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