Ingrand,

P.; Barbier, J.; Fusciardi, J. A comparison of direct, indirect, and fiberoptic

laryngoscopy to evaluate vocal cord paralysis after thyroid surgery. Thyroid. 1996

Feb; 6(1): 17-21; ISSN: 1050-7256.

UNITED-STATES. After thyroidectomy, the anesthesiologist usually performs a

laryngoscopy to detect laryngeal edema and nerve palsies. The goal of this study

was to compare three different methods of laryngeal examination after tracheal

extubation of the patients. For that purpose, between 1990 and 1995, a prospective

series of 1608 patients operated for thyroidectomy has been studied. The series was

divided into 4 groups. In group I (n = 200), four anesthesiologists have evaluated

the efficiency of the immediate postextubation direct laryngoscopy. In group II (n =

100), one anesthesiologist has compared the direct, indirect, and flexible

laryngoscopies in every patient in a fixed and timed fashion. In group III (n = 100),

the four examiners have evaluated the flexible laryngoscopy at a different timing so

as to eliminate the possible temporal relationship of the ease of visualization in

group II. In group IV (n = 1208), the four examiners have evaluated flexible

laryngoscopy, on a large scale, at any time during the 1-h stay in the recovery

room. Special attention was directed to the patients with known cardiovascular

diseases. Direct and indirect laryngoscopies were only effective in 76 and 73%,

respectively, of the patients, whereas flexible laryngoscopy was effective in 99.6%

of them. Flexible laryngoscopy was easy to perform in 96.5% of the patients

versus 65 and 55% with direct and indirect laryngoscopies. Finally, variations in

monitored cardiovascular parameters were significantly lower with flexible and

indirect laryngoscopies than with direct laryngoscopy. These mild variations

induced by flexible laryngoscopy were well tolerated by patients with known

cardiovascular diseases. Flexible laryngoscopy is the best method for an immediate

laryngoscopic examination after thyroidectomy.

367. Laghi Pasini, F.; Pastorelli, M.; Beermann, U.; de Candia, S.; Gallo, S.; Blardi, P.; Di

Perri, T. Peripheral neuropathy associated with ischemic vascular disease of the

lower limbs. Angiology. 1996 Jun; 47(6): 569-77; ISSN: 0003-3197.

UNITED-STATES. This paper deals with the possible identification of somatic

and autonomic nerve damage in patients with peripheral obliterative arterial disease

(POAD) at different stages of the disease, with a well-reproducible technique like

electroneurographic evaluation of nerve conduction. In 64 patients with intermittent

claudication, 19 patients with pain at rest, and 7 patients with trophic ulcers,

electroneurographic evaluation of motor (tibial and peroneal) and sensory

(superficial peroneal and sural) nerve conduction was performed. The median nerve

(motor and sensory) was used as control. A severe impairment of sural and

superficial peroneal nerve velocities was evident in many claudicant patients and in

all patients with pain at rest and trophic ulcers, with a progression in the conduction

abnormalities in advanced stages of the disease. Motor nerve conduction showed

only minor reductions in patients with claudication and pain at rest, although some

of them did show very poor velocity values. In 21 patients with intermittent

claudication and sensory nerve abnormalities, the autonomic fibers activity,

evaluated by the skin sympathetic response (SSR) test, was significantly

depressed, thus suggesting an involvement of the local autonomic system in the

ischemic disease. A correlation exists between the severity of the somatic nerve

damage and the stage of the vascular insufficiency. However, in the group of

claudicant patients, the evidence of similar ischemic threshold (claudication

distance) may be associated with a marked difference in the amount of somatic

nerve damage. The somatic and autonomic nerve alterations may play a relevant

role in the progression of the disease toward critical limb ischemia.

368. Laine, F. J. Cranial nerves III, IV, and VI. Top-Magn-Reson-Imaging. 1996 Apr;

8(2): 111-30; ISSN: 0899-3459.

UNITED-STATES. Movements of the eye are produced by six extraocular

muscles innervated by three cranial nerves: the oculomotor (III), the trochlear (IV),

and the abducens (VI). These cranial nerves are discussed together because of the

interrelated nuclear origins, neural pathways, and motor functions. The normal

anatomic pathway of these three nerves is presented. The clinical and pathologic

manifestations of lesions producing both isolated and complex palsies of these

nerves are discussed along with imaging correlation.

369. Landau, W. M. Reflex sympathetic dystrophy [letter; comment]. Mayo-Clin-Proc.

1996 May; 71(5): 524-5; ISSN: 0025-6196.

Note: Comment on: Mayo Clin Proc 1995 Nov;70(11):1124-6.

UNITED-STATES.

370. Lane, J. I.; Koeller, K. K.; Atkinson, J. D. MR imaging of the lumbar spine:

enhancement of the radicular veins. AJR-Am-J-Roentgenol. 1996 Jan; 166(1): 181-

5; ISSN: 0361-803X.

UNITED-STATES. Enhancement of lumbar nerve roots in the setting of

degenerative disk disease has been considered by several authors as evidence of a

breakdown in the blood-nerve barrier induced by nerve root compression [1-3].

Unfortunately, correlation between radicular enhancement and the clinically

determined level of radiculopathy has not been consistently shown [4, 5]. We

recently proposed that this phenomenon represents intravascular enhancement of

radicular veins that travel adjacent to or within the endoneurium of one or more

nerve roots of the cauda equina [4, 5]. Our purpose in this pictorial essay is to

illustrate the appearance of radicular vein enhancement so that this phenomenon will

not be misinterpreted as being clinically significant in the setting of degenerative

disk disease.

371. Lang, J. [Diagnosis and treatment of amblyopia]. Diagnose und Behandlung der

Amblyopie. Ther-Umsch. 1996 Jan; 53(1): 20-4; ISSN: 0040-5930.

SWITZERLAND. The word amblyopia means weak vision on a functional basis.

The most important form is strabismic amblyopia. Its diagnosis is easy in cases of

obvious unilateral strabismus; in cases with slight squint angles this may be

difficult. The most important treatment is carefully controlled occlusion of the

dominant eye.

372. Latronico, N.; Fenzi, F.; Recupero, D.; Guarneri, B.; Tomelleri, G.; Tonin, P.; De

Maria, G.; Antonini, L.; Rizzuto, N.; Candiani, A. Critical illness myopathy and

neuropathy. Lancet. 1996 Jun 8; 347(9015): 1579-82; ISSN: 0140-6736.

ENGLAND. BACKGROUND: Critically ill patients may develop muscle

weakness or paralysis during the course of sepsis and multiple-organ failure. We

studied peripheral nerve and muscle disorders (NMD) in comatose patients.

METHOD: Comatose patients who developed paralysis associated with absent

deep-tendon reflexes had electroneuromyography (ENMG) and muscle-nerve

biopsy specimens taken. Onset and duration of sepsis, multiple-organ dysfunction

and failure, biochemical alterations, and drugs potentially interfering with nerve-

muscle function were recorded. FINDINGS: 24 patients became quadriparetic or

quadriplegic; muscle changes were found in 23. Axonal neuropathy was found in

eight of 22 patients examined. All patients had prolonged sepsis and multiple-organ

dysfunction, but only 14 had multiple-organ failure. Drugs such as steroids,

neuromuscular-blocking agents, and aminoglycosides were not responsible for

paresis, and the part played by hyperglycaemia and hypoalbuminaemia is uncertain.

Attending physicians predicted a fatal outcome in all cases, although six of seven

survivors fully recovered within 115-210 days from the onset of paralysis.

INTERPRETATION: Comatose patients may become completely paralysed

because of NMD. The diagnosis is important to avoid unnecessary investigations

and unreasonably pessimistic prognosis. ENMG is essential for the diagnosis and

for planning further clinical management. Biopsy needs to be done only when it is

necessary to properly classify NMD.

373. Lauder, T. D.; Dillingham, T. R. The cervical radiculopathy screen: optimizing the

number of muscles studied. Muscle-Nerve. 1996 May; 19(5): 662-5; ISSN: 0148-

639X.

UNITED-STATES.

374. Lazareth, I. [False erythermalgia]. Les fausses erythermalgies. J-Mal-Vasc. 1996;

21(2): 84-7; ISSN: 0398-0499.

FRANCE. The differential diagnosis of erythermalgia is sometimes complicated by

the absence of consensus on proposed diagnostic criteria. Unwarranted diagnosis

can result from any clinical situation leading to burning sensations in the limbs.

This can occurs in patients with peripheral neuropathies who often experience

dysesthesia when going to bed when the legs are under the covers; in such cases,

redness and local warmth are missing. Venous insufficiency can also produce

sensations of warm feet, often at retiring, together with edema and an increase in

local heat. Algodystrophy, during the inflammatory phase can also mimic

erythermalgia with intense pain and local modifications. Nevertheless, the unilateral

aspect and persistence of the symptoms together with the post-traumatic situation

usually directs the diagnosis. Acrodynia is a rare disease caused by excessive

mercury intake and should be discussed in children. Vasomotor impairment in the

limbs is the main sign. The red color of the hands and feet is accompanied by

intense paroxysmal burn-type pain. The diagnosis is confirmed by high mercury

levels in urine. Fabry's disease is a hereditary sphingolipidosis transmitted on

chromosome X and occurs predominantly in men, often starting early in childhood

with burning sensation in the limbs. The diagnosis should be entertained in children

with pseudo-erythermalgia and is confirmed by chromatographic search for

abnormal sphingolipids in the urine.

375. Lechevalier, D.; Dubayle, P.; Crozes, P.; Magnin, J.; Gaillard, J. F.; Boyer, B.;

Pharaboz, C.; Eulry, F. [Magnetic resonance imaging in the warm and cold forms

of algodystrophy of the foot]. L'imagerie par resonance magnetique dans les

formes chaudes et froides de l'algodystrophie du pied. J-Radiol. 1996 Jun; 77(6):

411-7; ISSN: 0221-0363.

FRANCE. PURPOSE: To report magnetic resonance imaging abnormalities in

reflex sympathetic dystrophy of the foot. METHODS: Retrospective study of 22

algodystrophies of the foot, in warm phase in 17 cases, in cold phase in 5.

RESULTS: Algodystrophy in warm phase: Bone medullary abnormalities were

noted in 17 cases (decrease of signal intensity was found in T1 weighted images,

increase of signal intensity in T2 weighted images, in T1 and T2 with fat-

saturation, in T1 with gadolinium), located at the increased uptake technetium site

in 16 cases. T1 and T2 weighted images with fat-saturation and T1 with fat-

saturation after injection of gadolinium were pathological in all cases, T1 was

normal in 2 cases. Soft tissues abnormalities were noted in 11 cases, joint effusion

in 8 cases, synovial hypertrophy enhanced by gadolinium in 2 cases and a

subchondral linear area of hypointense signal on T1 and T2 images was not present

after gadolinium injection in 1 case. Six fractures were detected. Algodystrophy in

cold phase: no bone edema, no synovial hypertrophy, no joint effusion, no soft

tissues abnormalities, no fractures are detected. CONCLUSION: The results

suggest that the MRI has a considerable value in diagnosis during the warm phase

of reflex sympatetic dystrophy of the foot. The normal MRI findings during the

cold phase could be important to understand its etiology.

376. Lee, A. G. MRI in unexplained optic neuropathy [letter]. Ophthalmology. 1996 Aug;

103(8): 1163-4; ISSN: 0161-6420.

UNITED-STATES.

377. Lehtinen, I.; Kirjavainen, T.; Hurme, M.; Lauerma, H.; Martikainen, K.; Rauhala, E.

Sleep-related disorders in carpal tunnel syndrome. Acta-Neurol-Scand. 1996 May;

93(5): 360-5; ISSN: 0001-6314.

DENMARK. INTRODUCTION: Patients with carpal tunnel syndrome (CTS)

often wake up at night due to pain and numbness of affected fingers and hand. We

studied the sleep disorder caused by CTS. SUBJECTS AND METHODS: 34

consecutive patients referred for operative treatment of CTS answered to a sleep

questionnaire and the results were compared to a stratified random sample of 1600

Finns aged 36-50 year, whose response rate to the mailed questionnaires was

75.2% (n = 1186). Six CTS patients underwent a polygraphic sleep study before

and after operative treatment of CTS. RESULTS: CTS patients reported suffering

from poor sleep quality, fragmentary sleep and daytime sleepiness more often than

controls. Before operative treatment of CTS there were more nocturnal body

movements (p < 0.01) and awakenings lasted longer (p < 0.05) than after

operation. During preoperative sleep studies no drop in median nerve conduction

was detected during awakenings. CONCLUSIONS: Patients with CTS suffer from

fragmentary sleep. Although patients reported waking up for the pain or numbness

of hands no impairment in median and ulnar nerve conduction could be observed

during these awakenings. Operative treatment of hand entrapment significantly

reduced the number of nocturnal movements.

378. Lennerstrand, G.; Rydberg, A. Results of treatment of amblyopia with a screening

program for early detection. Acta-Ophthalmol-Scand-Suppl. 1996; (219): 42-5;

ISSN: 1395-3931.

DENMARK. The Swedish program for early intervention in children with visual

dysfunction, was evaluated with respect to the results of treatment of amblyopia.

One hundred and seventy-two amblyopic children were examined before and at 6,

12 and 18 months after the beginning of treatment. The mean visual acuity of the

amblyopic eye was 0.76 and the mean ratio of visual acuity between the two eyes

was 0.75 or better in all types of amblyopia. Children with strabismic amblyopia

showed slightly poorer treatment results than the other types, in spite of the fact that

their amblyopia had been detected earlier than the others. The results attest to the

importance of careful screening of monocular visual acuity in all children, which in

Sweden occurs at 4 years of age. This provides a good basis for detection and

treatment of amblyopia. Only one child in the group had a visual acuity at 0.2 and

may therefore be at risk for visual handicap if the better eye should be lost later in

life.

379. Lesprit, P.; Authier, F. J.; Gherardi, R.; Belec, L.; Paris, D.; Melliere, D.; Schaeffer,

A.; Godeau, B. Acute arterial obliteration: a new feature of the POEMS syndrome?

Medicine-Baltimore. 1996 Jul; 75(4): 226-32; ISSN: 0025-7974.

UNITED-STATES. The POEMS (polyneuropathy, organomegaly,

endocrinopathy, monoclonal gammapathy, and skin changes) syndrome is a rare

variant of plasma cell dyscrasia with multisystemic manifestations. We present 4

cases with arterial symptoms typical of acute arterial obliteration (AAO) and review

9 similar cases in the literature. The clinical course of AAO was unusual and

particularly severe when affecting the lower limbs; recurrent events required

amputations. As demonstrated by angiographic and histologic studies, thrombotic

and atheromatous lesions were the main pathologic features of AAO.

Atherosclerotic risk factors were absent or moderate in 3 of our cases, and no cause

of thrombosis other than the POEMS syndrome was found. A high production of

cytokines was found in all cases, with elevated serum levels of interleukin-1 beta

(9/9 samples), interleukin-6 (7/9 samples), and tumor necrosis factor-alpha (6/9

samples). We suggest that arterial manifestations should be added to the spectrum

of manifestations of the POEMS syndrome. Cytokines may mediate the POEMS

syndrome-associated AAO, as previously proposed for the other systemic

manifestations of this disorder.. 0; 0; 0.

380. Lesprit, P.; Mouloud, F.; Bierling, P.; Schaeffer, A.; Cesaro, P.; Brun Buisson, C.;

Godeau, B. Prolonged remission of SLE-associated polyradiculoneuropathy after a

single course of intravenous immunoglobulin. Scand-J-Rheumatol. 1996; 25(3):

177-9; ISSN: 0300-9742.

NORWAY. Polyradiculoneuropathy is a rare and potentially severe complication

of systemic lupus erythematosus (SLE). Treatment is not codified and response to

corticosteroid is inconstant. We report the case of a patient with severe SLE-

associated polyradiculoneuropathy and autoimmune thrombocytopenia. Dramatic

neurologic improvement and correction of thrombocytopenia were observed after a

single course of high-dose intravenous immunoglobulin infusions (IVIg, 2g/kg

body weight). Our case suggests that IVIg may be effective in the treatment of this

unusual condition.. 0.

381. Levin, L. A.; Louhab, A. Apoptosis of retinal ganglion cells in anterior ischemic optic

neuropathy. Arch-Ophthalmol. 1996 Apr; 114(4): 488-91; ISSN: 0003-9950.

UNITED-STATES. We identified retinal ganglion cells undergoing apoptosis, a

form of programmed cell death, in an eye of a 70-year-old man with anterior

ischemic optic neuropathy. The TUNEL (terminal deoxynucleotidyl transferase-

mediated deoxyuridine triphosphate-biotin end nick labeling) staining and the

presence of condensed, fragmented nuclear bodies were used to identify apoptotic

cells. Examination of TUNEL-stained retinal sections revealed occasional cells in

the ganglion cell layer with pyknotic nuclei and brown reaction product,

representing positive staining for chromosomal DNA breaks. Positive cells were

sparsely distributed, consistent with the limited time in which apoptotic cells are

identifiable before they are removed. The most likely explanation for these results is

that injury to the retinal ganglion cell axon induces apoptosis. To our knowledge,

this is the first report of human retinal ganglion cell apoptosis in an acute optic

neuropathy.. EC 2.7.7.31; 0; 58-85-5; 9007-49-2.

382. Levine, A. M.; Tulpule, A.; Espina, B.; Boswell, W.; Buckley, J.; Rasheed, S.; Stain,

S.; Parker, J.; Nathwani, B.; Gill, P. S. Low dose methotrexate, bleomycin,

doxorubicin, cyclophosphamide, vincristine, and dexamethasone with zalcitabine in

patients with acquired immunodeficiency syndrome-related lymphoma. Effect on

human immunodeficiency virus and serum interleukin-6 levels over time. Cancer.

1996 Aug 1; 78(3): 517-26; ISSN: 0008-543X.

UNITED-STATES. BACKGROUND: Use of multiagent chemotherapy has been

associated with complete remission (CR) in approximately 50% of patients with

newly diagnosed acquired immunodeficiency syndrome (AIDS)-lymphoma,

although additional AIDS-related complications may occur. Both chemotherapy and

antiretroviral therapy were employed in an attempt to ascertain if the combination

was safe, and associated with changes in human immunodeficiency virus (HIV)

p24 antigen levels during the course of treatment. METHODS: Low dose

methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, and

dexamethasone(M-BACOD) chemotherapy and zalcitabine (ddC) were employed in

28 patients. Since both vincristine and zalcitabine may cause peripheral neuropathy,

a Phase I/II study design was employed. Serum was analyzed for immune complex

dissociated (ICD) HIV p24 antigen and interleukin (IL)-6 levels during therapy.

RESULTS: CR was achieved in 14 of 25 patients (56%), with partial response

(PR) in 5 (20%). CRs were equivalent in patients with good or poor prognostic

indicators, including a history of AIDS prior to lymphoma (CR = 60%); and/or

CD4 lymphocytes < 200/mm3 (CR = 53%). Five patients with a CR subsequently

relapsed (36%); median survival of CR patients was 29.2 months (4.1-61+),

whereas that of all of the treated patients was 8.1 months. No significant peripheral

neuropathy or other toxicity was observed. Serum ICD p24 antigen levels either fell

(7/14) or remained consistently negative (2/14) in 9 of 14 patients (64%), whereas

36% experienced an increase. Elevated serum IL-6 levels at diagnosis were

associated with systemic "B" symptoms (P = 0.023), whereas changes in IL-6

correlated with response to therapy over time (P = 0.006). CONCLUSIONS:

Combination antineoplastic and zalcitabine antiretroviral therapy may be safely

administered to patients with AIDS-related lymphoma, resulting in CR in 56%, lack

of significant neurotoxicity, and favorable effect on HIV p24 antigen in 50%.

Elevation of serum IL-6 is associated with systemic "B" symptoms, whereas

changes in serum IL-6 may correlate with response.. 0; 0; 0; 0; 0; 11056-06-7;

23214-92-8; 50-02-2; 50-18-0; 57-22-7; 58-05-9; 59-05-2; 7481-89-2.

383. Levy, F. S.; Bircher, A. J.; Buchner, S. A. Delayed-type hypersensitivity to cow's

milk protein in Melkersson-Rosenthal syndrome: coincidence or pathogenetic role?

Dermatology. 1996; 192(2): 99-102; ISSN: 1018-8665.

SWITZERLAND. BACKGROUND. Intolerance to cow's milk protein is

frequently seen in children and rarely in adults. Non-IgE-mediated hypersensitivity

to cow's milk protein has been suspected in children based on in vitro evidence.

Food intolerance may play a pathogenetic role in some cases of Melkersson-

Rosenthal syndrome, which is often of unknown origin. OBJECTIVE. We

describe an adult female patient who developed a Melkersson-Rosenthal syndrome

and at the same time was found to have in vivo and in vitro evidence of a delayed-

type hypersensitivity to cow's milk protein. METHODS. Allergic and immunologic

examinations, including skin prick tests, patch tests, serology, lymphocyte

transformation tests and biopsies were performed. RESULTS. A positive patch test

to alpha-lactalbumin, a positive lymphocyte transformation test to whole cow's

milk and an immunohistology with infiltration of CD4+ and CD8+ T cells were

found. CONCLUSIONS. There is a possible pathogenetic relation between

delayed-type hypersensitivity to cow's milk protein and Melkersson-Rosenthal

syndrome in the patient presented.. 0; 0; 37341-29-0.

384. Lewallen, S.; Bakker, H.; Taylor, T. E.; Wills, B. A.; Courtright, P.; Molyneux, M.

E. Retinal findings predictive of outcome in cerebral malaria. Trans-R-Soc-Trop-

Med-Hyg. 1996 Mar; 90(2): 144-6; ISSN: 0035-9203.

ENGLAND. The pathogenesis of cerebral malaria is poorly understood. Direct and

indirect ophthalmoscope examinations of 141 Malawian children with strictly

defined cerebral malaria revealed 2 distinct and prognostically significant findings:

papilloedema and extramacular retinal oedema. The relative risk of death in patients

with papilloedema was 6.7 times that in patients without papilloedema.

Extramacular retinal oedema was associated with a 2.9 fold increase in the relative

risk of dying. The mortality rate in patients with neither of these signs was only

1.3% compared to an overall mortality rate of 9.2%. The clinical and laboratory

features associated with each of these ophthalmological findings were different,

suggesting that there may be at least 2 different pathogenetic processes in patients

with cerebral malaria.. 0.

385. L'Heveder, G.; Tea, S. H.; Jezequel, J.; Mabin, D. [Presymptomatic injury of the

recurrent laryngeal nerve in benign thyroid disease: contribution of

electromyography]. Atteinte recurrentielle presymptomatique au cours d'affections

benignes de la thyroide: apport de l'electromyographie. Neurophysiol-Clin. 1996;

26(2): 109-14; ISSN: 0987-7053.

NETHERLANDS. Diagnosis of recurrent laryngeal nerve palsy is usually possible

through a clinical, laryngoscopical and electromyographical approach, but at a

critical stage of the nerve injury. We observed four cases of benign thyroid tumoral

processes with a preoperative electromyographic examination showing neurogenic

abnormalities in the thyroarytenoid muscle without any clinical symptoms. We

presume that only laryngeal electromyography permits the diagnosis of mild, even

asymptomatic laryngeal recurrent nerve injury. A recurrent laryngeal nerve palsy

occurs in thyroid tumors, most often in malignant conditions, rarely in benign

ones. Nevertheless early forms of nerve injury with benign thyroid pathology could

be underrated. Since the functional prognosis of symptomatic laryngeal nerve palsy

is doubtful, laryngeal electromyography, through its ability to diagnose early nerve

injury, provides helpful indications in thyroid benign tumoral diseases for the

therapeutic decision.

386. Li, D.; Schauble, B.; Moll, C.; Fisch, U. Intratemporal facial nerve perineurioma.

Laryngoscope. 1996 Mar; 106(3 Pt 1): 328-33; ISSN: 0023-852X.

UNITED-STATES. Forty-two cases of perineurioma have been reported in the

literature. This report adds the first intratemporal facial nerve perineurioma to the

literature and reviews the others. Unlike schwannoma and neurofibroma, the

histological features of perineurioma demonstrate onion bulb-like structures with a

strong positive immunoreactivity for epithelial membrane antigen. The clinical

history of gradual facial nerve paresis was 15 years in the case presentation and the

clinical diagnosis of tumor was overlooked.

387. Li, R.; Chen, J.; Hammonds, G.; Phillips, H.; Armanini, M.; Wood, P.; Bunge, R.;

Godowski, P. J.; Sliwkowski, M. X.; Mather, J. P. Identification of Gas6 as a

growth factor for human Schwann cells. J-Neurosci. 1996 Mar 15; 16(6): 2012-9;

ISSN: 0270-6474.

UNITED-STATES. Schwann cells are one of the principal components of the

peripheral nervous system. They play a crucial role in nerve regeneration and can

be used clinically in the repair of injured nerves. We have established serum-free,

defined culture conditions that rapidly expand adult human Schwann cells without

fibroblast growth. We find that Gas6, a ligand for the Axl and Rse/Tyro3 receptor

protein tyrosine kinase family, stimulates human Schwann cell growth, increasing

both cell number and thymidine incorporation. Gas6 has synergistic effects with the

other known human Schwann cell mitogens, heregulin/glial growth factor and

forskolin. Addition of Gas6 causes phosphorylation of Axl and Rse/Tyro3

simultaneously and results in ERK-2 activation. A combination of Gas6 with

heregulin and forskolin, on a defined background, supports maximal Schwann cell

proliferation, while preserving the typical Schwann cell morphology and

expression of the Schwann cell markers S-100, glial fibrillary acidic protein, and

low-affinity nerve growth factor receptor. Gas6 mRNA is present in both spinal

motor neurons and large neurons of the dorsal root ganglia, and neural injury has

been reported to upregulate Rse/Axl in the schwann cell. This is the first

demonstration of a potentially important biological role for the human Gas6/Rse-

Axl system.. EC 2.7.1.-; EC 2.7.1.-; 0; 0; 0; 0; 0; 0; 143891-42-3.

388. Lin, S. S.; Lee, T. H.; Wapner, K. L. Plantar forefoot ulceration with equinus

deformity of the ankle in diabetic patients: the effect of tendo-Achilles lengthening

and total contact casting. Orthopedics. 1996 May; 19(5): 465-75; ISSN: 0147-

7447.

UNITED-STATES. Between 1993 and 1995, 93 neuropathic diabetes mellitus

patients with foot ulcers underwent a total contact cast (TCC) protocol. A randomly

chosen group of 21 patients (Group I) demonstrated ulcer healing in a mean time of

43.5 days. Despite 9 weeks of TCC, 15 patients (Group II) with forefoot ulcers

failed to heal. Physical examination of Group I revealed plantarflexion/dorsiflexion

range of motion of the ankle of 33.8 degrees / 1.9 degrees compared to 32.3

degrees / -10.5 degrees of Group II, demonstrating an ankle equinus deformity and

limited joint motion. Group II patients underwent a correction of the equinus

deformity with percutaneous tendo-Achilles lengthening (TAL), followed by a

TCC. All but one ulcer (93.3%) healed within 39.4 days. Four (19.0%) ulcers

recurred (at the same site) in Group I, compared to none in Group II at the latest

follow up of 17.3 months. Surgical correction with percutaneous TAL and TCC

results in healing of forefoot ulcer and helps prevent ulcer recurrence.

389. Lipsitz, L. A. An 85-year-old woman with a history of falls [clinical conference].

JAMA. 1996 Jul 3; 276(1): 59-66; ISSN: 0098-7484.

UNITED-STATES.

390. Lisak, R. P. Arthritis associated with circulating immune complexes following

administration of intravenous immunoglobulin therapy in a patient with chronic

inflammatory demyelinating polyneuropathy. J-Neurol-Sci. 1996 Jan; 135(1): 85-

8; ISSN: 0022-510X.

NETHERLANDS. Intravenous immunoglobulin is viewed as a relatively safe

treatment for several neurologic disorders. I report arthritis associated with elevated

circulating immune complexes and abnormalities of serum complement components

which is a rare complication with modern preparations of immunoglobulin-

gamma.. 0; 0.

391. Litt, A. W.; Licata, P.; Knopp, E. A.; Thomasson, D. M. Water excitation MPRAGE

MRI of VII and VIII cranial nerves. J-Comput-Assist-Tomogr. 1996 Mar; 20(2):

194-200; ISSN: 0363-8715.

UNITED-STATES. OBJECTIVE: Our goal was to compare magnetization

prepared rapid gradient echo--water excitation (MRPRAGE-WE) with conventional

spin echo (CSE) in the evaluation of the VII and VIII cranial nerves. METHODS:

One hundred three consecutive patients with symptoms referable to the VII/VIII

nerves were studied with CSE T1 and MPRAGE-WE following intravenous

gadolinium contrast agent. Each right and left nerve pair was independently

evaluated for the presence of an enhancing mass and for visualization of the nerves.

RESULTS: On the CSE images, 26 definite and 2 possible lesions were identified,

whereas 28 definite and 2 possible abnormalities were seen on the MPRAGE-WE.

Four cases were better identified on the MPRAGE-WE and one better seen on the

CSE. This difference was not statistically significant (p = 0.19). CSE demonstrated

the nerves partially in 23 instances and completely in 6; MPRAGE-WE showed the

nerves partially in 35 and completely in 73. This was highly significant (p<0.001).

CONCLUSION: With equivalent or slightly improved lesion detection and better

visualization of the nerves, MPRAGE-WE may replace CSE in studying the

VII/VIII nerves.. 7732-18-5.

392. Liu, G. T.; Galetta, S. L.; Rorke, L. B.; Bilaniuk, L. T.; Vojta, D. D.; Molloy, P. T.;

Phillips, P. C.; Needle, M.; Duhaime, A. C.; Sutton, L. N.; Volpe, N. J.

Gangliogliomas involving the optic chiasm. Neurology. 1996 Jun; 46(6): 1669-73;

ISSN: 0028-3878.

UNITED-STATES. We report three patients with gangliogliomas involving the

optic chiasm via distinct mechanisms. The ganglioglioma in one patient likely

originated in the temporal lobe and spread medially to involve the chiasm, and

diffuse spinal cord dissemination also occurred. Chiasmal involvement in this

manner and dissemination at presentation are unusual for gangliogliomas. The

tumor in a second patient was intrinsic to the hypothalmus and chiasm, while in the

third patient, it involved both optic tracts, and a cyst compressed the chiasm

laterally. Two patients developed severe bilateral visual loss, while the other had a

stable bitemporal hemianopsia. Two patients received radiotherapy, but one

continued to lose vision. Although gangliogliomas rarely involve chiasm, the

mechanisms by which they produce chiasmal visual loss may be diverse, and the

long-term visual prognosis is variable.. 33419-42-0.

393. Lobzin, I. u. V.; Zagriadskii, P. V.; Bazhukov, O. A.; Kotel'nikov, S. A. [The

neurological complications of diphtheria]. Nevrologicheskie oslozhneniia difterii.

Voen-Med-Zh. 1996 Jan; 317(1): 47-52; ISSN: 0026-9050.

RUSSIA.

394. Logak, M.; Feve, A.; Samson, Y.; Guillard, A.; Rancurel, G. [Contribution of

position emission tomography in a case of Marchiafava Bignami disease: Morel's

laminar sclerosis?]. Apport de la tomographie par emission de positons dans un

cas de maladie de Marchiafava Bignami: sclerose laminaire de Morel? Rev-Neurol-

Paris. 1996 Jan; 152(1): 47-50; ISSN: 0035-3787.

FRANCE. We report the case of a 26-year old alcoholic woman who abruptly

presented with confusion associated with a major hypertonia. She evolved into a

chronically vegetative state. Magnetic resonance imaging (MRI) was consistent

with the diagnosis of Marchiafava Bignami disease. The corpus callosum first

presented an oedematous aspect, then a central atrophy with an axial band. The

clinical presentation and the functional imaging strongly suggest an association

between the Marchiafava Bignami disease and diffuse cortical lesions, such as the

laminar sclerosis of Morel.

395. Longshore, R. C.; O'Brien, D. P.; Johnson, G. C.; Grooters, A. M.; Kroll, R. A.

Dysautonomia in dogs: a retrospective study. J-Vet-Intern-Med. 1996 May; 10(3):

103-9; ISSN: 0891-6640.

UNITED-STATES. Dysautonomia was diagnosed in 11 young (median age, 14-

months), predominantly medium- to large-breed dogs from 1988 to 1995. Clinical

signs caused by autonomic dysfunction of the urinary, alimentary, and ocular

systems were most common. Dysuria, mydriasis, absence of pupillary light

reflexes, decreased tear production, dry mucous membranes, weight loss, and

decreased anal tone were present in over 75% of affected dogs. Ocular

pharmacological testing with a dilute (0.1%) solution of pilocarpine was used to

demonstrate iris sphincter receptor function in all dogs. A low-dose (0.0375 mg/kg

s.c.) bethanechol test and pharmacological testing with phenylephrine and

epinephrine also demonstrated cholinergic and adrenergic receptor function in 4

dogs. All dogs died or were euthanized as a results of autonomic dysfunction.

Neuronal depletion, with associated gliosis and minimal inflammation were noted

histologically in the autonomic ganglia of each dog. The pelvic, ciliary, celiac,

cranial cervical, and cranial and caudal mesenteric ganglia were affected in all dogs.

The cause of autonomic failure in these dogs was not determined.

396. Lopes, J.; LeGuern, E.; Gouider, R.; Tardieu, S.; Abbas, N.; Birouk, N.;

Gugenheim, M.; Bouche, P.; Agid, Y.; Brice, A. Recombination hot spot in a 3.2-

kb region of the Charcot-Marie-Tooth type 1A repeat sequences: new tools for

molecular diagnosis of hereditary neuropathy with liability to pressure palsies and

of Charcot-Marie-Tooth type 1A. French CMT Collaborative Research Group. Am-

J-Hum-Genet. 1996 Jun; 58(6): 1223-30; ISSN: 0002-9297.

UNITED-STATES. Charcot-Marie-Tooth type 1A (CMT1A) disease and

hereditary neuropathy with liability to pressure palsies (HNPP) are autosomal

dominant neuropathies, associated, respectively, with duplications and deletions of

the same 1.5-Mb region on 17p11.2-p12. These two rearrangements are the

reciprocal products of an unequal meiotic crossover between the two chromosome

17 homologues, caused by the misalignment of the CMT1A repeat sequences

(CMT1A-REPs), the homologous sequences flanking the 1.5-Mb CMT1A/HNPP

monomer unit. In order to map recombination breakpoints within the CMT1A-

REPs, a 12.9-kb restriction map was constructed from cloned EcoRI fragments of

the proximal and distal CMT1A-REPs. Only 3 of the 17 tested restriction sites were

present in the proximal CMT1A-REP but absent in the distal CMT1A-REP,

indicating a high degree of homology between these sequences. The

rearrangements were mapped in four regions of the CMT1A-REPs by analysis of

76 CMT1A index cases and 38 HNPP patients, who where unrelated. A hot spot of

crossover breakpoints, located in a 3.2-kb region, accounted for three-quarters of

the rearrangements, detected after EcoRI/SacI digestion, by the presence of 3.2-kb

and 7.8-kb junction fragments in CMT1A and HNPP patients, respectively. These

junction fragments, which can be detected on classical Southern blots, permit

molecular diagnosis. Other rearrangements can also be detected by gene dosage on

the same Southern blots.

397. Lopez, J. M.; Pego Reigosa, R.; Alonso Losada, G.; Lopez Facal, S.; Marin Sanchez,

M.; Martinez Muniz, A. Bilateral infarction of the rostral pontine tegmentum as a

cause of isolated bilateral supranuclear sixth nerve palsy related to hypertension

[letter]. J-Neurol-Neurosurg-Psychiatry. 1996 Feb; 60(2): 238-9; ISSN: 0022-

3050.

ENGLAND.

398. Lopez Dominguez, J. M.; Casado Chocan, J. L.; Blanco Ollero, A.; Robledo Strauss,

A.; Viader Farre, C.; Diaz Espejo, C. [The use of FD-6 monoclonal antibody in

diagnosing and detecting the carriers of familial amyloidotic polyneuropathy type

I]. Utilidad del anticuerpo monoclonal FD-6 para el diagnostico y la deteccion de

portadores en la polineuropatia amiloidotica familiar tipo I. Neurologia. 1996 Mar;

11(3): 93-8; ISSN: 0213-4853.

SPAIN. Familial amyloidotic polyneuropathy type I (FAF-I) is caused by a

specific genetic mutation that gives rise to a transthyretin anomaly whose presence

in serum constitutes the biochemical marker for this disease. We studied the serum

of 7 patients and 16 asymptomatic members of their immediate families using

ELISA with FD-6 monoclonal antibody to detect the transthyretin anomaly.

Positive results were found for the 7 patients, including the 2 patients whose

disease was apparently sporadic, and 12 carriers were detected among the family

members. This technique makes sural nerve biopsy unnecessary for establishing a

diagnosis in patients whose clinical signs are consistent with FAP-I. Asymptomatic

carriers are also detected, facilitating appropriate genetic counseling.. 0; 0.

399. Louis, D. S.; Calkins, E. R.; Harris, P. G. Carpal tunnel syndrome in the work place.

Hand-Clin. 1996 May; 12(2): 305-8; discussion 308-11; ISSN: 0749-0712.

UNITED-STATES. The management of patients with the CTS that appears to be

related to occupational tasks is a complex issue. At this time, there is no definite

evidence to show that any job is the sole cause of an individual worker's symptom

complex. Management of patients in this situation requires surgical restraint

coupled with an understanding of the worker's overall lifestyle. The most effective

outcomes for all concerned appear to occur when there is cooperation among the

patient, the physician, and the employer or his or her representatives. The recent

prospective study by MacDougal that attempted to correlate job classifications with

surgical outcome may be a very positive step in this direction.

400. Ludin, H. P.; Diener, H. C.; Mumenthaler, M. [Migraine: diagnosis, differential

diagnosis and treatment]. Migrane: Diagnose, Differentialdiagnose und

Behandlung. Schweiz-Med-Wochenschr. 1996 Jan 20; 126(3): 86-91; ISSN:

0036-7672.

SWITZERLAND. Headache takes a large place in daily medical practice, and it is

important to try and establish the most precise possible diagnosis. An outline of the

diagnosis and differential diagnosis of migraine is followed by a discussion of

interval drug treatment forms and counselling. Today's recommended treatment for

attacks and the commonest sources of error in migraine management are

summarized.. 0; 0.

401. Lueder, G. T.; Arnoldi, K. Does "Touching Four" on the Worth 4-dot test indicate

fusion in young children? A computer simulation. Ophthalmology. 1996 Aug;

103(8): 1237-40; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: "Touching four" dots on the Worth 4-dot test is

used sometimes as an indication of fusion in young children. The authors examined

the reliability of this test. METHODS: A computer simulation of the Worth 4-dot

test generated images representing fusion, suppression, and alternate fixation.

Sixteen children, ranging in age from 32 to 48 months, were examined using this

test. RESULTS: None of the children could accurately describe the images

verbally. Alternate fixation could not be distinguished from fusion by asking the

subjects to touch the dots. Monocular suppression was identified accurately in all

subjects. CONCLUSION: Touching four dots on the Worth 4-dot test does not

distinguish fusion from alternate fixation in children with normal ocular alignment.

This has important implications regarding the diagnosis of monofixation syndrome

and assessment of the response to a prism adaptation trial in young children.

402. Luetje, C. M. Cochlear implantation in a patient after removal of an acoustic neuroma

[letter; comment]. Arch-Otolaryngol-Head-Neck-Surg. 1996 Feb; 122(2): 205;

ISSN: 0886-4470.

Note: Comment on: Arch Otolaryngol Head Neck Surg 1995 Apr;121(4):465-8.

UNITED-STATES.

403. Luft, D. [Diabetic cardiovascular neuropathy]. Diabetische kardiovaskulare

Neuropathie. Z-Arztl-Fortbild-Jena. 1996 Apr; 90(2): 139-43; ISSN: 0044-2178.

GERMANY. Impairment of the autonomic nervous system is frequently detectable

in diabetic patients. Symptoms and signs are less often observed but if present may

be very important with regard to quality of life, metabolic control, and prognosis.

Currently used methods to detect disturbances of the autonomic innervation of the

cardiovascular system are easily performed, noninvasive, reliable and reproducible.

They are not very time consuming and require only standard technical equipment

allowing the quick identification of patients at risk. Damage of the cardiovascular

autonomic nerve function may explain apparently disparate complaints and signs,

i.e. orthostatic hypotension, painless myocardial ischemia, complications during

anesthesia, postprandial hypotension and development of the diabetic food

syndrome. The diagnosis of asymptomatic cardiovascular neuropathy should

always induce attempts to intensify diabetes treatment because better metabolic

control may improve or at least stop deterioration of nerve function.

404. Lupski, J. R. DNA diagnostics for Charcot-Marie-Tooth disease and related inherited

neuropathies [editorial; comment]. Clin-Chem. 1996 Jul; 42(7): 995-8; ISSN:

0009-9147.

Note: Comment on: Clin Chem 1996 Jul;42(7):1021-5.

UNITED-STATES. 9007-49-2.

405. Lupski, J. R. DNA diagnostics for Charcot-Marie-Tooth disease and related inherited

neuropathies [editorial; comment]. Clin-Chem. 1996 Jul; 42(7): 995-8; ISSN:

0009-9147.

Note: Comment on: Clin Chem 1996 Jul;42(7):1021-5.

UNITED-STATES. 9007-49-2.

406. Lynch, T.; Sima, A. A. Glial cytoplasmic inclusions in multiple system atrophy

[letter]. Ann-Neurol. 1996 Mar; 39(3): 416; ISSN: 0364-5134.

UNITED-STATES.

407. Macho Fernandez, J. M.; Manero Ruiz, F. J.; Soriano Godes, J. J.; Otero Sierra, C.;

Ros Mendoza, L. H. [Atypical algodystrophy of the knee: apropos 2 partial cases].

Algodistrofia atipica de rodilla: a proposito de dos casos con forma parcial. Rev-

Clin-Esp. 1996 May; 196(5): 306-9; ISSN: 0014-2565.

SPAIN. Algodystrophy is a common entity which may present in many clinical

contexts. Its early diagnosis and therapy are of great prognostic interest. Apart from

the typical complete forms there are some other atypical forms, such as partial knee

algodystrophy, of difficult diagnosis. Its inclusion in the differential diagnosis of

gonalgia occurring in patients attended under many medical specialties is therefore

necessary. Two atypical cases of knee algodystrophy in its partial form are

reported. One of these cases relapsed at the heterolateral knee after 18 months of the

initial presentation; this second episode was also a partial form, a fact which we

have not seen reported. The reported cases are here discussed and the scarce

literature is reviewed, commenting on the difficulty of the early diagnosis compared

with other entities which may mimic the clinical picture, radiological and scanning

features of algodystrophy, such as aseptic osteonecrosis or stress fracture. The

diagnostic algorithm is discussed, pointing to the usefulness of magnetic resonance

(MR) in difficult cases to rule out other entities which would entail different

therapeutic modalities.. 7440-26-8.

408. Mackey, D. A.; Oostra, R. J.; Rosenberg, T.; Nikoskelainen, E.; Bronte Stewart, J.;

Poulton, J.; Harding, A. E.; Govan, G.; Bolhuis, P. A.; Norby, S.; Bleeker

Wagemakers, E. M.; Savontaus, M. L.; Chan, C.; Howell, N. Primary pathogenic

mtDNA mutations in multigeneration pedigrees with Leber hereditary optic

neuropathy [letter]. Am-J-Hum-Genet. 1996 Aug; 59(2): 481-5; ISSN: 0002-9297.

UNITED-STATES. 0.

409. Mackinnon, S. E.; Novak, C. B. Evaluation of the patient with thoracic outlet

syndrome. Semin-Thorac-Cardiovasc-Surg. 1996 Apr; 8(2): 190-200; ISSN:

1043-0679.

UNITED-STATES. Clinical evaluation is paramount in making the diagnosis of

thoracic outlet syndrome. Complaints of paresthesia and numbness will relate to the

nerve compression component of thoracic outlet syndrome, whereas the pain

associated with this syndrome is largely caused by muscle imbalance in the neck,

shoulders, and upper back. Utilization of a pain evaluation scale assists in

assessing a functional overlay to the pain complaints. Detailed sensory testing at

rest and after provocation of the patient's symptoms with overhead activity will

assist in the diagnosis. Radiographic test results are frequently normal in this

patient population. By contrast, vascular testing results are frequently abnormal in a

normal patient population. Electrodiagnostic tests are useful in ruling out other,

more distal nerve entrapments.

410. MacLean, I. C. Electric studies as a prognostic factor in the surgical treatment of carpal

tunnel syndrome surgery [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 521-2;

ISSN: 0363-5023.

UNITED-STATES.

411. Magliulo, G.; Cristofari, P.; Petti, R.; Marcotullio, D. Facial neuroma and magnetic

resonance imaging. An-Otorrinolaringol-Ibero-Am. 1996; 23(2): 147-52; ISSN:

0303-8874.

SPAIN. The aim of this paper is to present a new case of facial neuroma. Its

clinical appearance was uncommon, displaying symptoms characterized by sudden

facial paralysis. Diagnostic armamentarium is discussed emphasizing the

importance of magnetic resonance imaging (MRI). Therapeutical strategy with the

techniques of facial rehabilitation are contemplated.

412. Mainous, AG 3rd; Nelson, K. R. How often are preoperative electrodiagnostic studies

obtained for carpal tunnel syndrome in a Medicaid population? [letter]. Muscle-

Nerve. 1996 Feb; 19(2): 256-7; ISSN: 0148-639X.

UNITED-STATES.

413. Maisonobe, T.; Chassande, B.; Verin, M.; Jouni, M.; Leger, J. M.; Bouche, P.

Chronic dysimmune demyelinating polyneuropathy: a clinical and

electrophysiological study of 93 patients. J-Neurol-Neurosurg-Psychiatry. 1996

Jul; 61(1): 36-42; ISSN: 0022-3050.

ENGLAND. OBJECTIVES--To identify clinical, electrophysiological, and

immunological characteristics of chronic immune demyelinating polyneuropathy to

define for each group the appropriate therapeutic strategies. METHODS--The

clinical and electrophysiological data and the response to treatment of 93 patients

with an acquired chronic dysimmune demyelinating polyneuropathy (CDDP)

studied over a period of 10 years were reviewed. Two groups were identified:

group 1, comprising 64 patients with an idiopathic CDDP, of whom 13 had serum

monoclonal or polyclonal gammopathy without detectable antibodies directed

against the "myelin associated glycoprotein" (MAG), and group 2, comprising 29

patients with an IgM monoclonal gammopathy of undetermined significance

(MGUS) with antibodies binding to the MAG. RESULTS--Group 1 patients had

either a progressive or relapsing course. The relapsing course had more

pronounced distal slowing of motor conduction velocity. In group 1, there were no

significant clinical or electrophysiological differences between patients with or

without gammopathy. Patients with anti-MAG antibody (group 2) differed

significantly from group 1 patients, especially on the basis of electrophysiological

results. They had a more pronounced slowing of peroneal motor nerve conduction

velocity, a lower frequency of conduction block, and a distal accentuation of

conduction slowing, distinguishing them from those with idiopathic CDDP,

Charcot-Marie-Tooth polyneuropathy type 1A, and control subjects.

CONCLUSION--The idiopathic CDDP group is heterogeneous with probably

different subgroups. Patients with IgM MGUS polyneuropathy and anti-MAG

antibodies have characteristics which distinguish them significantly from other

CDDP and suggest different immune mechanisms and responses to treatment.. 0;

0; 0.

414. Makhmudov, U. B.; Kozlov, A. V.; Korshunov, A. G.; Turkin, A. M.; Ivanov, S. L.

[Malignant neurinoma of the gasserian ganglion]. Zlokachestvennaia nevrinoma

gasserova uzla. Zh-Vopr-Neirokhir-Im-N-N-Burdenko. 1996 Apr; (2): 33-5;

ISSN: 0042-8817.

RUSSIA.

415. Malessa, R.; Agelink, M.; Himmelmann, M.; Kloss, T.; Mertins, L.; Brockmeyer, N.

H. Nerve conduction changes in asymptomatic HIV-1 seropositive individuals in

the absence of other risk factors for neuropathy. Electromyogr-Clin-Neurophysiol.

1996 Jan; 36(1): 3-8; ISSN: 0301-150X.

BELGIUM. The major problem in determining the role of HIV-1 infection in the

pathogenesis of peripheral neuropathy is the difficulty in separating possible effects

of confounding factors such as other infections, malnutrition, neurotoxic

medication, drug abuse and antiretroviral treatment. We therefore selected 28

neurologically asymptomatic HIV-seropositive homosexual men (category A, CDC

1993) without other recognized reasons for peripheral nerve disease and 20 age,

sex and height matched healthy controls for a prospective nerve conduction study.

Nine (32%) HIV-seropositive patients had single nerve conduction abnormalities

and 2 (7%) had at least two abnormalities considered to be indicative of subclinical

neuropathy. Even patients with normal CD4 cell counts showed significantly lower

mean sural nerve conduction velocities and higher tibial distal motor latencies

compared to controls (ANOVA; p < 0.05). There was an overall trend toward more

frequent nerve conduction changes in subgroups with abnormal CD4 cell counts,

lymphocyte responsiveness or beta 2-microglobulin levels. Using strict selection

criteria subclinical nerve conduction changes can be found even in the absence of

symptomatic HIV-disease or potential confounding factors suggesting that HIV-1

plays a direct role in the pathogenesis of associated peripheral nervous system

disease.

416. Malessa, R.; Agelink, M.; Himmelmann, M.; Kloss, T.; Mertins, L.; Brockmeyer, N.

H. Nerve conduction changes in asymptomatic HIV-1 seropositive individuals in

the absence of other risk factors for neuropathy. Electromyogr-Clin-Neurophysiol.

1996 Jan; 36(1): 3-8; ISSN: 0301-150X.

BELGIUM. The major problem in determining the role of HIV-1 infection in the

pathogenesis of peripheral neuropathy is the difficulty in separating possible effects

of confounding factors such as other infections, malnutrition, neurotoxic

medication, drug abuse and antiretroviral treatment. We therefore selected 28

neurologically asymptomatic HIV-seropositive homosexual men (category A, CDC

1993) without other recognized reasons for peripheral nerve disease and 20 age,

sex and height matched healthy controls for a prospective nerve conduction study.

Nine (32%) HIV-seropositive patients had single nerve conduction abnormalities

and 2 (7%) had at least two abnormalities considered to be indicative of subclinical

neuropathy. Even patients with normal CD4 cell counts showed significantly lower

mean sural nerve conduction velocities and higher tibial distal motor latencies

compared to controls (ANOVA; p < 0.05). There was an overall trend toward more

frequent nerve conduction changes in subgroups with abnormal CD4 cell counts,

lymphocyte responsiveness or beta 2-microglobulin levels. Using strict selection

criteria subclinical nerve conduction changes can be found even in the absence of

symptomatic HIV-disease or potential confounding factors suggesting that HIV-1

plays a direct role in the pathogenesis of associated peripheral nervous system

disease.

417. Malessa, R.; Heimbach, M.; Brockmeyer, N. H.; Hengge, U.; Rascher, W.; Michel,

M. C. Increased neuropeptide Y-like immunoreactivity in cerebrospinal fluid and

plasma of human immunodeficiency virus-infected patients: relationship to HIV

encephalopathy. J-Neurol-Sci. 1996 Mar; 136(1-2): 154-8; ISSN: 0022-510X.

NETHERLANDS. Neuropeptide Y (NPY) is one of the most abundant and

phylogenetically best conserved peptides in the mammalian central and peripheral

nervous system where it plays an important role in the regulation of cardiovascular,

metabolic, endocrine, immunological and cognitive functions. In a prospective

study we determined neuropeptide Y-like immunoreactivity (NPY-LI) in

cerebrospinal fluid (CSF) and plasma of 95 HIV-seropositive (n = 49) or

seronegative (n = 46) patients who underwent diagnostic CSF examination. CSF

and plasma NPY-LI but not noradrenaline concentrations were higher in

seropositive than in seronegative patients indicating that raised levels of NPY-LI

did not result from a non-specific activation of the sympathetic nervous system.

Increased CSF NPY-LI was positively correlated with the degree of HIV

encephalopathy (P < 0.01, Kruskal-Wallis test). Inflammatory disorders of the

central nervous system and dementia due to other causes in HIV-seronegative

patients were not associated with increased CSF NPY-LI. Our data suggest that

increased CSF NPY-LI is a relatively specific phenomenon of HIV encephalopathy

and may be involved in the pathogenesis of HIV-related neurological dysfunction.

The links between retroviral infection and increased expression of neuropeptide Y

and their pathophysiological implications remain to be determined.. 0; 51-41-2.

418. Marangos, N. Hearing loss in multiple sclerosis: localization of the auditory pathway

lesion according to electrocochleographic findings. J-Laryngol-Otol. 1996 Mar;

110(3): 252-7; ISSN: 0022-2151.

ENGLAND. Multiple sclerosis is known to affect the myelin of the auditory

pathway resulting in acute hearing loss. Two cases of sudden deafness due to

multiple sclerosis have been evaluated by conventional audiometry, brainstem

auditory evoked response audiometry and transtympanic electrocochleography. The

abnormalities of the compound action potential in both patients (enhanced latency,

abnormal adaptation using fast stimulus rate) and the normal receptor potentials

(cochlear microphonic, summating potential), as well as the absence of brainstem

responses suggest a disturbance of synchronization at the level of the first auditory

neurone. The electrocochleography provides valuable information for the

topodiagnosis of this and other neural hearing losses, especially in the absence of

reliable brainstem responses.

419. Marcelli, C.; Perennou, D.; Cyteval, C.; Leray, H.; Lamarque, J. L.; Mion, C.;

Simon, L. Amyloidosis-related cauda equina compression in long-term

hemodialysis patients. Three case reports. Spine. 1996 Feb 1; 21(3): 381-5; ISSN:

0362-2436.

UNITED-STATES. STUDY DESIGN: These case reports illustrate the neurologic

manifestations due to beta 2 microglobulin amyloid deposition at the lumbar spine

level in long-term hemodialysis patients. OBJECTIVE: Radiologic investigations

suggested the amyloid origin of extradural soft tissue deposition, which was

confirmed by histologic examination after surgical excision. SUMMARY OF

BACKGROUND DATA: Although cervical myelopathy is a recently recognized

complication of long-term dialysis-related beta 2 microglobulin amyloidosis,

neurologic manifestations due to amyloid deposition at the lumbar spine level have

rarely been reported. METHODS: Three case reports of cauda equina compression

in long-term hemodialysis patients are presented. Follow-up radiography,

computed tomography, and magnetic resonance imaging were performed and

patients underwent surgical decompression of the thecal sac. RESULTS: In two

patients, the compression resulted from the development of a destructive

spondylarthropathy, and from the infiltration of extradural spaces and ligaments by

an abnormal soft tissue. The third patient had lumbar spinal stenosis due to multiple

disc protrusion and to hypertrophy of facet joints and ligamentum flavum.

Multilevel laminectomies enabled excision of an abnormal fibrous tissue

responsible for the thecal sac compression. Histologic examination of the excised

fibrous tissues disclosed amyloid deposits in intervertebral discs, apophysial joints,

and ligaments. CONCLUSIONS: In long-term hemodialysis patients, cauda equina

compression may develop as the consequence of beta 2 microglobulin amyloid

deposition in lumbar intervertebral discs, facet joints, and ligaments. Magnetic

resonance imaging is well suited to show the extent of the compression and

supports the argument for the amyloid origin of extradural soft tissue.

420. Marinelli, G.; Pajewski, L. A.; Porto, C.; Cerone, G. Network modelling applied to

temporal analysis of diagnostic processes. Ann-Ig. 1996 Mar; 8(2): 209-12; ISSN:

1120-9135.

ITALY.

421. Martin Martin, C.; Labella Caballero, T.; Frade Gonzalez, C.; Lozano Ramirez, A.

Tumor-associated paralysis of the mastoid portion of the facial nerve: report of

three unusual cases. Ear-Nose-Throat-J. 1996 Jun; 75(6): 365-8, 370, 372; ISSN:

0145-5613.

UNITED-STATES. Tumors of the mastoid portion of the facial nerve are rare and

often are misdiagnosed because they have subtle clinical manifestations. We have

described three cases of tumor affecting the mastoid portion of the facial nerve seen

in our hospital. In all cases, diagnosis proved problematic. Routine and careful use

of imaging techniques and a certain amount of intuition are necessary to reach the

correct diagnosis. Facial paralysis diagnosis requires histopathological examination

following mastoidectomy.

422. Martinelli, P.; Patuelli, A.; Minardi, C.; Cau, A.; Riviera, A. M.; Dal Pozzo, F.

Neuromyotonia, peripheral neuropathy and myasthenia gravis. Muscle-Nerve.

1996 Apr; 19(4): 505-10; ISSN: 0148-639X.

UNITED-STATES. A patient with neuromyotonia, peripheral neuropathy and

myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed

continuous muscle discharges of motor unit action potentials (MUAPs) in duplets

and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies

revealed mild axonal and demyelinating peripheral neuropathy. Plasma exchange

was followed by disappearance of clinical and electrophysiological signs of

neuromyotonia and MG, as well as peripheral neuropathy.

423. Maruo, T.; Iwashige, H.; Kubota, N.; Sakaue, T.; Ishida, T.; Honda, M.; Nemoto,

Y.; Usui, C. Results of surgery for paralytic exotropia due to oculomotor palsy.

Ophthalmologica. 1996; 210(3): 163-7; ISSN: 0030-3755.

SWITZERLAND. In 138 cases of paralytic exotropia due to oculomotor palsy,

transposition of the superior oblique muscle and resection of the medial rectus

muscle were carried out. Surgery was performed with or without recession of the

lateral rectus muscle. The long-term prognosis for 4 years or more was observed in

35 cases. We found that the same results could be obtained by selecting

transposition of the superior oblique muscle in cases of complete palsy and

resection of the medical rectus muscle in cases of incomplete palsy. There was no

benefit in combining resection of the medial rectus muscle when performing the

transposition of the superior oblique muscle. Regardless of which method was

used, a combination with recession of the lateral rectus muscle greatly improved the

effectiveness of the procedure.

424. Masdeu, J. C.; Moreira, J.; Trasi, S.; Visintainer, P.; Cavaliere, R.; Grundman, M.

The open ring. A new imaging sign in demyelinating disease. J-Neuroimaging.

1996 Apr; 6(2): 104-7; ISSN: 1051-2284.

UNITED-STATES. Because demyelinating disease of the brain occasionally

presents with large ring-enhancing lesions on computed tomography (CT) scans

and magnetic resonance images (MRIs), the authors sought to determine whether

the ring pattern differed from that found in other common brain lesions with ring

enhancement. Published MRI and CT scans of patients with adrenoleukodystrophy

(23), and multiple sclerosis or similar demyelinating disorders (21), as well as a

variety of tumors (44) and infections (44) matched to the demyelinating lesions by

year of publication, in which ring enhancement was evident, were photographed.

Photographs without diagnostic identification were presented randomly to two

independent observers. The observers rated the contrast enhancement pattern as (1)

open ring, with enhancement in the border of the lesion abutting the white matter;

(2) closed ring; or (3) uncertain. For all diagnostically certain cases (n = 112),

inter-rater agreement was excellent (kappa = 0.75). As an average of the two

reviewers, scans for 11 of 132 cases were read as uncertain; 89% of

adrenoleukodystrophy cases, 41% of the multiple sclerosis cases, 3% of tumors,

and 9% of infections were classified as having the open-ring pattern. Overall, 66%

of demyelinating lesions had an open-ring pattern compared with 7% of the non-

demyelinating lesions (chi2 = 41.2, p < 0.0001). An open-ring pattern of

enhancement is more likely to be associated with demyelinating lesions than with

nondemyelinating lesions.

425. Mass, E.; Wolff, A.; Gadoth, N. Increased major salivary gland secretion in familial

dysautonomia. Dev-Med-Child-Neurol. 1996 Feb; 38(2): 133-8; ISSN: 0012-

1622.

ENGLAND. Familial dysautonomia (FD), an autosomal recessive peripheral

nervous system disorder, affects almost exclusively children of Jewish Ashkenazi

origin and causes profound generalized autonomic dysfunction. Excessive drooling

is frequent and is traditionally attributed to swallowing difficulties. Although true

hypersalivation has been postulated, no quantitative assessment of the salivary

secretion rate has yet been reported. The authors determined this rate in 13 children

with FD and 28 healthy controls. Resting parotid, submandibular/sublingual and

unstimulated whole salivary secretion rates were significantly elevated in children

with FD. The known relation of salivary function with age was found in controls

only. This apparently major contribution of salivary hyperfunction to excessive

drooling in FD may be attributable to salivary gland denervation supersensitivity, as

this mechanism is present in the cardiovascular system and the pupil in FD.

426. Masur, H.; Schulte Oversohl, U.; Papke, K.; Oberwittler, C.; Vollmer, J. Involvement

of the autonomic nervous system in patients with syringomyelia--a study with the

sympathetic skin response. Electromyogr-Clin-Neurophysiol. 1996 Jan; 36(1): 43-

7; ISSN: 0301-150X.

BELGIUM. In order to determine the function of the autonomic nervous system in

syringomyelia, the sympathetic skin response (SSR) was performed in 13 patients

with syringomyelia and 20 healthy controls. SSR was recorded from both palms

and soles. In patients with syringomyelia, we found absent responses, prolonged

latencies and reduced amplitudes. SSRs could be recorded in 15 out of the

examined 26 upper extremities. The latencies were prolonged in 12 of these cases.

In the lower limbs, 11 SSRs could be obtained. In 4 of these cases the latencies

were prolonged. The SSR latencies recorded from the palms and soles were both

significantly prolonged (p < 0.05) and the amplitudes were reduced (p < 0.05) as

compared to normal persons. Our data strongly suggest involvement of the

autonomic nervous system in syringomyelia as assessed by the SSR response (in

upper and lower extremities). In our patients, the extent of autonomic dysfunction

was not related to the stage or the duration of disease.

427. Mathew, N. T.; Ravishankar, K.; Sanin, L. C. Coexistence of migraine and idiopathic

intracranial hypertension without papilledema. Neurology. 1996 May; 46(5): 1226-

30; ISSN: 0028-3878.

UNITED-STATES. Eighty-five patients with refractory transformed migraine type

of chronic daily headache (CDH) had spinal tap as a part of diagnostic work-up.

Twelve had increased intracranial pressure without papilledema, transient visual

obscurations, or visual field defects. The headache profile of these 12 patients was

not different from that of transformed migraine type of CDH. Acute headache

exacerbations responded to specific antimigraine agents such as ergotamine,

dihydroergotamine (DHE), and sumatriptan, whereas prophylactic antimigraine

medications were only partially helpful. Addition of agents such as acetazolamide

and furosemide, after the diagnosis of increased intracranial pressure, resulted in

better control of symptoms. These observations suggest a link between migraine

and idiopathic intracranial hypertension that needs further research. In refractory

CDH with migrainous features, a spinal tap to exclude coexistent idiopathic

intracranial hypertension without papilledema may be indicated.. 0; 103628-46-2;

113-15-5; 511-12-6; 54-31-9; 59-66-5.

428. Mathias, C. J. Disorders affecting autonomic function in parkinsonian patients. Adv-

Neurol. 1996; 69: 383-91; ISSN: 0091-3952.

UNITED-STATES. This chapter deals with certain aspects of autonomic

dysfunction in parkinsonian patients. It provides a classification of autonomic

disorders and a brief description of autonomic manifestations. An outline of

autonomic investigations, including those to diagnose the Shy-Drager syndrome

(multiple system atrophy), is provided. It concludes with comments on terminology

and on whether the parkinsonian syndromes with autonomic failure comprise single

or multiple entities.

429. Matsuda, M.; Ikeda, S.; Sakurai, S.; Nezu, A.; Yanagisawa, N.; Inuzuka, T.

Hypertrophic neuritis due to chronic inflammatory demyelinating

polyradiculoneuropathy (CIDP): a postmortem pathological study. Muscle-Nerve.

1996 Feb; 19(2): 163-9; ISSN: 0148-639X.

UNITED-STATES. A postmortem pathological study of a 65-year-old woman

with hypertrophic neuritis associated with hand tremor and limb ataxia is described.

There were many onion bulbs and loss of myelinated nerve fibers in the peripheral

nerves, including the facial and subserosal visceral nerves. The hypertrophic

neuritis was caused by chronic inflammatory demyelinating neuropathy (CIDP), in

which interstitial amorphous substances in the endoneurium and onion bulb

formation might contribute to nerve swelling. We speculate that visceral autonomic

nerves as well as somatic peripheral nerves are involved in patients with a long

clinical CIDP course and that peripheral nerve pathology in this disorder shows

more heterogeneous changes than previously recognized.

430. Matsumura, H.; Jimbo, Y.; Watanabe, K. Haemodynamic changes in early phase

reflex sympathetic dystrophy. Scand-J-Plast-Reconstr-Surg-Hand-Surg. 1996 Jun;

30(2): 133-8; ISSN: 0284-4311.

SWEDEN. We studied six patients with early phase reflex sympathetic dystrophy

(RSD). Osteoporotic changes were noted in the bones of the affected limb. Despite

higher temperatures indicated by thermography, laser speckle image sensing

showed no increase in blood flow on the skin surface. Digital subtraction

angiography showed arteriovenous shunting or increased density of perfused

vessels. Based on these results, we speculate that in RSD persistent vascular

contraction caused by pain leads to the formation of arteriovenous shunts in the

affected limb with an ischaemic state in the peripheral subcutaneous tissue which is

indicated by pain and swelling.

431. Matsumura, J. S.; Yao, J. S. Thoracic outlet arterial compression: clinical features and

surgical management. Semin-Vasc-Surg. 1996 Jun; 9(2): 125-33; ISSN: 0895-

7967.

UNITED-STATES.

432. Matsuoka, K. [Progress on diagnosis and therapy of diabetic neuropathies]. Nippon-

Naika-Gakkai-Zasshi. 1996 Apr 10; 85(4): 551-5; ISSN: 0021-5384.

JAPAN. EC 1.1.1.21.

433. Matsuyama, T.; Shimomura, T.; Kawata, K.; Ohnishi, H. [Huge skull base

neurofibroma: case report]. No-Shinkei-Geka. 1996 Jun; 24(6): 563-6; ISSN:

0301-2603.

JAPAN. A case of a huge skull base neurofibroma completely resected by a

combined subtemporal-infratemporal and basal subfrontal approach is reported. A

32-year-old female complained of left facial paresthesia and deformity since several

months before. Neurological examination revealed hypesthesia in the distribution of

the left trigeminal nerve second branch. CT and MRI images showed a huge tumor

in the infratemporal fossa, parapharyngeal space and middle fossa extending into

the cavernous sinus, compressing the left temporal lobe and distorting the left

hypothalamus and the brain stem. The patient tolerated trial balloon occlusion of the

left internal carotid artery for 45 minutes. Transmaxillary biopsy revealed the tumor

to be a neurofibroma. Through a combined basal subfrontal and a subtemporal-

infratemporal approach in two staged operations, total tumor resection was

performed. This tumor originated at the second branch of the trigeminal nerve. We

recommend these combined approaches and two staged operations for such a huge

skull base tumor as presented in this case.

434. Mauldin, C. C. Diagnosis of thoracic outlet syndrome in the emergency department

[letter]. South-Med-J. 1996 Sep; 89(9): 933; discussion 933-4; ISSN: 0038-4348.

UNITED-STATES.

435. Maurer, J.; Requardt, H.; Herrling, T.; Knollmann, F. D.; Schedel, H.; Vogl, T. J.;

Felix, R. [Theoretical principles and technical realization of high resolution nuclear

magnetic resonance tomography with the example of a dedicated coil system].

Theoretische Grundlagen und technische Realisation der hochauflosenden

Kernspintomographie (KST) am Beispiel eines dedizierten Spulensystems.

Bildgebung. 1996 Mar; 63(1): 40-6; ISSN: 1012-5655.

SWITZERLAND. The theory of high-resolution magnetic resonance imaging

(MRI) and the physical properties of a dedicated coil system with its clinical

application are reviewed. To evaluate the spatial resolution of the system, a

phantom sample was depicted by a transverse T1-weighted sequence (time of

repetition 500 ms, time of echo 25 ms, 256 x 256 matrix, 3 acquisitions, field of

view 25 mm2). Relative signal intensity decrease was less using the 5-cm coil, as

signal intensity field distribution depends on coil diameter. The phantom appeared

as an attainable resolution of 100-microns pixel width using the 2.5-cm coil. For

the 5-cm coil the pixel width was 200 microns, not accomplishing clear resolution

of the phantom. Coil head choice depends on the anatomic depth of the target

organ. Work-up of the skin and musculoskeletal lesions is the main indication for

high-resolution MRI using surface coils.

436. Mautner, V. F. Author's correction. Spinal tumors in patients with neurofibromatosis

type 2: MR imaging study of frequency, multiplicity and variety [letter]. AJR-Am-

J-Roentgenol. 1996 May; 166(5): 1231; ISSN: 0361-803X.

UNITED-STATES.

437. Mautner, V. F.; Lindenau, M.; Baser, M. E.; Hazim, W.; Tatagiba, M.; Haase, W.;

Samii, M.; Wais, R.; Pulst, S. M. The neuroimaging and clinical spectrum of

neurofibromatosis 2. Neurosurgery. 1996 May; 38(5): 880-5; discussion 885-6;

ISSN: 0148-396X.

UNITED-STATES. Neurofibromatosis 2 (NF2) is an autosomal dominant disease

predisposing to multiple tumors of the central and peripheral nervous system.

Bilateral vestibular schwannomas are the hallmark of the disease. To define the

clinical spectrum of the disease, we performed gadolinium-enhanced magnetic

resonance imaging of the brain and spine as well as neurological, dermatological,

and ocular examinations in 48 patients with NF2 diagnosed with the National

Institutes of Health diagnostic criteria. Patients were ascertained from patient

workshops and publications and from referral as a result of vestibular schwannoma

surgery. Vestibular schwannomas were found in 46 patients (96%, 43 bilateral and

3 unilateral), spinal tumors were found in 43 (90%), posterior subcapsular cataracts

were found in 30 (63%), meningiomas were found in 28 (58%), and trigeminal

schwannomas were found in 14 (29%). The presenting symptoms included hearing

loss or tinnitus in 15 patients (31%), multiple or nonspecific symptoms in 15

(31%), skin tumors in 12 (25%), and ocular symptoms in 6 (13%). When the

complete spine was imaged, spinal tumors were more common in patients with

NF2 than has previously been reported. This is a noteworthy finding, because

spinal tumors are a major cause of NF2 morbidity and mortality.

438. Maxted, G. H. Care of diabetic foot lesions [letter; comment]. Am-Fam-Physician.

1996 Jul; 54(1): 70; ISSN: 0002-838X.

Note: Comment on: Am Fam Physician 1996 Feb 1;53(2):601-11, 615-6.

UNITED-STATES.

439. McAuley, J. R.; Dickman, J. D.; Mustain, W.; Anand, V. K. Positional nystagmus in

asymptomatic human subjects. Otolaryngol-Head-Neck-Surg. 1996 Apr; 114(4):

545-53; ISSN: 0194-5998.

UNITED-STATES. Nystagmus produced by static placement of the head in

different orientations is termed positional nystagmus and is known to occur in

human subjects who are free of vestibular symptoms. This study provides

quantitative data for horizontal positional nystagmus occurrence in 49 normal

human subjects, in whom the number of nystagmus beats, the slow-phase velocity

of each beat, and distribution statistics were determined. A metric for the possible

differentiation of physiologic positional nystagmus from pathologic nystagmus is

described.

440. McBrien, F.; Spraggs, P. D.; Harcourt, J. P.; Croft, C. B. Abductor vocal fold palsy

in the Shy-Drager syndrome presenting with snoring and sleep apnoea. J-Laryngol-

Otol. 1996 Jul; 110(7): 681-2; ISSN: 0022-2151.

ENGLAND. The case of an elderly male with Shy-Drager syndrome is presented.

His presentation to the Sleep Clinic for assessment of snoring illustrates bilateral

abductor vocal fold palsy as a rare presentation of the syndrome. This case

emphasizes the need for thorough investigation of all patients with sleep-related

breathing disorders with video and sound recordings prior to anaesthesia and

surgery.

441. McCarthy, M. MRI simplifies diagnosis of peripheral nerve lesions [news]. Lancet.

1996 Sep 7; 348(9028): 674; ISSN: 0140-6736.

ENGLAND.

442. McDougall, A. J.; McLeod, J. G. Autonomic neuropathy, I. Clinical features,

investigation, pathophysiology, and treatment. J-Neurol-Sci. 1996 May; 137(2):

79-88; ISSN: 0022-510X.

NETHERLANDS. Autonomic dysfunction is a common complication of

peripheral neuropathies. It is often of little clinical importance, but some conditions

may cause profound disturbance of autonomic function, including postural

hypotension, impotence and impairment of heart rate and bladder and bowel

control. Autonomic function can be evaluated by a number of investigations, some

of which can be performed in a neurophysiology laboratory. Diseases that primarily

affect small nerve fibres or cause acute demyelination of small myelinated fibres are

most likely to cause autonomic dysfunction. Management includes treating the

underlying cause and symptomatic therapy.. 0.

443. Melin, J.; Usenius, J. P.; Fogelholm, R. Left ventricular failure and pulmonary edema

in acute multiple sclerosis. Acta-Neurol-Scand. 1996 May; 93(5): 315-7; ISSN:

0001-6314.

DENMARK. We describe a young woman who developed left ventricular failure

and pulmonary edema during fulminant onset of multiple sclerosis. One of the

numerous plaques was located in the left inferior cerebellar peduncle near the dorsal

motor vagal and solitary tract nuclei. Within two days pulmonary edema had

cleared, and within three days left ventricular function was normal. To our

knowledge, left ventricular failure has not earlier been verified in acute multiple

sclerosis.

444. Mensah, A.; Ba, M.; Gueye, S. M.; Sylla, C.; Ndoye, A. K.; Moreira, P.; Fall, A.;

Labou, I. [Neurologic aspects of vesico-vaginal fistula of obstetrical origin]. Les

aspects neurologiques de la fistule vesico-vaginale d'origine obstetricale. Prog-

Urol. 1996 Jun; 6(3): 398-402; ISSN: 1166-7087.

FRANCE. OBJECTIVE: To study the electromyographic alterations of the urethral

sphincter, perineal muscles and lower limbs observed in women with obstetric

vesicovaginal fistula. METHODS: We compared a group of 22 women with

obstetric vesicovaginal fistula with a control group of 10 women without fistula.

Each woman underwent a neurological examination of the perineum and lower

limbs and electromyography of the lower limbs and urethral sphincter. According

to the classification proposed by MENSAH, the fistulas were classified as simple

(54.5%), complex (31.8%) and complicated (13.6%). RESULTS: Twenty women

with VVF presented a peripheral nerve lesion. Five of these patients had a clinical

and electromyographic lesion; while the other fifteen patients had a subclinical

lesion. 68.18% of the fistula patients presented a clinical neuropathy in the

perineum. This lesion was detected on electromyography in all patients with fistula.

The severity of this denervation lesion ranged from moderate (36.37%) to severe

(63.63%). No neurological lesions were detected in the control group. The degree

of denervation has more severe in young women (21-25 years) and in primiparous

women. In contrast, the severity of denervation did not appear to be related to the

history of the fistula. The risk of treatment failure increased proportionally to the

severity of denervation. 73% of fistulas with severe denervation remained

"unsuturable", or required multiple operations with disappointing results in the

form of persistent vesicosphincteric dysfunction. CONCLUSION: Although the

psychosocial impairment and upper urinary tract repercussions have been well

evaluated, the peripheral neurological lesion has always been underestimated in the

management of obstetric vesicovaginal fistulas. The authors consider that the fistula

is only the apparent manifestation of "neurovesical damage", which remains the

decisive factor in the prognosis and which largely explains the high frequency of

treatment failure.

445. Mierzewska, H. [Mitochondrial diseases. Part II -- diagnosis and detailed review].

Choroby mitochondrialne. Czesc II -- Diagnostyka oraz czesc szczegolowa.

Neurol-Neurochir-Pol. 1996 Mar; 30(2): 279-92; ISSN: 0028-3843.

POLAND. The diagnosis of mitochondrial disease (MD) requires biochemical

investigations of body fluids, particularly with respect to lactate and pyruvate

elevation. Cardiological and ophtalmological examinations may also be helpful, as

well as cerebral imaging. Tissue sampling, especially muscle biopsy and DNA

analysis are important. Neuropathological findings consist of nonspecific

spongiform lesions of gray matter, neuronal loss and sometimes demyelination

with different topographic patterns. Some, more frequently occurring mitochondrial

diseases and therapeutic attempts are reviewed.. EC 1.8.1.4; 0.

446. Miki, A.; Nakajima, T.; Takagi, M.; Shirakashi, M.; Abe, H. Detection of visual

dysfunction in optic atrophy by functional magnetic resonance imaging during

monocular visual stimulation. Am-J-Ophthalmol. 1996 Sep; 122(3): 404-15; ISSN:

0002-9394.

UNITED-STATES. PURPOSE: To evaluate functional magnetic resonance

imaging as an objective method for detecting visual dysfunction in various

ophthalmologic disorders involving the optic nerve and the chiasm. METHODS:

We performed functional magnetic resonance imaging during monocular visual

stimulation on seven patients with visual field loss caused by lesions of the optic

nerve and the chiasm and on three normal control subjects with no visual field loss.

We correlated static threshold perimetry in the seven patients with the results of

functional magnetic resonance imaging. RESULTS: In the three normal control

subjects, we found good intrasubject similarity in areas of bilateral occipital lobe

activation between monocular stimulation of the right and left eyes. In the patients

with unilateral optic neuropathy, including glaucoma, stimulation of the affected

eye induced no activation of the primary visual cortex in the portion corresponding

to the central visual field defects and reduced activity of the associated visual

cortex. In the patients with chiasmal compression, monocular stimulation resulted

in a marked asymmetry of activation in the primary visual cortex, which

corresponded to the visual field abnormality. CONCLUSIONS: Functional

magnetic resonance imaging appears to be useful in confirming the clinical

diagnosis of optic atrophy because it can objectively disclose visual field loss, even

a small defect such as central scotoma.

447. Misra, S.; Ament, M. E. Orofacial lesions in Crohn's disease. Am-J-Gastroenterol.

1996 Aug; 91(8): 1651-3; ISSN: 0002-9270.

UNITED-STATES. We describe a female patient with Crohn's disease who

presented with longstanding gingival swelling. She went into remission after

surgery, but had labial involvement while in remission. A review of the literature is

presented with special reference to the clinical characteristics of orofacial Crohn's

disease and Melkersson-Rosenthal syndrome, which can mimic such presentation

of Crohn's disease.

448. Mod, G.; Kis, E.; Schneider, I.; Varga, S. M. [Glossopharyngeal neuralgia with

syncope]. Glossopharyngeus neuralgia syncopeval. Orv-Hetil. 1996 Jun 2;

137(22): 1199-203; ISSN: 0030-6002.

HUNGARY. Glossopharyngeal neuralgia an uncommon craniofacial pain

syndrome. An association with syncope is even less common. The authors give a

short case report of 74-year old woman, who has glossopharyngeal neuralgia

associated with syncope. During attacks 10 secundum asystolia was recorded in the

ECG. The electrophysiologic study of heart was normal, the carotid test resulted 3

secundum asystolia without clinical symptoms. The electroencephalogram and

computertomogram of brain were normal. Carbamazepine and demand pacemaker

were effective in controlling the symptoms of the patient. Finally, aetiology,

pathogenesis and treatment of glossopharyngeal neuralgia are discussed.

449. Mogyoros, I.; Kiernan, M. C.; Burke, D. Strength-duration properties of human

peripheral nerve. Brain. 1996 Apr; 119( Pt 2): 439-47; ISSN: 0006-8950.

ENGLAND. The strength-duration time constant (tau SD) is a property of nodal

membrane and, while it depends on a number of factors, its measurement may shed

light on axonal properties when taken in conjunction with measurements of axonal

excitability. For example, tau SD increases with demyelination as the exposed

membrane is enlarged by inclusion of paranodal and internodal membrane, it

decreases with hyperpolarization and it increases with depolarization. The present

study was undertaken in 20 normal volunteers to compare strength-duration curves

for compound sensory and muscle action potentials, to determine the most

appropriate curve fitting equation for the data, and to examine the reproducibility of

the calculated time constant on different days, for potentials of different amplitude

and at different sites along the nerve. Using a computerized threshold-tracking

system, stimulus intensity was adjusted to produce an antidromic compound

sensory action potential (CSAP) or an orthodromic muscle action potential of 30%

of maximum. Stimulus duration was increased every minute in 20 microseconds

steps from 20 microseconds to 1 ms. The time constant for compound sensory

potentials (665 +/- 182 microsecond) was longer than that for compound EMG

potentials (459 +/- 126 microseconds). Weiss's formula, which relates threshold

charge to stimulus duration, provided an accurate fit for the experimental data, and

the study validated that, using it, relatively few experimental measurements were

required to calculate the time constant. In repeated studies on the same subject, time

constants usually differed by < 400 microseconds for sensory axons and < 250

microseconds for motor axons. They were identical at different sites along the

nerve and did not alter with the size of the compound action potential. These

characteristics suggest that the determinations of strength-duration time constant

could be suitable for clinical usage.

450. Molina, J. A.; Benito Leon, J.; Bermejo, F.; Jimenez Jimenez, F. J.; Olivan, J.

Intravenous immunoglobulin therapy in sensory neuropathy associated with

Sjogren's syndrome [letter]. J-Neurol-Neurosurg-Psychiatry. 1996 Jun; 60(6):

699; ISSN: 0022-3050.

ENGLAND. 0.

451. Monti, L.; Biagi, S.; Pacini, M. [Raeder's syndrome (the paratrigeminal syndrome): its

etiology related to carotid artery dissection. A case report]. Sindrome di Raeder

(sindrome paratrigeminale): eziologia riferibile a dissecazione dell'arteria carotide

interna. Descrizione di un caso. Radiol-Med-Torino. 1996 Apr; 91(4): 473-5;

ISSN: 0033-8362.

ITALY.

452. Moore, T. E.; Yuh, W. T.; El Khoury, G. Y. Plantar compartments of the foot: MR

appearance in cadavers and diabetic patients [letter]. Radiology. 1996 Mar; 198(3):

909; ISSN: 0033-8419.

UNITED-STATES.

453. Morrison, G. A.; Sterkers, J. M. Unusual presentations of acoustic tumours. Clin-

Otolaryngol. 1996 Feb; 21(1): 80-3; ISSN: 0307-7772.

ENGLAND. A series of 238 consecutive patients with acoustic neuromas,

operated on in Paris has been studied to identify unusual presentations and varied

symptomatology. The most common history was that of a progressive unilateral

hearing loss (in 68.1%), with tinnitus (in 49.1%) or disequilibrium (in 49.1%) or

both. Sudden hearing loss (in 14.7%) or fluctuating hearing loss (in 6.3%), and a

single or repeated episodes of acute vertigo (in 8.8%) were seen less commonly.

Headaches occurred as an associated symptom in 10.5%, tinnitus was the sole

symptom in 2.8% and other uncommon symptoms included otalgia, facial nerve

palsy, facial or ocular pain, altered sensation in the face or eye, or tingling of the

tongue. Some 11.3% of patients presented with normal pure tone auditory

thresholds and a 100% speech discrimination score and of these patients acoustic

reflex thresholds were normal in 53% and brainstem auditory evoked responses

were suggestive of the retro-cochlear abnormality in only 76.2%. Amongst the less

common presentations, the initial symptoms mimicked such diagnoses as Meniere's

disease, benign positional vertigo, vertebro-basilar migraine, vertebro-basilar

insufficiency, Bell's palsy and Trigeminal neuralgia. Overall, 20.6% of patients

had unusual initial presenting symptoms, 36.5% of the symptoms were unusual

and these were found in isolation in 11.8% of patients. An awareness of the

spectrum of more subtle symptoms of acoustic tumours may lead to the correct

diagnosis at an earlier stage.

454. Moseley, I. Idiopathic duro-optic calcification [letter]. Clin-Radiol. 1996 Oct; 51(10):

741; ISSN: 0009-9260.

ENGLAND.

455. Mueller, M. J. Identifying patients with diabetes mellitus who are at risk for lower-

extremity complications: use of Semmes-Weinstein monofilaments. Phys-Ther.

1996 Jan; 76(1): 68-71; ISSN: 0031-9023.

UNITED-STATES. Research indicates that the SW monofilament is an

inexpensive, reliable, valid, and easy-to-use clinical indicator for identifying

patients who are at risk for developing foot ulcers and subsequent amputations.

Those patients unable to sense the 5.07 SW monofilament on any part of their foot

should be provided preventive care, including patient education and prescription of

appropriate therapeutic footwear.

456. Murakami, T.; Garcia, C. A.; Reiter, L. T.; Lupski, J. R. Charcot-Marie-Tooth disease

and related inherited neuropathies. Medicine-Baltimore. 1996 Sep; 75(5): 233-50;

ISSN: 0025-7974.

UNITED-STATES. Charcot-Marie-Tooth disease (CMT) was initially described

more than 100 years ago by Charcot, Marie, and Tooth. It was only recently,

however, that molecular genetic studies of CMT have uncovered the underlying

causes of most forms of the diseases. Most cases of CMT1 are associated with a

1.5-Mb tandem duplication in 17p11.2-p12 that encompasses the PMP22 gene.

Although many genes may exist in this large duplicated region, PMP22 appears to

be the major dosage-sensitive gene. CMT1A is the first autosomal dominant disease

associated with a gene dosage effect due to an inherited DNA rearrangement. There

is no mutant gene, but instead the disease phenotype results from having 3 copies

of a normal gene. Furthermore, these findings suggest that therapeutic intervention

in CMT1A duplication patients may be possible by normalizing the amount of

PMP22 mRNA levels. Alternatively, CMT1A can be caused by mutations in the

PMP22 gene. Other forms of CMT are associated with mutations in the MPZ

(CMT1B) and Cx32 (CMTX) genes. Thus, mutations in different genes can cause

similar CMT phenotypes. The related but more severe neuropathy, Dejerine-Sottas

syndrome (DSS), can also be caused by mutations in the PMP22 and MPZ genes.

All 3 genes thus far identified by CMT researchers appear to play an important role

in the myelin formation or maintenance of peripheral nerves. CMT1A, CMT1B,

CMTX, hereditary neuropathy with liability to pressure palsies (HNPP), and DSS

have been called myelin disorders or "myelino-pathies." Other demyelinating

forms, CMT1C and CMT-AR, may be caused by mutations of not yet identified

myelin genes expressed in Schwann cells. The clinically distinct disease HNPP is

caused by a 1.5-Mb deletion in 17p11.2-p12, which spans the same region

duplicated in most CMT1A patients. Underexpression of the PMP22 gene causes

HNPP just as overexpression of PMP22 causes CMT1A. Thus, 2 different

phenotypes can be caused by dosage variations of the same gene. It is apparent that

the CMT1A duplication and HNPP deletion are the reciprocal products of a

recombination event during meiosis mediated through the CMT1A-REPs. CMT1A

and HNPP could be thought of as a "genomic disease" more than single gene

disorders. Other genetic disorders may also prove to arise from recombination

events mediated by specific chromosomal structural features of the human genome

(102). Further studies on the recombination mechanism of CMT and HNPP might

reveal the causes of site specific homologous recombination in the human genome.

The discovery of the PMP22 gene in the 1.5-Mb CMT1A duplication/HNPP

deletion critical region also suggests that the clinical phenotype of chromosome

aneuploid syndromes may result from the effect of a small subset of dosage-

sensitive genes mapping within the region of aneuploidy. The understanding of the

molecular basis of CMT1 and related disorders has allowed accurate DNA

diagnosis and genetic counseling of inherited peripheral neuropathies and will make

it possible to develop rational strategies for therapy. As several loci for CMT2 have

been identified, the genes responsible for CMT2 will most likely be disclosed using

positional cloning and candidate gene approaches in the near future.. 0.

457. Murakami, T.; Garcia, C. A.; Reiter, L. T.; Lupski, J. R. Charcot-Marie-Tooth disease

and related inherited neuropathies. Medicine-Baltimore. 1996 Sep; 75(5): 233-50;

ISSN: 0025-7974.

UNITED-STATES. Charcot-Marie-Tooth disease (CMT) was initially described

more than 100 years ago by Charcot, Marie, and Tooth. It was only recently,

however, that molecular genetic studies of CMT have uncovered the underlying

causes of most forms of the diseases. Most cases of CMT1 are associated with a

1.5-Mb tandem duplication in 17p11.2-p12 that encompasses the PMP22 gene.

Although many genes may exist in this large duplicated region, PMP22 appears to

be the major dosage-sensitive gene. CMT1A is the first autosomal dominant disease

associated with a gene dosage effect due to an inherited DNA rearrangement. There

is no mutant gene, but instead the disease phenotype results from having 3 copies

of a normal gene. Furthermore, these findings suggest that therapeutic intervention

in CMT1A duplication patients may be possible by normalizing the amount of

PMP22 mRNA levels. Alternatively, CMT1A can be caused by mutations in the

PMP22 gene. Other forms of CMT are associated with mutations in the MPZ

(CMT1B) and Cx32 (CMTX) genes. Thus, mutations in different genes can cause

similar CMT phenotypes. The related but more severe neuropathy, Dejerine-Sottas

syndrome (DSS), can also be caused by mutations in the PMP22 and MPZ genes.

All 3 genes thus far identified by CMT researchers appear to play an important role

in the myelin formation or maintenance of peripheral nerves. CMT1A, CMT1B,

CMTX, hereditary neuropathy with liability to pressure palsies (HNPP), and DSS

have been called myelin disorders or "myelino-pathies." Other demyelinating

forms, CMT1C and CMT-AR, may be caused by mutations of not yet identified

myelin genes expressed in Schwann cells. The clinically distinct disease HNPP is

caused by a 1.5-Mb deletion in 17p11.2-p12, which spans the same region

duplicated in most CMT1A patients. Underexpression of the PMP22 gene causes

HNPP just as overexpression of PMP22 causes CMT1A. Thus, 2 different

phenotypes can be caused by dosage variations of the same gene. It is apparent that

the CMT1A duplication and HNPP deletion are the reciprocal products of a

recombination event during meiosis mediated through the CMT1A-REPs. CMT1A

and HNPP could be thought of as a "genomic disease" more than single gene

disorders. Other genetic disorders may also prove to arise from recombination

events mediated by specific chromosomal structural features of the human genome

(102). Further studies on the recombination mechanism of CMT and HNPP might

reveal the causes of site specific homologous recombination in the human genome.

The discovery of the PMP22 gene in the 1.5-Mb CMT1A duplication/HNPP

deletion critical region also suggests that the clinical phenotype of chromosome

aneuploid syndromes may result from the effect of a small subset of dosage-

sensitive genes mapping within the region of aneuploidy. The understanding of the

molecular basis of CMT1 and related disorders has allowed accurate DNA

diagnosis and genetic counseling of inherited peripheral neuropathies and will make

it possible to develop rational strategies for therapy. As several loci for CMT2 have

been identified, the genes responsible for CMT2 will most likely be disclosed using

positional cloning and candidate gene approaches in the near future.. 0.

458. Murakami, T.; Tsubaki, J.; Tahara, Y.; Nagashima, T. Gradenigo's syndrome: CT and

MRI findings. Pediatr-Radiol. 1996 Sep; 26(9): 684-5; ISSN: 0301-0449.

GERMANY. We report the case of an 8-year-old girl with Gradenigo's syndrome.

Involvement of the petrous portion of the left temporal bone was demonstrated by

CT and an inflammatory lesion of the left petrous apex was clearly shown by MRI,

which is useful in diagnosis and management of apical petrositis.. 0; 0; 0; 0;

61477-96-1; 67-43-6; 68401-81-0; 7440-54-2; 80529-93-7; 82219-78-1.

459. Murata, K.; Araki, S.; Okajima, F.; Saito, Y. Subclinical impairment in the median

nerve across the carpal tunnel among female VDT operators. Int-Arch-Occup-

Environ-Health. 1996; 68(2): 75-9; ISSN: 0340-0131.

GERMANY. Sensory nerve conduction velocities in the palm-to-finger (SCV-pf),

wrist-to-finger (SCV-wf), wrist-to-palm (SCV-wp), and wrist-to-elbow (SCV-we)

segments and the distribution of nerve conduction velocities in the right median

nerve were determined among 27 female operators aged 19-37 and 19 healthy

women (controls) aged 19-31 to estimate the prevalence of subclinical carpal tunnel

syndrome (CTS). Also, the WF/PF ratio, dividing the SCV-wf by the SCV-pf,

was calculated to assess abnormalities of nerve conduction within the carpal tunnel.

The operators were engaged in data entry in front of a visual display terminal

(VDT) for about 6 h/day, and their working duration was between 1 and 17 (mean

6) years. The SCV-wf, SCV-wp, and WF/PF ratio in the operators were

significantly lower than those in the controls. The rate of persons with the WF/PF

ratio of less than 90%, i.e., below normal limits in the 19 controls, was

significantly higher in the operator group (37%) than in the control group (0%).

The operators complained of more symptoms related to CTS than did the controls,

but any symptoms were not associated with slowing of nerve conduction velocities

in the operators. In the controls, the WF/PF ratio was not closely correlated with

skin temperature or age despite the presence of significant relations between skin

temperature and the SCV-wf, SCV-pf, and SCV-wp; the interpersonal variability of

the WF/PF ratio was much smaller than that of all SCVs. In the light of the present

and previous studies, the rate of VDT operators with subclinical CTS seems to be

high, independent of its symptoms. Also, the WF/PF ratio will be a useful and

reliable screening method for the early detection of CTS due to repetitive wrist and

finger movements involved in work.

460. Murofushi, T.; Halmagyi, G. M.; Yavor, R. A.; Colebatch, J. G. Absent vestibular

evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior

vestibular nerve involvement? Arch-Otolaryngol-Head-Neck-Surg. 1996 Aug;

122(8): 845-8; ISSN: 0886-4470.

UNITED-STATES. BACKGROUND: Benign paroxysmal positioning vertigo

(BPPV) is generally thought to be caused by canalolithiasis in the posterior

semicircular canal, an organ that is innervated by the inferior vestibular nerve. We

hypothesized that absent vestibular evoked myogenic potentials (VEMPs) would

indicate involvement of the inferior vestibular nerve and that posterior semicircular

canal-type BPPV could not develop after vestibular neurolabyrinthitis (VNL) in

patients with absent VEMPs. OBJECTIVE: To find out if VEMPs could be helpful

in evaluating involvement of the inferior vestibular nerve in acute VNL. DESIGN:

We reviewed the VEMP findings in 47 patients (34 men and 13 women) with acute

VNL, 10 of whom had then developed posterior semicircular canal-type BPPV.

RESULTS: While p13-n23, the first positive-negative peak of the VEMP, was

ipsilaterally present on stimulation of the unaffected side in all patients, it was

absent on the affected side in 16 patients (34%). The absence or presence of p13-

n23 was independent of the results of caloric tests, pure tone audiometry, and

auditory brain-stem responses. Typical posterior semicircular canal BPPV

developed in 10 of the 47 patients after the acute attack of VNL, always on the

same side as the neurolabyrinthitis. The p13-n23 potentials were preserved on

stimulation of the affected ear in all 10 patients with BPPV. CONCLUSIONS:

These results suggest that if VEMPs are absent from an ear that has suffered acute

VNL, then posterior semicircular canal BPPV is unlikely to develop as a

consequence of the VNL. The reason for this appears to be that the absence of

VEMPs is due to involvement of the inferior vestibular nerve or involvement of the

structures that it innervates.

461. Musharafieh, R.; Saghieh, S. Reflex sympathetic dystrophy revisited. Middle-East-J-

Anesthesiol. 1996 Feb; 13(4): 345-71; ISSN: 0544-0440.

LEBANON.

462. Nabavi, D. G.; Georgiadis, D.; Stogbauer, F.; Michael, N.; Husstedt, I. W. [Meralgia

paresthetica. A rare differential diagnosis of circumscribed alopecia]. Meralgia

paraesthetica. Seltene Differentialdiagnose der umschriebenen Alopezie. Dtsch-

Med-Wochenschr. 1996 Jun 21; 121(25-26): 834-8; ISSN: 0012-0472.

GERMANY. HISTORY AND CLINICAL FINDINGS. Two patients with

circumscribed alopecia on the lateral aspect of the thigh underwent a neurological

investigation after medical and dermatological examinations had failed to establish

the cause. Patient 1 also had neuralgia of the genitofemoral nerve after osteotomy of

the iliac crest; patient 2 had insulin-dependent diabetes mellitus. Within the affected

part of the skin both patients had sensory dysfunctions over the area of distribution

of the cutaneous lateral femoral nerve. Patient 2 additionally had sensory

dysfunctions in other areas of innervation. INVESTIGATIONS. Neurogram and

recordings of sensory evoked potentials revealed decreased amplitudes on the

affected side, establishing the diagnosis of meralgia paresthetica. TREATMENT

AND COURSE. The painful neuropathy was successfully treated in both patients

with carbamazepine (patient 1: 1.600 mg daily; patient 2: 900 mg daily).

CONCLUSION. Circumscribed alopecia can be caused by peripheral nerve

lesions. It should be considered in the differential diagnosis, particularly as the

cause can be easily established.. 0; 298-46-4.

463. Nadol, JB Jr; Diamond, P. F.; Thornton, A. R. Correlation of hearing loss and

radiologic dimensions of vestibular schwannomas (acoustic Neuromas). Am-J-

Otol. 1996 Mar; 17(2): 312-6; ISSN: 0192-9763.

UNITED-STATES. A retrospective analysis was performed of puretone

audiograms, speech-discrimination scores, and gadolinium-enhanced magnetic

resonance imaging scans of 75 patients with vestibular schwannomas (acoustic

neuroma). Sensorineural hearing loss was analyzed for low frequencies (250-500

Hz), midfrequencies (1,000-2,000 Hz), and high frequencies (4,000-8,000 Hz).

The largest tumor diameter in the cerebellopontine angle and the lateral extent of

invasion by tumor into the internal auditory canal were calculated from magnetic

resonance images. There were statistically significant correlations between the

largest tumor diameter and the severity of low-frequency sensorineural hearing loss

(p = 0.001). However, no significant correlations were found between the

following: largest tumor diameter and the severity of mid-frequency or high-

frequency sensorineural hearing loss or speech-discrimination scores and lateral

extent of invasion of the internal auditory canal and sensorineural hearing loss at all

frequencies or speech-discrimination scores (p > or = 0.05). The findings suggest

that nerve compression is not the only cause of hearing loss in vestibular

schwannoma.

464. Nakamura, K.; Yamamoto, T.; Yamashita, M. Small medullary infarction presenting as

painful trigeminal sensory neuropathy. J-Neurol-Neurosurg-Psychiatry. 1996 Aug;

61(2): 138; ISSN: 0022-3050.

ENGLAND.

465. Nakamura, Y.; Iwamoto, R.; Mekada, E. Expression and distribution of CD9 in

myelin of the central and peripheral nervous systems. Am-J-Pathol. 1996 Aug;

149(2): 575-83; ISSN: 0002-9440.

UNITED-STATES. CD9 is a member of the newly identified tetra-membrane-

spanning protein family. We show here that CD9 is a constituent of myelin in the

central and peripheral nervous systems. Expression of CD9 was detected in human

cerebral white matter and sciatic nerve by Northern and Western blotting. Myelin in

the central and peripheral nervous systems was strongly stained with a monoclonal

antibody against human CD9 antigen in paraffin-embedded sections. CD9 was

detected in adult nervous tissue but not in developing brain at less than 20 weeks of

gestation. Immunohistochemical studies indicated that expression of CD9 is

correlated with myelination and is somewhat delayed compared with expression of

myelin basic protein, a major component protein of myelin. In the central nervous

system, CD9 was detected along the outermost membrane of compact myelin but

not inside compact myelin or the periaxonal region. Although the membrane-

anchored form of heparin-binding epidermal-growth-factor-like growth factor

(proHB-EGF), which is identical to the diphtheria toxin receptor, forms a complex

with CD9 in some human and monkey cell lines, proHB-EGF was not detected in

myelin immunocytochemically. The distribution of CD9 in the outer surface of

myelin and its relatively late developmental appearance suggest that CD9 may

interact with the extracellular matrix or cell adhesion molecules and participate in the

maintenance of the entire myelin sheath.. 0; 0; 0; 147785-22-6; 62229-50-9.

466. Nakano, H.; Ohara, Y.; Bandoh, K.; Miyaoka, M. A case of central pontine

myelinolysis after surgical removal of a pituitary tumor. Surg-Neurol. 1996 Jul;

46(1): 32-6; ISSN: 0090-3019.

UNITED-STATES. CASE REPORT: We have experienced a case in which

surgical removal of a pituitary tumor from a male patient was followed by the

occurrence of hyponatremia, which in turn was later associated with central pontine

myelinolysis (CPM). A 4 X 3 X 3 cm pituitary tumor with hypothalamic extension

was removed via a transsphenoidal approach. The post-operative course was

uneventful until severe hyponatremia developed. To avoid drastic correction of

electrolyte levels, reestablishment of normal serum levels was spread over 1 week.

Following this, however, various neurologic symptoms such as pseudobulbar

palsy, quadriplegia, extrapyramidal symptoms, and mental symptoms appeared.

The case was diagnosed as CPM and extrapontine myelinolysis (EPM) on the basis

of the clinical course and symptoms, and high-dosage steroid therapy was

commenced. RESULTS: There was consequent gradual improvement in

symptoms. One month later, MRI revealed typical demyelination lesions in the

central pons and striatum. Abnormal electrolyte conditions easily occur in pituitary

tumors associated with hypothalamic extension in an altered hormone environment.

It is known that CPM and EPM result from drastic correction of hyponatremia.

CONCLUSIONS: The frequent measurement of electrolytes and cautious

correction of sodium imbalance are important for the prevention of CPM and EPM

in the postoperative management of patients who undergo surgery for a pituitary

tumor and whose high-dosage steroid therapy are effective.

467. Nakstad, P. H. MR imaging as the primary modality for neuroradiologic evaluation of

the lumbar spine [letter; comment]. Acta-Radiol. 1996 May; 37(3 Pt 1): 381-2;

ISSN: 0284-1851.

Note: Comment on: Acta Radiol 1996 May;37(3 Pt 1):373-80.

DENMARK.

468. Narayanan, V.; Diven, W.; Ahdab Barmada, M. Congenital fumarase deficiency

presenting with hypotonia and areflexia. J-Child-Neurol. 1996 May; 11(3): 252-5;

ISSN: 0883-0738.

UNITED-STATES. EC 4.2.1.2.

469. Nath, R. K.; Mackinnon, S. E.; Stelnicki, E. Reflex sympathetic dystrophy. The

controversy continues. Clin-Plast-Surg. 1996 Jul; 23(3): 435-46; ISSN: 0094-

1298.

UNITED-STATES. The chronic pain syndrome encompassed by the term RSD is

poorly understood. The confusion is caused in large part by frequent misdiagnosis

and excessive use of sympatholytic procedures in inappropriate circumstances.

Recently, pain specialists have redefined the specific criteria for regional pain

syndromes having sympathetic maintaining factors, emphasizing application of

placebo testing in diagnosis and attention to anatomic principles in pharmacologic

and surgical treatment. The authors believe that three-phase bone scanning is a

valuable adjunct to clinical judgment in making the proper diagnosis. Current

thinking suggests that sympathetic maintained pain exists but that it may comprise

only approximately 10% of regional pain cases. Once the appropriate diagnosis is

made, classically described sympatholytic procedures are reasonably used.

Alternative techniques, such as spinal cord stimulation, may have an important role

in refractory cases of sympathetically maintained pain.

470. Nathan, P. A. Outcome following conservative management of thoracic outlet

syndrome [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 528-9; ISSN: 0363-5023.

UNITED-STATES.

471. Nathan, P. A.; Keniston, R. C.; Meadows, K. D. Electrical studies as a prognostic

factor in the surgical treatment of carpal tunnel syndrome [letter]. J-Hand-Surg-

Am. 1996 May; 21(3): 526-7; ISSN: 0363-5023.

UNITED-STATES.

472. Nathan, P. A.; Keniston, R. C. Usefulness of preoperative nerve conduction studies

[letter]. J-Hand-Surg-Br. 1996 Apr; 21(2): 287-8; ISSN: 0266-7681.

SCOTLAND.

473. Navarro, B.; Sayas, M. J.; Atienza, A.; Leon, P. An unhappily married man with thick

soles [see comments]. Lancet. 1996 Jun 8; 347(9015): 1596; ISSN: 0140-6736.

Note: Comment in: Lancet 1996 Jul 27;348(9022):276.

ENGLAND. 7440-38-2.

474. Neary, W. J.; Newton, V. E.; Laoide Kemp, S. N.; Ramsden, R. T.; Griffith, G.;

Evans, D. G.; Harris, R.; Strachan, T. A clinical, genetic and audiological study of

patients and families with unilateral vestibular schwannomas. I. Clinical features of

neurofibromatosis in patients with unilateral vestibular schwannomas. J-Laryngol-

Otol. 1996 Jul; 110(7): 634-40; ISSN: 0022-2151.

ENGLAND. Ninety-three patients with unilateral vestibular schwannomas were

examined in a clinical, genetic and audiological study, to determine whether they

had features associated with neurofibromatosis Type 1 or neurofibromatosis Type

2. In 91 families, one patient only was found to be affected with a unilateral

vestibular schwannoma. Patients did have a few cafe-au-lait macules, but fewer

than six in number. None of the patients satisfied the cutaneous diagnostic criteria

for neurofibromatosis Type 1. Neither Lisch nodules nor presenile posterior

subcapsular lenticular opacities or cortical opacities were a feature. Five patients

with unilateral vestibular schwannomas are described where the clinical findings

raised the possibility of neurofibromatosis Type 2. It is suggested that certain

individuals with unilateral vestibular schwannomas are at risk of developing

neurofibromatosis Type 2. Furthermore, the possibility of neurofibromatosis Type

2 should be considered if more than one individual in a family is found to be

affected with a unilateral vestibular schwannoma.

475. Nedostup, A. V.; Vein, A. M.; Solov'eva, A. D.; Fedorova, V. I.; Morozova, N. S.;

Churganova, L. I. u.; Skiba, K. A. [State of autonomic regulation in patients with

mitral valve prolapse and dysfunction of sinus node]. Sostoianie vegetativnoi

reguliatsii u bol'nykh s prolapsom mitral'nogo klapana i disfunktsiei sinusovogo

uzla. Klin-Med-Mosk. 1996; 74(3): 35-9; ISSN: 0023-2149.

RUSSIA. This study was made of 62 patients with idiopathic mitral prolapse, 17

patients with vagus sinus dysfunction and 8 patients with combination of the above

disorders. Clinical, psychological and electrophysiological examinations revealed in

all the patients psychovegetative syndrome with paroxysmal and permanent

vegetative disorders, anxious-hypochondriacal and anxious-depressive conditions.

Vegetative disorders were characterized by definite discoordination of vegetative

regulation manifesting as hyperactivity of suprasegmental vegetative structures and

insufficiency of both sympathetic and parasympathetic vegetative regulation.

476. Neely, J. G.; Joaquin, A. H.; Kohn, L. A.; Cheung, J. Y. Quantitative assessment of

the variation within grades of facial paralysis. Laryngoscope. 1996 Apr; 106(4):

438-42; ISSN: 0023-852X.

UNITED-STATES. A completely objective, unambiguous outcome measure of

facial function is now available. A new automated computer-assisted clinimetric

system combines the crucial detection capabilities of the human observer and the

unique capacity of the computer to quantify the image light reflectance difference

observed during facial expression. The new system was applied to 27 patients with

a variety of diseases affecting the facial nerve. All subjects could be individually

and objectively ranked, and disease-specific profiles could be constructed. These

tasks are not possible with the House-Brackmann scale, because of the wide

variation within grades and the ambiguity between grades. With the automated

objective, unambiguous outcome measure, it may be possible to define individual

case progression, recovery, and outcome over the course of disease.

477. Netscher, D.; Polsen, C.; Thornby, J.; Choi, H.; Udeh, J. Anatomic delineation of the

ulnar nerve and ulnar artery in relation to the carpal tunnel by axial magnetic

resonance imaging scanning. J-Hand-Surg-Am. 1996 Mar; 21(2): 273-6; ISSN:

0363-5023.

UNITED-STATES. In a number of publications the distal branches of both the

ulnar artery and ulnar nerve have been identified as being positioned ulnar to the

hook of the hamate. We undertook a magnetic resonance imaging project in patients

who presented for carpal tunnel release to determine how far radially vital structures

of Guyon's canal may be located and if they may overlap the carpal tunnel at the

hook of the hamate. Cross-sectional magnetic resonance imaging scans of 20

patients were performed prior to carpal tunnel release with the wrist positioned in

neutral, flexed, and extended positions. Linear measurements were made either

radial or ulnar to the hook of the hamate taken as the reference point. In the neutral

position, the ulnar nerve was found to be, on average, 3.6 mm ulnar to the hook of

the hamate (range of 5.8 mm radial to 7.5 mm ulnar). The ulnar artery averaged 0.7

mm to the radial side of the hook of the hamate (range 7.8 mm radial to 2.8 mm

ulnar). Guyon's canal extended 28% of the way across the carpal ligament (range 9

to 63%). With the wrist in flexion and extension, there was an ulnar displacement

and a radial displacement, respectively, of these structures relative to the hook of

the hamate.

478. Newman, N. J. Optic neuropathy. Neurology. 1996 Feb; 46(2): 315-22; ISSN: 0028-

3878.

UNITED-STATES.

479. Nicholas, J. J.; Smith, W. F.; Andersson, G. B. Bacterial discitis caused by limb

gangrene requiring below-knee amputation. Arch-Phys-Med-Rehabil. 1996 Mar;

77(3): 301-4; ISSN: 0003-9993.

UNITED-STATES. Two patients presented with disabling back pain and were

unable to participate in physical therapy activities after being admitted to an acute

rehabilitation center. Both patients had bacterial discitis of the lumbar spine that was

apparently caused by infected ischemic limb tissue, ultimately removed at below-

knee amputation. The literature describes many cases of bacterial discitis infected

from many sources, but not from ischemic limb tissue requiring subsequent

amputation. Many such cases may exist, however, and earlier recognition of this

condition will enable appropriate treatment before vertebral destruction and/or

neurological sequelae.

480. Nickell, K.; Boone, T. B. Peripheral neuropathy and peripheral nerve injury. Urol-

Clin-North-Am. 1996 Aug; 23(3): 491-500; ISSN: 0094-0143.

UNITED-STATES. The peripheral nerves to the bladder can be altered by several

disease processes. Voiding symptoms alone are not reliable in predicting the exact

neurogenic bladder dysfunction. Urodynamic evaluation is crucial to optimize

therapy and to rule out concomitant pathology.

481. Nielsen, N. H.; Petersen, C. S. Herpes zoster-associated trigeminal neurotrophic ulcer

[letter]. Acta-Derm-Venereol. 1996 Mar; 76(2): 159-60; ISSN: 0001-5555.

NORWAY. 0; 378-44-9; 5593-20-4.

482. Nishikage, H.; Saito, A.; Nakano, Y.; Horie, C.; Hattori, T.; Yamamoto, J.; Kotake,

C.; Miya, I.; Numano, M.; Orihara, A. [Case of Fisher syndrome with pemphigus

treated by immuno-adsorption plasmapheresis]. Nippon-Naika-Gakkai-Zasshi.

1996 Feb 10; 85(2): 274-5; ISSN: 0021-5384.

JAPAN.

483. Noda, K.; Katayama, S.; Watanabe, C.; Yamamura, Y.; Nakamura, S.; Yonehara, S.;

Inai, K. Pure autonomic failure with motor neuron disease: report of a clinical

study and postmortem examination of a patient [letter]. J-Neurol-Neurosurg-

Psychiatry. 1996 Mar; 60(3): 351-2; ISSN: 0022-3050.

ENGLAND.

484. Norris, T.; Clarke, D. Care of diabetic foot lesions [letter; comment]. Am-Fam-

Physician. 1996 Jul; 54(1): 70, 72; ISSN: 0002-838X.

Note: Comment on: Am Fam Physician 1996 Feb 1;53(2):601-22, 615-6.

UNITED-STATES.

485. Novak, C. B.; Mackinnon, S. E. Thoracic outlet syndrome. Orthop-Clin-North-Am.

1996 Oct; 27(4): 747-62; ISSN: 0030-5898.

UNITED-STATES. Thoracic outlet syndrome (TOS) is the term commonly used

to describe patients with symptoms attributed to compression of the brachial plexus

and subclavian vein and artery in the region of the thoracic inlet/outlet. TOS

remains extremely controversial with respect to its existence, diagnosis,

conservative management, and surgical treatment. The diagnosis is based upon

clinical evaluation, reproduction of patient symptoms with arm elevation, and the

absence of other relevant pathology. Conservative management should be directed

towards correction of postural abnormalities and muscle imbalance in the

cervicoscapular region. Patient education compliance to a home exercise program

and behavioral modification at home, work, and sleep is necessary for a successful

outcome. Surgical decompression of the brachial plexus is best achieved through a

supraclavicular approach and should be reserved for the few patients in which

conservative management has failed to improve symptoms.

486. Nystrom, C.; Nystrom, O. [Estimated stress verifies autonomic dysfunction]. Skattad

stress verifierar autonom dysfunktion. Lakartidningen. 1996 Jul 10; 93(28-29):

2583-4; ISSN: 0023-7205.

SWEDEN.

487. O'Driscoll, C. M.; Irwin, A.; Saifuddin, A. Variations in morphology of the

lumbosacral junction on sagittal MRI: correlation with plain radiography. Skeletal-

Radiol. 1996 Apr; 25(3): 225-30; ISSN: 0364-2348.

GERMANY. OBJECTIVE: To identify on sagittal magnetic resonance imaging

(MRI) scans of the lumbar spine the features that indicate the presence of a

lumbosacral transitional vertebra (LSTV). DESIGN: One hundred consecutive

sagittal T1-and T2-weighted MRI scans of the lumbar spine were reviewed and

separated into four types depending upon the absence or presence of disc material

between what was considered to be the uppermost sacral segment and the

remainder of the sacrum, as follows: type 1: no disc material present; type 2: a

small residual disc, not extending for the whole anteroposterior (AP) diameter of

the sacrum; type 3: a well-formed disc extending for the whole AP diameter of the

sacrum; type 4: a well-formed disc extending for the whole AP diameter of the

sacrum with, in addition, an abnormal upper sagittal sacral outline. The

corresponding plain radiographs of each patient were than reviewed and assessed

for the presence of an LSTV. These were classified according to the method of

Castellvi et al. PATIENTS: All patients had been referred for MRI of the lumbar

spine, usually because of back pain with or without radiculopathy. There were 51

male and 49 female patients with a mean age of 42 years and an age range of 18-85

years. RESULTS AND CONCLUSIONS: With regard to sacral morphology on

MRI, 30 patients had type 1, 42 patients type 2, 16 patients type 3 and 12 patients

type 4 morphology. Fifteen patients had an LSTV. There was a good correlation

between the presence of a fused LSTV and a type 4 MRI appearance, indicating that

this type of LSTV can be identified on sagittal MRI scans.

488. Oganesyan, O. V.; Istomina, I. S.; Kuzmin, V. I. Treatment of equinocavovarus

deformity in adults with the use of a hinged distraction++ apparatus. J-Bone-Joint-

Surg-Am. 1996 Apr; 78(4): 546-56; ISSN: 0021-9355.

UNITED-STATES. We evaluated the results following the use of the Volkov-

Oganesyan-Povarov hinged distraction apparatus to correct equinocavovarus

deformity of the foot in sixty-five adults (eighty-three feet). The deformity was

secondary to ischemic and neuropathic changes after trauma to the foot and ankle,

poliomyelitis, Charcot-Marie-Tooth disease, or untreated clubfoot. The deformity

often was associated with severe neurotrophic changes, extensive scarring of the

skin, callosities, or osteomyelitis of the foot, all of which usually preclude

operative reconstruction. The average age of the patients was twenty-six years

(range, sixteen to fifty-six years). The average duration of follow-up was ten years

(range, three to nineteen years). The duration of distraction was three to thirteen

weeks, and the apparatus usually was kept in place for an additional two months

after the desired position of correction had been achieved. The patient then wore a

plaster cast for an average of eight weeks and custom-molded shoe-inserts for at

least one year. A plantigrade foot was obtained with this external transosseous

distraction device. Eight patients had inflammation of pin tracks, which was treated

with local injection of antibiotics into the soft tissues and application of bandages

soaked in antibiotics to the area around the pin sites. The pins were removed from

five of the eight patients, and the apparatus was removed from three additional

patients because of osteomyelitis. The results were classified as good in fifty-nine

feet, satisfactory in twenty, and unsatisfactory in four. In the four feet (three

patients) with an unsatisfactory result, a good result was obtained after an

arthrodesis was done.. 0.

489. Ohashi, T.; Kenmochi, M.; Kinoshita, H.; Ochi, K.; Yoshino, K.; Komatsuzaki, A.

Abnormal electrocochleography after excision of acoustic neuroma. Acta-

Otolaryngol-Suppl-Stockh. 1996; 522: 17-21; ISSN: 0365-5237.

NORWAY. Pre-, per- and post-operative ECoG findings obtained from a 43-year-

old woman with an acoustic neuroma were studied. Although CAP presented a

normal waveform in response to click during the whole process of the operation, it

was transformed into an abnormally broadened negative waveform in shape with a

mild hearing exacerbation, and has remained unchanged up to the present. This

broad response was considered to be a receptor potential, because it showed no

adaptational amplitude reduction. Additionally, postoperative ECoG to tone burst

stimuli demonstrated an increase in DC potential following the stimulus envelope.

Consequently, the broad negative response to click was considered to be mainly

composed of an enlarged negative SP. The generation mechanism underlying the

phenomenon of an abnormally increased negative SP found after the excision of an

acoustic neuroma is discussed, with reference to several items in the literature.

Similar responses in the postoperative ECoG of patients with acoustic neuromas

obtained by us in the past were given as additional examples. CAP as an indicator

for intraoperative monitoring can hardly predict postoperative auditory function. It

is to be hoped that a more reliable auditory monitoring method during the operation

will be established.

490. Ohkura, M.; Mitsudome, A.; Yasumoto, S.; Ogawa, A. [Werdnig-Hoffmann disease

type I with progressive ophthalmoplegia and ptosis]. No-To-Hattatsu. 1996 Jul;

28(4): 332-5; ISSN: 0029-0831.

JAPAN. Although pathological changes are observed in both the oculomotor

nucleus and abducens nucleus in autopsied cases of infantile progressive spinal

muscular atrophy, external and internal ocular palsy and ptosis have not been

previously reported clinically. We presented here two long-surviving cases on

respirators which gradually developed ophthalmoplegia and ptosis were presented.

From our observation of these cases, it was suggested that there are certain periods

of latencies between the occurrence of pathological changes and their clinical

manifestation and that the lack of clinical signs of upper cranial nerve involvement

in cases with Werdnig-Hoffmann type I is due to their short survival length.

491. Ohtsuka, K.; Sone, A.; Igarashi, Y.; Akiba, H.; Sakata, M. Vascular compressive

abducens nerve palsy disclosed by magnetic resonance imaging. Am-J-Ophthalmol.

1996 Sep; 122(3): 416-9; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To assess magnetic resonance imaging as a

diagnostic tool of neurovascular compression in a patient with abducens nerve

palsy. METHODS: We performed magnetic resonance imaging of the brainstem of

a 46-year-old patient with left abducens nerve palsy using spoiled gradient recalled

acquisition in the steady state (SPGR), which allows high-resolution T1-weighted

imaging and detection of the arteries across the plane of slices as a high-signal-

intensity area. RESULTS: Computed tomography of the brain was unremarkable

except for leftward shifting of the basilar artery. As disclosed by magnetic

resonance imaging with the SPGR, the right vertebral artery was shifted to the left

and joined with the left vertebral artery, and the left abducens nerve was

compressed by the vertebral artery. No other abnormal signals were seen in the

brainstem. CONCLUSIONS: These findings suggest that the abducens nerve palsy

in this patient was caused by vascular compression at the root exit zone. Magnetic

resonance imaging with the SPGR is useful for the diagnosis of vascular

compressive neuropathy.

492. Ok, E.; Akcicek, F.; Toz, H.; Coker, A.; Kursat, S.; Tokat, Y.; Arac, N. Goitre and

severe autonomic neuropathy due to secondary amyloidosis in a renal transplant

patient [letter]. Clin-Nephrol. 1996 May; 45(5): 361-2; ISSN: 0301-0430.

GERMANY. 0; 64-86-8.

493. Olney, R. K. Electric studies as a prognostic factor in the surgical treatment of carpal

tunnel surgery [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 522-3; ISSN: 0363-

5023.

UNITED-STATES.

494. Omori, K.; Kacker, A.; Slavit, D. H.; Blaugrund, S. M. Quantitative

videostroboscopic measurement of glottal gap and vocal function: an analysis of

thyroplasty type I. Ann-Otol-Rhinol-Laryngol. 1996 Apr; 105(4): 280-5; ISSN:

0003-4894.

UNITED-STATES. The goal of surgical medialization of the vocal fold is to attain

complete glottic closure. The purpose of this study is to quantify the glottal gap and

to examine the relationship between glottal gap and vocal function perioperatively in

thyroplasty type I. Glottal gap area was measured in 20 patients at the point of

maximum closure of vocal fold vibration in digitized laryngeal stroboscopic images

and was normalized by the square of vocal fold length. Glottal gap area thus

measured was correlated with results obtained from well-accepted acoustic,

aerodynamic, and perceptual measures of vocal function. The glottal gap was

significantly reduced after thyroplasty type I. In patients with small preoperative

glottal gaps, the amplitude of vocal fold vibration was significantly improved. This

study verifies that quantitative videostroboscopic measurement of the glottal gap is

a useful means of objective evaluation of glottic incompetence and of the results of

thyroplasty type I.

495. Onofrj, M.; Tartaro, A.; Thomas, A.; Gambi, D.; Fulgente, T.; Delli Pizzi, C.;

Bonomo, L. Long echo time STIR sequence MRI of optic nerves in optic neuritis.

Neuroradiology. 1996 Jan; 38(1): 66-9; ISSN: 0028-3940.

GERMANY. MRI of the optic nerves was obtained in 13 patients with acute optic

neuritis and 13 with a previous optic neuritis (ON), assessed by clinical features,

visual fields and visual evoked potentials. Results of the conventional short tau

inversion recovery (STIR) sequence obtained with a short echo time (STE-STIR;

22 ms) were compared with those of a long echo time (LTE-STIR: 80 ms)

sequence. The conventional STE-STIR sequence revealed lesions in the optic

nerves in 78.5% of acute and 58.8% of previous ON. The LTE-STIR sequence

showed abnormalities in 92.8% of acutely symptomatic nerves and 94.1% of

nerves with previous ON. The optic nerve lesions appeared significantly longer

with the LTE-STIR sequence than with the conventional STE-STIR sequences, in

both acute and previous ON.

496. Ooi, G. C.; Peh, W. C.; Fung, C. F. Case report: Acute haemorrhagic presentation of

trigeminal neuroma. Br-J-Radiol. 1996 Apr; 69(820): 363-5; ISSN: 0007-1285.

ENGLAND. The acute presentation of trigeminal neuroma, due to sudden

haemorrhage, in a 26-year-old Chinese man, is described. The clinical, CT and

MRI features are reviewed together with the other four cases reported in the

literature.

497. Oosterhuis, H. J. Acquired blepharoptosis. Clin-Neurol-Neurosurg. 1996 Feb; 98(1):

1-7; ISSN: 0303-8467.

NETHERLANDS. A review is given of the aetiology and possible treatment of

acquired (non-congenital), blepharoptosis, which is a common but not specific sign

of neurological disease. The diagnostic categories of upper eyelid drooping are

scheduled as (a) pseudo-ptosis due to a local process or overactivity of eye closure,

including blepharospasm, and (b) true ptosis due to a paresis of the eyelid levators

(m. tarsalis superior or m. levator palpebrae) or to a disinsertion of the m. levator

palpebrae (aponeurotic ptosis). A paresis of the m. tarsalis is due to a lesion in the

central, intermediate or peripheral neuron of the sympathetic chain and constitutes

one of the components of Horner's syndrome. A paresis of the m. levator

palpebrae may be due to a failure in central innervation, in oculomotor (n.III)

function, in neuromuscular transmission or to a lesion in the muscle itself.

498. O'Riordan, J. I.; Gallagher, H. L.; Thompson, A. J.; Howard, R. S.; Kingsley, D.

P.; Thompson, E. J.; McDonald, W. I.; Miller, D. H. Clinical, CSF, and MRI

findings in Devic's neuromyelitis optica. J-Neurol-Neurosurg-Psychiatry. 1996

Apr; 60(4): 382-7; ISSN: 0022-3050.

ENGLAND. OBJECTIVES: Since Devic's original description of neuromyelitis

optica in 1894 there has been much debate regarding its aetiology. A specific cause

has been identified in a minority of cases but in most the question has arisen

whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This

study was undertaken to help clarify this issue. METHODS: Neuromyelitis optica

was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral

optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic

nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody

status was documented. Patients underwent CSF examination and MRI of brain

and spinal cord. RESULTS: Twelve patients, with a mean age of presentation of

35.1 years, were seen. Eleven were women; vision was reduced to counting

fingers or worse in 10 patients and seven became confined to a wheelchair.

Examination of CSF showed local synthesis of oligoclonal bands in only two

patients and a neutrophil pleocytosis in two. A possible aetiology was identified in

five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and

possible acute disseminated encephalomyelitis (two). Six had non-specific

increases in various autoantibodies. Eleven patients underwent MRI of the brain

and spinal cord. In 10 there were diffuse abnormalities involving cervical and

thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in

five; in five there were multiple deep white matter lesions, and one patient had

minor age related changes. CONCLUSION: It is proposed that Devic's

neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI

features different from those found in classic multiple sclerosis. In most cases a

specific aetiology is not identified, but an immunological mechanism of tissue

damage seems likely.

499. Ormerod, L. D.; Rhodes, R. H.; Gross, S. A.; Crane, L. R.; Houchin, K. W.

Ophthalmologic manifestations of acquired immune deficiency syndrome-

associated progressive multifocal leukoencephalopathy. Ophthalmology. 1996 Jun;

103(6): 899-906; ISSN: 0161-6420.

UNITED-STATES. Purpose: Progressive multifocal leukoencephalopathy (PML)

is increasingly described as a late complication of the acquired immune deficiency

syndrome (AIDS). The purpose of this study is to evaluate retrospectively the

ophthalmologic, clinical, and investigational aspects of AIDS-associated PML.

Methods: The authors evaluated ten patients in whom ophthalmologic

manifestations developed in the course of AIDS-associated PML. Findings at

clinical examination and their progression over time, neuroimaging correlates, the

results of pathologic investigation, and visual outcomes were reviewed. Results:

Progressive multifocal leukoencephalopathy was the AIDS-defining illness in six of

ten patients. Homonymous visual field defects were the presenting symptom in

three patients and detected in six patients overall. Occipital blindness developed in

one patient. Cerebellar signs and brain stem nuclear and supranuclear palsies also

were common. Confluent white matter lesions with increased intensity on T2-

weighted magnetic resonance imaging were supratentorial in seven patients and

infratentorial in three patients. With incomplete data, the median survival time was

3 months from PML onset. Histopathologic confirmation of PML diagnosis was

available for nine of the ten patients. Conclusions: The development of progressive

retrochiasmal visual field defects, supranuclear and nuclear cranial nerve palsies, or

nystagmus ataxia in the relatively young patient should alert the ophthalmologist to

the possibility of PML, particularly in the presence of long-tract central nervous

system signs or dementia. Progressive multifocal leukoencephalopathy will often

be human immunodeficiency virus associated. Human immunodeficiency virus

encephalopathy, cerebral toxoplasmosis, lymphoma, and infarction need to be

discriminated. Effective therapy is required urgently for this devastating disease.

500. Ortiz, O.; Schochet, S. S.; Kotzan, J. M.; Kostick, D. Meningioma of the optic nerve

sheath. AJNR-Am-J-Neuroradiol. 1996 May; 17(5): 901-6; ISSN: 0195-6108.

UNITED-STATES. 0; 0; 0; 6284-40-8; 67-43-6; 7440-54-2; 86050-77-3.

501. Osterman, A. L.; Babhulkar, S. Unusual compressive neuropathies of the upper limb.

Orthop-Clin-North-Am. 1996 Apr; 27(2): 389-408; ISSN: 0030-5898.

UNITED-STATES. Compression neuropathies are one of the most common

causes of upper extremity pain. This article has addressed the diagnosis and

treatment of some of the more unusual neuropathies. As always, a thorough

knowledge of anatomy and its valuation, and a careful and impulsive physical

examination will guide the clinician to the appropriate diagnosis. Electrical studies,

done carefully and with the clinical diagnosis as a label, will often be confirmatory.

In cases not responsive to nonoperative measures, surgery to decompress the nerve

and to restore missing function will usually proved a modicum of patient

improvement.

502. Osterman, A. L.; Davis, C. A. Subcutaneous transposition of the ulnar nerve for

treatment of cubital tunnel syndrome. Hand-Clin. 1996 May; 12(2): 421-33; ISSN:

0749-0712.

UNITED-STATES. Subcutaneous transposition of the ulnar nerve has been

widely reported as a successful surgical treatment for ulnar neuropathy at the elbow

attributable to a variety of causes. Accepted indications for anterior transposition

include any anatomic lesion that interferes with or impinges on the nerve along its

native course. This may include a tumor, ganglion, osteophyte, valgus deformity or

instability, or subluxation of the nerve, as listed previously. The surgical technique

of ASCT also was described thoroughly earlier in this article. Points that warrant

emphasis include thorough decompression along the entire course of the nerve, an

attempt to preserve the venous plexus that accompanies the nerve, identification and

preservation of branches of the medial antebrachial cutaneous nerve, and resection

of a 3 to 6 cm segment of the medial intramuscular septum. Poor prognostic

indicators include age over 50 years; relatively advanced neuropathy, as noted by

electrical evidence of demyelination; or aggravating medical conditions, such as

diabetes or alcoholism. Complications include neuroma of the medial antebrachial

cutaneous nerve and resubluxation posterior to the medial epicondyle. In cases of

reoperation for recurrent or persistent symptoms, inadequate release, most

commonly at the medial intramuscular septum, was sited as the cause of failure in

over 90% of cases. In a few cases, compression was found at the site of a

fasciodermal sling. The majority of complications therefore were technical in nature

and probably could have been avoided by strict attention to basic principles.

Controversy surrounds the appropriate treatment for approximately half of patients

in whom no clearly definable cause can be found. These cases are either attributed

to "repetitive strain" or lumped into the "idiopathic" category. The pathophysiology

leading to neuropathy in these groups is poorly understood, so the rationale for

choosing one surgical procedure over another remains somewhat obscure. In the

absence of an anatomic lesion, proponents of in situ decompression believe

transposition involves unnecessary dissection, with attendant risks of

devasularization or injury to the nerve or surrounding structures. Advocates of

ASCT point out that the nerve may be compressed at any of several points along its

course, as outlined in Fig. 1. Unlike in situ decompression, therefore, a properly

performed anterior transposition assures adequate decompression at all points along

its course. Indications for subcutaneous versus submuscular transposition are even

less clear. Some believe submuscular transposition should be performed for more

severe neuropathy, when muscular atrophy is present. Other authors point out that

thin patients will be susceptible to repeated minor trauma if the nerve is left in a

subcutaneous position. Neither of these contentions is supported consistently by

available published data. In most cases of failed subcutaneous transposition,

submuscular transposition has been used as a salvage procedure simply to place the

nerve in an unscarred bed. Answers to the unresolved issues await well-designed

studies. Nevertheless, there is ample cause for optimism given that adherence to

basic principles has resulted in satisfactory results for 85% to 95% of patients

regardless of the procedure chosen.

503. Otto, S. R. Neurofibromatosis [letter]. J-Am-Acad-Audiol. 1996 Aug; 7(4): 304;

ISSN: 1050-0545.

CANADA.

504. Ouvrier, R. A. Hereditary neuropathies in children: the contribution of the new

genetics. Semin-Pediatr-Neurol. 1996 Jun; 3(2): 140-51; ISSN: 1071-9091.

UNITED-STATES. Although the prevalence of the hereditary motor and sensory

neuropathies in childhood is not clearly established and the age of presentation may

overlap the arbitrary boundary between pediatric and adult neurology, the recent

explosion of genetic information regarding these conditions has completely altered

our understanding and classification of these diseases. The current status of our

understanding of the molecular basis of the hereditary neuropathies which might

present in childhood is reviewed. The impact of this information on our concepts of

the mechanisms operative in the production of the clinical signs and symptoms in

these diseases is discussed.

505. Padua, L.; Lo Monaco, M.; Valente, E. M.; Tonali, P. A. A useful electrophysiologic

parameter for diagnosis of carpal tunnel syndrome. Muscle-Nerve. 1996 Jan;

19(1): 48-53; ISSN: 0148-639X.

UNITED-STATES. In 43 patients (50 hands) with clinical manifestations of mild-

moderate carpal tunnel syndrome (CTS) and 36 healthy volunteers (40 hands),

orthodromic sensory nerve conduction velocity (SNCV) was measured with

surface electrodes in the median nerve between the third digit and palm and between

the palm and wrist. These figures were used to calculate the ratio of distal to

proximal conduction (distoproximal ratio). All 90 hands were also subjected to

other nerve conduction studies used for diagnosis of CTS. All control hands

presented distoproximal ratios < 1.0 reflecting higher conduction rates in the

proximal segment. In contrast, 49 of 50 CTS hands (98%) presented reversed

ratios (> 1.0) indicating compromised proximal conduction. The sensitivity of this

test was significantly greater than that of other methods evaluated, including

comparative studies and segmental study of the palm-wrist portion of the median

nerve. Segmental study of median SNCV with calculation of the distoproximal ratio

is a sensitive technique for diagnosis of CTS in patients with normal findings in

standard nerve conduction studies.

506. Pal, B.; Keenan, J.; Misra, H. N.; Moussa, K.; Morris, J. Raynaud's phenomenon in

idiopathic carpal tunnel syndrome. Scand-J-Rheumatol. 1996; 25(3): 143-5; ISSN:

0300-9742.

NORWAY. Both carpal tunnel syndrome and Raynaud's phenomonon are

common conditions in the general population. These two different conditions

frequently cause similar symptoms such as tingling, numbness, and "deadness of

the fingers". They may also co-exist for instance in scleroderma or rheumatoid

arthritis. In order to study the association, if any, between these two conditions, we

studied 93 patients with idiopathic carpal tunnel syndrome confirmed in electro-

physiological tests with 57 control subjects, for the presence of Raynaud's

phenomenon by means of a previously validated questionnaire. Raynaud's

phenomenon was detected significantly more frequently (P = 0.002) in patients

with idiopathic carpal tunnel syndrome (36%) compared to control subjects (12%).

Thus there appears to be an association between these two conditions. The

mechanism for this is not clear. Sympathetic dysfunction may play a part.

Practitioners should be aware of the similarity of the symptoms and the possibility

that the two conditions may co-exist.

507. Paladini, D.; Dellantonio, R.; Cinti, A.; Angeleri, F. Axillary neuropathy in volleyball

players: report of two cases and literature review. J-Neurol-Neurosurg-Psychiatry.

1996 Mar; 60(3): 345-7; ISSN: 0022-3050.

ENGLAND. Two cases of isolated neuropathy, not consequent to acute trauma, of

the axillary nerve of young volleyball players are described. Interest in the

pathology derives from the rarity of such case reports and the fact that the

pathogenesis may be linked to a specific sporting activity. The lesion site is thought

to be in the quadrilateral space.

508. Panjwani, M.; Truong, L. D.; Eknoyan, G. Membranous glomerulonephritis

associated with inflammatory demyelinating peripheral neuropathies. Am-J-

Kidney-Dis. 1996 Feb; 27(2): 279-83; ISSN: 0272-6386.

UNITED-STATES. A 55-year-old man with chronic inflammatory demyelinating

polyradiculoneuropathy developed the nephrotic syndrome. Renal biopsy showed

stage I membranous glomerulonephritis. Review of the literature revealed the

association of these two rare syndromes, considered to be due to immunologic

dysfunction, in two other cases, as well as several cases of the acute form of

demyelinating peripheral polyradiculoneuropathy. The nephrotic syndrome appears

to be persistent in the chronic form of the peripheral neuropathy but reversible in its

acute form following immunosuppressive therapy. The possibility of a common

immunopathogenesis in the association of membranous glomerulonephritis and

inflammatory demyelinating peripheral neuropathies deserves further scrutiny.

509. Papierski, P. Ulnar neuropathy at the wrist associated with a recurrent branch through

the flexor carpi ulnaris tendon. J-Hand-Surg-Br. 1996 Jun; 21(3): 347-8; ISSN:

0266-7681.

SCOTLAND.

510. Paradiso, G.; Micheli, F.; Taratuto, A. L.; Parera, I. C. Familial bulbospinal

neuronopathy with optic atrophy: a distinct entity. J-Neurol-Neurosurg-Psychiatry.

1996 Aug; 61(2): 196-9; ISSN: 0022-3050.

ENGLAND. A 61 year old woman and her 58 year old brother presented with the

clinical picture of late onset progressive bulbar and spinal muscular atrophy with

family history of involvement in successive generations. The sister also had optic

neuropathy and the brother developed diabetes mellitus and sex hormone

abnormalities. Neurophysiological and histopathological studies showed a pattern

of motor and sensory neuronopathy. There was no abnormal expansion of CAG

repeats in the androgen receptor gene. This family seems to have a previously

unrecognised entity with the bulbospinal neuronopathy phenotype.

511. Parano, E.; Pavone, L.; Falsaperla, R.; Trifiletti, R.; Wang, C. Molecular basis of

phenotypic heterogeneity in siblings with spinal muscular atrophy. Ann-Neurol.

1996 Aug; 40(2): 247-51; ISSN: 0364-5134.

UNITED-STATES. We report on a family with childhood-onset spinal muscular

atrophy with intrafamilial phenotypic variation. Typical of a large majority of such

patients, both the child with spinal muscular atrophy type I and the child with type

II were missing both copies of the survival motor neuron telomeric gene

(SMN(T)). The more severely affected child, however, showed genotypic evidence

consistent with the de novo loss of DNA sequence in addition to that inherited by

both affected children. These data suggest that the intrafamilial phenotypic variation

in this family results from a new mutation event in the more severely affected child.

Examples of intrafamilial phenotypic variability are quite rare, but some reports

exist in the spinal muscular atrophy literature. We present evidence that one

explanation for this phenomenon is the occurrence of de novo deletion events at the

highly unstable disease locus.

512. Parry, D. M.; MacCollin, M. M.; Kaiser Kupfer, M. I.; Pulaski, K.; Nicholson, H.

S.; Bolesta, M.; Eldridge, R.; Gusella, J. F. Germ-line mutations in the

neurofibromatosis 2 gene: correlations with disease severity and retinal

abnormalities. Am-J-Hum-Genet. 1996 Sep; 59(3): 529-39; ISSN: 0002-9297.

UNITED-STATES. Neurofibromatosis 2 (NF2) features bilateral vestibular

schwannomas, other benign neural tumors, and cataracts. Patients in some families

develop many tumors at an early age and have rapid clinical progression, whereas

in other families, patients may not have symptoms until much later and vestibular

schwannomas may be the only tumors. The NF2 gene has been cloned from

chromosome 22q; most identified germ-line mutations result in a truncated protein

and severe NF2. To look for additional mutations and clinical correlations, we used

SSCP analysis to screen DNA from 32 unrelated patients. We identified 20

different mutations in 21 patients (66%): 10 nonsense mutations, 2 frameshifts, 7

splice-site mutations, and 1 large in-frame deletion. Clinical information on 47

patients from the 21 families included ages at onset and at diagnosis, numbers of

meningiomas, spinal and skin tumors, and presence of cataracts and retinal

abnormalities. We compared clinical findings in patients with nonsense or

frameshift mutations to those with splice-site mutations. When each patient was

considered as an independent random event, the two groups differed (P < or = .05)

for nearly every variable. Patients with nonsense or frameshift mutations were

younger at onset and at diagnosis and had a higher frequency and mean number of

tumors, supporting the correlation between nonsense and frameshift mutations and

severe NF2. When each family was considered as an independent random event,

statistically significant differences between the two groups were observed only for

mean ages at onset and at diagnosis. A larger data set is needed to resolve these

discrepancies. We observed retinal hamartomas and/or epiretinal membranes in nine

patients from five families with four different nonsense mutations. This finding,

which may represent a new genotype-phenotype correlation, merits further study.

513. Parry, G. J. AAEM case report #30: multifocal motor neuropathy. Muscle-Nerve.

1996 Mar; 19(3): 269-76; ISSN: 0148-639X.

UNITED-STATES. A 73-year-old man with a 16-year history of fasciculations

and 15 years of weakness in his right arm was diagnosed with focal motor neuron

disease. After 10 years of purely motor symptoms, he developed mild parasthesias

although his sensory examination remained normal. Reflexes were reduced or

absent in the weak muscles but were normal elsewhere. Nerve conduction was

studied in nerves innervating weak muscles and showed severe motor conduction

block. Sensory nerve conduction studies were minimally abnormal, showing

reduced amplitudes with normal velocities. Based on the clinical picture and the

presence of severe motor conduction block, the patient was diagnosed as multifocal

motor neuropathy. Treatment with high-dose intravenous immunoglobulin was

given with significant improvement in strength and partial resolution of the

conduction block. As this case demonstrates, this treatable disorder may

occasionally be mistaken for motor neuron disease although the resemblance is only

superficial, and it should never be mistaken for amyotrophic lateral sclerosis.

Multifocal motor neuropathy is an inflammatory, demyelinating neuropathy which,

like chronic inflammatory demyelinating polyneuropathy (CIDP), is probably

immune-mediated. It differs from typical CIDP by virtue of a marked predilection

for motor axons, a strikingly restricted distribution, and a protracted course.

Treatment with high-dose intravenous immunoglobulin is frequently helpful, but

other forms of immune manipulation are less effective.

514. Pascual Castroviejo, I.; Lopez Martin, V.; Martinez Bermejo, A.; Roche, C. [Behr's

syndrome. A report of 2 siblings studied by MR]. Sindrome de Behr. Presentacion

de dos hermanos con estudio por RM. An-Esp-Pediatr. 1996 Jun; 44(6): 593-5;

ISSN: 0302-4342.

SPAIN.

515. Passero, S.; Nuti, D. Auditory and vestibular system findings in patients with

vertebrobasilar dolichoectasia. Acta-Neurol-Scand. 1996 Jan; 93(1): 50-55; ISSN:

0001-6314.

DENMARK. Impairment of the auditory-vestibular system has been reported in

patients with vertebrobasilar dolichoectasia (VBD), but little is known about the

underlying cause of the symptoms. Auditory testing (pure tone audiometry,

auditory brain stem response and stapedius reflex) and vestibular tests (assessment

of nystagmus, eye tracking tests, caloric test and rotational test) were performed in

23 patients with auditory-vestibular symptoms and/or cranial nerve impairment

associated with VBD. Specific evidence of auditory and/or vestibular system

impairment was observed in 19 cases (83%). Among patients with abnormal test

findings, 47% had evidence suggesting peripheral impairment, 16% evidence

suggesting central dysfunction, and 37% evidence suggesting both peripheral and

central dysfunction. Although compression of the vestibulocochlear nerve plays an

important role in the genesis of the auditory-vestibular dysfunction in patients with

VBD, mechanism such as brain stem-cerebellar ischemia and impaired blood

supply to the vestibular labyrinth may be just as important.

516. Payner, T. D.; Tew, JM Jr. Recurrence of hemifacial spasm after microvascular

decompression. Neurosurgery. 1996 Apr; 38(4): 686-90; discussion 690-1; ISSN:

0148-396X.

UNITED-STATES. The success of medical and surgical treatment for hemifacial

spasm, and involuntary paroxysmal unilateral contraction of the facial muscles, has

been mixed. Although microvascular decompression has the greatest reported

success, symptom recurrence affects many patients in whom treatment was initially

successful. In this study, we report the results of 34 patients who underwent

microvascular decompression of the facial nerve from 1976 to 1989 as well as

review the literature concerning the incidence and timing of recurrence in more than

600 patients who underwent microvascular decompression. In this series, 94% of

34 patients had continuous relief of spasm after surgical treatment (mean duration,

> 6 yr). Of those patients whose spasms were completely initially relieved, 10.3%

developed some degree of recurrent spasm; however, no patient developed a

recurrence after 24 months without spasm. Our review of the literature discloses

that 86% of all recurrences occurred within 2 years of surgery. Patients who have

no recurrence of symptoms 2 years after surgical treatment have only a 1% chance

of developing recurrent hemifacial spasm. We also comment on possible causes of

treatment failure and recurrence of hemifacial spasm after surgical treatment.

517. Pellegrini, M.; O'Brien, T. J.; Hoy, J.; Sedal, L. Mycoplasma pneumoniae infection

associated with an acute brainstem syndrome. Acta-Neurol-Scand. 1996 Feb; 93(2-

3): 203-6; ISSN: 0001-6314.

DENMARK. A patient who developed an acute brainstem syndrome following

Mycoplasma pneumoniae respiratory infection is reported. MRI showed changes

consistent with brainstem demyelination. Clinical features and laboratory

investigations support an immune mediated mechanism with no evidence of

direction CNS invasion. On the basis of this case and a review of the literature, we

postulate two mechanisms for the development of M. pneumoniae associated CNS

disease: direct CNS invasion causing meningitis and an immune-mediated acute

disseminated encephalomyelitis (ADEM). This has obvious therapeutic

implications.. 0.

518. Penrith, M. L.; Tustin, R. C.; Thornton, D. J.; Burdett, P. D. Swayback in a blesbok

(Damaliscus dorcas phillipsi) and a black wildebeest (Connochaetes gnou). J-S-

Afr-Vet-Assoc. 1996 Jun; 67(2): 93-6; ISSN: 0301-0732.

SOUTH-AFRICA. Ataxia associated with myelopathy and low liver copper

concentrations is described in a blesbok (Damaliscus dorcas phillipsi) and black

wildebeest (Connochaetes gnou) from the Karoo Nature Reserve, Graaff-Reinet.

This syndrome, which occurs in neonatal and delayed forms, has been described in

sheep, goats, pigs and domesticated red deer, but reports in other ungulates,

including antelope, are very rare and generally unconfirmed by histopathological

examination. The 2 animals examined exhibited a typical pattern of Wallerian

degeneration in selected tracts of the spinal cord. Many blesbok in the reserve are

markedly pale in colour. Fading of the hair is also associated with copper

deficiency in ungulates. High lamb mortality occurs as a result of the ataxia and

threatens the survival of the blesbok herd in the reserve. Confirmation of low liver

copper concentrations in affected animals offers direction towards the solution of

the problem.. 0; 7440-50-8.

519. Perez Diaz, C. J.; Villarejo, F. J.; Pascual, A. M. Trigeminal neurinomas in infants:

report of two cases. Childs-Nerv-Syst. 1996 May; 12(5): 283-6; discussion 287;

ISSN: 0256-7040.

GERMANY. Two cases of trigeminal neurinoma in two infant girls aged 3 and 6

months are reported. Both presented with temporal cranial vault bulging at birth.

The 6-month-old patient suffered onset of focal fits 1 month before admission and

her neurological examination revealed no abnormalities. The 3-month-old patient

had right exophthalmus and a subcutaneous fronto-orbital plexiform neurofibroma

at birth. Neurological examination disclosed a sensory deficit of the first trigeminal

nerve division. She also had a family medical history of von Reckling-hausen's

disease. The incidence of trigeminal neurinomas in children is reviewed. The

patients in these two cases are the youngest recorded; the cases are the only ones

reported in infants. Clinical, radiological, and therapeutic aspects are discussed.

520. Perren, B. A.; Raisanen, J.; Good, W. V.; Crawford, J. B. Cytomegalovirus retinitis

and optic neuritis in a child with severe combined immunodeficiency syndrome.

Retina. 1996; 16(2): 117-21; ISSN: 0275-004X.

UNITED-STATES. BACKGROUND. Although cytomegalovirus (CMV)

infection of the retina and brain is common in patients with acquired

immunodeficiency syndrome (AIDS), it is exceedingly rare in patients with

immunodeficiencies due to other causes. This is the first report on ocular and

cerebral histopathology of disseminated CMV in a child with severe combined

immunodeficiency syndrome (SCID). METHODS. The authors examined by

routine histopathologic methods the eyes of a 2-year-old white boy with SCID and

bilateral CMV retinitis who died after failure of a third attempt at allogeneic bone

marrow transplantation (BMT). RESULTS. Cytomegalovirus inclusions were

found in the necrotic retinal remnants, in the hyperplastic and scarred retinal

pigment epithelium, and bilaterally in the optic nerves. There were infiltrates of

macrophages in response to the infection or the infused silicon, but no lymphoid

infiltrates. Cytomegalovirus inclusions also were found in brain tissue.

CONCLUSION. The histologic features resembled those of CMV retinitis and

optic neuritis in AIDS.

521. Perron, O.; Fassier, F.; Joncas, J. [Herniated disk and congenital spinal stenosis in the

adolescent]. Hernie discale et stenose congenitale du canal lombaire chez

l'adolescent. Rev-Chir-Orthop-Reparatrice-Appar-Mot. 1996; 82(1): 29-33; ISSN:

0035-1040.

FRANCE. PURPOSE OF THE STUDY: The goal of this study was to

demonstrate the relationship between herniated discs and congenital spinal stenosis.

A retrospective study was conducted on patients seen in our center over a five year

period. MATERIAL AND METHODS: The CT-Scan evaluation was essential for

the measurement of the diameter of the spinal canal. Data were compared with the

values obtained in ten normal subjects. RESULTS: Twelve complete charts of

adolescents with herniated discs were reviewed and in all cases a spinal stenosis

was established. The mean diameter of the canal was 8.1 mm for the patient group

compared with 18.1 mm for the control group. CONCLUSION: The relationship

of herniated discs and spinal stenosis found here is greater than the one reported in

the literature. This relationship accounts for the importance of neurological (9/12

patients) involvement found in our series in comparison with adults. This also

explains the failure of conservative treatment.

522. Persico, M.; Suozzo, R.; De Seta, M.; Montella, F.; Torella, R.; Gentile, S. Non-ulcer

dyspepsia and Helicobacter pylori in type 2 diabetic patients: association with

autonomic neuropathy. Diabetes-Res-Clin-Pract. 1996 Mar; 31(1-3): 87-92; ISSN:

0168-8227.

IRELAND. Diabetic patients often suffer from symptoms arising from the

gastrointestinal tract. Several factors are considered responsible for these

alterations, including abnormalities of gastric motility. Recently Helicobacter pylori

(HP) has been identified in a relevant aliquot of subjects with or without

gastrointestinal abnormalities, but only scarce and controversial data are available

on the prevalence of HP and the association between HP and chronic gastritis or

peptic ulcer in diabetic patients. In addition, the possible association between

alterations of gastric motility induced by autonomic neuropathy (AN) and the

presence of HP has never been evaluated in diabetic subjects. In this study we

document the presence of HP in the gastric biopsies of 73% out of a series of 29

patients affected by type 2 diabetes and non-ulcer dyspepsia (3 with oesophagitis,

10 with gastritis, 7 with bulbar duodenitis, and 9 with a normal endoscopy), with a

significantly higher prevalence (P < 0.01) in subjects with AN (74%) than in

subjects without AN (26%). Two other tests have been compared with the

histological evidence of HP (used as golden standard), i.e. the urease test (CP-test)

and the assay of anti-HP G-immunoglobulins, both of which were positive in a

significantly (P < 0.01) higher percentage of neuropathic patients in comparison

with non-neuropathic patients. The sensitivity and the specificity of the CP-test

were 96% and 100%, respectively. Similarly, both the sensitivity and the

specificity of the assay of IgG HP-Ab were 100%. Since patients affected by non-

ulcer dyspepsia and NIDDM complicated by autonomic neuropathy are under a

higher risk to be carriers of HP than non-neuropathic or non-diabetic patients. The

assay of serum IgG HP-Ab could be used as a screening method, thus avoiding the

more expensive and time-consuming endoscopy.. EC 3.5.1.5; 0.

523. Phadke, R. V.; Agarwal, P.; Sharma, K.; Chauhan, S. S. Idiopathic duro-optic

calcification--a new entity? Clin-Radiol. 1996 May; 51(5): 359-61; ISSN: 0009-

9260.

ENGLAND. This is a report of two patients with intracranial dural and optic

nerve/sheath calcification. CT and MR features of this previously unreported

condition are illustrated.

524. Pica, RA Jr; Rockwell, B. H.; Raji, M. R.; Dastur, K. J.; Berkey, K. E. Traumatic

internal carotid artery dissection presenting as delayed hemilingual paresis. AJNR-

Am-J-Neuroradiol. 1996 Jan; 17(1): 86-8; ISSN: 0195-6108.

UNITED-STATES. A case of internal carotid artery dissection presenting as

delayed right hemilingual paresis after blunt craniocervical trauma is presented.

Diagnosis is discussed with emphasis on MR and MR angiographic findings.

Mechanisms of injury and lower cranial nerve palsy are also briefly discussed.

525. Piccoli, B.; Braga, M.; Zambelli, P. L.; Bergamaschi, A. Viewing distance variation

and related ophthalmological changes in office activities with and without VDUs.

Ergonomics. 1996 May; 39(5): 719-28; ISSN: 0014-0139.

ENGLAND. This experimental study was conducted on 14 volunteer female clerks

to measure viewing distance variation and related ophthalmological changes in

office activities with (session 1) and without (session 2) VDU. A video camera

connected to an electronic elaborator was used to determine eye-screen distance of

the VDU operators during the task. Before and after each of the two work sessions

far refraction, far phorias, near point of accommodation (APP), and fusional

convergence were measured. Blinking rate of each subject was measured by a

transducer only during the "VDU session'. The objective measurements of the eye-

screen distance, whose range was between 48.42 and 65.33 cm, allowed a precise

quantification of the occupational visual load during session 1. The accommodation

was about 1.5-2D on average and the fusional convergence 10-13 delta on average.

The symptomatology shows a number of disturbances in session 1 (71.4%) clearly

higher than in session 2 (35.7%). The study of refraction before and after the task

has shown a slight excess of accommodation compared to the theoretical value in

both sessions, more marked in session 1. The APP shows no statistical

significative variation. A tendency to exophoria was registered at the beginning and

at the end of the trial and the fusional convergence clearly decreased, especially in

the first session.

526. Pierpaolo, L.; Mastronardi, L.; Strano, S.; Di Biasi, C.; Trasimeni, G. Neurovascular

conflict and essential arterial hypertension: MR evaluation [letter]. AJNR-Am-J-

Neuroradiol. 1996 Jan; 17(1): 195-6; ISSN: 0195-6108.

UNITED-STATES.

527. Pilo, L.; Ring, H.; Quinn, N.; Trimble, M. Depression in multiple system atrophy and

in idiopathic Parkinson's disease: a pilot comparative study. Biol-Psychiatry. 1996

May 1; 39(9): 803-7; ISSN: 0006-3223.

UNITED-STATES. Multiple system atrophy (MSA) is a disease causing

parkinsonism in which response to levodopa is classically absent, poor, or

transient. Idiopathic Parkinson's disease (IPD) itself, which responds favorably to

levodopa, has been associated with the development of disease-related depression.

Over and above the clinical and pathological characteristics of IPD, MSA causes

additional, more widespread, clinical and pathological deficits. We have compared

motor disability and mood in 12 patients with MSA and 12 with IPD. There was

more severe motor disability, but no clinical evidence of depression among the

MSA patients studied, and their Beck Depression Inventory scores did not differ

significantly from the group with IPD. We conclude that depression does not

appear to be more common in MSA than in IPD.

528. Piza Katzer, H.; Laszloffy, P.; Herczeg, E.; Balogh, B. [Complications of endoscopic

carpal tunnel operations]. Komplikationen bei endoskopischen Karpaltunnel-

Operationen. Handchir-Mikrochir-Plast-Chir. 1996 May; 28(3): 156-9; ISSN:

0722-1819.

GERMANY. This report is about four female patients admitted to our hospital

because of pain, dysaesthesia and paraesthesia following endoscopic release of the

carpal tunnel, who underwent revisional surgery. Intraoperatively, we found a

partial lesion of the median nerve in two cases and an incomplete release of the

ligament in the remaining two cases. The complete decompression and

epineurotomy of the median nerve and a nerve graft in two patients resulted in

clinical improvement.

529. Pla, M. E.; Dillingham, T. R.; Spellman, N. T.; Colon, E.; Jabbari, B. Painful legs

and moving toes associates with tarsal tunnel syndrome and accessory soleus

muscle. Mov-Disord. 1996 Jan; 11(1): 82-6; ISSN: 0885-3185.

UNITED-STATES. Painful legs, moving toes is a rare syndrome characterized by

leg pain and uncontrolled toe movements. We present a 35-year-old man with a 1-

year history of unilateral knee, calf, and medial ankle pain with spontaneous

movements of second through fifth toes. Electrodiagnostic studies showed an

absent lateral plantar nerve response consistent with a tarsal tunnel entrapment

neuropathy. Cine magnetic resonance imaging revealed a large accessory soleus

muscle compressing the flexor hallucis longus in the tarsal tunnel of the affected

extremity. Lidocaine block of the tibial nerve at the popliteal fossa did not stop

these movements, but blockade of the medial and lateral plantar nerves distal to the

medial malleolus stopped them temporarily. Treatment with foot orthotics and

cessation of running activity decreased the symptoms. We conclude that painful leg

and moving toes in this patient resulted from a compression neuropathy at the tarsal

tunnel possibly caused by a large adjacent accessory soleus muscle.

530. Plancher, K. D.; McGillicuddy, J. O.; Kleinman, W. B. Anterior intramuscular

transposition of the ulnar nerve. Hand-Clin. 1996 May; 12(2): 435-44; ISSN:

0749-0712.

UNITED-STATES. The surgical management of cubital tunnel syndrome is well

documented in the literature. Anterior intramuscular transposition of the ulnar nerve

is indicated for chronic cubital tunnel syndrome with symptoms refractory to

conservative therapy. Prompt diagnosis is essential to yield excellent results.

Extreme care must be exercised in the performance of anterior intramuscular

transposition. The surgeon must know the details of medial epicondylar anatomy

and pathophysiology, as well as all possible sites of potential nerve compression.

The placement of the transposed nerve in an intramuscular bed requires that all

fibrous septae are resected from the shallow trough created for the nerve to avoid

scar formation. Postoperatively, the arm is immobilized for 3 weeks, after which

range-of-motion exercises are begun. By the eighth postoperative week, most

patients are able to resume their regular activities, including manual labor.

Recurrence or persistence of symptoms postoperatively typically is traced to an

inadequate decompression of the nerve. Common sites of persistent ulnar nerve

compression include (1) the medial intermuscular septum, (2) the arcade of

Struthers, (3) fibrous bands immediately proximal or distal to the cubital tunnel, (4)

persistence or kinking at the arcuate ligament of Osborne, (5) Spinner's ligament or

other fascial slings, and (6) incomplete anterior transposition. Anterior

intramuscular transposition of the ulnar nerve is attractive for its relative ease of

dissection, simplicity, reliability, and low morbidity. Transposition of the nerve

into a shallow muscular trough deep only to the flexor-pronator fascia is a logical,

effective, and consistently reliable method of treating cubital tunnel syndrome

refractory to conservative management.

531. Plancher, K. D.; Peterson, R. K.; Steichen, J. B. Compressive neuropathies and

tendinopathies in the athletic elbow and wrist. Clin-Sports-Med. 1996 Apr; 15(2):

331-71; ISSN: 0278-5919.

UNITED-STATES. Overuse syndromes of the upper extremity in the athletic

population are a common and often difficult problem for physician and patient

alike. Optimal function of the upper extremity is tied intimately to success in many

sporting activities. Correct diagnosis and proper care require a thorough knowledge

of the pertinent anatomy, pathophysiology, and pathomechanics involved in each

disorder. Conservative care with rest, activity modification, and medication is

adequate for most athletic injuries. Surgical intervention may be indicated for

continuing pain, decreased performance, or to prevent chronic changes. Surgery

must be followed by thoughtfully prepared training and rehabilitation programs to

optimize the chances of a successful outcome.

532. Plumb, R.; Doolittle, G. C. Paraneoplastic pemphigus in a patient with non-Hodgkin's

lymphoma. Am-J-Hematol. 1996 May; 52(1): 58-9; ISSN: 0361-8609.

UNITED-STATES. Paraneoplastic pemphigus (PNP) is an autoimmune disorder

occurring in the setting of an underlying neoplasm in which patients have

polymorphous skin and mucous membrane lesions. We describe a patient with

non-Hodgkin's lymphoma who developed bullous, ulcerating lesions in an area

being treated with radiation therapy. The diagnosis of PNP was confirmed by

indirect immunofluorescence of the patient's serum on rat bladder. The disorder

was refractory to therapy, and ultimately the patient expired.. 0; 0; 446-86-6.

533. Plummer, E. S.; Albert, S. G. Focused assessment of foot care in older adults. J-Am-

Geriatr-Soc. 1996 Mar; 44(3): 310-3; ISSN: 0002-8614.

UNITED-STATES. OBJECTIVES: The purpose of this study was to investigate

the prevalence and characteristics of foot problems in non-diabetic individuals

compared with those in a diabetic population in order to develop recommendations

for preventive foot care in older people. DESIGN: Retrospective review of a

convenience sample of all patients referred to a Foot Care Service during a 24-

month period. SETTING: University Health Sciences Center. PARTICIPANTS: A

total of 308 patients aged 33 to 95 years (176 women and 132 men), of whom 183

had diabetes mellitus (DM) and 125 were without DM. INTERVENTION: Each

subject had a detailed history of foot care behavior and a thorough foot examination

for peripheral neuropathy (PN), peripheral vascular disease (PVD), and foot

deformities. PN was evaluated using the Semmes-Weinstein monofilament test.

PVD was graded by clinical measures and Doppler examination (ankle:brachial

index < 0.8). MEASUREMENTS: Forty-three percent of subjects with diabetes

had PN, 32% had PVD, and 19% had both conditions. Eighteen percent of subjects

without diabetes had PN, 21% had PVD, and 6% had both. The risk of foot

abnormalities in those without DM increased with age (r = .99, P = .0002). Thirty-

eight percent of non-diabetic patients older than age 60 had one or more of these

major risk factors and would be considered at high risk for the development of foot

ulcers or amputation. Ninety percent of the subjects reported inappropriate foot care

practices. Forty-seven percent of non-diabetic individuals with PN or PVD wore

inappropriate shoes. CONCLUSION: Older individuals without DM are at high

risk for foot-related disease and should receive the same foot care screening,

education, and follow-up as those with DM. Older people who have PN, PVD, or

physical and psychosocial limitations, may require referral to foot care specialists.

534. Polak, B. C.; Wijngaarde, R.; Bertelsmann, F. W. [Double vision as a result of

diabetic neuropathy]. Dubbelzien ten gevolge van diabetische neuropathie. Ned-

Tijdschr-Geneeskd. 1996 Apr 6; 140(14): 753-5; ISSN: 0028-2162.

NETHERLANDS.

535. Pollack, I. F.; Kinnunen, D.; Albright, A. L. The effect of early craniocervical

decompression on functional outcome in neonates and young infants with

myelodysplasia and symptomatic Chiari II malformations: results from a

prospective series. Neurosurgery. 1996 Apr; 38(4): 703-10; discussion 710; ISSN:

0148-396X.

UNITED-STATES. The indications for hindbrain decompression in neonates and

young infants with spinal dysraphism who experience brain stem dysfunction in

association with Chiari II malformations have remained controversial. This largely

reflects the fact that the postoperative outcome in such patients has often been poor,

which has supported the belief that much of the brain stem compromise in these

patients is congenital and inherently irreversible. However, in a previous

retrospective review of our operative results between 1975 and 1989, we noted that

a significant component of the brain stem dysfunction in these children was an

acquired phenomenon that potentially was reversible with prompt operative

intervention. Accordingly, we hypothesized that with early craniocervical

decompression, excellent functional outcome could be achieved in a majority of

neonates and young infants with symptomatic Chiari II malformations. On the basis

of this premise, we prospectively treated all such patients since 1989 with urgent

brain stem decompression after other potential causes for brain stem dysfunction,

such as progressive hydrocephalus, had been ruled out. All children underwent

limited suboccipital craniectomies, cervical laminectomies extending beneath the

inferior extent of the cerebellar tissue, and dural decompressions. The outcome in

these patients has been favorable in comparison with previous studies. Ten of the

13 children treated according to this protocol recovered normal or nearly normal

brain stem function shortly after decompression; 1 child had mild residual unilateral

lower cranial nerve paresis. None of these children required a tracheostomy for

ventilatory support, and only one required a temporary gastrostomy. The other

three children all exhibited bilateral vocal cord paralysis and severe central

hypoventilation by the time decompression was performed and failed to have any

meaningful recovery of function. We conclude that early recognition of the

symptoms of brain stem compromise in neonates and young infants with spinal

dysraphism coupled with urgent evaluation and decompression are effective in

producing prompt resolution of the brain stem dysfunction in most affected

patients. Conversely, the prognosis for recovery is poor in children who exhibit

bilateral vocal cord paralysis by the time of decompression.

536. Poole, G. V.; Thomae, K. R. Thoracic outlet syndrome reconsidered. Am-Surg. 1996

Apr; 62(4): 287-91; ISSN: 0003-1348.

UNITED-STATES. During a four year period, 50 patients were evaluated for

possible thoracic outlet syndrome (TOS). These 11 men and 39 women ranged in

age from 27 to 60 years, with a mean age of 38.6 years. Their symptoms had been

present from 3 months to 10 years (mean = 2.1 years). Twenty-seven had

previously undergone 33 operations, including carpal tunnel release, shoulder

arthroscopy, rotator cuff repair, cervical discectomy, and first rib resection, all

without benefit. Patients were evaluated by history, physical examination, and

radiographs of the cervical spine and chest. Additional studies such as

electromyography/nerve conduction studies, computed tomography, magnetic

resonance imaging, angiography, and myelography were obtained selectively. Only

12 patients were thought to have TOS, seven of whom underwent operation. Four

had complete resolution of symptoms; three were improved but had residual

symptoms for associated problems. Three patients who were not thought to have

TOS underwent first rib resection in other hospitals; none was improved after

surgery. The only study of positive value was evidence of unilateral subclavian

artery compression with shoulder positioning on physical examination. All other

studies were of value only if they demonstrated some other cause of the patient's

symptoms. Of the 35 patients without TOS in whom long-term follow-up was

obtained, four underwent appropriate operations with benefit, and 20 had good

results from physical therapy and nonoperative management. Patients whose

symptoms were work-related, and those who had engaged the services of a lawyer,

were less likely to demonstrate improvement, regardless of the treatment employed.

TOS is a relatively unusual cause of upper extremity pain and dysfunction. History

and physical examination are the most important diagnostic studies, and

radiographs of the chest and cervical spine and electromyography/nerve conduction

studies are useful to identify other causes of pain and disability. Careful selection of

patients for surgery can yield satisfactory results. A coordinated team of surgeons,

neurologists, and physical therapists is important in the management of these

patients.

537. Popova, L. M.; Avdiunina, I. A.; Alferova, V. P.; Piradov, M. A.; Pavlov, E. V.;

Plekhanova, S. A. [Respiratory insufficiency in adults with diphtheric

polyneuropathy]. Dykhatel'naia nedostatochnost' pri difteriinoi polineiropatii u

vzroslykh. Anesteziol-Reanimatol. 1996 Mar; (2): 9-13; ISSN: 0201-7563.

RUSSIA. Twenty-five adult patients with grave diphtheric polyneuropathy after

toxic diphtheria were followed up. Two symptom complexes of neurologic

disorders leading to the development of peripheral respiratory failure of different

severity were distinguished: 1) predominating glossopharyngeal paralysis and 2)

combination of glossopharyngeal paralysis with grave generalized sensorimotor

polyneuropathy (with pareses and paralyses of the respiratory muscles). The major

quantitative parameters of pulmonary functions associated with various

manifestations of respiratory failure are characterized. The significance of

information on the type of dysfunction of vocal cords is emphasized. A high

efficacy of a complex of respiratory reanimation used in the treatment of patients

with peripheral respiratory failure is demonstrated.

538. Porta Etessam, J.; Garcia Ruiz, F.; Hernandez Lain, A.; Luna, A.; Gomez, de la

Fuente E. [Atypical internuclear ophthalmoplegia in a patient with subacute

lymphocytic meningitis]. Oftalmoplejia internuclear atipica en un paciente con

meningitis linfocitaria subaguda. Rev-Neurol. 1996 Jul; 24(131): 838-40; ISSN:

0210-0010.

SPAIN. The local expression of tuberculous meningitis has an high incidence.

They respond to a Koch bacillus direct lesion or vasculitis disease. The clinic

implication of ocular movement is frequent and usually answers to a nervous trunk

direct injury. Exceptionally the clinic expression of the oculomotor disturbance is

due to a conjugate ocular tract or nucleus damage. The posterior internuclear

ophthalmoplegia is a well-known manifestation and correspond with a dwindle of

the medial longitudinal fasciculus electrical transmission and lies in falling of the

medial rectus to move synchronously with the contralateral rectus on attempted

lateral gaze to either side. In such a case the medial longitudinal fasciculus is

damaged between the sith nerve nucleous below and the opposite third nerve

nucleus above. A patient with AIDS B3 category who presents right internuclear

ophthalmoplegia and right eye vertical conjugate gaze impairment with left vertical

nystagmus due to a paramedial mesencephalic stroke as debut of a linfocitary

subacute meningitis is present.

539. Pozzati, A.; Pancaldi, L. G.; Di Pasquale, G.; Pinelli, G.; Bugiardini, R. Transient

sympathovagal imbalance triggers "ischemic" sudden death in patients undergoing

electrocardiographic Holter monitoring. J-Am-Coll-Cardiol. 1996 Mar 15; 27(4):

847-52; ISSN: 0735-1097.

UNITED-STATES. OBJECTIVES. The aim of this study was to investigate the

relation between "ischemic" sudden death (arrhythmic death preceded by ST

segment shift) and autonomic nervous system activity. Background. Mechanisms

precipitating sudden death are poorly known despite the importance of detecting

functional factors that may contribute to such a fatal event. METHODS. We

analyzed the tapes of eight patients (seven men and one woman with a mean age of

66 +/- 8 years) who had ischemic sudden death during ambulatory

electrocardiographic (Holter) monitoring. Four patients had unstable and four had

stable angina; none was taking antiarrhythmic drugs. Twenty patients with angina

and transient myocardial ischemia during Holter monitoring served as control

subjects. Arrhythmias, ST segment changes and heart rate variability were analyzed

by a computerized interactive Holter system. RESULTS. Five patients had

ventricular tachyarrhythmias (ventricular fibrillation in three, ventricular tachycardia

in two), and three had bradyarrhythmias (atrioventricular block in two, sinus arrest

in one) as the terminal event; all eight patients showed ST segment shift (maximal

change 0.46 +/- 0.16 mV; with ST elevation in two) that occurred 41 +/- 34 min

(mean +/- SD) before sudden death. The standard deviation of normal RR intervals

(SDNN) was 89 +/- 33 ms during the 10 +/- 6 h of Holter monitoring; 5 min before

the onset of the fatal ST shift, SDNN measurements were significantly lower than

during the initial 5-min period (48 +/- 10 vs. 29 +/- 9 ms; p=0.002). In control

patients, the SDNN was 102 +/- 39 ms during Holter monitoring, whereas it

measured 56 +/- 30 ms 5 min before the most significant episode of ST shift

(p<0.01 vs. 129 +/- 9 ms in the group with sudden death). CONCLUSIONS.

Autonomic dysfunction, as detected by a marked decrease in heart rate variability,

is present in the period (5 min) immediately preceding the onset of the ST shift

precipitating ischemic sudden death. These data suggest that sympathovagal

imbalance may trigger fatal arrhythmias during acute myocardial ischemia, thus

resulting in sudden death.

540. Prados, J.; Melguizo, C.; Aranega, A. E.; Escobar Jimenez, F.; Cobo, V.; Aranega,

A. Detection of circulating alpha-actinin in non-insulin-dependent diabetics with

autonomic dysfunction. Cardiology. 1996 Jul; 87(4): 283-6; ISSN: 0008-6312.

SWITZERLAND. The presence of cardiac proteins in serum has been related to

ischemic heart diseases such as acute myocardial infarction and angina pectoris,

which are more frequent in non-insulin-dependent diabetic patients. Silent

myocardial ischemia, which is also more frequent in these patients, occurs in

association with autonomic dysfunction. We used Western blot analysis to search

the myocardial protein alpha-actinin in sera from non-insulin-dependent diabetic

patients with or without autonomic dysfunction. Of the 24 diabetic patients with

neuropathy, 18 were positive for circulating alpha-actinin; this protein was found in

only 1 of the 22 diabetic patients without neuropathy. Our results showed a

significant correlation between non-insulin-dependent diabetic patients with

neuropathy and detectable circulating alpha-actinin in serum, and suggest that the

determination by immunoblotting of serum alpha-actinin in these patients may be an

effective method to detect myocardial cell impairment, and to identify diabetic

patients that may need special consideration.. 0; 0.

541. Prakash, P.; Sharma, P.; Rao, V. M.; Shastry, P.; Menon, V. Polaroid scotometer: a

new device to chart suppression scotomata. J-Pediatr-Ophthalmol-Strabismus.

1996 May; 33(3): 181-4; ISSN: 0191-3913.

UNITED-STATES. Suppression, an active inhibition of vision in one eye under

binocular conditions, is a sensory adaptation in concomitant strabismus. The

characteristics and extent of suppression differ not only in different types of

strabismus but also in the same type under different testing conditions, which may

differ in their degree of dissociation. The findings in exodeviations have thus varied

from regional suppression to hemiretinal trigger, and in esodeviations from two

point scotoma to single large scotoma. There is a need for a simple but less

dissociative test to chart the fields under binocular conditions. We have devised a

new, simple instrument based on polaroid dissociation. The findings corroborate

the findings of more sophisticated tests like phase-difference haploscope.

542. Preis, S.; Majewski, F.; Hantschmann, R.; Schumacher, H.; Lenard, H. G.

Goldenhar, Mobius and hypoglossia-hypodactyly anomalies in a patient: syndrome

or association? Eur-J-Pediatr. 1996 May; 155(5): 385-9; ISSN: 0340-6199.

GERMANY. The Mobius, Goldenhar and hypoglossia-hypodactyly anomalies are

usually sporadic conditions with a recurrence risk of about 2%. The combination of

Goldenhar and one or the two others is rare, whereas the concomitant occurrence of

Mobius and hypoglossia-hypodactyly, and/or Poland, and/or Klippel-Feil anomaly

is well known. Pathogenetically, vascular disruptions around the 4th embryonic

week have been hypothesized. In vivo and pathological studies as well as animal

models support this theory for all the above-mentioned combinations. Whether a

preceding blastogenetic alteration is an influencing factor or a disorganization

mutation, remains unclear. We describe a 3-year-old girl with bilateral anotia,

epidermoid on the right eye, 6th and 7th nerve palsy, hypoglossia, left

hypodactyly, and ventricular septal defect. CONCLUSION: We wish to emphasize

the aetiological relevance of vascular disruptions in this previously unreported

combination of Mobius, Goldenhar and hypoglossia-hypodactyly anomalies. The

concurrence of anomalies in this patient represents an association and not a

pleiotropic syndrome.

543. Preslan, M. W.; Novak, A. Baltimore Vision Screening Project. Ophthalmology. 1996

Jan; 103(1): 105-9; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: This study estimates the prevalence of common

visual disorders (amblyopia, strabismus, refractive errors) in a group of inner-city

school children. In addition, the study addresses the issue of access to care for

vision-screening programs, specifically for children with recognized difficulties in

obtaining routine medical care. METHODS: School children from an inner-city

elementary school were enrolled into a prospective vision-screening program

combining the identification arm (screening) and diagnostic/treatment arm

(ophthalmic examination). The screening consisted of Snellen E optotypes

presented at a 10-foot test distance. Each child failing the vision screening was

examined by an ophthalmologist at the school using standard protocol. This

allowed the authors to examine all children identified through the vision-screening

program. RESULTS: Six-hundred eighty children were screened during the 1993

to 1994 school year. Eleven percent (76) failed the vision screening and were

examined, 68 of whom failed the ophthalmic examination. The estimated

prevalence of visual morbidity was as follows: amblyopia, 3.9%; strabismus,

3.1%, and refractive errors, 8.2%. CONCLUSION: Amblyopia, strasbismus, and

refractive errors were found in relatively high frequencies for this population

sample of inner city children. These findings underscore the necessity of

comprehensive vision-screening programs that integrate follow-up care. Children

with limited access to specialized eye care must be provided with a mechanism for

obtaining these services.

544. [Progress on clinical study of diabetes mellitus (discussion)]. Nippon-Naika-Gakkai-

Zasshi. 1996 Apr 10; 85(4): 583-99; ISSN: 0021-5384.

JAPAN. 0; 0.

545. Provenzale, J. M.; VanLandingham, K. Cerebral infarction associated with Kearns-

Sayre syndrome-related cardiomyopathy. Neurology. 1996 Mar; 46(3): 826-8;

ISSN: 0028-3878.

UNITED-STATES. We present the clinical and neuroradiologic findings of a 31-

year-old man with Kearns-Sayre syndrome- related dilated cardiomyopathy who

experienced a left middle cerebral artery territory stroke, thought to be due to

cardiogenic embolism. The rate of clinically apparent cardiomyopathy in Kearns-

Sayre patients can be expected to increase as their survival is prolonged by the use

of cardiac pacemaker devices. Under these circumstances, stroke caused by

cardiogenic embolism, which is presently rare, may become more common.

546. Puri, V.; Rohtagi, A.; Parihar, P. S. Ponto-bulbar palsy with deafness (Vialetto-Van

Laere syndrome). Indian-Pediatr. 1996 Feb; 33(2): 140-2; ISSN: 0019-6061.

INDIA.

547. Quarles, R. H.; Dalakas, M. C. Do anti-ganglioside antibodies cause human peripheral

neuropathies? [see comments]. J-Clin-Invest. 1996 Mar 1; 97(5): 1136-7; ISSN:

0021-9738.

Note: Comment in: J Clin Invest 1996 Mar 1;97(5):1155-64.

UNITED-STATES. 0; 0; 0.

548. Quevedo, E.; Delvalle, A.; Higa, E.; Iffenecker, C.; Quillard, J.; Sterkers, J. M.;

Doyon, D. [Hemangioma of the facial nerve]. Hemangiomes du nerf facial. J-

Neuroradiol. 1996 Jun; 23(1): 26-32; ISSN: 0150-9861.

FRANCE. In this retrospective study the respective values of MRI and CT in the

location and nature diagnoses of facial nerve haemangiomas were evaluated. The

four male patients examined were 31, 44, 56 and 62 years old; they presented with

facial nerve pals and/or cochlear-vestibular dysfunction. The haemangiomas were

located in the internal auditory canal, the geniculate ganglion, the tympanic segment

of the facial nerve and the petrous bone apex. MRI revealed a tumoral process,

while CT showed intratumoral calcifications and provided a diagnosis of mass

nature in two cases. In the other cases the pre-operative diagnosis was neurinoma

of the VIIIth or VIIth cranial nerve. Histology ascertained the diagnosis. MRI is the

method of choice in cases of facial paralysis or cochlear-vestibular dysfunction if a

tumoral cause is suspected. Haemangioma is an uncommon tumour without

specific image, except for calcifications and neighbouring osseous reactions. It

must also be considered on the basis of clinical and topographical findings revealed

by CT and MRI imaging.

549. Rachbauer, F.; Sterzinger, W.; Frischhut, B. Suprascapular nerve entrapment at the

spinoglenoid notch caused by a ganglion cyst. J-Shoulder-Elbow-Surg. 1996 Mar;

5(2 Pt 1): 150-2; ISSN: 1058-2746.

UNITED-STATES. A 34-year-old man had right infraspinatus muscle palsy and

posterior aching of the shoulder caused by electromyographically confirmed

suprascapular nerve entrapment. Sonography and magnetic resonance imaging

revealed a cystic lesion at the spinoglenoid notch; this lesion was diagnosed as a

ganglion. Operative removal led to immediate pain relief and incomplete recovery of

the compressed branches of the suprascapular nerve.

550. Raffi, G. B.; Lodi, V.; Malenchini, G.; Missere, M.; Naldi, M.; Tabanelli, S.;

Violante, F.; Minak, G. J.; D'elia, V.; Montesi, M. Cumulative trauma disorders of

the upper limbs in workers on an agricultural farm. Arh-Hig-Rada-Toksikol. 1996

Mar; 47(1): 19-23; ISSN: 0004-1254.

CROATIA. The work associated with repetitive efforts and inadequate resting

periods, strong physical exertion, awkward postures or static positioning exposes

workers to the risk of cumulative trauma disorders of the upper limbs. These risk

factors are present in many agricultural activities. A study was carried out among

workers on an agricultural farm. The workers' histories were taken and they were

given periodical medical check-ups. The presence of upper limb disorders was

shown in a group of workers. A sample of 42 people was selected for the study by

means of specific tests: electromyography, ultrasonography and laser-doppler

flowmetry. The tests showed a high incidence of carpal tunnel syndrome and

microcirculation disorders. The study confirmed that electromyography,

ultrasonography and/or laser-doppler flowmetry are highly useful tools for

identifying cumulative trauma disorders.

551. Raina, U. K.; Taneja, S.; Lamba, P. A.; Bansal, R. L. Spontaneous extrusion of

extraocular cysticercus cysts. Am-J-Ophthalmol. 1996 Apr; 121(4): 438-41; ISSN:

0002-9394.

UNITED-STATES. PURPOSE: We treated two patients who had spontaneous

extrusion of cysticercus cysts and subconjunctival swelling. Additionally, one

patient had severe myositis and restriction of ocular movements. METHODS: The

patients were prescribed oral albendazole, 15 mg/kg of body mass per day.

Systemic corticosteroids were also given to the patient who had severe myositis.

RESULTS: Spontaneous extrusion of the cysts occurred in both patients within

three to five days of starting albendazole therapy. The conjunctival defects healed

without any surgical intervention. The ocular motility in the patient with myositis

returned to normal. CONCLUSION: As extrusion happened soon after the start of

albendazole therapy, it is uncertain whether this therapy played any role.

Additionally, it is not clear if systemic therapy must be continued in the absence of

systemic and neurologic manifestations.. 0; 0; 54965-21-8.

552. Randel, R. C.; Kearns, D. B.; Nespeca, M. P.; Scher, C. A.; Sawyer, M. H. Vocal

cord paralysis as a presentation of intrauterine infection with varicella-zoster virus.

Pediatrics. 1996 Jan; 97(1): 127-8; ISSN: 0031-4005.

UNITED-STATES.

553. Rask Anderson, H.; Nyberg, G.; Kinnefors, A.; Ekvall, L.; Bredberg, G. [Electric

stimulation of the brain stem. Treatment of deaf persons with destroyed auditory

nerve]. Elektrisk stimulering av hjarnstammen. Behandling av dova med forstord

horselnerv. Lakartidningen. 1996 Oct 2; 93(40): 3461-4; ISSN: 0023-7205.

SWEDEN.

554. Rastegar, D. A. Diagnosis of thoracic outlet syndrome in the emergency department

[letter]. South-Med-J. 1996 Sep; 89(9): 933; discussion 933-4; ISSN: 0038-4348.

UNITED-STATES.

555. Reichmann, H.; Vogler, L.; Seibel, P. Ragged red or ragged blue fibers. Eur-Neurol.

1996; 36(2): 98-102; ISSN: 0014-3022.

SWITZERLAND. Fibers called ragged red fibers are generally considered the

morphological characteristic of mitochondrial encephalomyopathies. These fibers

appear red in the modified Gomori trichrome (Tri) stain due to subsarcolemmal and

interfibrillar increase in mitochondrial number and volume. Other accepted

morphological abnormalities include partial cytochrome c oxidase deficiency and

subsarcolemmal increase in succinate dehydrogenase and NADH tetrazolium

reductase stain. We were interested to see which of these abnormalities would be

the most specific for mitochondrial cytopathies such as Kearns-Sayre syndrome

and chronic progressive external ophthalmoplegia. We analyzed five patients and

found 74 fibers compatible with mitochondrial abnormalities as defined above. The

modified Gomori Tri stain turned out to be the most specific and reliable

technique.. EC 1.3.99.1; EC 1.6.-; EC 1.9.3.1.

556. Reinecke, R. D. Retroequatorial placement of horizontal recti [letter]. J-Pediatr-

Ophthalmol-Strabismus. 1996 May; 33(3): 201-2; ISSN: 0191-3913.

UNITED-STATES.

557. Reiter, L. T.; Murakami, T.; Koeuth, T.; Pentao, L.; Muzny, D. M.; Gibbs, R. A.;

Lupski, J. R. A recombination hotspot responsible for two inherited peripheral

neuropathies is located near a mariner transposon-like element. Nat-Genet. 1996

Mar; 12(3): 288-97; ISSN: 1061-4036.

UNITED-STATES. The Charcot-Marie Tooth disease type 1A (CMT1A)

duplication and hereditary neuropathy with liability to pressure palsies (HNPP)

deletion are reciprocal products of an unequal crossing-over event between

misaligned flanking CMT1A-REP repeats. The molecular aetiology of this

apparently homologous recombination event was examined by sequencing the

crossover region. Through the detection of novel junction fragments from the

recombinant CMT1A-REPs in both CMT1A and HNPP patients, a 1.7-kb

recombination hotspot within the approximately 30-kb CMT1A-REPs was

identified. This hotspot is 98% identical between CMT1A-REPs indicating that

sequence identity is not likely the sole factor involved in promoting crossover

events. Sequence analysis revealed a mariner transposon-like element (MITE) near

the hotspot which we hypothesize could mediate strand exchange events via

cleavage by a transposase at or near the 3' end of the element.. 0; 9007-49-2.

558. Renault, F.; Chartier, J. P.; Harpey, J. P. [Contribution of the electromyogram in the

diagnosis of infantile spinal muscular atrophy in the neonatal period]. Apport de

l'electromyogramme au diagnostic d'amyotrophie spinale infantile en periode

neonatale. Arch-Pediatr. 1996 Apr; 3(4): 319-23; ISSN: 0929-693X.

FRANCE. BACKGROUND: The acute form of Werdnig-Hoffman disease,

infantile spinal muscular atrophy type I (SMA I), is characterized by severe

paralytic hypotonia with neurogenic electromyographic (EMG) pattern and specific

histologic features. PATIENTS: Four cases of very severe SMA I suffering from

generalized muscle weakness at birth were included in the study. RESULTS: The

neurogenic EMG pattern was observed at the first exam performed between D2 and

D46. The muscular biopsy performed between D18 and D45 showed only a mild

decrease of the muscle fiber size without grouping of fiber types. CONCLUSION:

In those forms of SMA I with a neonatal clinical onset, the diagnosis is assessed by

clinical and EMG findings while early muscular biopsy can be misleading. EMG is

the relevant diagnostic test which confirms the anterior horn cell disease and can

justify the DNA study.

559. Repka, M. X.; Claro, M. C.; Loupe, D. N.; Reich, S. G. Ocular motility in

Parkinson's disease. J-Pediatr-Ophthalmol-Strabismus. 1996 May; 33(3): 144-7;

ISSN: 0191-3913.

UNITED-STATES. INTRODUCTION: Parkinson's disease is associated with

multiple abnormalities of both the afferent and efferent visual systems.

Blepharospasm, paucity of blinking, apraxia of lid opening, visual neglect, reduced

vergence, reduced upgaze, and blurred vision are reported findings in these

patients. The association of these findings with the disease, and their duration,

severity, and treatment have not been systematically investigated. PATIENTS AND

METHODS: Patients with Parkinson's disease were prospectively examined. An

age-matched control group was recruited from accompanying family members and

volunteers. Data recorded included presence of visual complaint, the severity of the

Parkinson's disease by Hoehn and Yahr Stage (scale = 1 to 5), duration of disease,

pharmacologic therapy, visual acuity, ocular motility, accommodation, convergence

amplitudes, and the near point of convergence. RESULTS: Thirty-nine patients

were entered into each group, each with 21 men and 18 women. The average

patient had had the disease for 8.9 years with a severity index of 2.6. Asthenopia,

upgaze deficiency, and convergence insufficiency were significantly more common

in the patients with Parkinson's disease than in the controls. Mean geometric visual

acuity was poorer in the Parkinson's patients (20/39 compared with 20/28); P <

.001). DISCUSSION: Visual complaints were significantly more common in the

Parkinson's patients than in the age-matched controls. The frequency of ocular

abnormalities was not related to the duration of the disease. Increasing severity

seemed to be correlated with the presence of convergence insufficiency and a

decline in acuity.

560. Resnick, D. K.; Jannetta, P. J.; Lunsford, L. D.; Bissonette, D. J. Microvascular

decompression for trigeminal neuralgia in patients with multiple sclerosis. Surg-

Neurol. 1996 Oct; 46(4): 358-61; discussion 361-2; ISSN: 0090-3019.

UNITED-STATES. BACKGROUND: Microvascular decompression (MVD) of

the trigeminal nerve is a well-established procedure for the treatment of idiopathic

trigeminal neuralgia. Multiple sclerosis (MS) has long been considered a

contraindication for this procedure, due to the known polycentric nature of the

disease. Medical treatment followed by percutaneous procedures provide relief for

the great majority of these patients. There exists a small subgroup of patients with

trigeminal neuralgia who are diagnosed with MS only after a microvascular

decompression procedure has been performed. Furthermore, management of the

patient with known MS whose pain continues to recur, despite maximal medical

therapy and multiple percutaneous procedures, can be exceedingly difficult.

METHODS: Five patients with MS, three who had undergone multiple

unsuccessful percutaneous procedures and two in whom the diagnosis of MS had

not been established, underwent exploration of the cerebellopontine angle. Three

patients underwent MVD alone, and two (both with known MS) underwent MVD

and partial section of the trigeminal nerve. RESULTS: Patients who underwent

microvascular decompression alone did not have satisfactory relief of pain. Patients

who underwent partial sectioning of the nerve did better. CONCLUSIONS:

Patients with MS and symptoms of typical trigeminal neuralgia may benefit from

exploration of the cerebellopontine angle and partial sectioning of the nerve. MVD

alone fails to provide adequate or reliable relief of pain.

561. Richard, J. L. [How to detect diabetics with risk of foot complications]. Comment

d'epister les diabetiques a risque podologique? Diabetes-Metab. 1996 Jun; 22(3):

210-4.

FRANCE.

562. Richardson, J. K.; Ashton Miller, J. A. Peripheral neuropathy: an often-overlooked

cause of falls in the elderly. Postgrad-Med. 1996 Jun; 99(6): 161-72; ISSN: 0032-

5481.

UNITED-STATES. Peripheral neuropathy is common in the elderly and results in

impairments in distal proprioception and strength that hinder balance and

predispose them to falls. The loss of heel reflexes, decreased vibratory sense that

improves proximally, impaired position sense at the great toe, and inability to

maintain unipedal stance for 10 seconds in three attempts all suggest functionally

significant peripheral neuropathy. Physicians can help their patients with peripheral

neuropathy to prevent falls by teaching them and their families about peripheral

nerve dysfunction and its effects on balance and by advising patients to substitute

vision for the lost somatosensory function, correctly use a cane, wear proper shoes

and orthotics, and perform balance and upper extremity strengthening exercises.

563. Richter, V. M.; Bruser, P. [Surgical treatment of carpal tunnel syndrome: a comparison

between long and short incision and endoscopic release]. Die operative

Behandlung des Karpaltunnelsyndroms: Ein Vergleich zwischen langer und kurzer

Schnittfuhrung sowie endoskopischer Spaltung. Handchir-Mikrochir-Plast-Chir.

1996 May; 28(3): 160-6; ISSN: 0722-1819.

GERMANY. The results of open carpal tunnel release employing a short incision

(2.5 cm) were compared with those following a long incision (4.5 cm) in a

prospective randomized study. These results were then compared with those found

by Agee et al. (1992), following endoscopic carpal tunnel release. The criteria

examined included grip strength as well as key and pulp pinch strength, sensory

function and scar tenderness. Subjective symptoms such as tingling, numbness,

and nocturnal paraesthesia were specifically inquired into, and time off work was

noted. The examinations took place pre-operatively, as well as one, two, three, and

six weeks postoperatively. The endoscopic method demonstrated no advantages

when compared to the short incision. The long incision led to a temporary 10% loss

of strength only during the first three weeks. These results should be kept in mind

in the light of occasionally severe neurovascular complications following

endoscopic carpal tunnel release.

564. Ritz, G.; Rowland, W. D.; Rowland, J. W. Use of the Cam Walker in treating diabetic

ulcers. A case report. J-Am-Podiatr-Med-Assoc. 1996 Jun; 86(6): 253-6; ISSN:

8750-7315.

UNITED-STATES. This case study shows how a Cam Walker when equipped

with an arch filler can be another tool the physician may use to help heal diabetic

ulcerations. Even after other methods of conservative care have failed, the Cam

Walker with an arch filler has been shown to gradually decrease and heal diabetic

ulcerations on the plantar aspect of the foot. It accomplishes this by decreasing the

pressure on the ulcerative areas and by decreasing the velocity with which the foot

strikes the ground.

565. Roberson, JB Jr; Brackmann, D. E.; Hitselberger, W. E. Acoustic neuroma recurrence

after suboccipital resection: management with translabyrinthine resection. Am-J-

Otol. 1996 Mar; 17(2): 307-11; ISSN: 0192-9763.

UNITED-STATES. Thirty-five patients with recurrent acoustic tumors have been

studied before, during, and after revision surgery. Each had their original surgery

via a suboccipital approach. Six patients had undergone multiple suboccipital

surgeries. Revision surgery was accomplished using a translabyrinthine approach

in all patients. Total tumor removal was possible in 33 patients. No second

recurrences have occurred to date among the patients with total tumor removal.

Anatomic facial nerve integrity was maintained in 24 patients. All recurrent tumors

were present in the lateral internal auditory canal. Direct tumor access in this area is

not possible with the suboccipital approach without entering the vestibular

labyrinth. The suboccipital approach to acoustic neuromas is contraindicated if

tumor extends to the fundus of the internal auditory canal. Translabyrinthine

resection is the treatment of choice for recurrence of acoustic neuroma after primary

suboccipital resection.

566. Robin, C.; Levinger, N.; Salzmann, M.; Convert, G.; Fischer, G. [Neurinoma with

sudden-onset deafness (letter)]. Neurinome avec surdite brutale. Presse-Med.

1996 Jan 27; 25(3): 126; ISSN: 0755-4982.

FRANCE.

567. Roche, B.; Marti, M. C. [Pelvic pain of proctological origin]. Les algies pelviennes

d'origine proctologique. Schweiz-Med-Wochenschr. 1996 Feb 24; 126(8): 316-21;

ISSN: 0036-7672.

SWITZERLAND. Pelvic pain of anorectal origin should be carefully investigated.

The detailed clinical history may be sufficient to establish diagnosis and determine

the etiology of anorectal pain. The diagnosis should be confirmed by

complementary clinical investigations. The diagnosis of essential anorectal pain can

be established only after failure of measures instituted to correct organic lesions and

if pain persists. Among types of essential anorectal pain, coccygodinia and nervus

pudendus entrapment are responsive to precise and effective management.

568. Roche, P. H.; Figarella Branger, D.; Regis, J.; Peragut, J. C. Cauda equina

paraganglioma with subsequent intracranial and intraspinal metastases. Acta-

Neurochir-Wien. 1996; 138(4): 475-9; ISSN: 0001-6268.

AUSTRIA. A case of cauda equina paraganglioma is described; subsequent

intracranial and intraspinal metastases occurred after partial resection and adjunctive

radiotherapy. Cerebrospinal fluid dissemination is a rare complication of spinal

paragangliomas. Factors predictive of this unusual biological behaviour are

discussed.. 0.

569. Rodi, Z.; Denislic, M.; Vodusek, D. B. External anal sphincter electromyography in

the differential diagnosis of parkinsonism [letter]. J-Neurol-Neurosurg-Psychiatry.

1996 Apr; 60(4): 460-1; ISSN: 0022-3050.

ENGLAND.

570. Rogers, N. K.; Brand, C. S. Acoustic neuroma may impair vision [letter; comment].

BMJ. 1996 Feb 24; 312(7029): 511; ISSN: 0959-8138.

Note: Comment on: BMJ 1995 Oct 28;311(7013):1141-4.

ENGLAND.

571. Ropper, A. H. Chronic demyelinating polyneuropathy: Improvement after sepsis.

Neurology. 1996 Mar; 46(3): 848-50; ISSN: 0028-3878.

UNITED-STATES.

572. Rorick, M. B.; Chandar, K.; Colombi, B. J. Inflammatory trigeminal sensory

neuropathy mimicking trigeminal neurinoma. Neurology. 1996 May; 46(5): 1455-

7; ISSN: 0028-3878.

UNITED-STATES. Idiopathic trigeminal sensory neuropathy is a benign disorder.

We report two patients with transient MRI abnormalities, suggesting transient

inflammation of the trigeminal nerve caused temporary facial sensory symptoms.

573. Rosberg, H. E.; Ekerot, L. Multifocal neurilemomas in the same upper extremity. Case

report. Scand-J-Plast-Reconstr-Surg-Hand-Surg. 1996 Jun; 30(2): 153-6; ISSN:

0284-4311.

SWEDEN. Multifocal neurilemomas in different nerves of the same upper

extremity are rare. A 36-year-old woman presented with five neurilemomas

involving the main trunks of the median and radial nerve and digital nerves of her

left upper extremity. These were excised and she recovered without complications.

Fifteen years later she presented with several recurrences which were removed, and

two years after that she required a further operation to excise tumours. At the time

of writing she has at least five tumours on different nerves of her left arm but these

are causing her no problems.

574. Rose, M. R.; Whitehead, T.; Greenwood, R. Postmeningococcal lumbosacral

radiculopathy [letter]. J-Neurol-Neurosurg-Psychiatry. 1996 Apr; 60(4): 459-60;

ISSN: 0022-3050.

ENGLAND.

575. Rosenbaum, R. B. Electrical studies as a prognostic factor in the surgical treatment of

carpal tunnel syndrome [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 523-4; ISSN:

0363-5023.

UNITED-STATES.

576. Rosner, H.; Rubin, L.; Kestenbaum, A. Gabapentin adjunctive therapy in neuropathic

pain states. Clin-J-Pain. 1996 Mar; 12(1): 56-8; ISSN: 0749-8047.

UNITED-STATES. OBJECTIVE: This is a report of a trial of the new antiepileptic

agent gabapentin in patients with intractable neuropathic pain. DESIGN: A case

series of patients with a diagnosis of neuropathic pain whose previous management

was inadequate were given oral gabapentin in increasing doses and were followed

for a minimum of 2 months, monitored for efficacy and side effects. SETTING: An

outpatient pain management center located within a major university medical center.

PATIENTS: Convenience sample of patients referred for management of intractable

neuropathic pain. INTERVENTIONS: Simplification of existing pharmacologic

management, addition of gabapentin, and attempted reduction of opiate analgesic

doses. MAIN OUTCOME MEASURES: Patient self-reports and pain scores in

successive office visits. RESULTS: Gabapentin provides analgesic activity for

patients with neuropathic pain and has the advantage of a low side effect profile and

drug toxicity.. 0; 0; 60142-96-3.

577. Ross, B. G.; Fradet, G.; Nedzelski, J. M. Development of a sensitive clinical facial

grading system. Otolaryngol-Head-Neck-Surg. 1996 Mar; 114(3): 380-6; ISSN:

0194-5998.

UNITED-STATES. Clinicians require a reliable and valid method of evaluating

facial function after facial nerve injury. This tool should be clinically relevant and

easy to administer, provide a quantitative score for reporting purposes, and be

sensitive enough to detect clinically important change over time or with treatment.

The proposed facial grading system has all essential information, including precise

definitions for each item, presented on one page. The facial grading system is based

on the evaluation of resting symmetry, degree of voluntary excursion of facial

muscles, and degree of synkinesis associated with specified voluntary movement.

Different regions of the face are examined separately with the use of five standard

expressions. All items are evaluated on point scales, and a cumulative composite

score is tabulated. Construct validity was addressed by comparing the proposed

facial grading system to prerehabilitation and postrehabilitation treatment scores of

19 patients with varying degrees of facial nerve injury. All patients had documented

change in a controlled study of feedback training. The proposed system reports

results in a more continuous manner with a wider response range than the House-

Brackmann grades. Each component of the grading system is sensitive to change

and individually contributes to a change in the composite score. Tests of interrater

reliability are currently near completion.

578. Ross, C. N.; Reuter, H.; Scott, D.; Hamilton, D. V. Microangiopathic haemolytic

anaemia and systemic vasculitis. Br-J-Rheumatol. 1996 Apr; 35(4): 377-9; ISSN:

0263-7103.

ENGLAND. Two cases of systemic vasculitis complicated by microangiopathic

haemolytic anaemia (MAHA) are described: this association has not previously

been reported. Both patients had atypical presentations of their primary disease, one

with parotitis and one with a Guillain-Barre syndrome. Other causes of MAHA

were excluded and a possible link with macromolecular von Willebrands factor is

speculated upon.

579. Ross, D. E.; Thaker, G. K.; Buchanan, R. W.; Lahti, A. C.; Medoff, D.; Bartko, J.

J.; Moran, M.; Hartley, J. Association of abnormal smooth pursuit eye movements

with the deficit syndrome in schizophrenic patients. Am-J-Psychiatry. 1996 Sep;

153(9): 1158-65; ISSN: 0002-953X.

UNITED-STATES. OBJECTIVE: The authors' goal was to test the hypothesis

that abnormal smooth pursuit eye movements in schizophrenic patients are

associated with the deficit syndrome. METHOD: The eye movements of 24 normal

comparison subjects, 32 patients with nondeficit schizophrenia, and 11 patients

with deficit schizophrenia were tested with infrared oculography using foveapetal

step-ramp targets. RESULTS: The group of schizophrenic patients had normal

latency to pursuit onset, abnormally decreased open-loop acceleration and

abnormally decreased velocity during the periods of closed-loop acceleration and

steady-state pursuit. The subgroup of schizophrenic patients with the deficit

syndrome had particularly poor performance during the periods of open- and

closed-loop acceleration. CONCLUSIONS: Patients with schizophrenia have

abnormal smooth pursuit eye movements in response to a step-ramp stimulus, and

the defects are particularly pronounced in patients with the deficit syndrome.

Abnormal smooth pursuit eye movements in schizophrenia and related disorders

have been consistently linked with primary and enduring negative symptoms.

580. Ross, J. S.; Robertson, J. T.; Frederickson, R. C.; Petrie, J. L.; Obuchowski, N.;

Modic, M. T.; deTribolet, N. Association between peridural scar and recurrent

radicular pain after lumbar discectomy: magnetic resonance evaluation. ADCON-L

European Study Group. Neurosurgery. 1996 Apr; 38(4): 855-61; discussion 861-

3; ISSN: 0148-396X.

UNITED-STATES. The purpose of this study was to investigate the presence of

any correlation between recurrent radicular pain during the first six months

following first surgery for herniated lumbar intervertebral disc and the amount of

lumbar peridural fibrosis as defined by MR imaging. 197 patients who underwent

first-time single-level unilateral discectomy for lumbar disc herniation were

evaluated in a randomized, double-blind, controlled multicenter clinical trial.

Clinical assessments, performed by physicians blinded to patient treatment status,

were conducted preoperatively and at one and six months postoperatively. The

enhanced MR images of the operative site utilized in the analysis were obtained at

six months postoperatively. Radicular pain was recorded by the patient using a

validated visual analog pain scale in which 0 = no pain and 10 = excruciating pain.

The data obtained at the 6 month time point were analyzed for an association

between amount of peridural scars as measured by MR imaging and clinical failure

as defined by the recurrence of radicular pain. The results showed that the

probability of recurrent pain increases when scar score increases. Patients having

extensive peridural scar were 3.2 times more likely to experience recurrent radicular

pain than those patients with less extensive peridural scarring. In conclusion, this

prospective, controlled, randomized, blinded, multicenter study has demonstrated

that there is a significant association between the presence of extensive peridural

scar and the occurrence of recurrent radicular pain.. 0.

581. Rossier, J.; Hatt, M. [Atypical manifestation of progressive external ophthalmoplegia].

Manifestation atypique d'une ophtalmoplegie progressive externe. Klin-Monatsbl-

Augenheilkd. 1996 May; 208(5): 366-7; ISSN: 0023-2165.

GERMANY. A young woman presents a unilateral ptosis as first symptom of

progressive external ophthalmoplegia. We recommend the muscular biopsy to

confirm the diagnosis and analyse the mitochondrial DNA.. 0.

582. Roth, P.; Martin, V.; Khalife, H.; Gay, C.; Schaal, J. P.; Maillet, R.; Colette, C.

[Bilateral femoral neuropathy after laparotomy (letter)]. Femoroneuropathie

bilaterale apres laparotomie. J-Gynecol-Obstet-Biol-Reprod-Paris. 1996; 25(5):

540; ISSN: 0368-2315.

FRANCE.

583. Rothova, Z.; Handlova, R. [Anterior ischemic optic neuropathy after electric injury].

Predni ischemicka opticka neuropatie po urazu elektrickym proudem. Cesk-Slov-

Oftalmol. 1996 Feb; 52(1): 25-8.

CZECHOSLOVAKIA.

584. Rubin, D. A.; Towers, J. D.; Britton, C. A. MR imaging of the foot: utility of complex

oblique imaging planes. AJR-Am-J-Roentgenol. 1996 May; 166(5): 1079-84;

ISSN: 0361-803X.

UNITED-STATES. Successful MR imaging of the foot presents special challenges

to the radiologist. Accurate and confident diagnosis presupposes the ability to

produce high-resolution images of obliquely oriented, relatively small structures.

Orienting the foot within an appropriate local coil to bring such structures into an

orthogonal imaging plane, or even into a conventional oblique plane, may be

impossible or intolerably uncomfortable for the patient. The frequent result is

motion artifacts, which are accentuated when using a small field of view. However,

when patients are comfortably positioned, the anatomy of interest often lies in a

plane that is not orthogonal to any of the conventional imaging planes. Fortunately,

commercially available MR imaging equipment can produce images in complex

oblique planes with relative ease. In this pictorial essay, we discuss the technical

considerations for expedient diagnostic MR imaging of the complex anatomy of the

foot and illustrate our experiences with this technique.

585. Rubin, M.; Mackenzie, C. R. Clinically and electrodiagnostically pure sensory

demyelinating polyneuropathy. Electromyogr-Clin-Neurophysiol. 1996 Apr; 36(3):

145-9; ISSN: 0301-150X.

BELGIUM. A 35-year-old woman presented with a 4 year history of stepwise

sensory loss which progressed in an asymmetrical fashion (mononeuropathy

multiplex) and developed into a relatively symmetric polyneuropathy which was

purely sensory by clinical and electrodiagnostic criteria. Sural nerve biopsy

revealed demyelination with axonal sparing. Extensive laboratory evaluation failed

to reveal a definite cause. This case is unique among well-documented cases of

chronic inflammatory demyelinating polyneuropathy (CIDP) in that it is purely

sensory by both clinical and electrodiagnostic criteria, and may represent a distinct

entity rather than an extension of the spectrum of CIDP.

586. Ruckenstein, M. J.; Cueva, R. A.; Morrison, D. H.; Press, G. A prospective study of

ABR and MRI in the screening for vestibular schwannomas. Am-J-Otol. 1996 Mar;

17(2): 317-20; ISSN: 0192-9763.

UNITED-STATES. The advent of magnetic resonance imaging (MRI) has

significantly increased the clinician's ability to detect small vestibular

schwannomas. This had led to controversy in the evaluation of patients with

asymmetric sensorineural hearing loss, as some recent studies have suggested that

the auditory brainstem response (ABR) does not adequately detect small tumors of

the internal auditory canal and cerebellopontine angle. As these studies evaluated

ABR results in patients already diagnosed with vestibular schwannomas, they

could not determine the epidemiologic accuracy (validity) of the ABR as a screening

test for retrocochlear pathology. We report on the preliminary results of an ongoing

prospective study on the evaluation of patients with asymmetric sensorineural

hearing loss. All patients with asymmetry in two or more pure-tone thresholds of >

or = 15 decibels or asymmetry in speech discrimination scores of > or 15% or both

entered the study and underwent both an ABR examination and an enhanced MRI

scan. Based on preliminary results obtained from the first 47 patients entered in this

study, the ABR screening test for retrocochlear pathology was determined to have a

sensitivity of 63%, a specificity of 64%, a positive predictive value of 26%, and a

negative predictive value of 89%. All patients in whom ABR failed to diagnose a

vestibular schwannoma had unilateral hearing los. These results bring into question

the validity of ABR as a screening test for retrocochlear pathology, particularly in

cases of unilateral hearing loss. Continued patient enrollment in this study will

allow the confirmation of these results.

587. Rudnik Schoneborn, S.; Forkert, R.; Hahnen, E.; Wirth, B.; Zerres, K. Clinical

spectrum and diagnostic criteria of infantile spinal muscular atrophy: further

delineation on the basis of SMN gene deletion findings. Neuropediatrics. 1996

Feb; 27(1): 8-15; ISSN: 0174-304X.

GERMANY. With the evidence of deletions in the region responsible for

autosomal recessive spinal muscular atrophy (SMA) on chromosome 5, it is now

possible to further clarify the clinical and diagnostic findings in proximal SMA.

Homozygous deletions of the survival motor neuron (SMN) gene can be detected in

about 95% of patients with early onset SMA. In a series of more than 200 patients,

we tested 31 patients with atypical features of SMA who fulfilled at least one

exclusion criterion according to the diagnostic criteria of the International SMA

Consortium for the presence of SMN gene deletions. The patients were subdivided

into two groups: 1. Seven index patients being not deleted for the SMN gene who

belonged to a well-defined SMA plus variant that has already been shown to be

unlinked with chromosome 5q markers: diaphragmatic SMA, SMA plus

olivopontocerebellar hypoplasia, SMA with congenital arthrogryposis and bone

fractures. 2. Twenty-four patients with clinical signs of SMA and neurogenic

findings in EMG/muscle biopsy who had unusual features or other organ

involvement. In order to structure this heterogeneous group, each patient was

assigned to a subgroup according to the leading atypical feature. In 5 out of 8

unrelated patients with a history of preterm birth and/or perinatal asphyxia leading

to a picture of severe SMA in combination with respiratory distress and/or cerebral

palsy, no deletion of the SMN gene could be detected. There were five unrelated

patients with extended central nervous system involvement (cerebral atrophy, EEG

abnormalities, pyramidal tract signs, evidence of cerebellar involvement). Most of

these patients (4/5) proved to belong to SMA 5q on the basis of SMN gene deletion

findings. The same applied to a group of three patients with classical SMA in

association with congenital malformations (mainly heart defect). A fourth group of

three patients was characterized mainly by an unusual improvement of the

condition; in these patients no SMN gene deletions were present. In three index

patients a more complex syndrome of the CNS and other organs was suggested,

but the detection of SMN gene deletions in two of them made a coincidence of

features more likely. In addition, SMN gene deletions were found in two patients

with evidence of congenital fibre type dysproportion in one and extremely raised

CK activity ( > 10fold) in the other. While the confirmation of SMN gene deletions

is very useful in cases with diagnostic doubts, caution is required when offering

prenatal prediction with regard to SMA 5q in families with atypical features. There

is strong evidence that there are clinical entities resembling SMA which most likely

have another pathogenetic background.

588. Russell, B. G.; Jensen, H.; Kjaer, I. Ocular findings and sphenoid bone morphology

in tuberous sclerosis. Acta-Ophthalmol-Scand-Suppl. 1996; (219): 36-9; ISSN:

1395-3931.

DENMARK. The aim of the study was to focus on the correlation between the

ocular findings and the morphology of the sphenoid bone supporting the optic

nerve in patients with tuberous sclerosis. Ten patients participated in the

investigation. The sphenoid bone morphology was analyzed from existing

radiographs of the cranium. The results were compared to the individual

ophthalmologic diagnosis. Changes in the midline osseous structure, the sella

turcica and optic sulcus seem to be correlated with the severity of the ocular

findings.

589. Rutkove, S. B.; Matheson, J. K.; Logigian, E. L. Restless legs syndrome in patients

with polyneuropathy. Muscle-Nerve. 1996 May; 19(5): 670-2; ISSN: 0148-639X.

UNITED-STATES.

590. Ruttledge, M. H.; Andermann, A. A.; Phelan, C. M.; Claudio, J. O.; Han, F. Y.;

Chretien, N.; Rangaratnam, S.; MacCollin, M.; Short, P.; Parry, D.; Michels, V.;

Riccardi, V. M.; Weksberg, R.; Kitamura, K.; Bradburn, J. M.; Hall, B. D.;

Propping, P.; Rouleau, G. A. Type of mutation in the neurofibromatosis type 2

gene (NF2) frequently determines severity of disease. Am-J-Hum-Genet. 1996

Aug; 59(2): 331-42; ISSN: 0002-9297.

UNITED-STATES. The gene predisposing to neurofibromatosis type 2 (NF2) on

human chromosome 22 has revealed a wide variety of different mutations in NF2

individuals. These patients display a marked variability in clinical presentation,

ranging from very severe disease with numerous tumors at a young age to a

relatively mild condition much later in life. To investigate whether this phenotypic

heterogeneity is determined by the type of mutation in NF2, we have collected

clinical information on 111 NF2 cases from 73 different families on whom we have

performed mutation screening in this gene. Sixty-seven individuals (56.2%) from

41 of these kindreds revealed 36 different putative disease-causing mutations.

These include 26 proposed protein-truncating alterations (frameshift

deletions/insertions and nonsense mutations), 6 splice-site mutations, 2 missense

mutations, 1 base substitution in the 3' UTR of the NF2 cDNA, and a single 3-bp

in-frame insertion. Seventeen of these mutations are novel, whereas the remaining

19 have been described previously in other NF2 individuals or sporadic tumors.

When individuals harboring protein-truncating mutations are compared with cases

with single codon alterations, a significant correlation (P < .001) with clinical

outcome is observed. Twenty-four of 28 patients with mutations that cause

premature truncation of the NF2 protein, schwannomin, present with severe

phenotypes. In contrast, all 16 cases from three families with mutations that affect

only a single amino acid have mild NF2. These data provide conclusive evidence

that a phenotype/genotype correlation exists for certain NF2 mutations.. 0.

591. Sailer, S. M. The role of splinting and rehabilitation in the treatment of carpal and

cubital tunnel syndromes. Hand-Clin. 1996 May; 12(2): 223-41; ISSN: 0749-

0712.

UNITED-STATES. Successful treatment of patients suffering from carpal or

cubital tunnel syndrome requires a skilled collaborative effort by the physician,

therapist, and patient. Unfortunately, many factors influence an individual's

recovery and outcome. As medical professionals, we must educate the patient,

examine the physical and environmental factors involved in each case, and design

unique treatment plans in each instance.

592. Saim, L.; McKenna, M. J.; Nadol, JB Jr. Tubal and tympanic openings of the

peritubal cells: implications for cerebrospinal fluid otorhinorrhea. Am-J-Otol. 1996

Mar; 17(2): 335-9; ISSN: 0192-9763.

UNITED-STATES. Cerebrospinal fluid otorhinorrhea after surgery for

cerebellopontine angle tumors may persist despite obliteration of the mastoid,

middle ear, and tympanic orifice of the eustachian tube. In this study, histologic

sections of 120 adult temporal bones were examined by light microscopy to

determine the incidence of peritubal pneumatization and to demonstrate the

frequency of tubal and tympanic openings of the peritubal cells. The results of this

study suggest that the pathway for these persistent cerebrospinal fluid leaks may be

via the peritubal cells that open directly into the eustachian tube anterior to its

tympanic orifice. Peritubal pneumatization was present in 78 (65%) of the temporal

bones. Of the 57 specimens in which the openings of the peritubal cells could be

identified, in 52 (91%), the cells opened into the eustachian tube anterior to its

tympanic orifice, and in only five (9%), they opened into the middle ear. The

overall incidence of tubal openings in this study was 59%. In 13 temporal bones

(21%), the tubal opening were at a distance of > 5 mm anterior to the tympanic

orifice of the eustachian tube. Therefore, cerebrospinal leak may persist through

these tubal openings despite obliteration of the mastoid, middle ear, and tympanic

orifice of the eustachian tube. A case of persistent cerebrospinal fluid leak in which

extensive peritubal pneumatization was demonstrated by computed tomography

scan is presented. Successful control of the leak was obtained only after the tubal

openings of these cells several millimeters anterior to the tympanic orifice were

obliterated.

593. Saito, N.; Nishikawa, M.; Ota, A. [Progress on diagnosis and therapy of diabetic

gangrene]. Nippon-Naika-Gakkai-Zasshi. 1996 Apr 10; 85(4): 556-61; ISSN:

0021-5384.

JAPAN.

594. Sakai, K.; Maguchi, M.; Kohara, K.; Nishida, W.; Wakamiya, R.; Hara Nakamura,

N.; Mukai, M.; Yokoyama, A.; Hiwada, K. [A case of idiopathic orthostatic

hypotension manifesting sick sinus syndrome due to sympathetic nervous

dysfunction]. Nippon-Ronen-Igakkai-Zasshi. 1996 Feb; 33(2): 105-9; ISSN:

0300-9173.

JAPAN. A 67-year-old woman with idiopathic orthostatic hypotension was

presented. The patient started to experience faintness on standing since 1993.

During a physical examination, her systolic blood pressure fell from 148 to 50

mmHg on standing. Blood pressure responses to the mental arithmetic test and

hyperventilation stress were normal. However, cold pressor test failed to increase

blood pressure. These observations, with the finding that phase IV response on

Valsalva's maneuver was absent, indicate afferent sympathetic nervous

dysfunction. Peripheral neuropathy including diabetes mellitus and involvement of

central nervous system such as multiple system atrophy were excluded. Holter

ECG examination revealed a 3.9 second sinus arrest and bradycardia (total beats

88901/day). the blunted responses of the heart rate to atropine as well as

isoproterenol further suggested the presence of sick sinus syndrome. Amezinium

administration significantly improved her orthostatic hypotension and eliminated

sinus arrest. These findings indicate that sympathetic nervous dysfunction could

account for at least a part of the sick sinus syndrome in this patient.. 0; 0; 41658-

78-0.

595. Sakuma, A.; Kato, I.; Ogino, S.; Okada, T.; Takeyama, I. Primary position upbeat

nystagmus with special reference to alteration to downbeat nystagmus. Acta-

Otolaryngol-Suppl-Stockh. 1996; 522: 43-6; ISSN: 0365-5237.

NORWAY. A 24-year-old man was admitted to the University Hospital showing

severe dehydration that might have been the result of medicine-induced gastritis.

Wernicke's encephalopathy was suspected in this patient. On admission, primary

position upbeat nystagmus (PPUN) was found. The patient showed tongue

fasciculation and loss of gag reflex, which, however, soon returned to normal.

Electronystagmographic (ENG) findings were suggestive of lesions in the

brainstem. Based on neurological signs and symptoms, we concluded caudal

brainstem lesions might be a possible site responsible for the upbeat nystagmus.

This nystagmus, however, was later found to be reversed to downbeat nystagmus.

This is considered to have been due to predominant differences in vertical velocity

which are induced by gravity.

596. Salemi, G.; Savettieri, G.; Meneghini, F.; Di Benedetto, M. E.; Ragonese, P.;

Morgante, L.; Reggio, A.; Patti, F.; Grigoletto, F.; Di Perri, R. Prevalence of

cervical spondylotic radiculopathy: a door-to-door survey in a Sicilian municipality.

Acta-Neurol-Scand. 1996 Feb; 93(2-3): 184-8; ISSN: 0001-6314.

DENMARK. INTRODUCTION--Because of the limited information on cervical

spondylotic radiculopathy, we conducted a door-to-door two-phase survey in a

Sicilian municipality. MATERIAL AND METHODS--We first screened for cervical

spondylotic radiculopathy among the inhabitants of the municipality: (N = 7653, as

of the prevalence day, November 1, 1987). Study neurologists then investigated

those subjects suspected to have had a cervical spondylotic radiculopathy.

Diagnoses were bases on specified criteria. RESULTS--We found 27 subjects

affected by CSR (17 definite, 10 possible). Prevalence (cases per 1000 population)

was 3.5 in the total population; it increased to a peak at age 50-59 years, and

decreased thereafter. The age-specific prevalence was consistently higher in

women. CONCLUSIONS--Comparison with other prevalence studies shows

similar age-specific patterns, but different magnitudes, which may partly reflect

methodologic differences across studies.

597. Salgarello, T.; Tamburrelli, C.; Falsini, B.; Giudiceandrea, A.; Colotto, A. Optic nerve

diameters and perimetric thresholds in idiopathic intracranial hypertension. Br-J-

Ophthalmol. 1996 Jun; 80(6): 509-14; ISSN: 0007-1161.

ENGLAND. AIMS/BACKGROUND--Idiopathic intracranial hypertension (IIH)

is a central nervous disorder characterised by abnormally increased cerebrospinal

fluid (CSF) pressure leading to optic nerve compression. An indirect estimate of

increased CSF pressure can be obtained by the ultrasonographic determination of

optic nerve sheaths diameters. Computerised static perimetry is regarded as the

method of choice for monitoring the course of the optic neuropathy in IIH. The

aims were to compare the echographic optic nerve diameters (ONDs) and the

perimetric thresholds of patients with IIH with those of age-matched controls, and

to examine the correlation between these two variables in individual patients with

papilloedema. METHODS--Standardised A-scan echography of the mid orbital

optic nerve transverse diameters and automated threshold perimetry (Humphrey 30-

2) were performed in 20 patients with IIH with variable degree of papilloedema

(according to the Frisen scheme) and no concomitant ocular diseases. Echographic

and perimetric results were compared with those obtained from 20 age-matched

controls. RESULTS--When compared with controls, patients with IIH showed a

significant increase in mean ONDs and significantly reduced mean perimetric

sensitivities. In individual patients with papilloedema, the transverse ONDs

correlated negatively with Humphrey mean deviation values and positively with

pattern standard deviation values. CONCLUSION--These results indicate that OND

changes in IIH are associated with perimetric threshold losses, and suggest that IIH

functional deficits may be related to the degree of distension of optic nerve sheaths

as a result of an increased CSF pressure.

598. Salvesen, R.; Brautaset, N. J. Oculopharyngeal muscular dystrophy in Norway.

Survey of a large Norwegian family. Acta-Neurol-Scand. 1996 Apr; 93(4): 281-5;

ISSN: 0001-6314.

DENMARK. OBJECTIVES--Oculopharyngeal muscular dystrophy was first

described by Taylor in 1915. A substantial number of cases have been reported

from North America, the disorder being most frequent in Quebec, Canada.

MATERIAL--The onset of disease is in middle life, most often presenting with

ptosis and a slight degree of ophthalmoplegia, followed some years later by

dysphagia and often by proximal limb weakness. The course is slowly progressive,

but the dysphagia may become severe and has caused death by starvation in several

cases. The disorder is transmitted autosomally dominant in families. RESULTS--

The present report describes a large Norwegian family with 13 affected members of

whom we have examined eight. Transmission is autosomally dominant, mode of

presentation may be either by ptosis or by dysphagia. In rare cases gait difficulties

may be the presenting symptom. CONCLUSION--Electromyographic and muscle

biopsy examinations demonstrate a primary myogenic disorder.

599. Samii, K.; Cassinotti, P.; de Freudenreich, J.; Gallopin, Y.; Le Fort, D.; Stalder, H.

Acute bilateral carpal tunnel syndrome associated with human parvovirus B19

infection. Clin-Infect-Dis. 1996 Jan; 22(1): 162-4; ISSN: 1058-4838.

UNITED-STATES. Human parvovirus B19 has been described as a causative

agent of erythema infectiosum (a disease common in children), aplastic crisis in

patients with hemolytic disorders, and arthralgias and arthritis. Joint involvement

may be a prominent clinical feature of parvovirus B19 infection and may last for

several weeks. We describe three cases of acute bilateral carpal tunnel syndrome

associated with parvovirus B19 infection as evidenced by serological data and, in

one case, by detection of parvovirus B19 DNA in blood with use of PCR.. 0; 0.

600. Sangwan, Y. P.; Coller, J. A.; Barrett, R. C.; Murray, J. J.; Roberts, P. L.; Schoetz,

DJ Jr. Prospective comparative study of abnormal distal rectoanal excitatory reflex,

pudendal nerve terminal motor latency, and single fiber density as markers of

pudendal neuropathy. Dis-Colon-Rectum. 1996 Jul; 39(7): 794-8; ISSN: 0012-

3706.

UNITED-STATES. PURPOSE. This study was undertaken to determine the role

of abnormal distal rectoanal excitatory reflex (RAER) as a marker of pudendal

neuropathy and to compare results with pudendal nerve terminal motor latency

(PNTML) and single fiber density (SFD) estimation. METHODS. Fifteen female

patients (mean age, 47.1 (range, 20-70) years) referred to the pelvic floor

laboratory with pelvic floor disorders (fecal incontinence, 13 patients; constipation,

2 patients) were evaluated prospectively with neurophysiologic tests and balloon

reflex manometry for evidence of pudendal neuropathy. RESULTS. Pudendal

nerve terminal motor latency provided evidence of pudendal neuropathy in ten

patients (67 percent) and was normal in five patients (33 percent). Increased SFD

confirmed denervation of the external anal sphincter in 12 patients (80 percent),

being normal in 3 patients (20 percent). Distal RAER was abnormal in 13 patients

(87 percent) and was normal in 2 patients (13 percent). In ten patients (67 percent),

the three diagnostic modalities were in complete agreement, correctly identifying

neuropathy in nine patients (60 percent) and excluding nerve damage in one patient

(7 percent). Distal RAER was normal despite prolonged PNTML and increased

SFD in one patient (7 percent). In two patients (13 percent), distal RAER was

abnormal or absent despite normal PNTML and SFD. Pudendal nerve terminal

motor latency was normal in the presence of abnormal distal RAER and increased

SFD on electromyography in two patients (13 percent). CONCLUSIONS.

Abnormal distal RAER compares favorably with current neurophysiologic tests

used to diagnose pudendal neuropathy.

601. Sangwan, Y. P.; Coller, J. A.; Barrett, M. S.; Murray, J. J.; Roberts, P. L.; Schoetz,

DJ Jr. Unilateral pudendal neuropathy. Significance and implications. Dis-Colon-

Rectum. 1996 Mar; 39(3): 249-51; ISSN: 0012-3706.

UNITED-STATES. PURPOSE: Obstetric trauma and excessive defecatory

straining with perineal descent may lead to pudendal neuropathy with bilateral

increase in pudendal nerve terminal motor latencies (PNTML). We have frequently

observed unilateral prolongation of PNTML. Diagnostic and therapeutic

implications of unilateral pudendal neuropathy are discussed. METHODS: Records

of 174 patients referred to pelvic floor laboratory for anorectal manometry and

PNTML testing were reviewed. Computerized and manometry was performed

using dynamic pressure analysis, and PNTML was determined using a pudendal

(St. Mark's) electrode. RESULTS: No response was elicited from pudendal nerves

to electric stimulation from both sides in 14 patients (8 percent) and from one side

in 24 patients (13.8 percent). Bilateral PNTML determination was possible in only

136 patients (78 percent), of whom 83 patients (61 percent) had no evidence of

neuropathy, revealing normal PNTML on both sides. Of 53 patients (39 percent)

with delayed conduction in pudendal nerves, in 15 patients (28 percent), PNTML

was abnormally prolonged on both sides, with an abnormal mean value for

PNTML. In the remaining 38 patients (72 percent), PNTML was abnormal on one

side; in 27 patients with an abnormal mean PNTML and in 11 patients with a

normal mean PNTML. CONCLUSIONS: A significant number of patients with

pelvic floor disorders have only unilateral pudendal neuropathy. Patients with

unilaterally prolonged PNTML should be considered to have pudendal neuropathy,

despite normal value for mean PNTML. This fact may be relevant in planning

surgical treatment and in predicting prognosis of patients with sphincter injuries.

602. Sanna, P. P.; De Logu, A.; Williamson, R. A.; Hom, Y. L.; Straus, S. E.; Bloom, F.

E.; Burton, D. R. Protection of nude mice by passive immunization with a type-

common human recombinant monoclonal antibody against HSV. Virology. 1996

Jan 1; 215(1): 101-6; ISSN: 0042-6822.

UNITED-STATES. Herpes simplex viral disease is an important cause of

morbidity and mortality in man. Although the development of very effective

nucleoside analogs with a high therapeutic index has greatly improved the clinical

management of herpetic infections, the emergence of drug-resistant viral strains has

become a cause of serious concern both because of its clinical implications and in

terms of viral ecology. The present report is the first demonstration of the in vivo

protective activity of a type-common human recombinant monoclonal antibody

derived from a combinatorial antibody library. Athymic nude mice were infected

with HSV type 1 either intracutaneously in the flank or by corneal scarification.

Beside reducing morality rates when administered before infection, the antibody

dramatically and significantly prolonged survival times (P < 0.0001) when

administered up to 24 hr postinfection, a time when the virus had already reached

the peripheral nervous system. This suggests that the antibody may act, at least in

part, by interfering with axonal transport of the virus and/or with viral expression.

These results indicate that human recombinant antibodies isolated by antigen

selection from combinatorial libraries can be effective in vivo. Such antibodies

could complement antiviral chemotherapy and represent valuable tools for the

prophylaxis of infections by the herpes simplex viruses.. 0; 0; 0; 0; 0.

603. Saperstein, A. L.; Lusskin, R.; Doniguian, A. E.; Thomas, P. A.; Battista, A. F.

Malignant granular cell tumor mimicking herniated nucleus pulposus. Clin-Orthop.

1996 Mar; (324): 244-50; ISSN: 0009-921X.

UNITED-STATES. Peripheral nerve tumors may arise in any nerve, large or

small. Their presence is readily apparent when they are superficial, but when they

are located in a deep nerve, symptoms can mimic+ the nerve root pain of herniated

nucleus pulposus. This case of malignant granular cell tumor, probably of

Schwann cell origin, involved the sciatic and tibial nerves in the popliteal fossa.

Originally the pain was assumed to be produced by a herniated nucleus pulposus.

The discovery of the mass thus +was delayed. The mass gave a heterogeneous

signal on magnetic resonance imaging studies. A peripheral origin of nerve pain

should be considered whenever pain radiates to a limb. A complete physical

examination that includes palpation of the nerve to which pain is referred gives the

best clue as to the true cause of pain when a peripheral nerve tumor is present.

604. Satge, D. A decreased incidence of neuroblastomas in Down's syndrome and

overproduction of S-100 b protein. Med-Hypotheses. 1996 Apr; 46(4): 393-9;

ISSN: 0306-9877.

ENGLAND. Neuroblastoma, one of the most frequent solid tumors found in

childhood, is very rare in Down's syndrome subjects. This lack could possibly be

due to overproduction of S-100 b protein for the following reasons: 1) the gene

coding for S-100 b protein is situated on chromosome 21, and the protein is

overproduced via a gene dosage effect; 2) S-100 b protein is found in glial cells and

Schwann cells of the central and peripheral nervous system and has been shown to

have a differentiating effect on normal neural cells; 3) neuroblastomas with a stroma

rich in S-100 protein have a good prognosis. Preliminary studies demonstrated an

inhibition of growth of two human neuroblastoma cell lines in the presence of S-

100 b protein compared to controls. It is postulated that S-100 b protein may inhibit

the development of neuroblastomas in Down's syndrome either antenatally, or after

birth and may be a therapeutic agent against neuroblastoma.. 0; 0.

605. Savarese, D. M.; Gordon, J.; Smith, T. W.; Litofsky, N. S.; Licho, R.; Ragland, R.;

Recht, L. Cerebral demyelination syndrome in a patient treated with 5-fluorouracil

and levamisole. The use of thallium SPECT imaging to assist in noninvasive

diagnosis--a case report. Cancer. 1996 Jan 15; 77(2): 387-94; ISSN: 0008-543X.

UNITED-STATES. BACKGROUND. The use of 5-fluorouracil (5-FU) and

levamisole in patients with Stage III adenocarcinoma of the colon has now become

standard. There have been several reports of a multifocal cerebral demyelination

syndrome following 5-FU and levamisole administration. METHODS. We

describe a patient who developed focal neurologic symptoms while being treated

with levamisole and 5-FU in whom the diagnosis of central nervous system (CNS)

metastases was considered. RESULTS. A magnetic resonance imaging (MRI) scan

showed a diffuse, multifocal white matter process. Diagnostic evaluation did not

support a diagnosis of CNS metastasis. 201Thallium chloride single photon

emission computed tomography (SPECT) study was cold. A stereotactic brain

biopsy disclosed demyelination but not tumor. The patient had complete functional

resolution of symptoms with 1 month of dexamethasone therapy, although follow-

up MRI scans have shown persistent abnormality on T2-weighted images.

CONCLUSIONS. In patients receiving 5-FU and levamisole who develop focal

neurologic symptoms with an abnormal MRI scan, the diagnosis of CNS

metastasis should not be made without a thorough diagnostic evaluation. We

suggest the use of 201thallium chloride SPECT imaging to support the diagnosis of

multifocal leukoencephalopathy related to 5-FU and levamisole. In atypical cases, a

stereotactic brain biopsy may be required for confirmation.. 0; 14769-73-4; 51-21-

8.

606. Sawicki, P. T.; Muhlhauser, I.; Bender, R.; Pethke, W.; Heinemann, L.; Berger, M.

Effects of smoking on blood pressure and proteinuria in patients with diabetic

nephropathy. J-Intern-Med. 1996 Apr; 239(4): 345-52; ISSN: 0954-6820.

ENGLAND. OBJECTIVES: To investigate the effects of smoking on blood

pressure and proteinuria in hypertensive diabetic patients with nephropathy.

DESIGN: Controlled, randomized, cross-over study. SETTING: Tertiary care

centre, University Hospital of Dusseldorf, Germany. SUBJECTS: A total of 25

subjects were recruited, each of whom smoked at least 20 cigarettes a day: 10

normotensive healthy volunteers and 15 hypertensive type 1 (insulin-dependent)

diabetic outpatients with diabetic retinopathy and persistent micro- or

macroalbuminuria; 10 diabetic patients had normal autonomic function test, whilst

five patients showed signs of autonomic neuropathy. INTERVENTIONS:

Controlled smoking or nonsmoking over a period of 8 h on separate days. MAIN

OUTCOME MEASURES: Blood pressure was measured every 10 min with an

automatic device and urine samples were collected every 3 h. RESULTS: Systolic

blood pressure increased during smoking in controls (mean) (11.5 mmHg, P =

0.0001) and in diabetic patients without autonomic neuropathy (7.9 mmHg; P =

0.018), but not in patients with autonomic neuropathy (-2.4 mmHg; P = 0.792).

Diastolic blood pressure increased during smoking in controls (6.2 mmHg; P =

0.019) but not in diabetic patients (2.5 mmHg; P = 0.204. 0.2 mmHg; P = 0.956).

During smoking, median proteinuria and albuminuria increased in diabetic patients

without autonomic neuropathy (8.1 mg mmol-1 creatinine, P = 0.002; and 2.6 mg

mmol creatinine, P = 0.084). No significant changes in albuminuria or proteinuria

occurred in the other two groups. CONCLUSIONS: Smoking increases blood

pressure values in healthy subjects and in hypertensive patients with diabetic

nephropathy and without autonomic neuropathy. This effect of smoking may be

partly responsible for the faster progression of diabetic nephropathy in smoking

diabetic patients.

607. Scaioli, V.; Franzini, A.; Leocata, F.; Broggi, G. Hand dystonia and neuralgic pain

due to neurovascular contact to cervical spinal root [letter]. Mov-Disord. 1996 Jan;

11(1): 102-4; ISSN: 0885-3185.

UNITED-STATES.

608. Scarabino, T.; Giannatempo, G. M.; Perfetto, F.; Popolizio, T.; Salvolini, U.

[Magnetic resonance myelography with a fast-spin-echo sequence]. Mielografia

con Risonanza Magnetica con sequenza Fast Spin Echo. Radiol-Med-Torino. 1996

Mar; 91(3): 202-6; ISSN: 0033-8362.

ITALY. A major application of Magnetic Resonance Imaging (MRI) consists in the

"myelographic effect", characterized by high signal intensity of the CSF, which

permits to visualize spinal subarachnoid spaces and nerve root sheaths. Such new

ultrafast pulse sequences as fast spin echo (FSE) further enhance CSF high signal

intensity. In addition, suppressing background with the fat suppression technique

and with maximum intensity projection (MIP), a particular algorithm already used

in MR angiography, FSE sequences yield 3D myelography-like images. Our study

was aimed at assessing the clinical role and the reliability of this technique. Our

preliminary experience is based on 21 patients with pain in the lower back and legs

and on 5 healthy volunteers; all the myelography-like images were obtained with a

1.5-T MR system (Signa, General Electric). The parameters for FSE myelography-

like images included: TR = 6000, TE = 200, ETL = 32, thickness = 3 mm, matrix

= 256 x 224, Nex = 4, FOV = 20 cm, fat presaturation, no phase wrap, acquisition

time = 2 min 58 s. MR myelography sequences were acquired after a standard FSE

T1/T2-weighted exam. MR-myelography results confirmed the diagnosis of disk

herniation made on conventional T1/T2-weighted images, especially in thecal sac

compression and/or amputation of nerve root sheaths. MR myelography appeared

to be especially useful in migrated disk herniation or in abnormal origin of nerve

roots. Moreover, it allowed full and panoramic visualization of the subarachnoid

spaces and excellent definition of the thecal sac, nerve roots and nerve root sheaths.

In one patient only movement artifacts affected image quality. To conclude, even

though it fails to yield any functional information, MR myelography appears to be

an easy, rapid and noninvasive support to conventional MRI of the lumbar spine, to

replace conventional myelography.

609. Schabes, G. A.; Hillgen, JJ 4th. Pathologic mandibular fracture associated with

familial dysautonomia. J-Oral-Maxillofac-Surg. 1996 Jan; 54(1): 116-8; ISSN:

0278-2391.

UNITED-STATES.

610. Schepsis, A. A.; Lynch, G. Exertional compartment syndromes of the lower extremity.

Curr-Opin-Rheumatol. 1996 Mar; 8(2): 143-7; ISSN: 1040-8711.

UNITED-STATES. Compartment syndromes may be acute or chronic secondary

to exertion or exercise. The chronic or exertional type most commonly involves the

lower extremity, particularly the anterior compartment of the lower leg, and is the

subject of this review. Rarely, an exertional compartment syndrome may become

acute. The diagnosis is based on history, physical examination, and compartment

pressure measurements. The differential diagnosis of exertional leg pain includes

stress fractures, stress reaction, periostitis, claudication, popliteal artery

entrapment, and peripheral nerve entrapment. Unusual causes, such as a ganglion

of the proximal tibiofibular joint causing an anterior compartment syndrome, have

recently been reported.

611. Schmal, F.; Kumpf, W.; Stoll, W. [Effect of various electrode configurations and

voluntary, reproducible artifacts in computer-assisted analysis of nystagmus]. Der

Einfluss verschiedener Elektrodenanordnungen und willkurlicher, reproduzierbarer

Artefakte auf die computergestutzte Nystagmusanalyse. Laryngorhinootologie.

1996 Mar; 75(3): 148-53; ISSN: 0935-8943.

GERMANY. BACKGROUND: There is no detailed information in the current

literature about the best position for the electrodes with regard to

electronystagmography. The computer nystagmograph registers potential changes

resulting from electrodes on the forearm as nystagmus (pseudonystagmus). This

phenomenon led us to investigate more closely the effect of various electrode

arrangements and artifacts on the result of computer nystagmography. METHODS:

The following four electrode arrangements were investigated on 20 human subjects:

distance between the horizontal electrode and the lateral canthus (1 cm versus 2

cm), diagonal arrangement of the lateral electrodes, and changes to the lower

vertical electrode. Recorded parameters included calibration potential, rotary

nystagmus, post-rotary nystagmus, and optokinetic nystagmus. After determining

the basic activity with closed eyes, the influence of five human artifacts on the

computer nystagmography was investigated, namely blinking, contractions of the

masticatory muscles, swallowing, facial expression, and squinting. RESULTS:

The arrangement in which the horizontal electrodes were placed 1 cm away from

the lateral canthus showed the greatest calibration potential and the smallest degree

of human influence with regard to frequency and amplitude of the

pseudonystagmus since a larger potential does not have to be amplified as much as

a smaller one to achieve the same needle deflection. For this reason, the artifacts

experience a smaller degree of amplification. CONCLUSIONS: We think the

following arrangement of the electrodes guarantees the best signal reproduction: a

distance between horizontal electrodes and the lateral canthus of 1 cm. The medial

electrode is centered between the eyes on the bridge of the nose. The upper vertical

electrode is placed above the left eyebrow in the line with the middle of the pupil.

The lower vertical electrode is also in line with the middle of the pupil, 1 cm below

the lower eyelid. The result is a clear recording of a physiological nystagmus, e.g.

on rotation. In the absence of nystagmus, the analysis program is too unreliable and

produces a multiplicity of pseudonystagmi. To avoid this we have to improve the

recognition of nystagmus in the analysis program.

612. Schnarkowski, P.; Peterfy, C. G.; Johnston, J. O.; Weidner, N. Clear cell sarcoma

mimicking peripheral nerve sheath tumor. Skeletal-Radiol. 1996 Feb; 25(2): 197-

200; ISSN: 0364-2348.

GERMANY. 0; 67-43-6; 7440-54-2; 80529-93-7.

613. Schnell, O.; Stenner, T.; Standl, E.; Haslbeck, M. [The diagnostic value of the rate-

corrected QT interval in long-term type-1 diabetes mellitus]. Zum diagnostischen

Wert des frequenzkorrigierten QT-Intervalls bei langjahrigem Typ-I-Diabetes

mellitus. Dtsch-Med-Wochenschr. 1996 Jun 21; 121(25-26): 819-22; ISSN: 0012-

0472.

GERMANY. OBJECTIVE. To assess the relationship between rate-corrected QT

interval (QTc interval) and cardiac reflex tests in order to determine the value of

QTc interval measurements in the diagnosis of diabetic cardiac autonomic

neuropathy. INVESTIGATIONS. The QTc interval was measured in the resting

ECG of 97 type 1 diabetics (58 women, 39 men; mean age 35 +/- 12 years;

duration of diabetes 18 +/- 10 years; HbA1c 7.8 +/- 1.8%). Age-related results

were compared with five cardiac function tests (heart rate variation at rest and on

forced breathing; 30/15 ratio of heart rate; Valsalva manoeuvre; orthostasis).

RESULTS. The QTc interval was not prolonged ( < or = 440 ms) in 68 patients

(70%), while in 29 (30%) it was prolonged ( > 440 ms). No significant differences

regarding QTc interval were found between patients with autonomic cardiac

neuropathy ( > or = 2 abnormal function tests) and those without ( < 2 abnormal

function tests) (QTc interval 436 +/- 25 vs 426 +/- 19 ms). QTc intervals correlated

with the coefficients of variation for heart rate variation at rest and on forced

breathing and the 30/15 ratio of heart rate (p = 0.001; p = 0.001; p = 0.03), but not

with the results of the Valsalva manoeuvre and the orthostasis test.

CONCLUSION. Prolongation of the QTc interval in longstanding type 1 diabetes

does not provide a reliable indication of cardiac autonomic neuropathy and this

measure cannot replace conventional reflex tests for its diagnosis.

614. Schroder, H. A. [Supinator syndrome, an underdiagnosed disease (letter)].

Supinatorlogesyndrom, en underdiagnosticeret lidelse. Ugeskr-Laeger. 1996 Apr

15; 158(16): 2274-5; ISSN: 0041-5782.

DENMARK.

615. Schroeder, T. L.; Rainey, B. B.; Goss, D. A.; Grosvenor, T. P. Reliability of and

comparisons among methods of measuring dissociated phoria. Optom-Vis-Sci.

1996 Jun; 73(6): 389-97; ISSN: 1040-5488.

UNITED-STATES. Many methods of heterophoria measurement are available

clinically. This paper reviews several studies which have examined the reliability of

phoria measurements, and have compared various tests of phoria measurement.

Different methods of data analysis make comparison of studies difficult. Two

studies indicated 95% limits of agreement of 2 to 4 delta for reliability of modified

Thorington, von Graefe, and Maddox rod phoria tests. Studies using correlational

analysis to compare different measurement methods have found a high degree of

association of the results of these methods. Studies using statistical methods

assessing the agreement of test results found a high level of agreement among some

tests and a low level of agreement among some tests. Some of the various phoria

measurement methods differ in the technique used for dissociation, in the ability to

control accommodation adequately, in the level of proximal convergence induced,

or in the method by which the phoria is quantified. These differences can result in

different phoria measurements on the same patient.

616. Schuller, D. E.; Cadman, T. E.; Jeffreys, W. H. Recurrent headaches: what every

allergist should know. Ann-Allergy-Asthma-Immunol. 1996 Mar; 76(3): 219-26;

quiz 226-30; ISSN: 1081-1206.

UNITED-STATES. OBJECTIVE: To provide the allergist information regarding

the recognition, diagnosis, classification, and management of headaches. DATA

SOURCES: Literature and relevant articles pertaining to various types of headache

are reviewed and the clinical experience of the authors is presented.

CONCLUSIONS: After reading this article, the allergist should know the various

causes of headache, recognize the warning signs of serious neurologic disease, and

determine whether allergy or adverse food reactions are playing a role.

617. Sedwick, L. How to handle the pressure or too much of a good thing. Surv-

Ophthalmol. 1996 Jan; 40(4): 307-11; ISSN: 0039-6257.

UNITED-STATES. A 28-year-old non-obese woman with pseudotumor cerebri

and minimal visual loss who underwent two optic nerve sheath decompression

procedures. Different diagnosis and treatment options are discussed.

618. Sengpiel, F.; Blakemore, C. The neural basis of suppression and amblyopia in

strabismus. Eye. 1996; 10( Pt 2): 250-8; ISSN: 0950-222X.

ENGLAND. The neurophysiological consequences of artificial strabismus in cats

and monkeys have been studied for 30 years. However, until very recently no clear

picture has emerged of neural deficits that might account for the powerful

interocular suppression that strabismic humans experience, nor for the severe

amblyopia that is often associated with convergent strabismus. Here we review the

effects of squint on the integrative capacities of the primary visual cortex and

propose a hypothesis about the relationship between suppression and amblyopia.

Most neurons in the visual cortex of normal cats and monkeys can be excited

through either eye and show strong facilitation during binocular stimulation with

contours of similar orientation in the two eyes. But in strabismic animals, cortical

neurons tend to fall into two populations of monocularly excitable cells and exhibit

suppressive binocular interactions that share key properties with perceptual

suppression in strabismic humans. Such interocular suppression, if prolonged and

asymmetric (with input from the squinting eye habitually suppressed by that from

the fixating eye), might lead to neural defects in the representation of the deviating

eye and hence to amblyopia.

619. Seror, P. [Benign monomelic amyotrophy and localized spinal atrophy (letter)].

Amyotrophie monomelique benigne et atrophie spinale localisee. Presse-Med. 1996

Jan 20; 25(2): 82; ISSN: 0755-4982.

FRANCE.

620. Shandurina, A. N.; Panin, A. V.; Sologubova, E. K.; Kolotov, A. V.; Goncharenko,

O. I.; Nikol'skii, A. V.; Logunov, V. Y. u. Results of the use of therapeutic

periorbital electrostimulation in neurological patients with partial atrophy of the

optic nerves. Neurosci-Behav-Physiol. 1996 Mar; 26(2): 137-42; ISSN: 0097-

0549.

UNITED-STATES.

621. Shapira, O. M.; Kimmel, W. A.; Lindsey, P. S.; Shahian, D. M. Anterior ischemic

optic neuropathy after open heart operations. Ann-Thorac-Surg. 1996 Feb; 61(2):

660-6; ISSN: 0003-4975.

UNITED-STATES. BACKGROUND. The overall incidence of anterior ischemic

optic neuropathy after open heart operations at the Lahey Clinic is less than 0.5%.

However, during the 2-year period, March 1, 1990, to March 1, 1992, an

increased incidence (8 of 602 patients or 1.3%) of this complication was observed.

METHODS. A rigorous analysis was conducted of all 602 patients who underwent

operation during this period. RESULTS. No preoperative risk factors were

identified. The development of anterior ischemic optic neuropathy was associated

with prolonged cardiopulmonary bypass time, low hematocrit levels, excessive

perioperative body weight gain, and the use of epinephrine and amrinone. Other

hypothetical risk factors include systemic hypothermia, anemia, increased

intraocular pressure, and microembolization. Treatment options include the use of

corticosteroid medications, reduction of intraocular pressure, and optic nerve

fenestration, although recent evidence and our experience indicate that the

fenestration procedure is of no benefit. CONCLUSIONS. Because all methods of

treatment have had limited success, efforts to prevent this complication are of

paramount importance.. 51-43-4; 60719-84-8.

622. Shapiro, N. L.; Cunningham, M. J.; Parikh, S. R.; Eavey, R. D.; Cheney, M. L.

Congenital unilateral facial paralysis. Pediatrics. 1996 Feb; 97(2): 261-4; ISSN:

0031-4005.

UNITED-STATES.

623. Shawkat, F. S.; Harris, C. M.; Taylor, D. S.; Kriss, A. The role of ERG/VEP and eye

movement recordings in children with ocular motor apraxia. Eye. 1996; 10( Pt 1):

53-60; ISSN: 0950-222X.

ENGLAND. Ocular motor apraxia (OMA) is characterised by an intermittent

inability to initiate voluntary saccades, and a failure to produce optokinetic and

vestibular quick phases. Some patients have no other abnormalities (idiopathic

OMA), whereas in others it appears associated with a variety of neurological

conditions which may affect the sensory visual pathway. Electroretinograms

(ERGs), flash and pattern visual evoked potentials (VEPs) and eye movements

were assessed in 53 children with OMA (age range 17 days to 14 years) to

determine their efficacy in helping to distinguish between idiopathic and non-

idiopathic cases. Seven patients (13.2%) had idiopathic OMA and the remaining 46

(86.8%) had other associated clinical conditions. All patients had episodes of

absent quick phases ('lock up') during optokinetic nystagmus (OKN) and/or

vestibular testing. Flash ERGs were abnormal in only 7 patients (13.2%); 6 had

syndromes involving a pigmentary retinopathy (Joubert's, Bardet-Biedl, infantile

Refsum's, Kearns-Sayre's), and the seventh had a cone dystrophy with vermis

hypoplasia. VEPs were normal in all 7 patients with idiopathic OMA. Thirty-three

(72%) patients with OMA in association with neurological conditions had abnormal

VEPs and 13 had normal VEPs (28%). There was a significant positive correlation

between VEP abnormality and poor OKN gain. VEP/ERG testing and eye

movement studies are useful when OMA is suspected as they help in distinguishing

isolated idiopathic cases from those with more widespread neurological

abnormalities.

624. Sheean, G. L.; Kanabar, G.; Murray, N. M. Lumbrical-interosseous comparison in a

distal ulnar nerve lesion. Muscle-Nerve. 1996 May; 19(5): 673-4; ISSN: 0148-

639X.

UNITED-STATES.

625. Shelton, C.; Harnsberger, H. R.; Allen, R.; King, B. Fast spin echo magnetic

resonance imaging: clinical application in screening for acoustic neuroma.

Otolaryngol-Head-Neck-Surg. 1996 Jan; 114(1): 71-6; ISSN: 0194-5998.

UNITED-STATES. The advent of magnetic resonance imaging has greatly

improved our ability to diagnose acoustic tumors, but it is a relatively expensive

imaging modality. In the present climate of medical cost restraints, methods that

reduce costs but maintain quality are extremely desirable. We report a new magnetic

resonance imaging technique that uses fast spin echo without gadolinium. It

provides ultrahigh-resolution images of the internal auditory canal and

cerebellopontine angle. The sensitivity of this technique for the detection of acoustic

tumors is equivalent to conventional gadolinium-enhanced magnetic resonance

imaging, but the global cost is comparable with that of brain stem audiometry. In

our practice fast spin echo magnetic resonance imaging has replaced brain stem

audiometry as a screening modality to evaluate most acoustic tumor suspects. Also,

the intricate detail of the internal auditory canal anatomy provided by this technique

is useful in planning surgical removal of acoustic tumors.. 7440-54-2.

626. Sheth, R. D.; Riggs, J. E.; Ortiz, O. A. Raymond syndrome: a validation. Eur-Neurol.

1996; 36(3): 173-4; ISSN: 0014-3022.

SWITZERLAND.

627. Shewan, D.; Calaora, V.; Nielsen, P.; Cohen, J.; Rougon, G.; Moreau, H. mCD24, a

glycoprotein transiently expressed by neurons, is an inhibitor of neurite outgrowth.

J-Neurosci. 1996 Apr 15; 16(8): 2624-34; ISSN: 0270-6474.

UNITED-STATES. In the immune system, mCD24, the mouse homolog of the

human glycosyl phosphatidylinositol-anchored glycoprotein CD24, may play a role

in cell adhesion. In the nervous system, the function of mCD24 has not been

determined, but its transient expression by neurons suggests that it may be involved

in axon growth in development. Here we show that retinal ganglion cells (RGCs)

and dorsal root ganglion (DRG) neurons express mCD24 in the developing but not

adult mouse in vivo and in DRG neurons of the injured adult peripheral nervous

system (PNS). In vitro, mCD24 was expressed by immature neurons and by a

subpopulation of adult DRG neurons. To analyze the possible function of mCD24

in the nervous system, we prepared rat C6 glioma cells stably transfected or

retrovirally infected with mCD24 cDNA. The cells did not exhibit changes in their

adhesive properties or cell division rate after transfection or infection. When

mCD24-expressing cells were used as monolayer substrates for culturing RGCs

and DRG neurons, neurite outgrowth was inhibited, depending on neuronal age

and on the relative levels of mCD24 in the monolayer. This inhibition, however,

was not dependent on the expression of mCD24 by the neurons themselves,

because DRG neurons of a mouse deleted of the mCD24 gene showed the same

response. These results show that mCD24 interacts in a heterophilic manner with a

developmentally regulated molecule expressed by neurons, and they suggest that in

vivo, mCD24 may inhibit the further extension or collateral branching of axons in

late embryonic development.. 0.

628. Shindo, H.; Thomas, T. P.; Larkin, D. D.; Karihaloo, A. K.; Inada, H.; Onaya, T.;

Stevens, M. J.; Greene, D. A. Modulation of basal nitric oxide-dependent cyclic-

GMP production by ambient glucose, myo-inositol, and protein kinase C in SH-

SY5Y human neuroblastoma cells. J-Clin-Invest. 1996 Feb 1; 97(3): 736-45;

ISSN: 0021-9738.

UNITED-STATES. Defective tissue perfusion and nitric oxide production and

altered myo-inositol metabolism and protein kinase C activation have been invoked

in the pathogenesis of diabetic complications including neuropathy. The precise

cellular compartmentalization and mechanistic interrelationships of these

abnormalities remain obscure, and nitric oxide possesses both neurotransmitter and

vasodilator activity. Therefore the effects of ambient glucose and myo-inositol on

nitric oxide-dependent cGMP production and protein kinase C activity were studied

in SH-SY5Y human neuroblastoma cells, a cell culture model for peripheral

cholinergic neurons. D-Glucose lowered cellular myo-inositol content,

phosphatidylinositol synthesis, and phosphorylation of an endogenous protein

kinase C substrate, and specifically reduced nitric oxide-dependent cGMP

production a time- and dose-dependent manner with an apparent IC50 of

approximately 30 mM. The near maximal decrease in cGMP induced by 50 mM D-

glucose was corrected by the addition of protein kinase C agonists or 500 microM

myo-inositol to the culture medium, and was reproduced by protein kinase C

inhibition or downregulation, or by myo-inositol deficient medium. Sodium

nitroprusside increased cGMP in a dose-dependent fashion, with low

concentrations (1 microM) counteracting the effects of 50 mM D-glucose or protein

kinase C inhibition. The demonstration that elevated D-glucose diminishes basal

nitric oxide-dependent cGMP production by myo-inositol depletion and protein

kinase C inhibition in peripheral cholinergic neurons provides a potential metabolic

basis for impaired nitric oxide production, nerve blood flow, and nerve impulse

conduction in diabetes.. EC 1.14.13.39; EC 2.7.1.-; 10102-43-9; 50-70-4; 50-99-

7; 6917-35-7; 7665-99-8.

629. Shindo, M. L.; Zaretsky, L. S.; Rice, D. H. Autologous fat injection for unilateral

vocal fold paralysis. Ann-Otol-Rhinol-Laryngol. 1996 Aug; 105(8): 602-6; ISSN:

0003-4894.

UNITED-STATES. This study evaluates the efficacy of autologous fat injection

for medialization of the paralyzed vocal fold. In 21 patients with unilateral vocal

fold paralysis, autologous abdominal fat was injected into the thyroarytenoid

muscle to achieve medialization. All patients were followed up with serial

videolaryngoscopy and voice evaluation. At 2 months' follow-up, the voice was

judged to be excellent in 10 patients, slightly breathy but significantly better than

the preoperative voice in 6 patients, and markedly breathy in 4 patients. At 3 to 4

months' follow-up, of the 10 patients with excellent results, 5 maintained an

excellent voice, 3 had developed slight breathiness, and 1 had developed severe

breathiness. Long-term (6 to 12 months) results were available in 11 patients, and

all of them maintained the same voice quality that was noted during the 3 to 4

months' examination. Magnetic resonance imaging of the larynx was obtained in 7

patients at intervals ranging from 1 to 7 months and compared to the baseline scan

obtained at 1 week postoperative to assess the amount of fat remaining in the

muscle. The images showed fat volume to persist, but a decrease in the fat signal

was observed over time. The results suggest that the duration of medialization with

autologous fat is variable, but appears to last at least 2 to 3 months. This loss of

volume after 3 months seems to be due to absorption of the fat and possibly muscle

atrophy. Autologous fat injection is relatively safe and easy to perform, and is an

ideal method of temporary vocal fold medialization in patients in whom return of

vocal fold function is expected.

630. Shishov, A. S.; Virych, I. E.; Bagrov, F. I.; Latysheva, I. T. [The Guillain-Barre

syndrome in herpes zoster patients]. Sindrom Giiena--Barre u bol'nykh

opoiasyvaiushchim lishaem. Zh-Nevropatol-Psikhiatr-Im-S-S-Korsakova. 1996;

96(2): 26-9; ISSN: 0044-4588.

RUSSIA. A rare case of Guillain-Barre syndrome associated with herpes zoster

developed on the 7th day of eruption in the right dermatoma DII-III in a man of 51

years old. The peculiarities of the symptoms, current status, liquor changes have

been analysed basing on the data from foreign literature on 24 cases of Guillain-

Barre syndrome (polyradiculoneuritis) associated with herpes zoster.

631. Shoaib, B. O.; Patten, B. M. Human adjuvant disease: presentation as a multiple

sclerosis-like syndrome [see comments]. South-Med-J. 1996 Feb; 89(2): 179-88;

ISSN: 0038-4348.

Note: Comment in: South Med J 1996 Feb;89(2):251-2.

UNITED-STATES. Twenty-six women had a systemic disease with central

nervous system (CNS) involvement at a mean age of 39.2 years (range, 23 to 64

years) after receiving silicone breast implants (n = 25) or silicone fluid injections

into breasts (n = 1). The median latency period between breast surgery and onset of

symptoms was 5.71 years (range, 3 months to 15 years). All patients had evidence

of disseminated CNS lesions; 20 patients also had evidence of peripheral

neuropathy. Additional problems included myalgia (n = 24), joint stiffness (n =

23), arthralgia (n = 22), sicca complex (dry eyes and dry mouth) (n = 19),

headache (n = 16), skin rash (n = 15), joint swelling (n = 14), Raynaud's

phenomena (n = 14), fever (n = 13), hair loss (n = 12), allergies (n = 11),

sensitivity to sunlight (n = 10), and lymphadenopathy (n = 9). Magnetic resonance

imaging brain scans were abnormal in 22 of 26 patients (21, white matter lesions;

1, ischemic lesions; 4, cerebral atrophy). Spinal tap revealed oligoclonal bands in

18 of 23 patients. Visual evoked responses were delayed in 14 of 23 patients, and

autodirected antibodies were detected in 16 of 26. Sural nerve biopsy results

showed loss of myelinated fibers in 15 of 15. Seventeen of 24 patients (71%) who

had implant removal were found to have grossly ruptured implants. We believe our

patients had a new syndrome triggered by the foreign material in their body. This

syndrome appears as a systemic inflammatory autoimmune disease with central

nervous system involvement resembling multiple sclerosis.. 0; 0.

632. Shoaib, B. O.; Patten, B. M. Human adjuvant disease: presentation as a multiple

sclerosis-like syndrome [see comments]. South-Med-J. 1996 Feb; 89(2): 179-88;

ISSN: 0038-4348.

Note: Comment in: South Med J 1996 Feb;89(2):251-2.

UNITED-STATES. Twenty-six women had a systemic disease with central

nervous system (CNS) involvement at a mean age of 39.2 years (range, 23 to 64

years) after receiving silicone breast implants (n = 25) or silicone fluid injections

into breasts (n = 1). The median latency period between breast surgery and onset of

symptoms was 5.71 years (range, 3 months to 15 years). All patients had evidence

of disseminated CNS lesions; 20 patients also had evidence of peripheral

neuropathy. Additional problems included myalgia (n = 24), joint stiffness (n =

23), arthralgia (n = 22), sicca complex (dry eyes and dry mouth) (n = 19),

headache (n = 16), skin rash (n = 15), joint swelling (n = 14), Raynaud's

phenomena (n = 14), fever (n = 13), hair loss (n = 12), allergies (n = 11),

sensitivity to sunlight (n = 10), and lymphadenopathy (n = 9). Magnetic resonance

imaging brain scans were abnormal in 22 of 26 patients (21, white matter lesions;

1, ischemic lesions; 4, cerebral atrophy). Spinal tap revealed oligoclonal bands in

18 of 23 patients. Visual evoked responses were delayed in 14 of 23 patients, and

autodirected antibodies were detected in 16 of 26. Sural nerve biopsy results

showed loss of myelinated fibers in 15 of 15. Seventeen of 24 patients (71%) who

had implant removal were found to have grossly ruptured implants. We believe our

patients had a new syndrome triggered by the foreign material in their body. This

syndrome appears as a systemic inflammatory autoimmune disease with central

nervous system involvement resembling multiple sclerosis.. 0; 0.

633. Shukla, P. C.; Carlton, FB Jr. Diagnosis of thoracic outlet syndrome in the emergency

department. South-Med-J. 1996 Feb; 89(2): 212-7; ISSN: 0038-4348.

UNITED-STATES. The use of the emergency department (ED) as a source for

primary care has increased the number of patients seen in this setting with even

chronic symptoms such as pain, paresthesias, and weakness in the upper extremity.

This group may include individuals with thoracic outlet syndrome (TOS). We were

concerned that TOS may be underdiagnosed in the ED because of physician

unfamiliarity with the signs and symptoms of TOS. Hence, we retrospectively

studied cases of TOS seen at the ED of the University hospital during a 29-year

period. We believe this is the first report in the English language literature to reflect

the assessment and management of TOS in the ED. The study data include clinical

presentation, diagnostic tests, and management of TOS. Lack of thorough

evaluation resulted in underdiagnosis of TOS in our ED. We recommend that ED

personnel pay close attention to patients with symptoms of long duration and that

ED physicians be aware of TOS presentation and its management.

634. Sicherer, S. H.; Sampson, H. A. Auriculotemporal syndrome: a masquerader of food

allergy. J-Allergy-Clin-Immunol. 1996 Mar; 97(3): 851-2; ISSN: 0091-6749.

UNITED-STATES.

635. Siegel, I. M. Cavus versus planus in the neuropathic foot (peroneal muscular atrophy

reconsidered) [letter]. Muscle-Nerve. 1996 Jun; 19(6): 797; ISSN: 0148-639X.

UNITED-STATES.

636. Sieradzki, J.; Koblik, T.; Otfinowski, J.; Friedlein, J.; Legutko, J.; Stoch, A.;

Cencora, A.; Mrowiecki, A.; Gacka, J. [Diabetic foot syndrome in clinical practice.

II. Results after one year at the Diabetic Foot Consulting Unit]. Zespol stopy

cukrzycowej w praktyce klinicznej II. Wyniki rocznej dzialalnosci Gabinetu Stopy

Cukrzycowej. Przegl-Lek. 1996; 53(1): 6-8; ISSN: 0033-2240.

POLAND. The study included 46 patients with the diabetic foot syndrome and

ulcerations, and 40 patients with high-risk foot. Mean duration of hospitalization of

the patients with ulcerations was 54 days, mean daily glycemia decreased from

162.5 mg% to 114.9 mg%. Ulcerations were completely healed in 93.5% of

patients, whereas high and partial foot amputation was high and 3.2% of patients,

respectively. The annual amputation rate was 2.3% in the entire group of diabetic

foot patients (86). The present results indicate the necessity for adoption of a

multidisciplinary approach to the problem of diabetic foot. It may be added that

cooperation in glycemia normalization and patients education may decrease the

number of amputations, and hence the degree of physical disability in diabetic foot

patients.

637. Silver, N. C.; Barker, G. J.; MacManus, D. G.; Miller, D. H.; Thorpe, J. W.;

Howard, R. S. Decreased magnetisation transfer ratio due to demyelination: a case

of central pontine myelinolysis [letter]. J-Neurol-Neurosurg-Psychiatry. 1996 Aug;

61(2): 208-9; ISSN: 0022-3050.

ENGLAND.

638. Simon, J. W.; Waldman, J. B.; Couture, K. C. Cerebellar astrocytoma manifesting as

isolated, comitant esotropia in childhood. Am-J-Ophthalmol. 1996 May; 121(5):

584-6; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: We encountered a 4 1/2-year-old girl with gradual

onset of intermittent, comitant esotropia in the absence of diplopia and other

neurologic findings. METHODS: Because of the patient's relatively advanced age

and lack of response to hyperopic correction for accommodative esotropia,

computed tomography of the head was performed. RESULTS: A large cerebellar

astrocytoma was identified and successfully resected. After strabismus surgery,

fusion was reestablished. CONCLUSIONS: The onset of comitant esotropia in an

older child may indicate an underlying neurologic disorder.

639. Simpson, R. L.; Fern, S. A. Multiple compression neuropathies and the double-crush

syndrome. Orthop-Clin-North-Am. 1996 Apr; 27(2): 381-8; ISSN: 0030-5898.

UNITED-STATES. Multiple compressions along a nerve have a cumulative effect

on condition, both antegrade and retrograde. This nerve compromise renders the

nerve more susceptible to a second source of compression. The proximal source of

compression may be subclinical yet partially responsible for the cumulative

compression syndrome. The primary "crush" may be anatomic or metabolic in

origin. Strict attention to the patient's history and physical findings permit

separation of the multiple compressions, making the choice of surgical

decompression easier. Provocative testing and work simulation are important in

examination, as is EMG testing. Complete medical evaluation can aid in diagnosing

patients presenting with peripheral nerve compression and additional risk factors.

Surgical decompression of a nerve with multiple levels of compression may not

relieve all symptoms. Dynamic compressions in the work place and other

nonsurgical types of compression must be identified so the prognosis for complete

recovery is improved.

640. Sklar, E. M.; Schatz, N. J.; Glaser, J. S.; Post, M. J.; ten Hove, M. MR of vasculitis-

induced optic neuropathy. AJNR-Am-J-Neuroradiol. 1996 Jan; 17(1): 121-8;

ISSN: 0195-6108.

UNITED-STATES. PURPOSE: To describe the MR characteristics of optic

neuropathy caused by vasculitis. METHODS: Nine cases of optic neuropathy with

diagnosis of vasculitis (six with systemic lupus erythematosis and one each with

rheumatoid arthritis, Sjogren disease, and radiation vasculitis) were reviewed

retrospectively. Patients were 31 to 62 years old, and all but one were women. All

patients had MR imaging through the orbits and anterior visual pathways, five with

fat suppression, with and without gadopentetate dimeglumine. Five patients also

had MR imaging of the entire brain. The size and enhancement of various segments

of the optic nerve and anterior visual pathways were studied. RESULTS: MR

imaging with contrast material showed enhancement and enlargement of segments

of the optic nerves and/or chiasm in six of the nine patients (all but three with

systemic lupus erythematosis). Enlargement of a segment of the anterior visual

pathway never occurred without enhancement, but enhancement alone did occur in

three cases. Of the five patients who had MR imaging of the whole brain,

abnormalities were seen in three: periventricular hyperintensity in two and a lacunar

infarct in one; none had vessel abnormalities. CONCLUSION: Because the MR

enhancement seen represents disruption of the blood-brain barrier within the optic

nerve, MR imaging with gadopentetate dimeglumine and fat suppression should be

performed to detect increased permeability of the blood-brain barrier in acute optic

neuropathy.. 0; 0; 0; 6284-40-8; 67-43-6; 86050-77-3.

641. Skoog, I.; Berg, S.; Johansson, B.; Palmertz, B.; Andreasson, L. A. The influence of

white matter lesions on neuropsychological functioning in demented and non-

demented 85-year-olds. Acta-Neurol-Scand. 1996 Feb; 93(2-3): 142-8; ISSN:

0001-6314.

DENMARK. White matter lesions on computed tomography of the head were

studied in relation to neuropsychological functioning in subjects from a

representative sample of non-demented (n = 134) and demented (n = 98) 85-year-

olds. Non-demented subjects with white matter lesions (n = 46) scored

significantly lower in tests of verbal ability (Synonyms), spatial ability (Block

Design, Clock Test), perceptual speed (Identical forms), secondary memory

(Thurstone Picture Memory), basic arithmetic (Coin Test) and the global cognitive

screening test Mini-Mental State Examination than non-demented subjects without

white matter lesions (n = 88). Demented subjects with white matter lesions (n = 67)

scored significantly lower in tests of spatial ability (Block Design and Clock Test)

and secondary memory (free recall in the MIR memory test, Ten-word memory test

I and II) and in the Mini-Mental State Examination than demented subjects without

white matter lesions (n = 31). It is concluded that white matter lesions contribute to

cognitive decline in both non-demented and demented elderly subjects.

642. Sladky, J. T. Immune neuropathies in childhood. Baillieres-Clin-Neurol. 1996 Mar;

5(1): 233-44; ISSN: 0961-0421.

ENGLAND. In contradistinction to older populations, immune-mediated disorders

(principally demyelinating processes) account for nearly half of peripheral

neuropathies in childhood. The largest single diagnostic entity is GBS, which

makes up approximately 25% of sensorimotor neuropathies in patients under 18

years of age. The clinical features are similar to those encountered in adults,

although the prognosis in youngsters appears to be better than in older populations.

Despite the absence of prospective data, plasmapheresis seems to be an effective

modality for hastening recovery during GBS in children. The use of human

immunoglobulin has gained acceptance for the treatment of GBS in adults, but

insufficient data exist to draw firm conclusions about it role in the management of

paediatric GBS. CIDP is the second most common cause of chronic sensorimotor

neuropathy in children. The clinical manifestations of this disorder are extremely

variable, and it can mimic the phenotype of several genetically determined

neuropathies. The prognosis in this disorder is also relatively good, although a

small number of children have significant neurological disability or treatment side-

effects. Other immune-mediated neuropathies are relatively infrequent in our

experience. When they occur, they are often associated with collagen-vascular

diseases or bone marrow transplantation. Peripheral neuropathy in association with

HIV infection in children appears to be rare.. 0; 53-03-2.

643. Sladky, J. T. Immune neuropathies in childhood. Baillieres-Clin-Neurol. 1996 Mar;

5(1): 233-44; ISSN: 0961-0421.

ENGLAND. In contradistinction to older populations, immune-mediated disorders

(principally demyelinating processes) account for nearly half of peripheral

neuropathies in childhood. The largest single diagnostic entity is GBS, which

makes up approximately 25% of sensorimotor neuropathies in patients under 18

years of age. The clinical features are similar to those encountered in adults,

although the prognosis in youngsters appears to be better than in older populations.

Despite the absence of prospective data, plasmapheresis seems to be an effective

modality for hastening recovery during GBS in children. The use of human

immunoglobulin has gained acceptance for the treatment of GBS in adults, but

insufficient data exist to draw firm conclusions about it role in the management of

paediatric GBS. CIDP is the second most common cause of chronic sensorimotor

neuropathy in children. The clinical manifestations of this disorder are extremely

variable, and it can mimic the phenotype of several genetically determined

neuropathies. The prognosis in this disorder is also relatively good, although a

small number of children have significant neurological disability or treatment side-

effects. Other immune-mediated neuropathies are relatively infrequent in our

experience. When they occur, they are often associated with collagen-vascular

diseases or bone marrow transplantation. Peripheral neuropathy in association with

HIV infection in children appears to be rare.. 0; 53-03-2.

644. Small, K. W.; Pollock, S. C.; Vance, J. M.; Stajich, J. M.; Pericak Vance, M. Ocular

motility in North Carolina autosomal dominant ataxia. J-Neuroophthalmol. 1996

Jun; 16(2): 91-5; ISSN: 1070-8022.

UNITED-STATES. The term "vestibulocerebellar ataxia" has been applied to a

rare, autosomal dominant, late-onset disease with unusual ocular motility findings.

We examined the ocular motility of 18 family members from two different kindreds

and found 11 affected individuals. Both families in the present study, one of which

was originally described by Farmer and Mustian, as well as the family reported by

Farris et al., originated from Johnston County, North Carolina. We suspect that all

three of these families have a common ancestral origin. The age of onset of the

disorder was 31-60 years in the individuals examined. Ataxia, vertigo, diplopia,

oscillopsia, and tinnitus were common complaints. Although a variety of eye

movement abnormalities have previously been described in this disease, the most

prominent and consistent findings in our patients were (a) abnormal smooth

pursuits, (b) inability to suppress the vestibuloocular reflex (VOR), and (c) gaze-

evoked nystagmus. These findings suggest that the cerebellar flocculus may be the

primary site of pathology.

645. Smit, J. J.; Baas, F.; Hoogendijk, J. E.; Jansen, G. H.; van, der Valk MA; Schinkel,

A. H.; Berns, A. J.; Acton, D.; Nooter, K.; Burger, H.; Smith, S. J.; Borst, P.

Peripheral neuropathy in mice transgenic for a human MDR3 P-glycoprotein mini-

gene. J-Neurosci. 1996 Oct 15; 16(20): 6386-93; ISSN: 0270-6474.

UNITED-STATES. We have generated mice transgenic for a human MDR3 mini-

gene, under control of a hamster vimentin promoter. Expression of the MDR3

transgene was found in mesenchymal tissues, peripheral nerves, and the eye lens.

These MDR3 transgenic mice have a slowed motor nerve conduction and

dysmyelination of their peripheral nerves. An extensive dysmyelination in some

transgenic strains results in a severe peripheral neuropathy with paresis of the hind

legs. How expression of the MDR3 transgene causes these abnormalities is

unknown. The MDR3 gene encodes a large glycosylated plasma membrane protein

with multiple transmembrane spanning domains, which are involved in the

translocation of the phospholipid phosphatidylcholine through the hepatocyte

canalicular membrane. The ability of the MDR3 P-glycoprotein to alter

phsopholipid distribution in the plasma membrane of Schwann cells may cause the

damage. It is also possible, however, that the presence of a large glycoprotein in

the cell membrane may be sufficient to severely disturb myelination of peripheral

nerves.. 0.

646. Smith, G. D.; Mathias, C. J. Differences in cardiovascular responses to supine

exercise and to standing after exercise in two clinical subgroups of Shy-Drager

syndrome (multiple system atrophy). J-Neurol-Neurosurg-Psychiatry. 1996 Sep;

61(3): 297-303; ISSN: 0022-3050.

ENGLAND. BACKGROUND: In chronic autonomic failure of varying

aetiologies, there are differences in the cardiovascular responses to supine leg

exercise and to standing after exercise. Whether this occurs between the different

subgroups with Shy-Drager syndrome (SDS) is unknown. METHODS: Fourteen

patients with the cerebellar form (SDS-C) and 11 patients with parkinsonian

features (SDS-P) were studied. RESULTS: Both groups had a similar degree of

autonomic failure and postural hypotension. Their responses were compared with

nine patients with idiopathic Parkinson's disease (IPD) and 15 normal subjects

(controls), all with normal autonomic function. With supine exercise, blood

pressure and heart rate rose similarly in controls and patients with IPD and there

was no fall in blood pressure on standing after exercise. In both SDS groups there

were abnormal responses to exercise: blood pressure fell in SDS-C, but did not fall

or rise in SDS-P. Heart rate increased similarly in both SDS groups, calculated

systemic vascular resistance fell similarly, but cardiac index rose more in SDS-P

than SDS-C. Resting plasma noradrenaline concentrations were subnormal in both

forms of SDS, and did not increase with exercise. Postural hypotension was

enhanced after exercise to the same extent in SDS-C and SDS-P. CONCLUSIONS:

The greater cardiovascular abnormalities in response to exercise in SDS-C suggests

that cerebellar or brain stem autonomic pathways are impaired to a greater extent in

SDS-C than in SDS-P. Pooling SDS subgroups, therefore, may obscure

pathophysiological differences to certain stimuli. Clinically when postural

hypotension is being assessed, separation of the subgroups may not be essential, as

they responded similarly.

647. Snoek, G. J. [Autonomic dysreflexia: a life-threatening complication in patients with a

high-level spinal cord injury]. Autonome dysreflexie: een levensbedreigende

complicatie bij patienten met een hoge dwarslaesie. Ned-Tijdschr-Geneeskd. 1996

Aug 24; 140(34): 1729-32; ISSN: 0028-2162.

NETHERLANDS. A 37-year-old patient with a traumatic spinal cord injury at the

level CVI is described, suffering from a urinary tract infection which was

complicated by autonomic dysreflexia. Autonomic dysreflexia occurs only in

patients with a spinal cord injury at TVI or above. A variety of afferent stimuli

(mostly emerging from bladder or bowel) can induce an uncontrolled sympathetic

reaction of the spinal cord below the laesion level that is isolated from the

vasomotor centre. This reaction induces vasoconstriction below the laesion.

Compensating mechanisms are insufficient to compensate for the vasoconstriction

below the laesion. As a result there is a fast rise in blood pressure up to extreme

values with a serious risk of cardiac and cerebral complications. Knowledge of

these phenomena as well as of the preventive and therapeutic measures is essential

since the prevalence of spinal cord injury patients in the population is increasing.

648. Sommer, C.; Myers, R. R. Vascular pathology in CCI neuropathy: a quantitative

temporal study. Exp-Neurol. 1996 Sep; 141(1): 113-9; ISSN: 0014-4886.

UNITED-STATES. An experimental neuropathy in rats produced by tying loosely

constrictive ligatures around one sciatic nerve evokes pain-related behavior that

follows a reproducible time course. We have previously investigated the pathologic

changes in the sciatic nerve in this model, and a striking feature was protrusion and

proliferation of endoneurial endothelial cells. In the present study, we quantified

these changes at time points from 1 day to 12 weeks after surgery. One day after

surgery, vessels were distended, shown by an increase of the slope of a graph in

which the vessel areas were plotted on the x axis and luminal areas on the y axis.

From Day 7, the slope was reduced; i.e., vessel walls had thickened and lumina

were smaller. By Day 14, narrowing of lumina was observed in the contralateral

(control) nerve also. We suggest that endothelial cell proliferation, known to be

induced by cytokines produced by macrophages, indicates increased activity and

pathogenic significance of these cytokines in the CCI model.

649. Spickard, A. 3rd; Engel, J. Z. Low back pain with progressive weakness: a case of

cauda equina syndrome from lumbar disc herniation. Tenn-Med. 1996 Sep; 89(9):

338-9; ISSN: 1088-6222.

UNITED-STATES.

650. Spiegel, J. R.; Sataloff, R. T.; Hawkshaw, M. Vocal fold paralysis. Ear-Nose-Throat-

J. 1996 Feb; 75(2): 72; ISSN: 0145-5613.

UNITED-STATES.

651. Spillane, R. M.; Whitman, G. J.; Chew, F. S. Peroneal nerve ganglion cyst. AJR-Am-

J-Roentgenol. 1996 Mar; 166(3): 682; ISSN: 0361-803X.

UNITED-STATES.

652. Spinner, R. J.; Lins, R. E.; Spinner, M. Compression of the medial half of the deep

branch of the ulnar nerve by an anomalous origin of the flexor digiti minimi. A case

report. J-Bone-Joint-Surg-Am. 1996 Mar; 78(3): 427-30; ISSN: 0021-9355.

UNITED-STATES.

653. Spowart, K. M. Visual screening programme for preschool children [letter]. Br-J-

Ophthalmol. 1996 Jul; 80(7): 678; ISSN: 0007-1161.

ENGLAND.

654. Starr, A.; Picton, T. W.; Sininger, Y.; Hood, L. J.; Berlin, C. I. Auditory neuropathy.

Brain. 1996 Jun; 119( Pt 3): 741-53; ISSN: 0006-8950.

ENGLAND. Ten patients presented as children or young adults with hearing

impairments that, by behavioural and physiological testing, were compatible with a

disorder of the auditory portion of the VIII cranial nerve. Evidence of normal

cochlear outer hair cell function was provided by preservation of otoacoustic

emissions and cochlear microphonics in all of the patients. Auditory brainstem

potentials showed evidence of abnormal auditory pathway function beginning with

the VIII nerve: the potentials were absent in nine patients and severely distorted in

one patient. Auditory brainstem reflexes (middle ear muscles; crossed suppression

of otoacoustic emissions) were absent in all of the tested patients. Behavioural

audiometric testing showed a mild to moderate elevation of pure tone threshold in

nine patients. The extent of the hearing loss, if due to cochlear receptor damage,

should not have resulted in the loss of auditory brainstem potentials. The shape of

the pure tone loss varied, being predominantly low frequency in five patients, flat

across all frequencies in three patients and predominantly high frequency in two

patients. Speech intelligibility was tested in eight patients, and in six was affected

out of proportion to what would have been expected if the pure tone loss were of

cochlear origin. The patients were otherwise neurologically normal when the

hearing impairment was first manifest. Subsequently, eight of these patients

developed evidence for a peripheral neuropathy. The neuropathy was hereditary in

three and sporadic in five. We suggest that this type of hearing impairment is due to

a disorder of auditory nerve function and may have, as one of its causes, a

neuropathy of the auditory nerve, occurring either in isolation or as part of a

generalized neuropathic process.

655. Steel, D. H.; Harrad, R. A. Unilateral congenital ptosis with ipsilateral superior rectus

muscle overaction. Am-J-Ophthalmol. 1996 Oct; 122(4): 550-6; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: Congenital ptosis may be accompanied by

weakness of the ipsilateral superior rectus muscle. We report the finding of a

hypertropia of the ipsilateral eye in patients with isolated unilateral congenital ptosis

that became manifest only in upgaze. METHODS: Seventy consecutive patients

with congenital ptosis were recalled and 58 reexamined. Examination included

assessment of visual acuities, palpebral apertures, levator muscle function, and an

orthoptic examination. Particular attention was paid to the assessment of upgaze.

RESULTS: Thirty-eight patients had an isolated unilateral congenital ptosis. Four

patients had bilateral ptosis, six had upgaze deficits, and ten others had a variety of

other syndromes associated with ptosis. Seventeen of the 38 patients with isolated

unilateral congenital ptosis were found to have an ipsilateral hypertropia on upgaze.

The size of the vertical deviation varied from 5 to 30 prism diopters and, in the

more severe cases, produced a cosmetic problem that became more noticeable after

successful ptosis surgery. In one of these patients, a superior rectus muscle

posterior fixation suture was effective in reducing the hypertropia. CONCLUSION:

Of a number of possible causes for the ipsilateral hypertropia in upgaze in patients

with unilateral congenital ptosis that we observed either a misdirection syndrome

within the superior division of the oculomotor nerve or an exaggerated Bell's reflex

is the most likely.

656. Steffen, H.; Eifert, B.; Aschoff, A.; Kolling, G. H.; Volcker, H. E. The diagnostic

value of optic disc evaluation in acute elevated intracranial pressure.

Ophthalmology. 1996 Aug; 103(8): 1229-32; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: Ophthalmologists often are asked to evaluate the

optic disc for evidence of acute increased intracranial pressure (ICP). The authors

studied the incidence of papilledema in a population of patients with a documented

acute increase in ICP. METHODS: Included in this study were 37 patients with

acute elevated ICP due to a spontaneous hemorrhage or craniocerebral trauma. In

all patients, the ICP was monitored continuously. Fundus examination was

performed twice daily on at least 7 consecutive days. RESULTS: According to the

level and duration of the ICP, the patients were divided into three groups. Group 1

included 13 patients who had a slightly elevated ICP (range, 20-30 mmHg) on at

least 3 consecutive days. In this group, 3 of 13 patients demonstrated venous

congestion on the fifth or sixth day. No swelling of the optic disc was seen in this

group. Group 2 included seven patients with an elevated ICP, with values ranging

from 30 to 70 mmHg lasting for at least 3 consecutive days. In this group, one

patient had a blurred disc margin on the sixth day. Group 3 included 17 patients

with shortlasting ICP values, ranging from 30 to 60 mmHg for less than 72 hour.

Neither papilledema nor abnormalities of fundus vessels were seen in this group.

CONCLUSION: Papilledema in acute elevation of ICP is an uncommon event. Its

absence does not preclude the presence of ICP elevation.

657. Steiner, I.; Cohen, O. Peripartum Bell's palsy [letter]. Lancet. 1996 Apr 20;

347(9008): 1121-2; ISSN: 0140-6736.

ENGLAND.

658. Stoll, A. [A case from practice (345). 1. Suspicion of right-sided choroid melanoma--

right-sided ocular melanosis. 2. Suspicion of type II neurofibromatosis--status

following surgery of right-sided acoustic neurinoma in 1992]. Der Fall aus der

Praxis (345). 1. Verdacht auf Aderhautmelanom rechts--Melanosis culi rechts. 2.

Verdacht auf Neurofibromatosis Typ II--Status nach Akustikusneurinomoperation

rechts 1992. Schweiz-Rundsch-Med-Prax. 1996 Mar 26; 85(13): 420-1; ISSN:

0369-8394.

SWITZERLAND.

659. Stoll, G. Immunocytochemistry as a diagnostic tool. Baillieres-Clin-Neurol. 1996 Mar;

5(1): 129-42; ISSN: 0961-0421.

ENGLAND. ICC is a powerful technique to gain information about pathological

processes in peripheral nerve beyond the limits of classical histopathology. ICC can

aid in the identification of cellular infiltrates (T cells, B cells and macrophages), in

the definition of their state of activation (MHC class I and II molecule expression),

in the localization of cellular adhesion molecules (vascular and intercellular

adhesion molecule 1) involved in trafficking of inflammatory cells through the

endothelium and, finally, in the identification of locally produced cytokines

(interferon gamma, interleukins and tumour necrosis factor). Moreover, ICC can

identify pathological deposits, such as immunoglobulins and amyloid, within

nerves and can be used to study the expression of myelin sheath proteins as they

change in disease. In this chapter, basic immunocytochemical findings in

experimental diseases of the peripheral nervous system, such as WD and immune-

mediated demyelination, are summarized and discussed in the context of similar

observations in sural nerve biopsies.. 0; 0.

660. Stromland, K. Present state of the fetal alcohol syndrome. Acta-Ophthalmol-Scand-

Suppl. 1996; (219): 10-2; ISSN: 1395-3931.

DENMARK.

661. Studer, E. M.; Harms, M.; Masouye, I.; Peclard, N. [Nail changes within the scope of

reflex dystrophy]. Nagelveranderungen im Rahmen einer Reflexdystrophie.

Hautarzt. 1996 Mar; 47(3): 206-8; ISSN: 0017-8470.

GERMANY. We describe a 16-year-old boy with reflex sympathetic dystrophy

after a fracture of the right hand. He had inflammation of the proximal nail folds

and arrested nail growth of digits 3, 4 and 5 on the right hand. After 2 months

lymphatic drainage treatment, the changes of the nails had disappeared.

662. Suarez, G. A. [Diagnosis and treatment of the neurovegetative disorders: autonomic

neuropathies]. Diagnostico y tratamiento de los trastornos del sistema

neurovegetativo: neuropatias autonomicas. Rev-Neurol. 1996 Jun; 24(130): 671-5;

ISSN: 0210-0010.

SPAIN.

663. Suarez, G. A. [Peripheral neuropathies: diagnosis and treatment]. Neuropatias

perifericas: diagnostico y tratamiento. Rev-Neurol. 1996 Jun; 24(130): 676-80;

ISSN: 0210-0010.

SPAIN. 0; 0.

664. Suhr, O.; Danielsson, A.; Rydh, A.; Nyhlin, N.; Hietala, S. O.; Steen, L. Impact of

gastrointestinal dysfunction on survival after liver transplantation for familial

amyloidotic polyneuropathy. Dig-Dis-Sci. 1996 Oct; 41(10): 1909-14; ISSN:

0163-2116.

UNITED-STATES. Liver transplantation is the only effective treatment of familial

amyloidotic polyneuropathy type I (FAP). The aim of the present investigation was

to identify factors at the time of submission for transplantation that had impact on

survival, with special reference to gastrointestinal disturbances. All 28 liver-

transplanted FAP patients evaluated at Umea University Hospital were included in

the study. A modified body mass index was used to assess nutritional status.

Intestinal examinations were performed to diagnose bile acid malabsorption, gastric

retention, and bacterial contamination of the small bowel. A significantly improved

survival rate was found for patients in a good nutritional state (P = 0.002).

Peripheral neurological symptoms were unrelated to survival, whereas increased

mortality was found for patients with bile acid malabsorption (P < 0.05). Bacterial

contamination and gastric retention were common complications of the disease. In

conclusion, malabsorption and malnutrition have a profound impact on the outcome

of liver transplantation for familial amyloidotic polyneuropathy.

665. Summers, C. G.; Oetting, W. S.; King, R. A. Diagnosis of oculocutaneous albinism

with molecular analysis. Am-J-Ophthalmol. 1996 Jun; 121(6): 724-6; ISSN: 0002-

9394.

UNITED-STATES. PURPOSE: To use molecular analysis to diagnose

oculocutaneous albinism in a patient with an atypical clinical presentation.

METHODS: A 34-year-old woman with a history of strabismus and absent

cutaneous pigment underwent comprehensive ophthalmic examination, visual-

evoked potentials to detect altered optic decussation, and molecular analysis.

RESULTS: Examination showed fine nystagmus, iris transillumination, foveal

hypoplasia, and corrected visual acuity of 20/25 in each eye. Misrouting of the

retinostriate fibers was demonstrated with visual-evoked potentials. Mutations in

the tyrosinase gene established the diagnosis of oculocutaneous albinism 1 even

though the patient had atypical clinical features. CONCLUSIONS: Molecular

analysis can establish the diagnosis of oculocutaneous albinism 1 in the patient with

atypical ocular features.. EC 1.14.18.1.

666. Szirmai, A.; Ribari, O. [Etiology, investigation and therapy of facial nerve palsy]. A

nervus facialis periferias benulasanak okairol, vizsgalatarol es kezeleserol. Orv-

Hetil. 1996 Jul 14; 137(28): 1525-9; ISSN: 0030-6002.

HUNGARY. The authors give a description of the causes of the peripheral facial

palsy (traumas, viral and bacterial infections, tumors and palsies of unknown

origin). Emphasis is given to those anamnestic data, which are useful in the

differential diagnostic procedure. Diagnostic methods (modern otologic

investigation technics, imaging methods, electrodiagnostic tests), which may help

in the precise diagnosis, are discussed. Authors survey the guidelines in the

medical and surgical treatment, as well as the electrotherapy of the peripheral facial

palsy. The prognostic factors of the disease are also discussed. The author's

opinion, that the effectiveness of the treatment is good, and consequently to treat

the patients is necessary, and gives high success rate to the doctor.

667. Szoke, G.; Renyi Vamos, A.; Bider, M. A. Osteoarticular manifestations of congenital

insensitivity to pain with anhydrosis. Int-Orthop. 1996; 20(2): 107-10; ISSN:

0341-2695.

GERMANY. We report the case history of a boy who suffered from congenital

insensitivity to pain with anhydrosis. We discuss the orthopaedic disorders

occurring in 21 cases reported in the literature.

668. Taha, J. M.; Tew, JM Jr. Comparison of surgical treatments for trigeminal neuralgia:

reevaluation of radiofrequency rhizotomy. Neurosurgery. 1996 May; 38(5): 865-

71; ISSN: 0148-396X.

UNITED-STATES. In this study, we reevaluate the results of radiofrequency

rhizotomy and review the effectiveness of other surgical procedures for the

treatment of trigeminal neuralgia. Five hundred patients with trigeminal neuralgia

underwent radiofrequency rhizotomy at the University of Cincinnati Medical

Center, Cincinnati, OH, between 1981 and 1986. Their results are compared with

those of patients reported in the literature who underwent radiofrequency rhizotomy

(6205 patients), glycerol rhizotomy (1217 patients), balloon compression (759

patients), microvascular decompression (MVD) (1417 patients), and partial

trigeminal rhizotomy (250 patients). Comparisons were based on the following

outcome parameters: technical success, pain relief and recurrence, facial numbness,

dysesthesia, corneal anesthesia, keratitis, trigeminal motor dysfunction, permanent

cranial nerve deficit, intracranial hemorrhage or infarction, perioperative morbidity,

and perioperative mortality. We found that MVD had the lowest rate of technical

success. Radiofrequency rhizotomy and MVD had the highest rates of initial pain

relief and the lowest rates of pain recurrence. Glycerol rhizotomy had the highest

rate of pain recurrence. Balloon compression had the highest rate of trigeminal

motor dysfunction. Balloon compression and MVD had the lowest rates of corneal

anesthesia or keratitis. MVD had the lowest rates of facial numbness and

dysesthesia. All percutaneous procedures had similar rates of dysesthesia. Posterior

fossa exploration had the highest rates of permanent cranial nerve deficit,

intracranial hemorrhage or infarction, and perioperative morbidity and mortality. On

the basis of our experience and a review of the literature, we conclude the

following: 1) percutaneous techniques and posterior fossa exploration offer

advantages and disadvantages, 2) radiofrequency rhizotomy is the procedure of

choice for most patients undergoing first surgical treatments, and 3) MVD is

recommended for healthy patients who have isolated pain in the first ophthalmic

trigeminal division or in all three trigeminal divisions and patients who desire no

sensory deficit.

669. Takahashi, J.; Kitamura, K.; Miyata, M. Spontaneous nystagmus in normal subjects.

ORL-J-Otorhinolaryngol-Relat-Spec. 1996 Jan; 58(1): 42-5; ISSN: 0301-1569.

SWITZERLAND. We investigated the incidence of spontaneous nystagmus in 30

normal volunteers using electronystagmography (ENG). In all subjects, ENG was

performed with the eyes open in the primary position, with the eyes closed, and

with the eyes open in total darkness. Spontaneous nystagmus was detected in 5

(16.7%) of 30 subjects. In 15 subjects who underwent 2 ENG examinations, 4

subjects (26.7%) exhibited spontaneous nystagmus. Caloric testing was performed

in 26 of 30 subjects. Spontaneous nystagmus was detected in 3 subjects who had a

right-left difference in caloric response of over 40%, and in 2 of 23 subjects who

had a difference of less than 40%. Our results showed that spontaneous nystagmus

was detected in more subjects when the ENG examinations were repeated. Thus, a

single ENG examination cannot exclude the possibility of spontaneous nystagmus.

670. Takahashi, Y.; Abe, T.; Kojima, K.; Ochiai, S.; Miyagi, J.; Shigemori, M. Tolosa-

Hunt syndrome with atypical intrasellar and juxtasellar lesions--two case reports.

Kurume-Med-J. 1996; 43(2): 165-70; ISSN: 0023-5679.

JAPAN. Two patients with Tolosa-Hunt syndrome (THS) who had atypical

lesions in the intrasellar and juxtasellar regions are reported. They manifested with

painful ophthalmoplegia. Magnetic resonance imaging (MRI) commonly showed an

increase in the volume of the cavernous sinus occupied by homogeneously well-

enhanced lesions in both cases. These lesions extended to the intrasellar and

juxtasellar regions with meningela enhancement. Follow-up MRI after steroid

treatment demonstrated normalized or decreased size of the cavernous sinus. These

findings suggested nonspecific inflammatory granulomatosis with atypical

extension.

671. Takamatsu, I. [Bilateral vocal cord paralysis in children]. Nippon-Jibiinkoka-Gakkai-

Kaiho. 1996 Jan; 99(1): 91-102; ISSN: 0030-6622.

JAPAN. Eighteen infantile cases with bilateral vocal cord paralysis were treated at

our hospital from 1970 to 1993. All cases were diagnosed using a flexible

fiberscope to examine the larynx. Direct laryngoscopy was performed under

general anesthesia for the definite diagnosis and differential diagnosis from

laryngomalacia, subglottic stenosis, tracheal stenosis, or laryngeal web. Bilateral

vocal cord paralysis in children is a rare disease, there have been few and reported

cases. Eight cases were male and 10 cases were female. Thirteen cases were

congenital, 4 cases acquired and 1 case was unknown. The characteristic symptoms

of bilateral vocal cord paralysis include normal or near normal phonation with

inspiratory stridor which may progress to complete respiratory obstruction.

Associated anomalies and diseases included 3 cases of immature infant, 2 of

myelomeningocele, and single cases of Arnold-Chiari malformation, cerebral palsy,

hydrocephalus, laryngomalacia, William's syndrome, Wiedemann-Beckwith

syndrome, hypoxia, esophageal hiatus hernia, gastroesophageal reflex, spina

bifida, COFS syndrome, and cerebral atrophy. Laryngeal function was completely

recovered in seven cases following growth of the children incompletely recovered

in five cases, and 2 cases retained right vocal cord paralysis. Tracheostomy was

performed in 2 cases. One case died from the original disease, and the other one

case was unknown. Swallowing function, phonation and development were good.

Our experience suggests that the airway with bilateral vocal cord paralysis in

children can be managed well without the need for tracheostomy.

672. Takanashi, J.; Sugita, K.; Matsubayashi, J.; Sato, K.; Niimi, H. Availability of

frequency-selective fat-saturation pulse (Fat-Sat) MRI in childhood optic neuritis.

Pediatr-Neurol. 1996 Jan; 14(1): 64-5; ISSN: 0887-8994.

UNITED-STATES. A 2-year-old boy with acute optic neuritis, confirmed by

gadolinium-DTPA enhancement of the optic nerve using frequency-selective fat-

saturation pulse magnetic resonance imaging (Fat-Sat MRI), is reported. Because it

is difficult in very young children to sufficiently evaluate visual acuity, visual field,

and retroocular pain on eye movement, and visual evoked potential during

wakefulness, Fat-Sat MRI will be useful for revealing optic nerve inflammation and

for monitoring treatment.. 0; 0; 67-43-6; 80529-93-7.

673. Takeda, H.; Kumakawa, K. [Sound lateralization testing based on interaural intensity

difference discrimination and interaural time difference discrimination in acoustic

neurinoma patients--comparison of ABRS]. Nippon-Jibiinkoka-Gakkai-Kaiho.

1996 Jan; 99(1): 18-27; ISSN: 0030-6622.

JAPAN. Sound lateralization is considered useful in diagnosing retrocochlear

lesions. Interaural time difference (ITD) discrimination has been considered more

important than interaural intensity difference (IID) discrimination in diagnosing

retrocochlear lesions. The purpose of this study was to determine whether testing

sound lateralization by IID and ITD discrimination is useful in patients with

acoustic neurinomas (AN). Twenty patients (15 to 74 years old) with unilateral AN

were studied by testing IID and ITD discrimination using a self-recording device by

delivering 500 Hz band noise through head-phones. Auditory brainstem response

(ABR) testing and magnetic resonance imaging (MRI) were also performed in all

patients. IID discrimination abnormalities were found in 25% of all patients, and

ITD discrimination abnormalities in 85%. Five patients (25%) showed both IID and

ITD discrimination abnormalities. One of the three patients with both normal IID

and ITD discrimination showed waves I and V, and two of them showed waves I,

III and V. One of the five patients with both IID and ITD discrimination

abnormalities showed no response, two of them showed only wave I, the other two

showed waves I, III and V. Two patients with latency prolongation between waves

III and V had both IID and ITD discrimination abnormalities, and seven patients

with latency prolongation between waves I and III had normal ITD discrimination

or slightly abnormal ITD discrimination and normal IID discrimination. Three of

the four patients with large tumors (> 3 cm) had both IID and ITD discrimination

abnormalities, while two patients with small ear tumors (< 1 cm) had both normal

IID discrimination and normal ITD discrimination. Normal ITD discrimination or

slightly abnormal ITD discrimination was found in the majority of patients with

wave III. Impairment of ITD discrimination was well correlated with wave III

abnormalities. Patients with latency prolongation between waves III and V showed

a high degree of ITD discrimination abnormality. Impairment of IID discrimination

was related to a high degree of cochlear nerve damage or brainstem damage. We

emphasize that IID discrimination is important in the neurological diagnosis of AN,

in addition to ITD discrimination. Sound lateralization testing was a useful

screening test for diagnosing AN, because tumor size influenced lateralization in

every patient, and ITD discrimination was closely correlated with the ABR

findings.

674. Takizawa, S.; Shinohara, Y. Magnetic resonance imaging in Avellis' syndrome. J-

Neurol-Neurosurg-Psychiatry. 1996 Jul; 61(1): 17; ISSN: 0022-3050.

ENGLAND.

675. Takolander, R.; Rauwerda, J. A. The use of non-invasive vascular assessment in

diabetic patients with foot lesions. Diabet-Med. 1996; 13 Suppl 1: S39-42; ISSN:

0742-3071.

ENGLAND.

676. Tam, J. K.; Bradley, WG Jr; Goergen, S. K.; Chen, D. Y.; Pema, P. J.; Dubin, M.

D.; Teresi, L. M.; Jordan, J. E. Patterns of contrast enhancement in the pediatric

spine at MR imaging with single- and triple-dose gadolinium. Radiology. 1996 Jan;

198(1): 273-8; ISSN: 0033-8419.

UNITED-STATES. PURPOSE: To assess patterns of nerve root and spinal cord

contrast enhancement in the pediatric spine at magnetic resonance (MR) imaging

with single- and triple-dose gadolinium. MATERIALS AND METHODS: In three

control patients with no suspected pathologic spinal condition and 19 patients with

a suspected condition, spinal cords were evaluated prospectively for potential

spread of tumor to cerebrospinal fluid ("drop metastases") (n = 18) or Guillain-

Barre syndrome (n = 1). After enhancement with 0.1 mmol/kg gadolinium, patients

without definite drop metastases (n = 8) received a booster of 0.2 mmol/kg

gadolinium 30-40 minutes later; clinical follow-up was obtained 12 1/2 to 19

months later. RESULTS: Drop metastases appeared as nodular areas of

enhancement in 11 patients. Vascular enhancement related to the spinal cord surface

and emerging nerve roots was observed in images obtained in all control patients,

as well as in patients with negative findings at lumbar puncture and at clinical or

MR imaging follow-up examination (n = 6). Vascular and nerve root enhancement

increased with triple-dose gadolinium and was greater in patients after radiation

therapy (n = 17) than in control patients (n = 3). CONCLUSION: Use of triple-

dose gadolinium did not result in detection of additional cases of drop metastases..

0; 0; 0; 112188-16-6; 7440-54-2.

677. Tang, X. F.; Zhang, X. J. Guillain-Barre syndrome or "new" Chinese paralytic

syndrome in northern China? Electroencephalogr-Clin-Neurophysiol. 1996 Apr;

101(2): 105-9; ISSN: 0013-4694.

IRELAND. A serial study of clinical and magnetic stimulation motor evoked

potentials (MEP) was accomplished in 44 patients with the acute flaccid paralytic

syndrome which occurred in Northern China in 1991. Control data were provided

by 70 healthy subjects from the same area. The cases came from the same area

where a so-called new "Chinese paralytic syndrome" had been reported. We found

the clinical features of these 44 patients to be similar to those of classical Guillain-

Barre. Prolongation of MEP latency at 2 sites or on 2 occasions was found in 36

patients of whom 26 showed obvious clinical and electrophysiological recovery

within 4-8 weeks. Three cases showed reduced MEP amplitude with normal

latency, but in 2 of them the amplitude recovered in 2-8 weeks. Only 2 cases had

no response at all time. We think 41 patients (93.7%) had predominant nerve

demyelination. The 3 other patients (6.8%) showed axonal degeneration which is

within the range found in previous reports of classical Guillain-Barre. We conclude

that the acute paralytic syndrome seen in the summer of 1991 in Northern China

represents a classical Guillain-Barre syndrome with demyelination of motor and

sensory fibers. There is no reason to consider any special nomenclature such as

"Chinese paralytic syndrome" or "acute motor axonal neuropathy.".

678. Tani, Y.; Inoue, K.; Kasahara, H.; Nishioka, J.; Hukuda, S. Asymptomatic big toe

changes in diabetic patients with early Charcot knees--a report of 2 cases. Acta-

Orthop-Scand. 1996 Jun; 67(3): 302-4; ISSN: 0001-6470.

NORWAY.

679. Tate, B. J.; Kelly, J. W.; Rotstein, H. Scleredema of Buschke: a report of seven cases.

Australas-J-Dermatol. 1996 Aug; 37(3): 139-42; ISSN: 0004-8380.

AUSTRALIA. Scleredema of Buschke is an uncommon dermatosis characterized

by thickened, indurated skin, sometimes with erythema. Histopathology shows

thickened dermal collagen with a mild infiltrate of mucin in the deeper dermis.

Seven adults with scleredema are presented, four females and three males, and their

mean age at diagnosis was 54 years. All had diabetes mellitus for an average of 13

years prior to the onset of scleredema. Complications of diabetes, especially

retinopathy (n = 5), neuropathy (n = 4) and peripheral vascular disease (n = 3),

were present in five patients. One patient died (cause not established), and another

has life-threatening cardiomyopathy. We have no evidence that the scleredema was

a significant aetiologic factor in either case, despite published reports of fatalities

related to scleredema. Three patients were followed up for more than 1 year and,

irrespective of therapy, the scleredema did not resolve in any patient.. 0; 9007-34-

5.

680. Tekin, N.; Kural, N.; Kaya, T. Diagnostic value of renal arteriography in polyarteritis

nodosa. Turk-J-Pediatr. 1996 Jan; 38(1): 101-5; ISSN: 0041-4301.

TURKEY. Polyarteritis Nodosa (PAN) is a rare disease in childhood. No single

pattern of clinical presentation characterizes this disease, but abdominal pain,

central or peripheral nervous system disease, arthritis, myalgia and skin lesions

occur at some time during the course of the illness. In this case a 16-year-old boy

who presented with abdominal pain, elevated sedimentation rate associated with

hypertension, and a high level of renin, all of which were detected during his

hospitalization, suggested the diagnosis of PAN, and renal angiography was

performed. Characteristic renal aneurysms were visualized and the diagnosis was

confirmed.. EC 3.4.23.15.

681. Tenembaum, S. N.; Reisin, R. C.; Taratuto, A. L.; Fejerman, N. Spastic paraparesis

and sensory neuropathy. Muscle-Nerve. 1996 May; 19(5): 649-53; ISSN: 0148-

639X.

UNITED-STATES. A 12-year-old developed a slowly progressive spastic gait at

the age of 3. A marked loss of pain and temperature sensations led to a mutilating

acropathy starting at age 5. Electrodiagnostic studies revealed a symmetric, axonal,

predominantly sensory neuropathy, and magnetic resonance imaging ruled out

compression of spinal cord. Sural nerve biopsy disclosed a predominant

involvement of unmyelinated and a global loss of myelinated fibers, particularly

larger ones. Clinical, electrodiagnostic and pathological findings of this case most

likely represent an example of the "Cavanagh's variant", an unusual but distinct

entity within the hereditary sensory and autonomic neuropathies.

682. Terrono, A. L.; Millender, L. H. Management of work-related upper-extremity nerve

entrapments. Orthop-Clin-North-Am. 1996 Oct; 27(4): 783-93; ISSN: 0030-5898.

UNITED-STATES. Peripheral nerve symptoms are common in the worker. Great

care must be given to obtain an accurate diagnosis. Diagnostic labels should not be

used unless one is sure of the diagnosis. A detailed evaluation of the worker, job,

and medical and psychosocial conditions must be performed. Nonoperative

treatment is primary. Understanding at-risk patients and managing them carefully

can decrease disability and improve results following treatment. The results from

surgery are less successful in work-related disorders and disability is often

prolonged. Surgery should only be performed for clear diagnoses and clear

indications after adequate nonoperative treatment, with careful consideration of the

job to which the worker will return.

683. Testa, D.; Filippini, G.; Farinotti, M.; Palazzini, E.; Caraceni, T. Survival in multiple

system atrophy: a study of prognostic factors in 59 cases. J-Neurol. 1996 May;

243(5): 401-4; ISSN: 0340-5354.

GERMANY. The various clinical features of multiple system atrophy (MSA) make

the diagnosis of the disease difficult, especially in its early stages, when signs of

differentiated neuroanatomical system involvement have not yet appeared. Mortality

studies may be affected by the variability of the diagnostic criteria and selection

bias. We used strict clinical and MRI criteria to diagnose MSA in 59 patients.

Patients with parkinsonian and cerebellar onset were compared. Median survival

time from the onset of the first motor symptom was 7.5 years. Our results indicated

a trend (P = 0.09) for the Northwestern University Disability Scale score to

correlate with mortality, but we failed to find other characteristics identifying

subgroups or predictors for survival.

684. Theisen, S. K.; Podell, M.; Schneider, T.; Wilkie, D. A.; Fenner, W. R. A

retrospective study of cavernous sinus syndrome in 4 dogs and 8 cats. J-Vet-

Intern-Med. 1996 Mar; 10(2): 65-71; ISSN: 0891-6640.

UNITED-STATES. Cavernous sinus syndrome (CSS) is characterized by deficits

in more than one of the cranial nerves (CN) that traverse the cavernous sinus at the

base of the cranial vault: CN III (oculomotor), IV (trochlear), VI (abducens), and

the first two branches of CN V (trigeminal). Records from 4 dogs and 8 cats with

CSS diagnosed over a 14-year period were reviewed. The most common clinical

signs were ophthalmoparesis or ophthalmoplegia, mydriasis with no direct or

consensual pupillary light reflexes, ptosis, decreased corneal sensation, and

decreased retractor oculi reflex. All cats had initial signs referable to a left CSS

lesion (one had bilateral CSS), whereas in all dogs the lesions were localized to the

right cavernous sinus. Median ages at diagnosis were 9 and 10 years of age for

dogs and cats, respectively. Cerebel lomedullary cisternae cerebrospinal fluid

analysis in 6 animals was useful as a sensitivebut nonspecific diagnostic test of an

intracranial inflammatory or neoplastic lesion. Magnetic resonance imaging scans

provided a more definitive diagnostic test in all dogs, revealing a contrast-

enhancing mass on T1 weighted scans in the region of the cavernous sinus. A

definitive pathological diagnosis was obtained in 2 dogs: a primary intracranial

neoplasm and a metastatic intracranial neoplasm. A definitive diagnosis was

obtained in 6 cats: metastatic neoplasm (n = 1), primary intracranial neoplasm (n =

1), primary intracranial infectious disease (n = 2), and associated systemic

infectious disease (n = 2). The prognosis associated with CSS in dogs and cats was

considered guarded to poor.

685. Thimineur, M. A.; Saberski, L. Complex regional pain syndrome type I (RSD) or

peripheral mononeuropathy? A discussion of three cases. Clin-J-Pain. 1996 Jun;

12(2): 145-50; ISSN: 0749-8047.

UNITED-STATES. OBJECTIVE: Peripheral nerve pathology commonly results in

symptoms that suggest a diagnosis of complex regional pain syndrome (CRPS)

type I (RSD). We briefly review common symptoms of peripheral nerve pathology

(referred pain, hyperpathia, and autonomic changes) and present three illustrative

cases of peripheral nerve injury misdiagnosed and treated as RSD. The

nonspecificity of current taxonomy regarding CRPS as it relates to the three cases is

emphasized. DESIGN: The study is case series. SETTING: All three of the cases

were diagnosed and treated for their painful symptoms at a university hospital clinic

that provides multispecialty evaluations for painful conditions. PATIENTS: The

three patients all had work-related injuries resulting in pain, hyperpathia, and

autonomic changes in one of their upper extremities. Their injuries were

representative of common peripheral nerve lesions, one being a neuroma, one an

irritative lesion, and one an entrapment. RESULTS AND CONCLUSIONS: The

clinical entity of CRPS quite apparently encompasses symptomatology caused by

peripheral nerve entrapment, irritative lesions, and neuroma. As such, its use as a

diagnostic end point may overlook these treatable conditions. As illustrated in these

cases, peripheral nerve pathology may prove a diagnostic challenge and alternative

techniques of investigation other than electrophysiologic studies are often helpful.

686. Thimineur, M. A.; Saberski, L. Complex regional pain syndrome type I (RSD) or

peripheral mononeuropathy? A discussion of three cases. Clin-J-Pain. 1996 Jun;

12(2): 145-50; ISSN: 0749-8047.

UNITED-STATES. OBJECTIVE: Peripheral nerve pathology commonly results in

symptoms that suggest a diagnosis of complex regional pain syndrome (CRPS)

type I (RSD). We briefly review common symptoms of peripheral nerve pathology

(referred pain, hyperpathia, and autonomic changes) and present three illustrative

cases of peripheral nerve injury misdiagnosed and treated as RSD. The

nonspecificity of current taxonomy regarding CRPS as it relates to the three cases is

emphasized. DESIGN: The study is case series. SETTING: All three of the cases

were diagnosed and treated for their painful symptoms at a university hospital clinic

that provides multispecialty evaluations for painful conditions. PATIENTS: The

three patients all had work-related injuries resulting in pain, hyperpathia, and

autonomic changes in one of their upper extremities. Their injuries were

representative of common peripheral nerve lesions, one being a neuroma, one an

irritative lesion, and one an entrapment. RESULTS AND CONCLUSIONS: The

clinical entity of CRPS quite apparently encompasses symptomatology caused by

peripheral nerve entrapment, irritative lesions, and neuroma. As such, its use as a

diagnostic end point may overlook these treatable conditions. As illustrated in these

cases, peripheral nerve pathology may prove a diagnostic challenge and alternative

techniques of investigation other than electrophysiologic studies are often helpful.

687. Thomas, P. K. Undiagnosed neuropathies: the impact of ancillary investigations.

Baillieres-Clin-Neurol. 1996 Mar; 5(1): 157-70; ISSN: 0961-0421.

ENGLAND. Before the advances in knowledge about peripheral nerve disorders

that have accumulated over the past two to three decades, a high proportion of

chronic neuropathies seen in tertiary referral centres remained undiagnosed. With

intensive investigation, it is now possible to reduce this proportion to 13-14%. The

category for which an aetiological diagnosis is most elusive is that of chronic

axonal polyneuropathies, although some multifocal neuropathies resist explanation.

An aetiological diagnosis is achieved for most demyelinating neuropathies. Some

examples of chronic idiopathic axonal polyneuropathy (CIAP) may be of genetic

origin; others may have an autoimmune basis. Advances in the molecular genetics

and immunopathology of peripheral nerve disorders are likely to lead to the

elucidation of some of those neuropathies that currently have to be classified as

cryptogenic.

688. Thomas, P. K.; Claus, D.; Jaspert, A.; Workman, J. M.; King, R. H.; Larner, A. J.;

Anderson, M.; Emerson, J. A.; Ferguson, I. T. Focal upper limb demyelinating

neuropathy. Brain. 1996 Jun; 119( Pt 3): 765-74; ISSN: 0006-8950.

ENGLAND. Observations are presented on nine selected patients with chronic

upper limb demyelinating neuropathy to illustrate the range of manifestations that

may be observed. In three, the involvement was purely motor, in five, mixed motor

and sensory and, in one, virtually purely sensory; in seven the symptoms were

unilateral and in two bilateral. The presence of reduced nerve conduction velocity

and conduction block and the response to treatment in seven of the cases indicate

that they represented examples of chronic inflammatory demyelinating

polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve

biopsy in two cases. The presentation in one patient was accompanied by forearm

swelling initially suspected of being a tumour but shown to be due to muscle

hypertrophy. This was probably the consequence of recurrent muscle cramps and

fasciculation and possibly neuromyotonia. The patient with predominant sensory

involvement restricted to the upper limbs demonstrates that sensory CIDP can

present focally. In one patient with monomelic motor and sensory involvement,

nerve biopsy showed multifocal areas of hypertrophic demyelinating neuropathy

distally in the ulnar nerve without inflammatory infiltration. This patient failed to

respond to therapy. Response in the others was satisfactory, although one patient

with a monomelic motor neuropathy showed a severe deterioration after being

given corticosteroids; he subsequently improved with intravenous human

immunoglobulin therapy.. 37758-47-7.

689. Tick paralysis--Washington, 1995. MMWR-Morb-Mortal-Wkly-Rep. 1996 Apr 26;

45(16): 325-6; ISSN: 0149-2195.

UNITED-STATES. Tick paralysis (tick toxicosis)--one of the eight most common

tickborne diseases in the United States (1)--is an acute, ascending, flaccid motor

paralysis that can be confused with Guillain-Barre syndrome, botulism, and

myasthenia gravis. This report summarizes the results of the investigation of a case

of tick paralysis in Washington.

690. Tick paralysis--Washington, 1995. From the Centers for Disease Control and

Prevention. JAMA. 1996 May 15; 275(19): 1470; ISSN: 0098-7484.

UNITED-STATES.

691. Tiffin, P. A.; MacEwen, C. J.; Craig, E. A.; Clayton, G. Acquired palsy of the

oculomotor, trochlear and abducens nerves. Eye. 1996; 10( Pt 3): 377-84; ISSN:

0950-222X.

ENGLAND. There have been few studies primarily concerned with the relative

frequencies, aetiologies and prognoses of ocular motor palsies. Those published

have emanated largely from neurological tertiary referral centres rather than primary

ophthalmology departments. We have performed a retrospective study of all

patients with acquired III, IV or VI cranial nerve palsy who were seen in the

orthoptic department at Ninewells Hospital, Dundee, over the 9 year period from

1984 to 1992. A total of 165 cases were identified. VI nerve palsies accounted for

the majority of patients (57%), with IV nerve palsies (21%) occurring more

frequently than III nerve palsies (17%) and multiple palsies (5%). Thirty-five per

cent of cases were of unknown aetiology and 32% of vascular aetiology. The

incidence of sinister pathology-neoplasia (2%) and aneurysm (1%)-was

surprisingly low. Fifty-seven per cent of all patients made a total recovery (in a

median time of 3 months) and 80% made at least a partial recovery. The results are

contrasted with those of previous studies and the value of associated symptoms and

of further investigation in the assessment of these patients is discussed.

692. Timmerman, V.; Lofgren, A.; Le Guern, E.; Liang, P.; De Jonghe, P.; Martin, J. J.;

Verhalle, D.; Robberecht, W.; Gouider, R.; Brice, A.; Van Broeckhoven, C.

Molecular genetic analysis of the 17p11.2 region in patients with hereditary

neuropathy with liability to pressure palsies (HNPP). Hum-Genet. 1996 Jan;

97(1): 26-34; ISSN: 0340-6717.

GERMANY. Hereditary neuropathy with liability to pressure palsies (HNPP) is in

most cases associated with an interstitial deletion of the same 1.5-Mb region at

17p11.2 that is duplicated in Charcot-Marie-Tooth type 1A (CMT1A) patients.

Unequal crossing-over following misalignment at flanking repeat sequences

(CMT1A-REP), either leads to tandem duplication in CMT1A patients or deletion in

HNPP patients. With the use of polymorphic DNA markers located within the

CMT1A/HNPP duplication/deletion region we detected the HNPP deletion in 16

unrelated HNPP patients, 11 of Belgian and 5 of French origin. In all cases, the

1.5-Mb size of the HNPP deletion was confirmed by EcoRI dosage analysis using

a CMT1A-REP probe. In the 16 HNPP patients, the same 370/320-kb EagI

deletion-junction fragments were detected with pulsed field gel electrophoresis

(PFGE), while in CMT1A patients, a 150-kb EagI duplication-junction fragment

was seen. Thus, PFGE analysis of EagI-digested DNA with a CMT1A-REP probe

allows direct detection of the HNPP deletion or the CMT1A duplication for DNA

diagnostic purposes.. 9007-49-2.

693. Tomono, Y.; Takeuchi, S.; Nose, T. Acoustic neurinoma with a large organized

hematoma--case report. Neurol-Med-Chir-Tokyo. 1996 Jun; 36(6): 380-3; ISSN:

0470-8105.

JAPAN. A 66-year-old female presented with a large organized hematoma within

an acoustic neurinoma. She had suffered from diminished hearing for 20 years and

had headache 1 week before presentation. Computed tomography demonstrated an

inhomogeneously high density cerebellopontine angle mass, and magnetic

resonance imaging showed a mass with heterogeneous intensity and gadolinium-

diethylenetriaminepenta-acetic acid enhancement of only the peripheral surface of

the mass and the inner parts of the internal auditory meatus. At operation the

majority of the mass was soft and feature-less with a firm capsule, and a yellowish

soft tumor was removed from the perimeatal area. Histological examination showed

the mass was an acoustic neurinoma with a large organized hematoma. Extensive

hemorrhage from an abnormal vascularity in the tumor had repeated followed by

granulomatous organization.

694. Toyry, J. P.; Niskanen, L. K.; Lansimies, E. A.; Partanen, K. P.; Uusitupa, M. I.

Autonomic neuropathy predicts the development of stroke in patients with non-

insulin-dependent diabetes mellitus. Stroke. 1996 Aug; 27(8): 1316-8; ISSN:

0039-2499.

UNITED-STATES. BACKGROUND AND PURPOSE: Our aim was to

determine the predictive factors for stroke in patients with non-insulin-dependent

diabetes mellitus (NIDDM). METHODS: We studied 133 patients with NIDDM at

the time of diagnosis and 5 and 10 years later. RESULTS: The number of new fatal

or nonfatal strokes was 19 (14.7%; 14 after 5-year examination). High initial

fasting blood glucose (odds ratio [OR], 1.2; 95% confidence interval [CI], 1.04 to

1.4) and the use of beta-blocking agents (OR, 6.7; 95% CI, 2.1 to 21.5) at baseline

and the presence of parasympathetic neuropathy (OR, 6.7; 95% CI, 1.5 to 29.9),

or sympathetic autonomic nervous dysfunction (OR, 1.1; 95% CI, 1.01 to 1.2),

hypertriglyceridemia (OR, 5.7; 95% CI, 1.1 to 31.0), or use of beta-blocking

agents (OR, 6.4; 95% CI, 1.3 to 31.2), and high fasting plasma glucose (OR, 1.2;

95% CI, 1.0 to 1.5) determined at 5-year examination predicted the development of

stroke. CONCLUSIONS: Autonomic neuropathy is an independent risk factor for

stroke in NIDDM.. 0; 0; 57-88-5.

695. Toyry, J. P.; Niskanen, L. K.; Mantysaari, M. J.; Lansimies, E. A.; Uusitupa, M. I.

Occurrence, predictors, and clinical significance of autonomic neuropathy in

NIDDM. Ten-year follow-up from the diagnosis. Diabetes. 1996 Mar; 45(3): 308-

15; ISSN: 0012-1797.

UNITED-STATES. Little is known about the occurrence and predictive factors of

autonomic neuropathy and its relationship to cardiovascular mortality in NIDDM

patients, and no long-term follow-up studies including nondiabetic control subjects

are available. A total of 133 patients with newly diagnosed NIDDM (70 men) and

144 control subjects (62 men) were examined at baseline and after 5 and 10 years

of follow-up. Deep-breathing tests (baseline, 5-year, and 10-year) and active

orthostatic tests (5- and 10-year) were performed. Criteria for autonomic

neuropathy were parasympathetic (expiration-to-inspiration ratio </- 1.10),

sympathetic (systolic blood pressure decrease >/- 30 mmHg in the orthostatic test),

and combined autonomic neuropathy (parasympathetic with sympathetic

neuropathy). The frequency of parasympathetic neuropathy (NIDDM patients

versus control subjects) was 4.9 vs. 2.2% (P = 0.224) at baseline, 19.6 vs. 8.5%

(P = 0.017) at 5 years, and 65.0 vs. 28.0% (P < 0.001) at 10 years of follow-up.

The frequency of sympathetic neuropathy was 6.8 vs. 5.6% (P = 0.709) at 5 years

and 24.4 vs. 9.0% (P = 0.003) at 10 years of follow- up. These figures for

combined autonomic neuropathy were 2.1 vs. 1.8% (P = 0.869) at 5 years and

15.2 vs. 4.2% (P = 0.007) at 10 years of follow-up. NIDDM patients with

parasympathetic neuropathy at the 10-year examination showed worse glycemic

control and higher insulin values than those without parasympathetic neuropathy.

Furthermore, in our subjects, women were more prone to have parasympathetic

neuropathy than men. Parasympathetic neuropathy at baseline was more frequent in

those who died from a cardiovascular cause than those who did not (13 vs. 3%, P

= 0.045). Similarly, sympathetic autonomic nervous dysfunction at the 5-year

examination predicted the 10-year cardiovascular mortality. In conclusion, the

frequency of autonomic neuropathy in NIDDM patients increases sharply with

time. The development of autonomic neuropathy is connected with poor glycemic

control. Interestingly, a high insulin level seems to have a predictive role in the

development of parasympathetic autonomic neuropathy irrespective of obesity and

glycemia.. 0; 11061-68-0.

696. Trobe, J. D.; Beck, R. W.; Moke, P. S.; Cleary, P. A. Contrast sensitivity and other

vision tests in the optic neuritis treatment trial. Am-J-Ophthalmol. 1996 May;

121(5): 547-53; ISSN: 0002-9394.

UNITED-STATES. PURPOSE: To determine the intercorrelation, prevalence of

abnormality, and incremental detection value of vision tests in optic neuritis.

METHODS: We calculated the linear correlation of paired vision tests and

prevalence of abnormal test values from baseline and six-month measurements of

Snellen visual acuity, Pelli-Robson contrast sensitivity, Humphrey Field Analyzer

mean deviation, and Farnsworth-Munsell 100-hue color vision in 438 patients

entered in the Optic Neuritis Treatment Trial from 1988 to 1991. The incremental

detection value of nonvisual acuity tests was defined as their frequency of

abnormality when visual acuity was 20/20 or better. RESULTS: All four vision-test

results were highly intercorrelated at baseline and at six months. At baseline,

contrast sensitivity had the highest prevalence of abnormality, but all vision tests

were so often abnormal that differences were not clinically relevant. At six months,

when visual recovery had occurred, contrast sensitivity was most often abnormal

(2.2 X visual acuity; 1.8 X mean deviation; 1.5 X Farnsworth-Munsell 100-hue

color vision test); when contrast sensitivity, mean deviation, or Farnsworth-

Munsell 100-hue color vision was normal, visual acuity was 20/25 or better in 98%

of patients. CONCLUSIONS: The high intercorrelation of four vision tests

suggests that optic neuritis affects a broad range of visual functions. Among non-

visual acuity tests, Pelli-Robson contrast sensitivity proved to be a particularly

practical and sensitive indicator of visual dysfunction in optic neuritis.. 0.

697. Troost, B. T. Ophthalmoplegic migraine. Biomed-Pharmacother. 1996; 50(2): 49-51;

ISSN: 0753-3322.

FRANCE. Ophthalmoplegic migraine is a rare condition usually beginning in

infancy or childhood and characterized by paralysis of ocular cranial nerves in

association with vascular headache. The diagnosis is one of exclusion. Most

patients should have high resolution magnetic resonance imaging (MRI) or

magnetic resonance angiography (MRA) to eliminate the possibility of aneurysm,

tumor, or granulomatous inflammation.

698. Truffert, A.; Dumas, J. J.; Dandelot, J. B. [Interhemispheric disconnection, Balint's

syndrome and persistent anarthria: Marchifava-Bignami disease with white matter

hemorrhage]. Dysconnexion interhemispherique, syndrome de Balint et troubles

arthriques persistants: maladie de Marchiafava-Bignami avec hemorrhagie de la

substance blanche. Rev-Neurol-Paris. 1996 Mar; 152(3): 174-80; ISSN: 0035-

3787.

FRANCE. A 37-year-old alcoholic right-handed man developed a complex

neuropsychological picture following a mild head injury and a severe confusional

state. Prominent features were Balint's syndrome, signs of interhemispheric

deconnection, and speech disorders with anarthria and dysprosody. Iterative CT

scans showed pathognomonic hypodensities of the genu and splenium of corpus

callosum, confirmative of Machiafava-Bignami disease. After a two years follow-

up, a favourable outcome was observed despite haemmoragic transformation of

bilateral necrotic lesions of the parietal white matter, an exceptional

neuropathological fact. This case is demonstrative of the possibility of articulate

speech impairment when lesions of both corpus callosum and subcortical white

matter are present. It also raises several aetiopathogenic problems which are

discussed.

699. Turgut, M.; Benli, K.; Ozgen, T.; Saglam, S.; Bertan, V.; Erbengi, A. Twenty-five

years experience in the treatment of trigeminal neuralgia. Comparison of three

different operative procedures in forty-nine patients. J-Craniomaxillofac-Surg.

1996 Feb; 24(1): 40-5; ISSN: 1010-5182.

SCOTLAND. A series of 49 patients with trigeminal neuralgia (TN) were treated

by three different surgical procedures: (1) peripheral ablative procedures in 10

patients; (2) percutaneous rhizotomy in 17 patients and (3) intracranial rhizotomy

(IR) in 22 patients. On the basis of surgical treatment, the concept that

neurovascular compression is a mechanical factor in the aetiology of TN was

supported in 14 to 18 patients who underwent posterior fossa exploration. The

results support the conclusion that retromastoid craniectomy with IR is the

procedure of choice for the majority of patients with TN.

700. Turp, J. C.; Gobetti, J. P. Trigeminal neuralgia versus atypical facial pain. A review of

the literature and case report. Oral-Surg-Oral-Med-Oral-Pathol-Oral-Radiol-Endod.

1996 Apr; 81(4): 424-32; ISSN: 1079-2104.

UNITED-STATES. Trigeminal neuralgia and atypical facial pain are common

conditions of facial pain. Although these two pain conditions are classically well

separated in textbooks, a straightforward diagnosis may not always be possible

because of the overlapping clinical signs and symptoms. In this article, a

comparison and differentiation between the clinical and diagnostic features of these

two pain conditions are presented. The general characteristics, etiologic

characteristics, pathophysiology, differential diagnostic criteria, and therapeutic

options of trigeminal neuralgia and atypical facial pain are described. A case report

demonstrates the difficulties that can arise in the diagnosis and differentiation

between the two disease entities. The article underscores the responsibility

clinicians have in correctly diagnosing and managing patients with facial pain

conditions.. 0; 298-46-4.

701. Tuulonen, A.; Jonas, J. B.; Valimaki, S.; Alanko, H. I.; Airaksinen, P. J.

Interobserver variation in the measurements of peripapillary atrophy in glaucoma.

Ophthalmology. 1996 Mar; 103(3): 535-41; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: The purpose of this retrospective study is to

compare the measurements of intrapapillary and peripapillary parameters between

two observers and test the usefulness of measuring different types of crescents.

METHODS: Optic disc photographs of 23 eyes of 23 patients with glaucoma and

23 age-matched normal eyes were measured in Oulu and in Erlangen using manual

planimetric techniques. The authors measured the following magnification corrected

intrapapillary and peripapillary areas: optic disc, neuroretinal rim, cup: disc area

ratio, scleral ring, central (zone beta), and peripheral peripapillary atrophy (zone

alpha). Twenty-one patients with glaucoma had a follow-up of 3.2 years (range,

1.1-4.7 years), and follow-up for 19 control eyes was 3.7 years (range, 2.5-5.9

years). The measurements were performed in a masked fashion for the diagnosis

and temporal sequence of the photographs. RESULTS: Central peripapillary

atrophy (zone beta) was statistically significantly largest in primary open-angle

glaucoma in both centers (Oulu, P=0.003; Erlangen, P=0.004), whereas normal

and exfoliative eyes did not differ significantly from each other. The results for

peripheral peripapillary atrophy (zone alpha) and scleral ring were less consistent.

Despite statistically significant interobserver correlations ranging from r=0.30

(scleral ring area; P=0.0472) to r=0.97 (optic disc area; P=0.0001), the means of

all parameters, except for zone alpha and beta, differed statistically significantly

between the two observers. CONCLUSIONS: The central peripapillary atrophy, or

zone beta, is the most reproducible parameter when measuring peripapillary atrophy

in glaucoma. Nonetheless, its measurement is of limited usefulness in the

recognition of glaucoma or progression of glaucomatous nerve damage.

702. Tychsen, L.; Boothe, R. G. Latent fixation nystagmus and nasotemporal asymmetries

of motion visually evoked potentials in naturally strabismic primate. J-Pediatr-

Ophthalmol-Strabismus. 1996 May; 33(3): 148-52; ISSN: 0191-3913.

UNITED-STATES. PURPOSE: To determine whether macaque monkeys who

had onset of natural, alternating esotropia in early infancy have the eye movement

abnormalities and motion visually evoked potential (VEP) abnormalities observed

in human infantile esotropes. METHODS: Recordings were obtained of horizontal

pursuit eye movements and fixation nystagmus under conditions of monocular

viewing. Motion VEPs in response to horizontal motion were recorded with the

animals sedated to reduce the possibility of eye movement artifact. RESULTS: The

strabismic monkeys had a naso-temporal asymmetry of horizontal pursuit favoring

nasally directed motion when viewing with either eye. When fixating stationary

targets, latent nystagmus was apparent; the eyes drifted conjugately and the slow

phase of the nystagmus was always nasally directed with respect to the fixating

eye. Motion VEPs were characterized by a strong horizontal asymmetry with the

directional bias inverted approximately 180 deg in the two eyes. These eye

movement and motion VEP asymmetries were not observed in a normal macaque.

CONCLUSIONS: Macaque monkeys who have infantile esotropia possess

asymmetries of horizontal pursuit and motion VEPs like those documented in

strabismic humans. Macaques with infantile esotropia appear to be an appropriate

animal model for study of neural mechanisms in strabismus.

703. Ubaidullaev, A. M.; Gafurov, B. G.; Kaiumkhodzhaeva, M. A. [Psychoautonomic

disorders in bronchial asthma patients]. Psikhovegetativnye narusheniia u

bol'nykh bronkhial'noi astmoi. Ter-Arkh. 1996; 68(3): 44-7; ISSN: 0040-3660.

RUSSIA. The results of psychological and vegetative investigations in 142

patients with bronchial asthma (BA) demonstrated that BA gives rise to

psychovegetative syndrome resultant from parasympathetic orientation of the

vegetative tone and reactivity, weak vegetative support of muscular and mental

activity. These vegetative shifts occur in line with enhanced ascending nonspecific

activation and anxious-hypochondriac traits of personality.

704. Uberall, M. A.; Renner, C.; Edl, S.; Parzinger, E.; Wenzel, D. VEP and ERP

abnormalities in children and adolescents with prepubertal onset of insulin-

dependent diabetes mellitus. Neuropediatrics. 1996 Apr; 27(2): 88-93; ISSN:

0174-304X.

GERMANY. Visual evoked sensory (VEP) and event-related potentials (ERP)

were assessed in 29 adolescents with insulin-dependent diabetes mellitus (IDDM)

and in 29 controls matched for age and gender. Data were compared with clinical

and psychometric measures, age at onset, duration of disease, and metabolic

control. Analysis revealed no latency differences for the first cortical VEP

component (P50) but a steadily increasing latency delay for subsequent VEP (N80,

P100, N150, P200) and ERP components (P300) in the IDDM group compared to

healthy controls. IDDM subjects showed highly significant latency prolongations (p

< 0.001) for P100, N150 and P200 and P300 compared with healthy controls. A

pathological VEP/ERP latency delay of more than 3 SD above the reference value

range was observed in 21 IDDM patients (72.4%). Psychometric outcome

measures in IDDM subjects showed no significant performance deficits on the

Raven SPMs relative to non-diabetic controls. In contrast to VEP and ERP

anomalies, which were highly interrelated, there was no tendency for

neurophysiological and psychometric abnormalities to be contemporarily present.

Neither electrophysiological nor psychometric measures were correlated with age at

onset, IDDM duration, quality of metabolic control, or the presence of peripheral

neuropathy. These findings give evidence that 1) higher cognitive functions are

frequently affected in adolescents even with prepubertal IDDM onset, 2)

neurophysiological ERP analysis seems to detect minor neurocognitive restrictions,

presently not affecting psychometric outcome, 3) altered neurophysiological

parameters were present in more than 70% of IDDM subjects studied, and 4)

functional CNS disturbances affecting neurocognition are apparently not correlated

with metabolic parameters previously thought to be important predictors of CNS

outcome, suggesting the presence of multifactorial influences affecting

neurocognition in IDDM subjects.

705. Uchida, K.; Baba, H.; Maezawa, Y.; Chen, Q.; Imura, S. Lumbosacral dumb-bell

neurilemmoma approached by microsurgical interlaminar foraminotomy. A case

report. Int-Orthop. 1996; 20(2): 119-22; ISSN: 0341-2695.

GERMANY. The case history of a patient with a dumb-bell neurilemmoma arising

from the L5 nerve root is described. The tumour extended into the outlet of the

neural foramen at L5-S1 on the left and was also compressing the dural sac and the

S1 nerve root. A limited laminotomy at L4-5 and L5-S1, with preservation of the

neural arch, was followed by microsurgical medial foraminotomy at L5-S1 which

allowed visualisation of the nerve root and enucleation of the tumour. The

technique maintains stability of the posterior elements and spinal movement, and is

recommended for the removal of this type of tumour.

706. Uchuya, M.; Graus, F.; Vega, F.; Rene, R.; Delattre, J. Y. Intravenous

immunoglobulin treatment in paraneoplastic neurological syndromes with

antineuronal autoantibodies. J-Neurol-Neurosurg-Psychiatry. 1996 Apr; 60(4):

388-92; ISSN: 0022-3050.

ENGLAND. OBJECTIVE: To evaluate the effect of intravenous high dose human

immunoglobulin (IVIg) therapy on the clinical course and autoantibody titres of

patients with neurological paraneoplastic syndromes. METHODS: Twenty two

patients with paraneoplastic encephalomyelitis and sensory neuronopathy syndrome

associated with anti-Hu antibodies (18) or paraneoplastic cerebellar degeneration

(PCD) with anti-Yo antibodies (four), were treated with 1-26 (mean 5.8) cycles of

IVIg. The Rankin scale was used to evaluate the response. RESULTS: The only

serious toxicity was one case of haemolytic anaemia. Twenty one patients were

evaluable for therapeutic response. One patient, with subacute sensory

neuronopathy (SSN), improved for at least 15 months, 10 remained stable (eight

with anti-Hu and two with anti-Yo antibodies), and 10 deteriorated (eight with anti-

Hu and two with anti-Yo antibodies). In seven of the 10 patients who stabilised,

the syndrome had already made a plateau when the treatment was started but three

patients (one with anti-Hu and two with anti-Yo antibodies) who had still been

progressing stabilised for six, eight, and more than 48 months, including one

patient with SSN who achieved stabilisation when the neurological dysfunction

was only moderate (Rankin scale = 3). Another patient with SSN and initial stable

response worsened when IVIg was reduced and improved when it was increased.

No significant predictive factors of outcome could be identified but improvement or

stabilisation was more frequent in patients with isolated involvement of the

peripheral nervous system (62%) than in patients with evidence of CNS damage

(37%) at the onset of treatment. Stabilisation in patients with CNS involvement was

only achieved when the neurological dysfunction was already severe (Rankin scale

> 3). The titres of autoantibodies did not change significantly. CONCLUSION:

Treatment with IVIg at the doses given in the present protocol was not effective in

paraneoplastic CNS syndromes associated with antineuronal antibodies. The role of

this regime in the treatment of SSN should be further evaluated.. 0; 0; 0; 0; 0; 0; 0;

138545-05-8.

707. Uncini, A.; Sabatelli, M.; Mignogna, T.; Lugaresi, A.; Liguori, R.; Montagna, P.

Chronic progressive steroid responsive axonal polyneuropathy: a CIDP vaariant or

a primary axonal disorder? Muscle-Nerve. 1996 Mar; 19(3): 365-71; ISSN: 0148-

639X.

UNITED-STATES. Five patients presented with chronic,, progressive,

predominantly motor polyneuropathy. CSF protein content was increased in 4

patients. Motor conduction velocities and EMG were consistent with axonal

involvement. Sural nerve conductions were normal in all cases and sural nerve

biopsy performed in 1 patient was normal. Serum antibodies to GM1, GD1a,

GD1b, and GM2 were negative. All patients improved after steroid treatment and 3

completely recovered. Because of therapeutic implications it is important to

differentiate these patients from those with chronic idiopathic axonal neuropathies.

It is unclear whether this is a primary axonal, probably immune-mediated,

polyneuropathy, or whether it represents one extreme of the chronic inflammatory

demyelinating polyradiculoneuropathy spectrum characterized by severe axonal

loss. We suggest that the term "chronic inflammatory polyneuropathy,"

encompassing cases from pure demyelinating to pure axonal neuropathies

responsive to steroids, should be reinstated and that, like in Guillain-Barre

syndrome, different subtypes should be individuated.. 0; 0; 53-03-2.

708. Urbaniak, J. R.; Desai, S. S. Complications of nonoperative and operative treatment of

carpal tunnel syndrome. Hand-Clin. 1996 May; 12(2): 325-35; ISSN: 0749-0712.

UNITED-STATES. The popularity of treatment of carpal tunnel syndrome has

resulted in a significant number of complications. A rational plan of therapy is

available for each category of complications of carpal tunnel syndrome. This article

presents 14 of the most frequently encountered complications of treatment.

Prevention and management of these complications are discussed.. 0.

709. Usai, P.; Usai Satta, P.; Savarino, V.; Boy, M. F. Autonomic neuropathy in adult

celiac disease [letter]. Am-J-Gastroenterol. 1996 Aug; 91(8): 1676-7; ISSN: 0002-

9270.

UNITED-STATES.

710. Ushiyama, M.; Ikeda, S.; Suzuki, T.; Yazawa, M.; Yanagisawa, N.; Tsujino, S. Acute

pandysautonomia: mass spectrometric and histopathological studies of the

sympathetic nervous system during long term L-threo-3,4-dihydroxyphenylserine

treatment. J-Neurol-Neurosurg-Psychiatry. 1996 Jul; 61(1): 99-102; ISSN: 0022-

3050.

ENGLAND. Stable isotope labelled L-threo-3,4-dihydroxyphenylserine (L-DOPS)

infusion tests and histopathological studies of the rectal autonomic nerves were

performed in a patient with acute pandysautonomia. A pronounced increase in

blood pressure occurred and stable isotope labelled noradrenaline appeared in the

plasma during L-DOPS infusion in the acute stage, but decreased during the next

three years. Noradrenergic nerve fibres in the rectal mucosa showed no recovery,

and so clinical improvement had occurred without apparent significant regeneration

of the peripheral autonomic nerves.. 23651-95-8; 51-41-2.

711. Vaeckenstedt, J.; Friederich, N. F. [Shoulder problems in leisure athletes: physical

examination, diagnosis and therapy. Sensible procedures in daily practice].

Schulterprobleme beim Hobbysportler: Untersuchung, Diagnose und Therapie.

Sinnvolles Vorgehen in der taglichen Praxis. Schweiz-Med-Wochenschr. 1996 Mar

16; 126(11): 447-56; ISSN: 0036-7672.

SWITZERLAND. At first sight the anatomy of the shoulder may seem simple (see

Fig. 1). However, for the physician treating a patient suffering from shoulder pain,

the scapulo-thoracal interplay of 5 joints and 19 muscles, providing a wide and

varied range of motion, may constitute an obstacle difficult to overcome. In the

leisure athlete acute injuries must be distinguished from degenerative disease.

Contact sports in particular tend to involve risks of falling on the shoulder, injuring

the shoulder girdle or the elbow and wrist, sometimes with major consequences:

complex fractures, dislocations, ligament and tendon lesions or joint instabilities.

Thorough, rapid and cost-effective diagnostic evaluation of the athlete, involving

clinical examination (function tests), radiographic imaging (shoulder a.p., y-view)

and in selected cases ultrasonography (compared with the other side) may be

necessary in starting early and effective therapy.

712. Vallat, J. M. Electron microscopy as a tool to diagnose neuropathies. Baillieres-Clin-

Neurol. 1996 Mar; 5(1): 143-56; ISSN: 0961-0421.

ENGLAND. Ultrastructural examination of a peripheral nerve biopsy may be

particularly useful and sometimes indispensable for identification of the type of

nerve lesion and of the aetiologies of peripheral neuropathies. The ultrastructural

findings have anyway to be correlated with the clinical findings, the

electrophysiological examination and the laboratory investigations. In this

presentation, the various causes of peripheral neuropathies for which nerve biopsy

study by electron microscope can provide diagnostic information are discussed.

The principal aetiologies that will benefit from such an ultrastructural study are

toxic, infectious, haemopathic, genetic and storage disorders. Sometimes the

electron microscopic examination will help to determine not only the cause of the

peripheral neuropathy, but also the mechanism of nerve lesions. Thus, in peripheral

nerve pathology, ultrastructural study is an efficient tool among other available,

complementary techniques.

713. Valmaggia, C.; Zaunbauer, W.; Gottlob, I. Elevation deficit caused by accessory

extraocular muscle. Am-J-Ophthalmol. 1996 Apr; 121(4): 444-5; ISSN: 0002-

9394.

UNITED-STATES. PURPOSE: To report an elevation deficit, which was caused

by an accessory extraocular muscle, in a 6-year-old boy. METHODS: Computed

tomography and magnetic resonance imaging were used to confirm an accessory,

fusiform, well-defined, solid structure in the retrobulbar space. RESULTS: A

supernumerary intraconal muscle was detected between the annulus of Zinn and the

posterior part of the left globe. CONCLUSION: This rare anomaly may represent

an atavistic retractor bulbi muscle.

714. Van, den Berg L.; Hays, A. P.; Nobile Orazio, E.; Kinsella, L. J.; Manfredini, E.;

Corbo, M.; Rosoklija, G.; Younger, D. S.; Lovelace, R. E.; Trojaborg, W.;

Lange, D. E.; Goldstein, S.; Delfiner, J. S.; Sadiq, S. A.; Sherman, W. H.; Latov,

N. Anti-MAG and anti-SGPG antibodies in neuropathy. Muscle-Nerve. 1996 May;

19(5): 637-43; ISSN: 0148-639X.

UNITED-STATES. We compared the binding of human antibodies from patients

with neuropathy to the myelin-associated glycoprotein (MAG), to its cross-reactive

glycolipid sulfoglucuronyl paragloboside (SGPG), and to sections of peripheral

nerve. Titers were correlated with the clinical presentation and results of

electrophysiological and pathological studies. Most patients had a predominantly

sensory or sensorimotor demyelinating neuropathy and highly elevated antibodies

to both MAG and SGPG, but 2 had highly elevated antibodies to MAG alone, and

1 to SGPG alone. Two patients had predominantly motor neuropathy and highly

elevated antibodies to SGPG which reacted with MAG by Western blot but not by

enzyme-linked immunosorbent assay. One patient had amyotrophic lateral sclerosis

and antibodies to SGPG but not to MAG. These studies indicate that the

neuropathic syndrome associated with anti-MAG or -SGPG antibodies are more

heterogeneous than previously suspected, and that although most of the antibodies

react with both MAG and SGPG, some may react with MAG or SGPG alone.. 0;

0; 0; 0; 0.

715. van, der Laan L.; Goris, R. J. Reflex sympathetic dystrophy after a burn injury.

Burns. 1996 Jun; 22(4): 303-6; ISSN: 0305-4179.

ENGLAND. Reflex sympathetic dystrophy (RSD) is a disease that can appear after

minor trauma or operation to an extremity. The injury may vary from a simple

contusion to a fracture. The prevalence of burns as a cause of RSD, within a

population of 829 patients with RSD, was studied retrospectively. Prospectively,

we documented the medical history, signs and symptoms of all patients with RSD,

seen by our department during the period from January 1984 to 31 December 1994.

Four patients had developed RSD after a burn injury, resulting in a prevalence of

0.5 per cent. Though the clinical signs of early RSD are similar to those of a

(thermal) burn, alertness to recognize inflammatory signs, in combination with the

increase in complaints after exercise, is necessary for early diagnosis and treatment

of the complicating RSD.

716. van, der Meer JW; Koopmans, P. P.; Lutterman, J. A. Antibiotic therapy in diabetic

foot infection. Diabet-Med. 1996; 13 Suppl 1: S48-51; ISSN: 0742-3071.

ENGLAND. 0.

717. van Domburg, P. H.; Gabreels Festen, A. A.; Gabreels, F. J.; de, Coo R.;

Ruitenbeek, W.; Wesseling, P.; ter Laak, H. Mitochondrial cytopathy presenting as

hereditary sensory neuropathy with progressive external ophthalmoplegia, ataxia

and fatal myoclonic epileptic status. Brain. 1996 Jun; 119( Pt 3): 997-1010; ISSN:

0006-8950.

ENGLAND. We present six adult patients from three separate families, with a

remarkably uniform heredo-ataxic syndrome, developing in three stages and ending

in early death. The initial stage is determined by severe sensory neuropathy. The

second stage is characterized further by progressive external ophthalmoplegia

(PEO), probably caused by ocular myopathy, and progressive ataxia. During a

short last stage there is epilepsia, and particularly myoclonic status epilepticus, of

which four patients died unexpectedly. Sural nerve biopsies showed severe loss of

myelinated fibres in a rather early stage of disease. Skeletal muscle biopsies (and a

specimen of ocular muscle) revealed ragged-red fibres. Autopsy examination in two

patients revealed multisystemic involvement of the nervous system, with, in

particular, degeneration of spinal dorsal columns and spinocerebellar tracts.

Pedigree data were compatible with an autosomal recessive disorder. Additional

findings, particularly elevation of CSF lactate, suggested mitochondrial cytopathy

as an essential feature of the multisystem degeneration in these patients.. 0.

718. van Doorn, L. G.; Kranendonk, S. E. Partial unilateral phrenic nerve paralysis caused

by a large intrathoracic goitre. Neth-J-Med. 1996 Jun; 48(6): 216-9; ISSN: 0300-

2977.

NETHERLANDS. Intrathoracic goitres may cause a variety of symptoms caused

by compression of the trachea, neural structures, blood vessels and the

oesophagus. A case history is presented of a patient with a recurrent goitre after

subtotal thyroidectomy who displayed partial unilateral phrenic paralysis, which

subsided after a second subtotal thyroidectomy. Compression of the phrenic nerve

appears to be a very rare manifestation of an intrathoracic goitre and thus far has

never been reported.

719. van Everdingen, J. [Diplopia due to diabetic neuropathy (letter)]. Dubbelzien ten

gevolge van diabetische neuropathie. Ned-Tijdschr-Geneeskd. 1996 Aug 17;

140(33): 1704-5; ISSN: 0028-2162.

NETHERLANDS.

720. van Leeuwen, J. P.; Harhangi, B. S.; Thewissen, N. P.; Thijssen, H. O.; Cremers, C.

W. Delays in the diagnosis of acoustic neuromas. Am-J-Otol. 1996 Mar; 17(2):

321-5; ISSN: 0192-9763.

UNITED-STATES. From the medical files of 164 consecutive patients who

underwent surgical treatment for a unilateral acoustic neuroma between 1980 and

1992, we collected data on the delay until the diagnosis was made. A distinction

was made between the patient's and general practitioner's delay (delay 1) and the

delay after the specialist's first visit until the radiologic diagnosis (delay 2). The

average delay was 35.7 months (SD, 62.2) for delay 1 and 15.2 months (SD,

36.3) for delay 2. Specialist's delay (otolaryngologist or neurologist) was divided

into a delay of a maximum of 12 months (134 patients) and a longer delay (30

patients). In 27 of the 30 patients, no specific tests had been performed, and in the

remaining three, the test results were inconclusive. Reasons for not conducting

further tests included familial hearing impairment, Meniere's disease, otosclerosis,

and alcoholism. In cases in which the specialist had not made the diagnosis within

1 year, it took an average of 6 months extra to make the diagnosis of an acoustic

neuroma, usually with a fairly short patient delay. The specialist's delay remained

constant in the period of investigation, with the possibility of magnetic resonance

imaging (MRI) scanning only in the last 2 years. In view of the increasing

accessibility of MRI, it is now recommended if possible to perform MRIs in all

patients with symptoms suspicious for an acoustic neuroma.

721. Vance, J. M.; Speer, M. C.; Stajich, J. M.; West, S.; Wolpert, C.; Gaskell, P.;

Lennon, F.; Tim, R. M.; Rozear, M.; Othmane, K. B.; et, a. l. Misclassification

and linkage of hereditary sensory and autonomic neuropathy type 1 as Charcot-

Marie-Tooth disease, type 2B [letter; comment]. Am-J-Hum-Genet. 1996 Jul;

59(1): 258-62; ISSN: 0002-9297.

Note: Comment on: Am J Hum Genet 1995 Oct;57(4):853-8.

UNITED-STATES.

722. Vandenberghe, A.; Latour, P.; Chauplannaz, G.; Chapon, F.; Pouget, J.; Dumas, R.;

Laguenay, A.; Ollagnon, E.; Bost, M.; Duthel, S.; Chazot, G.; Boucherat, M.

Molecular diagnosis of Charcot-Marie-Tooth 1A disease and hereditary neuropathy

with liability to pressure palsies by quantifying CMT1A-REP sequences:

consequences of recombinations at variant sites on chromosomes 17p11.2-12 [see

comments]. Clin-Chem. 1996 Jul; 42(7): 1021-5; ISSN: 0009-9147.

Note: Comment in: Clin Chem 1996 Jul;42(7):995-8.

UNITED-STATES. The most frequent form of Charcot-Marie-Tooth disease

(CMT1A; OMIM118.220) is the result of a duplication on chromosome 17 in pll.2-

p12. This region contains PMP22, a gene expressed in peripheral myelin. The

mutation results from an unequal crossing-over involving repeated sequences,

CMT1A-REP, located on both sides of the duplicated region. The reciprocal

product of this recombination is a deletion of the same region, which is associated

with hereditary neuropathy with liability to pressure palsies (HNPP;

OMIM162.500). Proximal and distal CMT1A-REP sequences can be distinguished

by the presence of a variant EcoRI site. We quantified the number of these repeat

sequences in 36 CMT1A and 40 HNPP patients. CMT1A-REP sequences are

involved in almost all of the mutations. The majority of recombination breakpoints

occur distally from the variant EcoRI site. However, a few have a breakpoint

proximal to this site, which creates the risk of misinterpretation with respect to a

duplicated/deleted status.. 9007-49-2.

723. Vandenberghe, A.; Latour, P.; Chauplannaz, G.; Chapon, F.; Pouget, J.; Dumas, R.;

Laguenay, A.; Ollagnon, E.; Bost, M.; Duthel, S.; Chazot, G.; Boucherat, M.

Molecular diagnosis of Charcot-Marie-Tooth 1A disease and hereditary neuropathy

with liability to pressure palsies by quantifying CMT1A-REP sequences:

consequences of recombinations at variant sites on chromosomes 17p11.2-12 [see

comments]. Clin-Chem. 1996 Jul; 42(7): 1021-5; ISSN: 0009-9147.

Note: Comment in: Clin Chem 1996 Jul;42(7):995-8.

UNITED-STATES. The most frequent form of Charcot-Marie-Tooth disease

(CMT1A; OMIM118.220) is the result of a duplication on chromosome 17 in pll.2-

p12. This region contains PMP22, a gene expressed in peripheral myelin. The

mutation results from an unequal crossing-over involving repeated sequences,

CMT1A-REP, located on both sides of the duplicated region. The reciprocal

product of this recombination is a deletion of the same region, which is associated

with hereditary neuropathy with liability to pressure palsies (HNPP;

OMIM162.500). Proximal and distal CMT1A-REP sequences can be distinguished

by the presence of a variant EcoRI site. We quantified the number of these repeat

sequences in 36 CMT1A and 40 HNPP patients. CMT1A-REP sequences are

involved in almost all of the mutations. The majority of recombination breakpoints

occur distally from the variant EcoRI site. However, a few have a breakpoint

proximal to this site, which creates the risk of misinterpretation with respect to a

duplicated/deleted status.. 9007-49-2.

724. Verdon, M. E. Overuse syndromes of the hand and wrist. Prim-Care. 1996 Jun; 23(2):

305-19; ISSN: 0095-4543.

UNITED-STATES. Overuse syndromes are one of the most common occupational

illnesses treated by primary care providers. Their pathophysiology parallels that of

tenosynovitis. Occupational risk factors for overuse syndromes include repetition,

high force, awkward joint posture, direct pressure, and vibration. Initial treatment

is aimed at preventing fibrosis through rest, immobilization, and anti-inflammatory

agents. Treatment must include identification and adjustment of occupational risk

factors. Specific overuse syndromes are discussed, including tenosynovitis of the

dorsal wrist extensor compartments and flexor tendons of the wrist, trigger finger,

and carpal tunnel syndrome.

725. Verheggen, R.; Markakis, E.; Muhlendyck, H.; Finkenstaedt, M. Symptomatology,

surgical therapy and postoperative results of sphenoorbital, intraorbital-

intracanalicular and optic sheath meningiomas. Acta-Neurochir-Suppl-Wien. 1996;

65: 95-8; ISSN: 0065-1419.

AUSTRIA. A series of 7 patients with optic sheath meningiomas, 3

intracanalicular and intraorbital, 2 intraosseus meningiomas of the sphenoid wing

involving the optic canal, and 4 sphenoorbital meningiomas were reported. The

choice of a surgical approach to the orbit was appropriate to the location and size of

the tumour relative to the optic nerve. The most common complaints were

proptosis, reduction of visual acuity and paresis of eye muscles. Patients with optic

sheath meningiomas are threatened postoperatively by visual loss whereas the high

recurrence rate has to be taken into consideration in cases of sphenoorbital

meningiomas.

726. Verrotti, A.; Chiarelli, F.; Morgese, G. Autonomic dysfunction in newly diagnosed

insulin-dependent diabetes mellitus children. Pediatr-Neurol. 1996 Jan; 14(1): 49-

52; ISSN: 0887-8994.

UNITED-STATES. In order to evaluate the presence of electrophysiologic signs

of autonomic dysfunction (AD) in newly diagnosed diabetic children,

cardiovascular reflex tests were performed in 55 (30 female, 25 male) newly

diagnosed insulin-dependent diabetes mellitus (IDDM) patients aged 10.3-20.7

years (mean +/- S.D.: 15.2 +/- 5.6). Ten (18.2%) diabetic children had

cardiovascular AD, defined as abnormal results in 2 of 5 tests. Autonomic function

tests were assessed at entry and after 12, 24, and 36 months of the study. All

diabetic children received human insulin and followed an intensive insulin treatment

(3 or 4 injections per day), associated with a teaching program of self-management

of the disease. In the 3 years of follow-up, all children improved the quality of

metabolic control (glycosylated hemoglobin, HbA1c: 10.3 +/- 1.1% versus 7.7 +/-

0.9; P < .01) and manifested no significant difference between baseline and follow-

up values of autonomic function tests which remained unchanged in spite of this

improvement. Cardiovascular autonomic dysfunction can be present in newly

diagnosed IDDM children and it seems to be stable in children who follow an

intensive insulin injection therapy.. 0; 11061-68-0.

727. Vibert, D.; Hausler, R.; Safran, A. B.; Koerner, F. Diplopia from skew deviation in

unilateral peripheral vestibular lesions. Acta-Otolaryngol-Stockh. 1996 Mar;

116(2): 170-6; ISSN: 0001-6489.

NORWAY. Vertical diplopia from skew deviation is well described in brainstem

lesions. The phenomenon can also result from peripheral vestibular lesions. During

the past years, we have observed these ocular changes in the acute stage following

unilateral vestibular neurectomy and labyrinthectomy (n = 13), as well as in series

of patients suffering from idiopathic sudden unilateral peripheral vestibular or

cochleo-vestibular deficit (n = 5). Diplopia from skew deviation was noted

immediately following ablative vestibular procedures; in patients with idiopathic

vestibular deficit, it was observed as an associated sign. In all patients, clinical

evaluation revealed an acute unilateral peripheral vestibular loss, with spontaneous

nystagmus toward the unaffected ear and absence of nystagmic response to caloric

testing on the affected ear. Skew deviation was measured using the Hess-Weiss

test, which is based on the haploscopic principle. Static visual vertical was

evaluated with the original methods of vertical frame and Maddox rod techniques.

Photographs were made of the ocular fundi, to measure the degree of cyclotorsion

of both eyes. In our patients, we found skew deviation with hypotropia of the eye

that was ipsilateral to the affected ear and conjugated cyclotorsion and tilt of the

static visual vertical on the side of the affected ear. Skew deviation was the first

sign to disappear within a few days; conjugated cyclotorsion and tilt of the static

visual vertical persisted for weeks to months. The eye-head postural reaction,

consisting of head tilt, conjugated eye cyclotorsion, skew deviation, and alteration

of vertical perception directed toward the side of the lesion, is known as the Ocular

Tilt Reaction (OTR). The mechanism is presumably related to a lesion of the

otolithic organs and/or to changes in the afferent graviceptive pathways. In man,

the OTR is often mild and unrecognized, masked by spontaneous nystagmus and

marked neuro-vegetative symptoms. Our observations indicate that skew deviation,

as a part of the OTR, occurs in patients with sudden peripheral vestibular lesions,

whether surgical or non-surgical in origin.

728. Villablanca, P.; Curran, J.; Arnold, A.; Lufkin, R. Orbit and optic nerve. Top-Magn-

Reson-Imaging. 1996 Apr; 8(2): 87-110; ISSN: 0899-3459.

UNITED-STATES. The optic nerve is a fiber tract of the brain, and is not a true

cranial nerve. Primary pathologic processes seen in the central nervous system and

its linings can therefore affect the optic nerve. Along their course the fibers of the

visual pathways are also susceptible to secondary extrinsic processes. The MR

appearance of many of the primary and secondary processes that affect the optic

nerve are characteristic. Where the imaging features of a process are not

characteristic, a fairly narrow differential diagnosis can be suggested depending on

the imaging appearance and anatomic location of the pathologic entity. This article

is a categorical overview of the disease entities that may affect the optic nerve and

tracts. Pathologic processes are organized according to the typical sites of

involvement along the visual pathways, from the retina to the occipital cortex.

Medical imaging, pathologic, clinical, and epidemiological highlights are presented.

729. Visser, L. H.; van, der Meche FG; Meulstee, J.; Rothbarth, P. P.; Jacobs, B. C.;

Schmitz, P. I.; van Doorn, P. A. Cytomegalovirus infection and Guillain-Barre

syndrome: the clinical, electrophysiologic, and prognostic features. Dutch Guillain-

Barre Study Group. Neurology. 1996 Sep; 47(3): 668-73; ISSN: 0028-3878.

UNITED-STATES. Guillain-Barre syndrome (GBS) is usually preceded by

infections, in particular cytomegalovirus (CMV) and Campylobacter jejuni

infection. We studied the clinical and electrophysiologic features of 20 CMV-

associated GBS patients and compared the findings with earlier established data of

C. jejuni-related GBS patients (n = 43) and of GBS patients without these

infections (n = 71). The patients all participated in the Dutch GBS trial in which we

compared the effect of intravenous immune globulins and plasma exchange. We

demonstrate that CMV-related GBS patients have a different clinical pattern in

comparison with the other two GBS groups. They are significantly younger,

initially have a severe course indicated by a high frequency of respiratory

insufficiency, and often develop cranial nerve involvement and severe sensory loss.

This is in contrast to C. jejuni infection, which is associated with motor GBS. Both

infections are associated with delayed recovery compared with the GBS patients

without these infections.

730. Vita, G.; Savica, V.; Milone, S.; Trusso, A.; Bellinghieri, G.; Messina, C. Uremic

autonomic neuropathy: recovery following bicarbonate hemodialysis. Clin-

Nephrol. 1996 Jan; 45(1): 56-60; ISSN: 0301-0430.

GERMANY. Autonomic function was followed in 8 chronic uremic patients on

periodic hemodialysis over a period of almost eight years. The cardiovascular

autonomic testing included R-R interval variation test, deep breathing, Valsalva

manoeuvre, heart rate and blood pressure responses to standing, sustained

handgrip. The patients were investigated on entry into the study and after 18, 56,

and 92 months. Six months after the study at time 56 months, they switched from

acetate to bicarbonate dialysis. The response to deep breathing test was

significantly reduced at time 18 months versus baseline (P = 0.014), but

significantly increased at time 92 months versus 56 months (P = 0.042). A

significant decrease was found in the systolic blood pressure response to standing

between baseline and 18 months (P = 0.014) and in the response to handgrip test

between 18 and 56 months (P = 0.014). Multivariate analysis of the autonomic

tests by a pattern recognition method (Bayesian analysis) showed that, at the time

of entry into the study, two out of eight patients had autonomic damage. At 18 and

56 months, 6/8 patients had autonomic dysfunction. At the last time of

investigation, 30 months after the onset of bicarbonate dialysis, all the patients

showed a reversal of autonomic damage. Age and duration of dialysis on entry did

not affect autonomic function. The present study is the first demonstration that

autonomic neuropathy can recover after long-term dialysis. Since chronic

hypoxemia is a cause of polyneuropathy, we postulate that: 1) hypoxemia in

dialysis patients may have a role in the pathogenesis of uremic polyneuropathy, and

particularly of autonomic dysfunction; 2) in patients on bicarbonate dialysis, a

greater hemodynamic stability with less hypoxemia may lead to a recovery of

autonomic function.. 0; 0; 0.

731. Vitali, C.; Genovesi Ebert, F.; Romani, A.; Jeracitano, G.; Nardi, M.

Ophthalmological and neuro-ophthalmological involvement in Churg-Strauss

syndrome: a case report. Graefes-Arch-Clin-Exp-Ophthalmol. 1996 Jun; 234(6):

404-8; ISSN: 0721-832X.

GERMANY. BACKGROUND: It is well known that different types of eye

involvement may develop during the course of systemic vasculitides. METHODS:

We report here a case of Churg-Strauss syndrome (allergic granulomatous angiitis)

characterized by the presence of multiple ophthalmological and neuro-

ophthalmological lesions, i.e., mononeuritis of the fourth cranial nerve, multifocal

choroidal ischaemia, and bilateral ischaemic optic neuropathy. RESULTS:

Ischaemic lesions in the posterior ciliary plexus and chorio-retinal circulation,

which appeared simultaneously after a phase of disease activity, were documented.

CONCLUSION: The simultaneous occurrence of multiple ocular features in a

patient with Churg-Strauss syndrome suggests that regional vasculitis may be the

pathological mechanism underlying the multiple ophthalmological lesions in this

disorder.

732. Vladutiu, C. [The incidence of refractive errors in strabismus with an early onset].

Incidenta viciilor de refractie in strabismele cu debut precoce. Oftalmologia. 1996

Apr; 40(2): 153-7; ISSN: 1120-0875.

ROMANIA. The paper represents one study effected at a number of two hundred

and fifty children under three years old, examinated in Ophthalmological Clinic

between 1992 and 1995. It was examinated strabismic deviation, visual acuity,

state of binocular-eyesight refraction. Most of children (59%) present to

hypermetropia under three dioptry; 23.3% medium hypermetropia (3-6 dioptries).

Little percentage under 10% present others refraction vices: big hypermetropia,

astigmatism, anisometropia, myopia. After optic correction and occlusion, most of

deviations remained unaltered requiring surgical subsequent correction. This fact

certifys the preponderence of neurogenic pathogenesis and not adjustment in

strabismus with precocious debut.

733. Vladutiu, C.; Mihai, E. [Prismatic and surgical block in nystagmus]. Blocarea

prismatica si chirurgicala a nistagmusului. Oftalmologia. 1996 Apr; 40(2): 115-9;

ISSN: 1120-0875.

ROMANIA. After the speech of some teoretical notions (classification, ethyology,

clinical description, treatment) about nystagmus are presented the summaries of

dossiers of five patients consultated in our clinic with the diagnostic of nystagmus.

At two from them realised the complete blockade at the contractions with prisms

and at two from en the partial blockade too with prisms. One of them presenting

nystagmus associate with strabismus was surgical solved.

734. Voinea, L.; Samson, M.; Iliescu, M. [The loss of a right muscle in the orbit intra- or

postoperatively]. Pierderea unui muschi drept in orbita intra sau postoperator.

Oftalmologia. 1996 Apr; 40(2): 120-4; ISSN: 1120-0875.

ROMANIA. One child four and half years old remains with a divergent deviation

by forty-five degree with absence of adduction, after an operation of convergent

strabismus at the left eye. Considering that is about by one postoperatorial paralytic

strabismus, computer tomography praises the right intern muscle retracted at the

back of orbit, but it doesn't adheres at the eyeball. It considers that the divergent

strabismus was caused by the slide of postoperatorial muscle. Looking middle line

of the muscle, parallel with the middle wall of the orbit, it praises the right intern

muscle retracted and after the excision of the granulation tissue all round, displays

the muscle with its normal lenght. It cuts 3.5 milimeters from this muscle and it

practices the retroposition of the extern right muscle with 7 milimeters. It obtains

postoperatorial orthophoria. It talks about possibility of lossen of a right muscle in

orbit intra- and post-operatorial and the measures that are imposed in these

situations.

735. Volmink, J.; Lancaster, T.; Gray, S.; Silagy, C. Treatments for postherpetic neuralgia-

-a systematic review of randomized controlled trials. Fam-Pract. 1996 Feb; 13(1):

84-91; ISSN: 0263-2136.

ENGLAND. BACKGROUND. A number of different therapies have been used

for postherpetic neuralgia. We decided to conduct a systematic review of existing

randomized controlled trials. OBJECTIVE. To determine the efficacy of available

therapies for relieving the pain of established postherpetic neuralgia. METHODS.

We performed a systematic review, including meta-analysis, of existing

randomized controlled trials. Eleven published trials and one unpublished trial were

identified which met the inclusion criteria and were included in the current review.

RESULTS. Pooled analysis of the effect of tricyclic antidepressants demonstrate

statistically significant pain relief (OR 0.15, CI 0.08-0.27). Pooling of the results

of the three trials comparing the effects of capsaicin and placebo could not be done

due to heterogeneity. This heterogeneity was mainly attributable to an unpublished

trial which differed in terms of the dose and duration of treatment. When this study

was omitted, no heterogeneity was found and the pooled analysis revealed a

statistically significant benefit (OR 0.29, 95% CI 0.16-0.54). However, problems

with blinding in patients using capsaicin may have accounted for the positive effect.

One small study of vincristine iontophoresis compared to placebo also yielded a

favourable result (OR 0.05, 95% CI 0.01-0.26). Other treatment evaluated include

lorazepam, acyclovir, topical benzydamine, and acupuncture. We found no

evidence that these are effective in relieving pain associated with postherpetic

neuralgia. CONCLUSION. Based on evidence from randomized trials, tricyclic

anti-depressants appear to be the only agents of proven benefit for established

postherpetic neuralgia.. 0; 0.

736. Wallace, C. J.; Sevick, R. J. Multifocal white matter lesions. Semin-Ultrasound-CT-

MR. 1996 Jun; 17(3): 251-64; ISSN: 0887-2171.

UNITED-STATES. There is a long differential diagnosis for multifocal white

matter lesions on MR. The most common causes are prominent Virchow-Robin

spaces, white matter ischemic change, and multiple sclerosis, but many other

causes have been reported. Most of these are related to vascular or other

demyelinating etiologies, but infectious/inflammatory disease, trauma, and

neoplastic and other unusual causes may also be responsible. Typical imaging

features of the more common multifocal white matter disorders are outlined, and the

rarer causes are discussed briefly. An approach to imaging differential diagnosis is

given, with emphasis on the differences between white matter ischemic lesions and

multiple sclerosis.

737. Wallace, M. R.; Moss, R. B.; Beecham, HJ 3rd; Grace, C. J.; Hersh, E. M.;

Peterson, E.; Murphy, R.; Shepp, D. H.; Siegal, F. P.; Turner, J. L.; Safrin, S.;

Carlo, D. J.; Levine, A. M. Early clinical markers and CD4 percentage in subjects

with human immunodeficiency virus infection. J-Acquir-Immune-Defic-Syndr-

Hum-Retrovirol. 1996 Aug 1; 12(4): 358-62; ISSN: 1077-9450.

UNITED-STATES. In a clinical trial involving asymptomatic, HIV-seropositive

subjects treated with either the HIV-1 immunogen (an inactivated, gp120-depleted

HIV-1 virus in incomplete Freund's adjuvant) or an adjuvant control, we examined

the relationship between changes in the percentage of CD4 cells over time and early

clinical markers of HIV disease progression. Subjects who had an early clinical

event were more likely to have a greater decline in the percentage of CD4 cells than

those subjects who did not have a clinical event (p = 0.054). The greatest decline in

CD4 percentage occurred within 10 weeks prior to a clinical event (mean 11%

decrease from baseline). Subjects from the quartile with the greatest decline in CD4

percentage had a fivefold greater risk of having a clinical event than subjects from

the quartile with the second largest decline (p = 0.045). These results demonstrate a

relationship between changes in the percentage of CD4 cells and early clinical

events. Further validation of this association may be useful in clinical monitoring

and in evaluating therapies to treat HIV infection.. 0; 0; 0; 0.

738. Wallace, M. R.; Moss, R. B.; Beecham, HJ 3rd; Grace, C. J.; Hersh, E. M.;

Peterson, E.; Murphy, R.; Shepp, D. H.; Siegal, F. P.; Turner, J. L.; Safrin, S.;

Carlo, D. J.; Levine, A. M. Early clinical markers and CD4 percentage in subjects

with human immunodeficiency virus infection. J-Acquir-Immune-Defic-Syndr-

Hum-Retrovirol. 1996 Aug 1; 12(4): 358-62; ISSN: 1077-9450.

UNITED-STATES. In a clinical trial involving asymptomatic, HIV-seropositive

subjects treated with either the HIV-1 immunogen (an inactivated, gp120-depleted

HIV-1 virus in incomplete Freund's adjuvant) or an adjuvant control, we examined

the relationship between changes in the percentage of CD4 cells over time and early

clinical markers of HIV disease progression. Subjects who had an early clinical

event were more likely to have a greater decline in the percentage of CD4 cells than

those subjects who did not have a clinical event (p = 0.054). The greatest decline in

CD4 percentage occurred within 10 weeks prior to a clinical event (mean 11%

decrease from baseline). Subjects from the quartile with the greatest decline in CD4

percentage had a fivefold greater risk of having a clinical event than subjects from

the quartile with the second largest decline (p = 0.045). These results demonstrate a

relationship between changes in the percentage of CD4 cells and early clinical

events. Further validation of this association may be useful in clinical monitoring

and in evaluating therapies to treat HIV infection.. 0; 0; 0; 0.

739. Wang, H. C.; Tsai, M. D. Compressive ulnar neuropathy in the proximal forearm

caused by a gouty tophus. Muscle-Nerve. 1996 Apr; 19(4): 525-7; ISSN: 0148-

639X.

UNITED-STATES. 69-93-2.

740. Warner, L. E.; Hilz, M. J.; Appel, S. H.; Killian, J. M.; Kolodry, E. H.; Karpati, G.;

Carpenter, S.; Watters, G. V.; Wheeler, C.; Witt, D.; Bodell, A.; Nelis, E.; Van

Broeckhoven, C.; Lupski, J. R. Clinical phenotypes of different MPZ (P0)

mutations may include Charcot-Marie-Tooth type 1B, Dejerine-Sottas, and

congenital hypomyelination. Neuron. 1996 Sep; 17(3): 451-60; ISSN: 0896-6273.

UNITED-STATES. Hereditary demyelinating peripheral neuropathies consist of a

heterogeneous group of genetic disorders that includes hereditary neuropathy with

liability to pressure palsies (HNPP), Charcot-Marie-Tooth disease (CMT),

Dejerine-Sottas syndrome (DSS), and congenital hypomyelination (CH). The

clinical classification of these neuropathies into discrete categories can sometimes

be difficult because there can be both clinical and pathologic variation and overlap

between these disorders. We have identified five novel mutations in the myelin

protein zero (MPZ) gene, encoding the major structural protein (P0) of peripheral

nerve myelin, in patients with either CMT1B, DSS, or CH. This finding suggests

that these disorders may not be distinct pathophysiologic entities, but rather

represent a spectrum of related "myelinopathies" due to an underlying defect in

myelination. Furthermore, we hypothesize the differences in clinical severity seen

with mutations in MPZ are related to the type of mutation and its subsequent effect

on protein function (i.e., loss of function versus dominant negative).. 0.

741. Watanabe, Y.; Takeda, S. Computerized electro-nystagmography. Acta-Otolaryngol-

Suppl-Stockh. 1996; 522: 26-31; ISSN: 0365-5237.

NORWAY. A computerized electro-nystagmography (ENG) system was

developed on the basis of a new idea for the performance of ENG. In this system,

all the functions of conventional ENG was performed by a personal computer (PC)

except for the initial setting of an analog amplifier. The eye movement signals from

the ENG amplifier were displayed on the graphic screen and recorded on a

magnetic or magneto-optical disc. By using a special graphic screen, eye

movements, even fine nystagmus, can be more clearly observed than they can on

the PC's conventional graphic screen. Examiners can select the most suitable

display condition according to the specific purpose(s) of the ENG examination. As

a laser printer prints the recorded eye movements simultaneously with data

sampling, we can observe eye movements in the same manner as on the recorded

paper of a conventional ENG. Moreover, this system has a very powerful function

for the analysis of such nystagmus responses as caloric test, VOR test and

optokinetic nystagmus and it can be gotten at a lower cost than conventional ENG.

These technical and economical features indicate that the computerized ENG system

is a very useful and convenient device for observing, recording and analyzing ENG

examinations.

742. Waters, K. A.; Woo, P.; Mortelliti, A. J.; Colton, R. Assessment of the infant airway

with videorecorded flexible laryngoscopy and the objective analysis of vocal fold

abduction. Otolaryngol-Head-Neck-Surg. 1996 Apr; 114(4): 554-61; ISSN: 0194-

5998.

UNITED-STATES. Accurate diagnosis of upper airway abnormalities by flexible

laryngoscopy in infants is hampered by rapid laryngeal motion and lack of patient

cooperation. This study evaluates the added role of videorecorded flexible

laryngoscopy and the objective measurement of vocal fold abduction in improving

the diagnosis of upper airway abnormalities in infants. Seventy-eight infants had

videorecorded flexible laryngoscopy performed as part of their evaluation of a

suspected airway disorder. These recordings were reviewed by three

otolaryngologists for confirmation of the clinical diagnosis. From the video image,

the maximum angle of vocal fold abduction was measured with image analysis

software. Of 78 patients 40 had supraglottic or glottic abnormalities, 9 had nasal or

nasopharyngeal obstruction, 9 had subglottic abnormalities (diagnosed subsequent

to videolaryngoscopy), and 15 patients had normal findings on examination. Of

those with laryngeal abnormalities, laryngomalacia was the most common

diagnosis (23 of 78). Vocal fold paralysis was present in 4 patients. A separate

group (9 of 78) of patients was identified as having symmetric bilateral limitation of

vocal fold abduction. Laryngeal dyskinesia was diagnosed in these 9 patients. The

mean values of maximal vocal fold abduction were as follows: (1) normals, 59.5

degrees; (2) laryngomalacia, 57.0 degrees; (3) paralysis, 26.6 degrees; and (4)

incomplete abduction with laryngeal dyskinesia, 27.6 degrees. Videolaryngoscopy

is a valuable tool for documentation, parent education, and analysis of infant

laryngeal abnormalities. Repeat viewing of the video examination and frame-by-

frame analysis improve the diagnostic accuracy. Using this approach, we have

calculated the anterior glottic abduction angle in the normal and abnormal infant

larynx. In addition, we have identified a group of infants with incomplete abduction

of the vocal folds that appears to be different from that found in vocal cord

paralysis.

743. Waters, M. F.; Jacobs, J. M. Leprous neuropathies. Baillieres-Clin-Neurol. 1996 Mar;

5(1): 171-97; ISSN: 0961-0421.

ENGLAND. Once a terrifying disease, leprosy today has a very hopeful

prognosis, provided that it is diagnosed early and treated with modern multidrug

chemotherapy, any immunological reactions being recognized quickly and

controlled well to prevent (further) peripheral nerve damage after commencing

treatment. The diagnosis should be considered in all patients who present with

peripheral neuropathy and/or anaesthetic skin lesions who come from or have lived

in the tropics and subtropics. Although M. leprae cannot yet be grown in vitro, it is

readily grown in experimental animals. A complete gene library has been

developed, much of the genome mapped and a number species-specific and

common mycobacterial antigens identified. The intricacies of the host-parasite

relationship, especially of cell-mediated immunity, and of the important

immunological reactions of ENL and reversal reaction have been widely

investigated. Modern MDT has caused a dramatic fall in prevalence, although the

world annual case detection rate remains at around 600,000 new patients, many

being at an early stage of the disease. WHO has launched a campaign to eliminate

leprosy as a significant public health risk by the 2000 (with a prevalence of less

than 1:10 000 population), which may well be achieved in some endemic countries.

Leprosy will, however, remain an important cause of peripheral neuropathy for at

least several more decades.

744. Wehling, P.; Cleveland, S. J.; Heininger, K.; Schulitz, K. P.; Reinecke, J.; Evans, C.

H. Neurophysiologic changes in lumbar nerve root inflammation in the rat after

treatment with cytokine inhibitors. Evidence for a role of interleukin-1. Spine. 1996

Apr 15; 21(8): 931-5; ISSN: 0362-2436.

UNITED-STATES. STUDY DESIGN. The present study was designed to

evaluate the effect of cytokine inhibitors in experimental allergic radiculitis.

OBJECTIVE. Evaluation of the effect of cytokine inhibitors in experimental allergic

radiculitis. SUMMARY OF BACKGROUND DATA. A number of cytokines are

known to be involved in hyperalgesia and may play a role in radiculitis.

Corticosteroids and other cytokine inhibitors antagonize their effects. METHODS.

Experimental allergic radiculitis was induced in rats by injection of bovine myelin

from the peripheral nervous system. The sham group subsequently received saline

injections; the treatment groups received either prednisolone or interleukin-1

receptor antagonist. Treatment effect was assessed on the basis of motor

performance and neurophysiologic parameters. RESULTS. Treatment ameliorated

the symptoms of experimental allergic radiculitis. Prednisolone appeared to be

somewhat more effective than interleukin-1 receptor antagonist. CONCLUSIONS.

Because interleukin-1 receptor antagonist specifically blocks the effects of

interleukin-1 at its receptors, the present results imply that interleukin-1 is a causal

factor in the model of experimental radiculitis used. Its specificity and apparent lack

of side effects make interleukin-1 receptor antagonist an attractive candidate

treatment for the human disease.. 0; 0; 0; 0; 0; 50-24-8.

745. Wei, M. E.; Campbell, S. H.; Taylor, C. Precipitous visual loss secondary to optic

nerve toxoplasmosis as an unusual presentation of AIDS. Aust-N-Z-J-Ophthalmol.

1996 Feb; 24(1): 75-7; ISSN: 0814-9763.

AUSTRALIA. Life-threatening conditions requiring urgent medical treatment

rarely present to the ophthalmologist. This case report describes a patient with

precipitious visual loss as the primary complaint and which subsequently led to the

diagnosis of Toxoplasmic papillitis and life-threatening cerebral involvement as an

initial manifestation of AIDS.. 0; 0; 58-05-9; 58-14-0; 68-35-9.

746. Weissgold, D. J.; Galetta, S. L. Horner's syndrome after vertical ramus osteotomy:

report of a case. J-Oral-Maxillofac-Surg. 1996 Jul; 54(7): 913-4; ISSN: 0278-

2391.

UNITED-STATES.

747. Wenzel, R.; Bartenstein, P.; Dieterich, M.; Danek, A.; Weindl, A.; Minoshima, S.;

Ziegler, S.; Schwaiger, M.; Brandt, T. Deactivation of human visual cortex during

involuntary ocular oscillations. A PET activation study. Brain. 1996 Feb; 119( Pt

1): 101-10; ISSN: 0006-8950.

ENGLAND. Prompted by the observation of decreased glucose metabolism in the

striate and extrastriate visual cortex in a patient with opsoclonus, we studied the

influence of involuntary eye movements on visual cortex activity. Repeated

measurements of cerebral blood flow (CBF) by PET were performed in 12 healthy

volunteers using H2(15)O-bolus technique after ear canal irrigation with ice cold or

warm (44 degrees C) water with the subjects eyes closed. In addition to blood flow

increases in areas involved in central vestibular processing, statistical subtraction

analysis revealed a nearly symmetrical, bilateral, highly significant decrease in the

occipital cortex covering Brodmann areas 17, 18, and 19 after ice water stimulation

of either ears. Region of interest analysis revealed in all subjects a mean decrease in

regional CBF (rCBF) of 12.8% (range 4.6-21.0%) in these areas. A similar but

less pronounced effect (mean rCBF decrease in visual cortex 4.8%, range 1.1-

11.5%) was observed after warm water irrigation. The observations suggest that

deactivation of the visual cortex is induced by involuntary ocular oscillations. This

deactivation is not dependent on changes of the retinal input (eyes closed). The

physiological significance of this hitherto unknown phenomenon may be the

protection from inadequate visual input (oscillopsia) during involuntary ocular

oscillations.. 0; 154-17-6; 29702-43-0; 7732-18-5.

748. Wetzel, F. T. Surgery for herniated vertebral disks. JAMA. 1996 Feb 21; 275(7): 513-

4; ISSN: 0098-7484.

UNITED-STATES.

749. Whitaker, E. J.; Luchetti, C. J. Treating the oral sequelae of an acoustic neuroma. J-

Am-Dent-Assoc. 1996 Feb; 127(2): 231-3; ISSN: 0002-8177.

UNITED-STATES. This article describes the treatment of oral complications that

arose after surgery for the removal of an acoustic brain tumor. The authors contend

that a thorough oral examination can pinpoint the cranial nerves that have been

affected by the tumor or the surgery, and that dental appliances can offer special

protection after surgery.

750. Wiegand, D. A.; Ojemann, R. G.; Fickel, V. Surgical treatment of acoustic neuroma

(vestibular schwannoma) in the United States: report from the Acoustic Neuroma

Registry. Laryngoscope. 1996 Jan; 106(1 Pt 1): 58-66; ISSN: 0023-852X.

UNITED-STATES. In 1989, the Acoustic Neuroma Association established a

multisurgeon, multi-institutional registry to collect data related to the treatment of

patients with acoustic neuroma. This report analyzes information from the 1579

surgically treated patients who were entered in the registry between January 1,

1989, and February 28, 1994.

751. Wieslander, G.; Norback, D.; Edling, C. Local cold exposure of the hands from

cryosectioning work in histopathological and toxicological laboratories: signs and

symptoms of peripheral neuropathy and Raynaud's phenomenon. Occup-Environ-

Med. 1996 Apr; 53(4): 276-80; ISSN: 1351-0711.

ENGLAND. OBJECTIVES: To study relations between cryosectioning work,

skin temperature, early signs of neuropathy in the hands, and vasospastic and

neurological symptoms. Microtome work is carried out at histological and

toxicological laboratories all over the world. It implicates local cold exposure of -20

degrees C on the hands of exposed laboratory technicians. METHODS: Thirty nine

laboratory technicians who use microtomes at the preclinical and clinical

laboratories at the University of Uppsala, Sweden were studied. An equal number

of nonexposed laboratory technicians served as controls, matched for workplace,

sex, age, and smoking habits. Information on symptoms, type of work, and

personal factors were assessed by means of a self administered questionnaire. Two

point discrimination ability was tested, and vibration perception threshholds were

measured for both hands by a bio-thesiometer. Also, skin temperature was

measured during cryosectioning work in those 15 technicians performing

cryosectioning work during the study period. RESULTS: Most laboratory

technicians did not use any gloves during cryosectioning work, and direct contact

with frozen materials sometimes occurred and resulted in a rapid cooling of the

skin. In six of 15 exposed subjects (40%), the mean skin temperature during

microtome work was below 20 degrees C. A later rise in skin temperature, due to a

compensatory vasodilation, was found in two subjects. The group exposed to cold

had signs of early neuropathy on the right hand, indicated both by vibrametry and

two point discrimination test. Significant work related differences in clinical signs

within the group exposed to cold was also found. No differences between exposed

and nonexposed people were found for symptoms of Raynaud's phenomenon,

numbness, or musculosceletal complaints. CONCLUSION: Our study shows that

cryosectioning laboratory work may cause adverse health effects--for example,

peripheral neuropathy--and measures should be taken to protect the hands from the

local cold exposure.

752. Wiet, R. J.; Micco, A. G.; Bauer, G. P. Complications of the gamma knife. Arch-

Otolaryngol-Head-Neck-Surg. 1996 Apr; 122(4): 414-6; ISSN: 0886-4470.

UNITED-STATES.

753. Wiezer, M. J.; Franssen, H.; Rinkel, G. J.; Wokke, J. H. Meralgia Paraesthetica:

differential diagnosis and follow-up. Muscle-Nerve. 1996 Apr; 19(4): 522-4;

ISSN: 0148-639X.

UNITED-STATES.

754. Williams, C. R.; O'Flynn, E.; Clarke, N. M.; Morris, R. J. Torticollis secondary to

ocular pathology. J-Bone-Joint-Surg-Br. 1996 Jul; 78(4): 620-4; ISSN: 0301-

620X.

ENGLAND. We report a series of 15 children, six male and nine female, of

average age 20 months, seen at a paediatric orthopaedic clinic with torticollis.

Orthopaedic examination revealed a normal range of neck movement in all cases but

in seven there was palpable tightness in the absence of true shortening or

contracture of the sternomastoid muscle. The patients were prospectively referred

for ocular examination. In five of the 15 an ocular cause for the torticollis was

detected with underaction of the superior oblique muscle in three, paresis of the

lateral rectus muscle in one and nystagmus in one. Another two patients were found

to have an abnormal ocular examination which was thought to be unrelated to their

torticollis. Three of the patients with ocular torticollis required extra-ocular muscle

surgery to abolish the head tilt and one of these had a tight sternomastoid muscle.

Two of the non-ocular group had surgical release of the sternomastoid muscle; in

the rest, the condition either resolved with physiotherapy or required no active

treatment. We recommend that all patients with torticollis and no clear orthopaedic

cause are referred for ocular assessment since it is not possible clinically to

distinguish ocular from non-ocular causes.

755. Williams, J. R. Optic neuritis in a child. Pediatr-Emerg-Care. 1996 Jun; 12(3): 210-2;

ISSN: 0749-5161.

UNITED-STATES. A previously healthy four-year-old girl developed bilateral

loss of vision over one day. Her course of optic nerve abnormalities, a related

neurologic episode, and the response to corticosteroid therapy is presented. The

diagnosis of optic neuritis in a child is made infrequently in an emergency

department, but it must be considered in any patient presenting with an acute

nontraumatic loss of vision. The characteristic findings associated with optic

neuritis, and a review of childhood cases, are discussed.. 0; 0.

756. Willison, H. J.; O'Hanlon, G. M.; Paterson, G.; Veitch, J.; Wilson, G.; Roberts, M.;

Tang, T.; Vincent, A. A somatically mutated human antiganglioside IgM antibody

that induces experimental neuropathy in mice is encoded by the variable region

heavy chain gene, V1-18 [see comments]. J-Clin-Invest. 1996 Mar 1; 97(5): 1155-

64; ISSN: 0021-9738.

Note: Comment in: J Clin Invest 1996 Mar 1;97(5):1136-7.

UNITED-STATES. IgM paraproteins associated with autoimmune peripheral

neuropathy and anti-Pr cold agglutinins react with sialic acid epitopes present on

disialylated gangliosides including GD1b, GT1b, GQ1b, and GD3. A causal

relationship between the paraprotein and the neuropathy has never been proven

experimentally. From peripheral blood B cells of an affected patient, we have

cloned a human hybridoma secreting an antidisialosyl IgM mAb, termed Ha1, that

shows identical structural and functional characteristics to its serum counterpart.

Variable region analysis shows Ha1 is encoded by the same VH1 family heavy

chain gene, V1-18, as the only other known anti-Pr antibody sequence and is

somatically mutated, suggesting that it [correction of is] arose in vivo in response to

antigenic stimulation. In the rodent peripheral nervous system, Ha1

immunolocalizes to dorsal root ganglia, motor nerve terminals, muscle spindles,

myelinated axons, and nodes of Ranvier. After intraperitoneal injection of affinity-

purified antibody into mice for 10 d, electrophysiological recordings from the

phrenic nerve-hemidiaphragm preparation demonstrated impairment of nerve

excitability and a reduction in quantal release of neurotransmitter. These data

unequivocally establish that an antidisialosyl antibody can exert pathophysiological

effects on the peripheral nervous system and strongly support the view that the

antibody contributes to the associated human disease.. 0; 0; 0; 0; 0; 0.

757. Wilson, J. R. Electrical studies as a prognostic factor in the surgical treatment of carpal

tunnel syndrome [letter]. J-Hand-Surg-Am. 1996 May; 21(3): 524-6; ISSN: 0363-

5023.

UNITED-STATES.

758. Wohrle, J. C.; Amoiridis, G.; Heye, N.; Przuntek, H. Acute multifocal motor

neuropathy with early spontaneous recovery: a distinct syndrome from Guillain-

Barre syndrome? Electromyogr-Clin-Neurophysiol. 1996 Jan; 36(1): 37-41; ISSN:

0301-150X.

BELGIUM. We describe a case of acute multifocal motor neuropathy with normal

sensory conduction studies in the nerve segments of severe motor conduction

block. Antiganglioside antibodies were not detected in serum and the patient

recovered spontaneously. The clinical picture and course of time of the illness

allowed the diagnosis of a Guillain-Barre syndrome (GBS). The

electrophysiological findings closely matched the typical findings of chronic

multifocal motor neuropathy with persistent conduction block. From these

similarities, we conclude that acute and chronic forms of acquired demyelinating

motor neuropathies have to be accepted as variants of acute GBS and chronic

inflammatory demyelinating polyneuropathy (CIDP), respectively. We suggest that

the conduction block cannot always be attributed to antiganglioside antibodies, as

chronic cases without antiganglioside antibodies have also been reported and

further elevation of antibody titres has been seen after spontaneous recovery.. 0; 0.

759. Wokke, J. H.; Morris, J. H.; Donaghy, M. Lymphoma, paraproteinaemia, and

neuropathy [clinical conference]. J-Neurol-Neurosurg-Psychiatry. 1996 Jun; 60(6):

684-9; ISSN: 0022-3050.

ENGLAND. 0.

760. Wokke, J. H.; van, den Berg LH; van Schaik, J. P. Sixth nerve palsy from a CNS

lesion in chronic inflammatory demyelinating polyneuropathy [letter]. J-Neurol-

Neurosurg-Psychiatry. 1996 Jun; 60(6): 695-6; ISSN: 0022-3050.

ENGLAND.

761. Wolansky, L. J.; Stewart, V. A.; Pramanik, B. K.; Heary, R. F.; Cho, E. S.;

Schulder, M.; Pak, J. Giant paraganglioma of the cauda equina in adolescence:

magnetic resonance imaging demonstration. J-Neuroimaging. 1996 Jan; 6(1): 54-6;

ISSN: 1051-2284.

UNITED-STATES. A case of giant paraganglioma of the cauda equina is

presented. This is only the second reported case in a minor and the first with

magnetic resonance imaging. At 13 cm long, this is the largest paraganglioma of the

cauda equina ever recorded.

762. Wolf, H. K.; Buslei, R.; Schmidt Kastner, R.; Schmidt Kastner, P. K.; Pietsch, T.;

Wiestler, O. D.; Bluhmke, I. NeuN: a useful neuronal marker for diagnostic

histopathology. J-Histochem-Cytochem. 1996 Oct; 44(10): 1167-71; ISSN: 0022-

1554.

UNITED-STATES. The monoclonal antibody A60 specifically recognizes the

DNA-binding, neuron-specific protein NeuN, which is present in most neuronal

cell types of vertebrates. In this study we demonstrate the potential use of NeuN as

a diagnostic neuronal marker using a wide range of formalin-fixed, paraffin-

embedded human surgical and autopsy specimens from the central and peripheral

nervous system. After microwave antigen retrieval, almost all neuronal populations

revealed strong immunoreactivity for NeuN in nuclei, perikarya, and some

proximal neuronal processes, whereas more distal axon cylinders and dendritic

ramifications were not stained. The stain greatly enhanced the gray matter

architecture. NeuN immunoreactivity was not detected in Purkinje cells, most

neurons of the internal nuclear layer of the retina, and in sympathetic chain ganglia.

We examined nine gangliogliomas and 14 dysembryoplastic neuroepithelial tumors,

one ganglioneuroma, and one dysplastic cerebellar gangliocytoma. The neuronal

component of all of these lesions showed marked immunoreactivity for NeuN. In

addition, NeuN immunoreactivity was focally seen in one of seven

medulloblastomas with prominent neuronal differentiation. There was no staining

of non-neuronal structures. The results indicate that NeuN immunoreactivity is a

sensitive and specific neuronal marker in formalin-fixed, paraffin-embedded

tissues, and may be useful in diagnostic histopathology.. 0; 0; 0; 0; 0; 50-00-0.

763. Wolin, M. J.; Trent, R. G.; Lavin, P. J.; Cornblath, W. T. Oculopalatal myoclonus

after the one-and-a-half syndrome with facial nerve palsy. Ophthalmology. 1996

Jan; 103(1): 177-80; ISSN: 0161-6420.

UNITED-STATES. PURPOSE: The one-and-a-half syndrome is an eye

movement disorder characterized by a unilateral gaze palsy and an ipsilateral

internuclear ophthalmoplegia. The authors describe a previously unrecognized

association between the one-and-a-half syndrome and oculopalatal myoclonus

(OPM). METHODS: Five clinical cases are presented, with pertinent physical

findings and radiologic studies. RESULTS: A previously unrecognized association

of the one-and-a-half syndrome with subsequent development of OPM appears to

exist. Involvement of the facial nerve in patients with the one-and-a-half syndrome

may be a predictor of the subsequent development of OPM. CONCLUSION:

Patients with the one-and-a-half syndrome and facial nerve palsy should be

followed closely for possible future development of OPM.

764. Wood, RP 2nd; Milgrom, H. Vocal cord dysfunction. J-Allergy-Clin-Immunol. 1996

Sep; 98(3): 481-5; ISSN: 0091-6749.

UNITED-STATES.

765. Woodruff, J. M. Pathology of the major peripheral nerve sheath neoplasms. Monogr-

Pathol. 1996; 38: 129-61; ISSN: 0077-0922.

UNITED-STATES.

766. Wright, K. W. Partially accommodative esotropia: should you overcorrect and cut the

plus? [letter]. Arch-Ophthalmol. 1996 Nov; 114(11): 1429-31; ISSN: 0003-9950.

UNITED-STATES.

767. Wyman, D. O. You're the flight surgeon. Thoracic outlet syndrome. Aviat-Space-

Environ-Med. 1996 May; 67(5): 494-5; ISSN: 0095-6562.

UNITED-STATES.

768. Yagi, T.; Ohyama, Y. Three-dimensional analysis of nystagmus induced by neck

vibration. Acta-Otolaryngol-Stockh. 1996 Mar; 116(2): 167-9; ISSN: 0001-6489.

NORWAY. The role of dorsal neck proprioceptive inputs to vestibular

compensation was investigated in 11 patients with unilateral vestibular dysfunction.

Subjects had neither spontaneous nystagmus nor disequilibrium, indicating that

they were well compensated. Vibratory stimulation to the dorsal neck produced

marked nystagmus. All subjects showed horizontal component directed towards the

contralateral side of the lesion. Vertical and torsional components of the nystagmus

were exhibited by 10 and 7 subjects, respectively. These results indicate that the

neck vibration caused a discompensation in vestibularly well compensated subjects

after unilateral dysfunction. In addition, the influence of neck afferent on the

vestibulo-ocular reflex pathways, not only of the horizontal, but also the vertical

systems, is discussed.

769. Yagihashi, S.; Yamagishi, S.; Wada, R.; Sugimoto, K.; Baba, M.; Wong, H. G.;

Fujimoto, J.; Nishimura, C.; Kokai, Y. Galactosemic neuropathy in transgenic

mice for human aldose reductase. Diabetes. 1996 Jan; 45(1): 56-9; ISSN: 0012-

1797.

UNITED-STATES. We studied the functional consequences of an enhanced

polyol pathway activity, elicited with galactose feeding, on the peripheral nerve of

transgenic mice expressing human aldose reductase. Nontransgenic littermate mice

were used as controls. With a quantitative immunoassay, the expression level of

human aldose reductase in the sciatic nerve was 791 +/- 44 ng/mg protein (mean +/-

SE), about 25% of that in human sural nerve. When the transgenic mice were fed

food containing 30% galactose, significant levels of galactitol accumulated in the

sciatic nerve. Galactose feeding of nontransgenic littermate mice led to a 10-fold

lower accumulation of galactitol. Galactose feeding for 16 weeks caused a

significant and progressive decrease in motor nerve conduction velocity in

transgenic mice to 80% of the level of galactose-fed littermate mice, which was not

significantly different from that of galactose-free littermate mice. A morphometric

analysis of sciatic nerve detected > 10% reduction of mean myelinated fiber size but

no alterations of myelinated fiber density in galactose-fed transgenic mice compared

with other groups. The functional and structural changes that develop in galactose-

fed transgenic mice are similar to those previously reported in diabetic animals. The

results of these studies suggest that transgenic mice expressing human aldose

reductase may be a useful model not only for defining the role of the polyol

pathway in diabetic neuropathy but also for identifying and characterizing effective

inhibitors specific for human aldose reductase.. EC 1.1.1.21; 0; 26566-61-0; 608-

66-2.

770. Yaginuma, Y.; Kawamura, M.; Ishikawa, M. Landry-Guillain-Barre-Strohl syndrome

in pregnancy. J-Obstet-Gynaecol-Res. 1996 Feb; 22(1): 47-9; ISSN: 1341-8076.

JAPAN. We report here a case of Landry-Guillain-Barre-Strohl syndrome

(LGBSs) in pregnancy. The patient had rubella viral infection, and the patient's

inspiratory ability rapidly weakened. In the end, aggressive plasmapheresis

prevented the need for respiratory support and the inherent risk it poses in terms of

maternal morbidity and mortality.

771. Yamada, S.; Lonser, R. R.; Iacono, R. P.; Morenski, J. D.; Bailey, L. Bypass

coaptation procedures for cervical nerve root avulsion. Neurosurgery. 1996 Jun;

38(6): 1145-51; discussion 1151-2; ISSN: 0148-396X.

UNITED-STATES. In the past, patients with cervical spinal nerve root avulsions

were resigned to accept a natural crippling from upper extremity neurological

deficits. Recently, bypass coaptation procedures have resulted in functional return

of denervated muscles after such avulsions, much to the appreciation of patients.

Presented are 12 patients with avulsion of cervical spinal nerve roots that form

either the brachial plexus upper trunk (n = 7), lower trunk (n = 1), or all three

trunks (n = 4). The patients underwent the new bypass coaptation procedures with

complete or partial return of motor and sensory function, which otherwise would

be totally nonfunctional. The most dramatic results were noted in those patients

who underwent operations within 6 weeks of injury. The results of these

procedures offer patients a valid therapeutic modality for an enhanced quality of life

after cervical nerve root avulsion.

772. Yamawaki, M.; Vasquez, A.; Ben Younes, A.; Yoshino, H.; Kanda, T.; Ariga, T.;

Baumann, N.; Yu, R. K. Sensitization of Lewis rats with sulfoglucuronosyl

paragloboside: electrophysiological and immunological studies of an animal model

of peripheral neuropathy. J-Neurosci-Res. 1996 Apr 1; 44(1): 58-65; ISSN: 0360-

4012.

UNITED-STATES. Antibodies against sulfoglucuronosyl glycosphingolipids

(SGGLs) are known to be present in sera of patients with chronic polyneuropathy

associated with IgM paraproteinemia. We recently studied rats sensitized with

sulfoglucuronosyl paragloboside (SGPG), a major SGGL species, emulsified with

keyhold limpet hemocyanin and Freund's adjuvant. The titer of the IgM class

antibodies against SGPG increased up to 1:1,600, while that of the IgG class

increased up to 1:800 2 weeks after sensitization. The antibodies showed a high

degree of antigenic specificity; no cross-reactivity with other brain glycolipids could

be detected. They, however, reacted with human myelin-associated glycoprotein

(MAG) by Western blot analysis, but not with rat MAG. These animal models

showed minor but clear clinical signs of neuropathy, consisting of mild tail muscle

tone loss and walking disabilities. Electrophysiological examination of the sciatic

nerves revealed nerve conduction abnormalities which consisted of conduction

block and mild decrease in conduction velocity. Thus, our results support the

concept that anti-SGPG antibodies may play an important pathogenetic role in this

type of chronic neuropathy.. 0; 0.

773. Yang, J.; Letts, M. Thoracic outlet syndrome in children. J-Pediatr-Orthop. 1996 Jul;

16(4): 514-7; ISSN: 0271-6798.

UNITED-STATES. Thoracic outlet syndrome, although uncommon in children,

may be precipitated during rapid growth in adolescence, especially in association

with a cervical rib. Four children exhibited the aching, tiredness in the limb, and

occasional paresthesia in adolescence. Two had a cervical rib requiring removal,

after which the symptoms disappeared. The other two were treated nonoperatively

with shoulder-strengthening exercises and avoidance of posturing that elicited the

symptoms. It is proposed that in a child, the anatomy of the thoracic outlet is

constantly changing with growth and development of the structures that form the

thoracic outlet, as well as those that traverse the outlet itself. A conservative

approach to these symptoms is warranted in the first instance to allow further

growth and remodeling of the thoracic outlet, which may be sufficient to

accommodate the nerve roots and brachial plexus and alleviate the symptoms.

Excision of the cervical rib in the presence of increasing and chronic symptoms can

be quit beneficial.

774. Yang, J.; Simonson, T. M.; Ruprecht, A.; Meng, D.; Vincent, S. D.; Yuh, W. T.

Magnetic resonance imaging used to assess patients with trigeminal neuralgia. Oral-

Surg-Oral-Med-Oral-Pathol-Oral-Radiol-Endod. 1996 Mar; 81(3): 343-50; ISSN:

1079-2104.

UNITED-STATES. To assess the value of magnetic resonance imaging in the

evaluation of trigeminal neuralgia, 51 patients were studied by magnetic resonance

imaging after a trigeminal protocol. Clinical and magnetic resonance imaging results

were correlated. Seventeen (33%) nonvascular abnormalities and 27 (53%)

vascular contacts or compressions of the trigeminal nerve were demonstrated. Of

the patients younger than of 29 and 39 years of age, 100% and 45%, respectively,

had a tumor or multiple sclerosis compared with 20% and 18% of those older than

40 and 60 years of age, respectively. One third of the patients with pain in more

than one branch of the trigeminal nerve had tumors. On the basis of this study,

magnetic resonance imaging may be useful in discovering underlying pathoses

associated with trigeminal neuralgia if patients have failed to respond to an initial

conservative treatment. The patients most likely to exhibit significant magnetic

imaging resonance findings are young and with pain in more than one trigeminal

branch.

775. Yeh, Y. F.; Hsieh, F. Y.; Chen, S. T.; Ng, S. H. Magnetic resonance images of

Tolosa-Hunt syndrome before and after steroid therapy. J-Formos-Med-Assoc.

1996 Jul; 95(7): 572-4; ISSN: 0929-6646.

TAIWAN. Tolosa-Hunt syndrome (THS) is characterized by painful

ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous

sinus. Steroid therapy dramatically reverses the clinical signs and symptoms of

THS. We report a typical case of THS, in which MRI showed a mass lesion in the

right cavernous sinus with intermediate signal intensity on T1-weighted images,

hypointensity on T2-weighted images and positive gadolinium enhancement. This

24-year-old man had three episodes of painful opthalmoplegia in a period of 9

months. The side of the involved cavernous sinus was different in the first and the

second episodes of THS. The symptoms lasted for 1 month and the patient

recovered spontaneously from these two episodes. In the third episode, the right

pupil was also involved and the light reflex was lost. After steroid therapy, the

ocular symptoms improved rapidly within 48 hours and the mass lesion in the right

cavernous sinus on MRI was markedly diminished by the 7th day of treatment..

50-02-2.

776. Yen, M. Y.; Wei, Y. H.; Liu, J. H. Stargardt's type maculopathy in a patient with

11778 Leber's optic neuropathy. J-Neuroophthalmol. 1996 Jun; 16(2): 120-3;

ISSN: 1070-8022.

UNITED-STATES. A 13-year-old boy presented with a 2-month history of

blurred vision. Visual acuity was 20/200 in both eyes. Ophthalmoscopic

examination revealed normal discs and "beaten bronze atrophy" in the maculae.

Subsequently, progressive vision loss with optic atrophy occurred over the next

few years. Fluorescein angiographic findings were compatible with Stargardt's

maculopathy. Because his cousin developed sequential vision loss diagnosed as

Leber's hereditary optic neuropathy, molecular genetic analysis was performed on

blood mitochondrial DNA (mtDNA) from our patient, his cousin with vision loss,

and another three asymptomatic cousins. The results showed that they all harbored

homoplasmic G to A point mutations at nucleotide position 11778 of the ND4 gene

in mtDNA.. 0.

777. Yokota, T.; Inaba, A.; Yuki, N.; Ichikawa, T.; Tanaka, H.; Saito, Y.; Kanouchi, T.

The F wave disappears due to impaired excitability of motor neurons or proximal

axons in inflammatory demyelinating neuropathies. J-Neurol-Neurosurg-

Psychiatry. 1996 Jun; 60(6): 650-4; ISSN: 0022-3050.

ENGLAND. OBJECTIVES--Investigation of pathophysiology of F wave

disappearance in demyelinating neuropathies. METHODS--The peripheral motor

nerve conduction was studied by motor evoked potential (MEP) on transcranial

magnetic stimulation as well as conventional nerve conduction studies before and

after the treatment in 26 patients with inflammatory demyelinating neuropathies. In

addition, serum antiganglioside antibodies in the acute or active stage were

examined. RESULTS--The F wave was abolished in 10 patients. Seven of the 10

patients showed motor evoked potentials (MEPs) on transcranial magnetic

stimulation that ranged from 1-4 mV. In six of them the F wave reappeared in the

recovery stage, but the MEP size did not change. This may be caused by humoral

factors, because the F wave reappeared immediately after plasma exchange or

intravenous immunoglobulin treatment. A correlation of F wave disappearance with

the presence of serum antiganglioside antibodies was found. CONCLUSIONS--

The major pathophysiology of F wave disappearance in demyelinating neuropathies

is impairment of motor neuron excitability or prolonged refractoriness of the most

proximal axon for backfiring. The conventional interpretation that absent F waves

suggest a conduction block at the proximal site is often inadequate.. 0; 0; 37758-

47-7.

778. Yoshimura, T.; Satake, M.; Kobayashi, T. Connexin43 is another gap junction protein

in the peripheral nervous system. J-Neurochem. 1996 Sep; 67(3): 1252-8; ISSN:

0022-3042.

UNITED-STATES. That many cells express more than one connexin (Cx) led us

to examine whether Cxs other than Cx32 are expressed in the PNS. In addition to

Cx32 mRNA, Cx43 and Cx26 mRNAs were detected in rat sciatic nerve by

northern blot analysis. Cx43 mRNA, but not Cx26 mRNA, was expressed in both

the primary Schwann cell culture and immortalized Schwann cell line (T93). The

steady-state levels of the Cx43 mRNA in the primary Schwann cell culture

increased 2.0-fold with 100 microM forskolin, whereas that of Po increased 7.0-

fold. Immunoreactivity to Cx43 was detected on western blots of cultured Schwann

cells, T93 cells, and sciatic nerves but not on blots of PNS myelin.

Immunohistochemical study using human peripheral nerves revealed that anti-Cx43

antibody stained cytoplasm around nucleus of Schwann cells but not myelin,

confirming western blot results. Although Po expression was markedly decreased

by crush injury of the sciatic nerves, Cx43 expression showed no apparent change.

Developmental profiles showed that Cx43 expression in the sciatic nerve increased

rapidly after birth, peaked at about postnatal day 6, and then decreased gradually to

a low level. In adult rats, the Cx43 mRNA value was much lower than that of

Cx32. These findings suggest that Cx43 is localized in Schwann cell bodies and

that, compared with Po, its expression is less influenced by axonal contact and

cyclic AMP levels. The high expression on postnatal day 6 indicates that Cx43 may

be related to PNS myelination. Cx43 is another gap junction, but its function

appears to differ from that of Cx32, as judged by the differences in their

localization and developmental profiles.. 0; 0.

779. Yoshioka, R.; Dyck, P. J.; Chance, P. F. Genetic heterogeneity in Charcot-Marie-

Tooth neuropathy type 2. Neurology. 1996 Feb; 46(2): 569-71; ISSN: 0028-3878.

UNITED-STATES. Charcot-Marie-Tooth neuropathy type 2 (CMT2) is a common

inherited axonal neuropathy. The locus for one form of CMT2 (CMT2A) is

assigned to the short arm of chromosome 1. There is genetic heterogeneity in

CMT2 because additional pedigrees do not demonstrate linkage to chromosome 1

and are designated as CMT2B. Further clinical heterogeneity is suggested by

CMT2 pedigrees with diaphragm and vocal cord weakness and are designated as

CMT2C. To address the possible genetic distinction between CMT2A and CMT2C,

we tested markers from the CMT2A locus for linkage in a large CMT2C pedigree.

There was no evidence to support linkage of the CMT2C gene to the region of the

CMT2A locus on chromosome 1. CMT2C is not an allelic variant of CMT2A. This

analysis provides further evidence for genetic heterogeneity within inherited axonal

neuropathies.. 0; 0.

780. Yuki, N.; Takahashi, M.; Saito, K.; Yoshino, H. Traveler's diarrhea, Campylobacter

jejuni, and acute motor axonal neuropathy [letter]. Ann-Neurol. 1996 Mar; 39(3):

416-7; ISSN: 0364-5134.

UNITED-STATES.

781. Zawadzka Tolloczko, W. [Usefulness of EEG examination in the diagnosis of multiple

sclerosis with particular reference to its prognostic importance in retrobulbar

neuritis]. Przydatnosc badania eeg w diagnostyce stwardnienia rozsianego, ze

szczegolnym uwzglednieniem jego znaczenia rokowniczego w przypadkach

pozagalkowego zapalenia nerwu wzrokowego. Neurol-Neurochir-Pol. 1996 Jan;

30(1): 29-37; ISSN: 0028-3843.

POLAND. It is known from literature that 40-60% of eeg recordings from SM

patients show changes. These changes are: slow generalised activity, focal

changes, and sharp waves and spikes occurring in bursts. It is established that optic

neuritis often precedes the occurrence of neurological focal changes in SM. The

goal of this work was the analysis of eeg recordings from SM patients before and

after treatment during disease exacerbation and from patients with optic neuritis

without neurological changes. Eeg recordings in optic neuritis showed changes

similar to those observed in SM. Follow-up studies proved that patients with

changes in eeg during neuritis were at a risk group; absence of such changes does

not exclude the possibility of SM. Patients after neuritis should periodically

undergo neurological examination.

782. Zbar, R. I.; Smith, R. J. Vocal fold paralysis in infants twelve months of age and

younger. Otolaryngol-Head-Neck-Surg. 1996 Jan; 114(1): 18-21; ISSN: 0194-

5998.

UNITED-STATES. Seventeen cases of unilateral or bilateral vocal fold paralysis

were diagnosed in infants younger than 12 months from 1991 to 1994 at the

University of Iowa Hospitals and Clinics. Eight (47%) children with left vocal fold

paralysis had a history of prior thoracic surgery--two to repair complex congenital

anomalies and six to ligate a patent ductus arteriosus. During the study period, a

total of 81 patent ductus arteriosus ligations were performed, yielding a 7.4%

postoperative incidence of vocal fold paralysis. Seven (41%) children had

idiopathic vocal fold paralysis (3 right, 1 left, 3 bilateral). Two (12%) children had

VFP caused by central nervous system pathology (1 right, 1 bilateral).

Tracheotomy was not required in any case. Prognosis for vocal fold paralysis

varied with cause. With left vocal fold paralysis caused by thoracic surgery, no

improvement was noted after an average follow-up of 6 months; with idiopathic

vocal fold paralysis infants improved within an average of 6 weeks of diagnosis;

with vocal fold paralysis caused by central nervous system pathology, treatment of

the underlying condition was followed by return of vocal cord function.

Irrespective of cause, the morbidity associated with vocal fold paralysis is minimal.

Although tracheotomy is not required, careful airway observation is important.

Differences and similarities of these results with other studies are discussed.

783. Zbaren, P.; Becker, M. Schwannoma of the brachial plexus. Ann-Otol-Rhinol-

Laryngol. 1996 Sep; 105(9): 748-50; ISSN: 0003-4894.

UNITED-STATES.

784. Zenone, T.; Bastion, Y.; Salles, G.; Rieux, C.; Morel, D.; Felman, P.; Espinouse, D.;

Bryon, P. A.; Coiffier, B. POEMS syndrome, arterial thrombosis and

thrombocythaemia. J-Intern-Med. 1996 Aug; 240(2): 107-9; ISSN: 0954-6820.

ENGLAND. The case of a 22-year-old man with polyneuropathy, endocrinopathy,

skin change and monoclonal gammopathy of IgG-lambda type is described. There

was no solitary plasmocytoma, osteosclerotic myeloma or Castleman's disease.

However, significant thrombocytosis occurred and the patient developed arterial

thrombosis, these were attributed to essential thrombocythaemia in the absence of

other aetiological factors.

785. Ziegler, D. Diagnosis and management of diabetic peripheral neuropathy. Diabet-Med.

1996; 13 Suppl 1: S34-8; ISSN: 0742-3071.

ENGLAND. EC 1.1.1.21; 0; 0; 0; 82964-04-3.

786. Zimmern, P. E.; Tanelian, D. Re: Urological symptomatology in patients with reflex

sympathetic dystrophy [letter]. J-Urol. 1996 Nov; 156(5): 1782-3; ISSN: 0022-

5347.

UNITED-STATES.

787. Zislina, N. N.; Shamshinova, A. M.; Kozhevnikova, O. M.; Loran, S. I.

[Comprehensive electrophysiological study of the system of vision in children and

adolescents with congenital myopia and diseases of the retina and optic nerve].

Kompleksnoe elektrofiziologicheskoe issledovanie zritel'noi sistemy u detei i

podrostkov s vrozhdennoi blizorukost'iu, zabolevaniiami setchatki i zritel'nogo

nerva. Vestn-Oftalmol. 1996 Jan; 112(1): 20-3; ISSN: 0042-465X.

RUSSIA. Visual evoked potentials were analyzed and general and local

electroretinography carried out in 83 children and adolescents (145 eyes) aged 7 to

16 with different ocular diseases. The latencies of P100 component of evoked

potentials reliably varied in different clinical groups. The role of

electrophysiological studies in the differential diagnosis of pathological processes

of different localization is shown, as is the role of time characteristics of evoked

potentials in discrimination between the functional and organic disorders in the

ocular system.

788. Zuurmond, W. W.; Langendijk, P. N.; Bezemer, P. D.; Brink, H. E.; de Lange, J. J.;

van loenen, A. C. Treatment of acute reflex sympathetic dystrophy with DMSO

50% in a fatty cream. Acta-Anaesthesiol-Scand. 1996 Mar; 40(3): 364-7; ISSN:

0001-5172.

DENMARK. Acute Reflex Sympathetic Dystrophy (acute RSD) was defined using

a reproducible classification. Elevated temperature of the affected extremity

("calor"), measured by the dorsal side of the observer's hand and mentioned by the

patient, pain ("dolor") measured by the Visual Analogue Scale (VAS), redness

("rubor"), edema ("tumor") and limited active range of motion ("functio laesa"), all

contributed to the classification system. Patients scoring 4 to 5 positive symptoms

were considered to have acute RSD. A prospective, randomized and double blind

study was performed in 32 patients, all suffering from acute RSD. In all of these

patients the primary injury was the result of a previous accident. One patient was

taken out of the study because of his surgery. The study involved treatment with a

fatty cream with 50% dimethyl sulfoxide (DMSO, group A), or without DMSO

(placebo, group B), both for 2 months. All patients received physiotherapy applied

within pain limits. Application of the creams resulted in both groups in an

improvement of RSD-scores and VAS-scores after 2 months. However, the

improvement of the RSD score in patients of group A (DMSO-group) was

significantly (P < 0.01) better compared to group B. The results suggest a certain

activity of DMSO 50% cream in patients suffering from RSD and is, therefore,

recommendable.. 0; 0; 0; 0; 67-68-5.