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Evidence that Charcot-Marie-tooth disease with tremor coincides with the Roussy-Levy syndrome.
Can J Neurol Sci. 1984 Nov; 11(4 Suppl):534-40.CJ

Abstract

We report data on 3 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 EMG signs of 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 evidenced reduced internodal lengths and segmental demyelination. Other data from the literature on RLS are reviewed and discussed. 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.

Authors

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Pub Type(s)

Case Reports
Comparative Study
Journal Article

Language

eng

PubMed ID

6509399

Citation

Barbieri, F, et al. "Evidence That Charcot-Marie-tooth Disease With Tremor Coincides With the Roussy-Levy Syndrome." The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques, vol. 11, no. 4 Suppl, 1984, pp. 534-40.
Barbieri F, Filla A, Ragno M, et al. Evidence that Charcot-Marie-tooth disease with tremor coincides with the Roussy-Levy syndrome. Can J Neurol Sci. 1984;11(4 Suppl):534-40.
Barbieri, F., Filla, A., Ragno, M., Crisci, C., Santoro, L., Corona, M., & Campanella, G. (1984). Evidence that Charcot-Marie-tooth disease with tremor coincides with the Roussy-Levy syndrome. The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques, 11(4 Suppl), 534-40.
Barbieri F, et al. Evidence That Charcot-Marie-tooth Disease With Tremor Coincides With the Roussy-Levy Syndrome. Can J Neurol Sci. 1984;11(4 Suppl):534-40. PubMed PMID: 6509399.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Evidence that Charcot-Marie-tooth disease with tremor coincides with the Roussy-Levy syndrome. AU - Barbieri,F, AU - Filla,A, AU - Ragno,M, AU - Crisci,C, AU - Santoro,L, AU - Corona,M, AU - Campanella,G, PY - 1984/11/1/pubmed PY - 1984/11/1/medline PY - 1984/11/1/entrez SP - 534 EP - 40 JF - The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques JO - Can J Neurol Sci VL - 11 IS - 4 Suppl N2 - We report data on 3 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 EMG signs of 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 evidenced reduced internodal lengths and segmental demyelination. Other data from the literature on RLS are reviewed and discussed. 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. SN - 0317-1671 UR - https://www.unboundmedicine.com/medline/citation/6509399/Evidence_that_Charcot_Marie_tooth_disease_with_tremor_coincides_with_the_Roussy_Levy_syndrome_ L2 - http://www.diseaseinfosearch.org/result/6365 DB - PRIME DP - Unbound Medicine ER -