Document Detail


Evidence that Charcot-Marie-tooth disease with tremor coincides with the Roussy-Levy syndrome.
MedLine Citation:
PMID:  6509399     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
F Barbieri; A Filla; M Ragno; C Crisci; L Santoro; M Corona; G Campanella
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article    
Journal Detail:
Title:  The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques     Volume:  11     ISSN:  0317-1671     ISO Abbreviation:  Can J Neurol Sci     Publication Date:  1984 Nov 
Date Detail:
Created Date:  1985-02-06     Completed Date:  1985-02-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0415227     Medline TA:  Can J Neurol Sci     Country:  CANADA    
Other Details:
Languages:  eng     Pagination:  534-40     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Charcot-Marie-Tooth Disease / complications*,  pathology
Child
Female
Gait
Humans
Hypertrophy
Male
Middle Aged
Movement Disorders / genetics*
Muscular Atrophy / complications*
Pedigree
Peripheral Nerves / pathology*
Reflex, Abnormal / genetics*
Syndrome
Tremor / complications*,  pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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