J Korean Neurol Assoc.
1994 Mar;12(1):170-174.
Is Roussy-Levy Syndrome the same as Charcot-Marie-Tooth Disease?
- Affiliations
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- 1Department of Neurololgy, University of Ulsan, College of Medicine, Asan Medical Center, Korea.
- 2Department of Neurology, College of Medicine, Seoul National University, Korea.
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.
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