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.