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http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uid\

s=7664521 & dopt=Abstract

A case with hereditary motor and sensory neuropathy with multiple

cranial nerves involvement].

[Article in Japanese]

Nakamura N, Oka N, Nakamura S, Akiguchi I, Kashii S

Department of Neurology, Yasui Hospital.

We described a 62-year-old man presenting motor and

sensory neuropathy with multiple cranial

nerve involvement. He was admitted to our hospital for

visual loss and gait disturbance. Visual

disturbance appeared at age of 32 and have slowly

progressed until admission. At age of 55, he

noticed walking difficulty, which has progressed. On

neurological examinations we found cranial

nerve disturbance including the 2nd, 3rd, 4th, 5th, 6th,

7th and 12th nerves. Muscle atrophy and

sensory disturbance were observed in four limbs, most

strikingly at the distal lower limbs. The

electromyographic study indicated chronic denervation in

four limbs. The histological examination

on sural nerve biopsy specimens showed neuronal type

degeneration accompanied with a severe

loss of myelinated fibers and proliferation of

perineurial cells in this nerve. We diagnosed the

present case as HMSN type 6 documented by Dyck. To our

knowledge, the present case has

been the initial case accompanied with multiple cranial

nerve involvements.

PMID: 7664521, UI: 95393662

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