Clinical profile and outcome of children with opsoclonus-myoclonus syndrome.

The opsoclonus-myoclonus syndrome is a distinct disorder characterized by opsoclonus, myoclonus, and ataxia, along with marked irritability and behavioral changes. Worldwide, data on its epidemiology, clinical features, and outcome are scarce. The aim of the study was to determine the clinical profile and outcome of children with this disorder. A retrospective study of all children admitted with a diagnosis of opsoclonus-myoclonus from 2000 to 2012 was done. Outcome was assessed on follow-up by direct assessment and by telephonic interview. Eleven patients with a diagnosis of opsoclonus-myoclonus were admitted over a 12-year period. Of the 11, 4 had paraneoplastic etiology. Children with paraneoplastic opsoclonus had more relapses and a poor outcome as compared to an idiopathic group. Paraneoplastic opsoclonus had a poor outcome in our experience.