Document Detail


A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside.
MedLine Citation:
PMID:  2843079     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report 2 patients with a treatable, immune-mediated motor polyneuropathy associated with antibodies to defined neural antigens. In these patients asymmetrical weakness developed in one arm and progressed over 2 to 3 years to involve the other arm, legs, and trunk. Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis. However, repeated electrophysiological testing eventually showed multifocal conduction blocks in motor but not sensory fibers compatible with patchy selective demyelination. Serum testing by thin-layer chromatography and enzyme-linked immunosorbent assay revealed that both patients had high titers of antibody directed against GM1 and other gangliosides. Initial therapeutic trials of prednisone (100 mg daily for 4 to 6 months) and plasmapheresis were unsuccessful. Treatment with cyclophosphamide, however, was followed by marked improvement in strength in both patients.
Authors:
A Pestronk; D R Cornblath; A A Ilyas; H Baba; R H Quarles; J W Griffin; K Alderson; R N Adams
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of neurology     Volume:  24     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1988 Jul 
Date Detail:
Created Date:  1988-10-13     Completed Date:  1988-10-13     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  73-8     Citation Subset:  IM    
Affiliation:
Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD 21205.
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MeSH Terms
Descriptor/Qualifier:
Adult
Autoantibodies / analysis*
Autoimmune Diseases / drug therapy*,  immunology
Cyclophosphamide / therapeutic use*
Female
G(M1) Ganglioside / immunology*
Humans
Infusions, Intravenous
Male
Middle Aged
Motor Neurons / immunology*
Neuromuscular Diseases / drug therapy*,  immunology
Synaptic Transmission / drug effects
Grant Support
ID/Acronym/Agency:
1RO1 NS23719/NS/NINDS NIH HHS; P01 NS22849/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/Autoantibodies; 37758-47-7/G(M1) Ganglioside; 50-18-0/Cyclophosphamide

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


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