Is Roussy-Levy Syndrome the same as Charcot-Marie-Tooth Disease?

J Korean Neurol Assoc > Volume 12(1); 1994 > Article

Journal of the Korean Neurological Association 1994;12(1): 170-174.

Roussy-Levy Syndrome은 Charcot-Marie-Tooth Disease와 같은 것인가?

이재홍, 나덕렬,박성호,이광우

울산대학교 신경과. 서울중앙병원 신경과. 서울대학교 신경과.

Is Roussy-Levy Syndrome the same as Charcot-Marie-Tooth Disease?

Jae Hong Lee, M.D., Duk Lyul Na, M.D., Seong Ho Park, M.D.,Kwang Woo Lee, M.D.,

Department of Neurololgy, University of Ulsan, College of Medicine, Asan Medical Center Department of Neurology, College of Medicine, Seoul National University

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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