Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies-Reference-Cited by-同舟云学术

Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies

Published:1986-08 Issue:3 Volume:13 Page:264-266
ISSN:0317-1671
Container-title:Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques
language:en
Short-container-title:Can. j. neurol. sci.

Author:

Ronen Gabriel M.,Lowry Noel,Wedge John H.,Sarnat Harvey B.,Hill Alan

Abstract

ABSTRACT:A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.

Publisher

Cambridge University Press (CUP)

Subject

Clinical Neurology,Neurology,General Medicine

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