Autoantibodies in childhood opsoclonus-myoclonus syndrome.
Opsoclonus-myoclonus syndrome or Dancing Eye Syndrome (OMS/DES) is a rare neurological disorder of children, which associates with neuroblastoma (NB) in approximately 50% of cases. We examined sera from five patients with (OMS-NB(+)) and five without NB (OMS-NB(-)) for autoantibodies. OMS-NB(-) IgG bound to the surface of a NB cell line, whereas IgG from OMS-NB(+) and from NB patients without OMS/DES bound only to permeabilised cells. Both OMS-NB(+) and OMS-NB(-) reduced proliferation of NB cells. We also present a case report of a child with OMS/DES without NB who made a complete recovery without treatment. Serum antibodies at presentation bound to the surface and decreased NB cell proliferation but had decreased 9 weeks later when the child was asymptomatic. These results demonstrate that sera from some OMS/DES patients contain IgG antibodies that are potentially pathogenic.
Blaes
F
F
Department of Neurology, Justus-Liebig University, Giessen, Germany.
Pike
M G
MG
Lang
B
B
D000704:Analysis of Variance; D000818:Animals; D001323:Autoantibodies / Q000097:blood* / Q000494:pharmacology; D015220:Calcium Channels / Q000276:immunology; D002460:Cell Line; D049109:Cell Proliferation / Q000187:drug effects*; D002531:Cerebellum / Q000378:metabolism; D002675:Child, Preschool; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D005968:Glutamate Decarboxylase / Q000378:metabolism; D006801:Humans; D007223:Infant; D008297:Male; D009447:Neuroblastoma / Q000097:blood / Q000150:complications / Q000276:immunology; D053578:Opsoclonus-Myoclonus Syndrome / Q000097:blood / Q000150:complications / Q000276:immunology*; D015221:Potassium Channels / Q000378:metabolism; D011485:Protein Binding / Q000187:drug effects; D011863:Radioimmunoassay / Q000379:methods; D051381:Rats; D013777:Tetrazoles / Q000378:metabolism; D013997:Time Factors
D001323:Autoantibodies; D015220:Calcium Channels; D015221:Potassium Channels; D013777:Tetrazoles; C037703:3-aminopropyl-5-tetrazole; D005968:Glutamate Decarboxylase