Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis

Abstract

Opsoclonus-myoclonus syndrome is a distinct neurologic disorder characterized by opsoclonic eye movements, multifocal myoclonus, and ataxia, traditionally described as "dancing eyes, dancing feet." A presenting sign in 2% of children with neuroblastoma, it usually heralds a favorable prognosis for the tumor. Although opsoclonus-myoclonus syndrome usually presents at initial diagnosis or relapse, there are reports of delayed presentation, usually a few months after diagnosis. This report describes a patient with ganglioneuroblastoma who developed recurrent symptoms of opsoclonus-myoclonus syndrome 9 years after completing treatment, without evidence of recurrent tumor. Believed to be autoimmune in origin, opsoclonus-myoclonus syndrome frequently responds to immunomodulatory therapies, such as steroids or intravenous immunoglobulin. This patient did not respond adequately to either agent, so plasmapheresis, a less commonly used modality in opsoclonus-myoclonus syndrome, was attempted. His symptoms resolved after he received therapy with a combination of plasmapheresis and steroids over a 1-year period. After being slowly weaned off all therapy, he has been symptom-free now for over 3 years. Armstrong MB, Robertson PL, Castle VP. Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis.