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New Autoantibodies in Pediatric Opsoclonus–Myoclonus Syndrome
Author(s) -
KIRSTEN A.,
BECK S.,
FÜHLHUBER V.,
KAPS M.,
KREUTZ T.,
KORFEI M.,
SCHMITT S.,
PREISSNER K.T.,
BLAES F.
Publication year - 2007
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1196/annals.1423.027
Subject(s) - autoantibody , opsoclonus , neuroblastoma , myoclonus , pathogenesis , medicine , immunology , ataxia , cerebellar ataxia , pathology , antibody , biology , cell culture , genetics , psychiatry
: 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, α‐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 .