Document Detail

Clinical and experimental features of MuSK antibody positive MG in Japan.
MedLine Citation:
PMID:  17718696     Owner:  NLM     Status:  MEDLINE    
We investigated the presence of antibodies (Abs) against muscle-specific tyrosine kinase (MuSK) in Japanese myasthenia gravis (MG) patients. MuSK Abs were found in 23 (27%) of 85 generalized seronegative MG (SNMG) patients but not in any of the ocular MG patients. MuSK Ab-positive patients were characterized as having female dominance (M:F, 5:18), age range at onset 18 to 72 (median 45) years old, and prominent oculobulbar symptoms (100%) with neck (57%) or respiratory (35%) muscle weakness. Limb muscle weakness was comparatively less severe (52%), thymoma absent. Most patients had good responses to simple plasma exchange and steroid therapy. MuSK IgG from all 18 patients was exclusively the IgG 4 subclass and bound mainly with the MuSK Ig 1-2 domain. Serial studies of 12 individuals showed a close correlation between the variation in MuSK Ab titers and MG clinical severity (P = 0.01 by Kruskal-Wallis). MuSK Ab titers were sharply decreased in patients who had a good response to early steroid therapy or simple plasma exchange, but there was no change, or a rapid increase on exacerbation after thymectomy. Measurement of MuSK Ab titers aids in the diagnosis of MG and the monitoring of clinical courses after treatment.
K Ohta; K Shigemoto; A Fujinami; N Maruyama; T Konishi; M Ohta
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European journal of neurology : the official journal of the European Federation of Neurological Societies     Volume:  14     ISSN:  1468-1331     ISO Abbreviation:  Eur. J. Neurol.     Publication Date:  2007 Sep 
Date Detail:
Created Date:  2007-08-27     Completed Date:  2007-09-26     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  9506311     Medline TA:  Eur J Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  1029-34     Citation Subset:  IM    
Clinical Research Center, Utano National Hospital, Kyoto, Japan.
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MeSH Terms
Analysis of Variance
Antibodies / blood*
Epitope Mapping
Japan / epidemiology
Middle Aged
Myasthenia Gravis / blood*,  immunology*
Radioimmunoassay / methods
Receptor Protein-Tyrosine Kinases / immunology*
Receptors, Cholinergic / immunology*
Reg. No./Substance:
0/Antibodies; 0/Receptors, Cholinergic; EC protein, human; EC Protein-Tyrosine Kinases

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