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Surface‐binding autoantibodies to cerebellar neurons in opsoclonus syndrome
Author(s) -
Blaes Franz,
Fühlhuber Verena,
Korfei Martina,
Tschernatsch Marlene,
Behnisch Wolfgang,
Rostasy Kevin,
Hero Barbara,
Kaps Manfred,
Preissner Klaus T.
Publication year - 2005
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.20539
Subject(s) - opsoclonus , autoantibody , neuroblastoma , myoclonus , cerebellum , pathogenesis , antibody , immunology , medicine , neuroscience , chemistry , biology , cell culture , genetics
Childhood opsoclonus‐myoclonus syndrome can occur with or without associated neuroblastoma. An autoimmune pathogenesis has been discussed for both forms. We show here that the majority of children with opsoclonus‐myoclonus syndrome (10/14) have autoantibodies binding to the surface of isolated rat cerebellar granular neurons. In some patients, these antibodies are masked by IgG binding to ubiquitous surface antigens, which could be removed by preincubation with the nonneuronal control cell line HEK 293. A newly introduced competitive binding assay showed that the surface binding is directed against the same autoantigen in different patients. Therefore, we hypothesize that opsoclonus‐myoclonus syndrome may be the result of an autoimmune process against a neuronal surface protein. Ann Neurol 2005