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J Korean Neurol Assoc. 1994;12(1):170-174.
- Is Roussy-Levy Syndrome the same as Charcot-Marie-Tooth Disease?
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Jae Hong Lee, M.D., Duk Lyul Na, M.D., Seong Ho Park, M.D.,Kwang Woo Lee, M.D.,
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Department of Neurololgy, University of Ulsan, College of Medicine,
Asan Medical Center
Department of Neurology, College of Medicine, Seoul National University
- Roussy-Levy Syndrome은 Charcot-Marie-Tooth Disease와 같은 것인가?
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이재홍, 나덕렬,박성호,이광우
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울산대학교 신경과. 서울중앙병원 신경과. 서울대학교 신경과.
- 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.
Keywords :
- 초록
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