Is Roussy-Levy Syndrome the same as Charcot-Marie-Tooth Disease?.
- Author:
Jae Hong LEE
1
;
Duk Lyul NA
;
Seong Ho PARK
;
Kwang Woo LEE
Author Information
1. Department of Neurololgy, University of Ulsan, College of Medicine, Asan Medical Center, Korea.
- Publication Type:Brief Communication
- MeSH:
Biopsy;
Charcot-Marie-Tooth Disease*;
Demyelinating Diseases;
Humans;
Microscopy, Electron;
Sural Nerve;
Tooth Diseases
- From:Journal of the Korean Neurological Association
1994;12(1):170-174
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
We report data on 2 members of a family affected by a dominantly inherited disorder closely resembling Roussy-Levy syndrome(RLS) Electrophysiological findings showed a marked decrease of motor and sensory conduction velocities and mild neurogenic damage. Light and electron microscopy of sural nerve biopsy showed a hypertrophic neuropathy with diffuse onion-bulb 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 1) is strongly supported.
