|
KMID : 0359719940120010170
|
|
Journal of the Korean Neurological Association
1994 Volume.12 No. 1 p.170 ~ p.174
|
|
|
Is Roussy-Levy Syndrome the Same as Charcot-Marie-Tooth Disease?
|
|
|
|
|
Abstract
|
|
|
We report data on 2 members of a family affected by a dominantly ingerited disorder closely resembling Roussy-Levy syndrome(RLS). Electrophysiological findings showed a marked decrease of motor and sensory conduction velocities and ild neurogenic
damage. Light and electron microscopy of sural nerve biopsy showed a hypertrophic neuropathy with diffuse onion-buld formations and marked decrease of large size fibers. Teased fiber preparations revealed reduced internodal lengths and segmental
demyelination. The hypothesis that RLS is not a disease entity-but a hypertrophic-type of charcot-Marie Tooth disease with essential tremor(HMSN type 10 is strongly supported.
|
|
|
KEYWORD
|
|
|
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
  
|