Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome.
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.
Department of Neurology, Justus-Liebig-University, Giessen, Germany. franz.blaes@neuro.med.uni-giessen.de
Franz
F
Blaes
Verena
V
Fühlhuber
Martina
M
Korfei
Marlene
M
Tschernatsch
Wolfgang
W
Behnisch
Kevin
K
Rostasy
Barbara
B
Hero
Manfred
M
Kaps
Klaus T
KT
Preissner
D000954:Antigens, Surface / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D016022:Case-Control Studies; D002460:Cell Line; D002531:Cerebellum / Q000473:pathology*; D002675:Child, Preschool; D003163:Competitive Behavior / Q000502:physiology; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D006801:Humans; D007223:Infant; D008297:Male; D009474:Neurons / Q000276:immunology*; D020361:Paraneoplastic Syndromes, Nervous System / Q000276:immunology* / Q000473:pathology*
D000954:Antigens, Surface; D001323:Autoantibodies