Opsoclonus-myoclonus syndrome: a new era of improved prognosis?

Publication date: Available online 27 March 2017
Source:Pediatric Neurology
Author(s): Armine Galstyan, Colin Wilbur, Kathryn Selby, Juliette Hukin
BackgroundOpsoclonus-myoclonus syndrome (OMS) is an autoimmune neurologic disorder characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. While long-term outcomes have historically been poor, including motor and cognitive disabilities, the advent of new and more aggressive immunotherapy regimens may be improving prognosis in OMS.MethodsA retrospective chart review was performed of all children diagnosed with OMS at BC Children's Hospital from 2000-2010. Neurologic outcomes were compared to those previously reported in the literature.ResultsTwelve children with OMS were identified, 4 of whom had an associated neuroblastoma. Two-thirds of patients received initial treatment with a combination of corticosteroids, intravenous immunoglobulin (IVIG), and an additional immunosuppressant agent. After a median follow-up of 3 years from diagnosis, 10 patients had no or minimal neurologic abnormalities. Two patients had poor outcome with significant cognitive impairment.ConclusionsThe majority of patients in this series were treated with early multimodal immunotherapy and neurologic outcomes were better than most historical reports. This is consistent with recent studies that suggest multimodal immunotherapy regimens may be improving the prognosis in this challenging disease. However, some did well with less aggressive treatment and further studies are required to determine optimal treatment approach.



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