New autoantibodies in pediatric opsoclonus myoclonus syndrome.

Abstract:

:Opsoclonus-myoclonus syndrome (OMS) is a rare neurologic disorder comprising the main symptoms of eye-movement disturbances, muscle jerks, and severe ataxia. In children and adults, some cases are associated with a tumor as a paraneoplastic syndrome, whereas in children the paraneoplastic form is almost exclusively associated with neuroblastoma. The detection of autoantibodies in some OMS sera led to the hypothesis that the syndrome is of autoimmune origin. Beside autoantibodies against intracellular proteins, such as anti-Hu, alpha-enolase, and KHSRP, specific binding of autoantibodies to the surface of neuroblastoma cells and cerebellar granular neurons have been found. Antiproliferative and proapoptotic effects of these autoantibodies on neuroblastoma cell lines were noted as well. These results support the concept of a humoral autoimmune process in the pathogenesis of OMS.

journal_name

Ann N Y Acad Sci

authors

Kirsten A,Beck S,Fühlhuber V,Kaps M,Kreutz T,Korfei M,Schmitt S,Preissner KT,Blaes F

doi

10.1196/annals.1423.027

subject

Has Abstract

pub_date

2007-09-01 00:00:00

pages

256-60

eissn

0077-8923

issn

1749-6632

pii

1110/1/256

journal_volume

1110

pub_type

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