Document Detail


Identification of autoantigens in pediatric opsoclonus-myoclonus syndrome.
MedLine Citation:
PMID:  20477144     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Pediatric opsoclonus-myoclonus syndrome (OMS) is a rare disease, including eye movement disturbances, muscle jerks, ataxia and developmental disturbances as the main symptoms. In 50% of patients, OMS is associated with a neuroblastoma as a paraneoplastic neurological syndrome. Since autoantibodies have been detected in some patients and OMS patients respond to immunosuppression, an autoimmune etiology has been suspected in OMS. A variety of autoantibodies have been reported in association with OMS. In paraneoplastic OMS, some patients have anti-Hu antibodies, directed against a group of RNA-binding proteins expressed in neurons and tumor cells. Autoantibodies against alpha-enolase and KH-type splicing regulatory protein could also be demonstrated. However, these autoantibodies are not specific for OMS. By contrast, autoantibodies against surface epitopes of cerebellar granular cells and neuroblastoma cells have been demonstrated exclusively in OMS patients, and these autoantibodies have pathogenic effects on neuroblastoma cells. Taken together, OMS is associated with surface-binding autoantibodies with pathogenic effects, indicating a humoral immune-mediated process as the underlying cause for OMS.
Authors:
Franz Blaes; Verena F?hlhuber; Klaus T Preissner
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Expert review of clinical immunology     Volume:  3     ISSN:  1744-8409     ISO Abbreviation:  Expert Rev Clin Immunol     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2010-05-18     Completed Date:  2010-06-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101271248     Medline TA:  Expert Rev Clin Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  975-82     Citation Subset:  -    
Affiliation:
Justus-Liebig University, Department of Neurology, Am Steg 14, 35385 Giessen, Germany. franz.blaes@neuro.med.uni-giessen.de
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