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