Response to rituximab in a child with neuroblastoma and opsoclonus-myoclonus

Abstract

Opsoclonus-myoclonus (OM) is a paraneoplastic syndrome of probable autoimmune origin. Despite current therapies aimed at decreasing autoantibody formation, OM is difficult to control and may impact long-term neurologic outcome. We present a case of a 19-month-old patient who initially presented with OM, neuroblastoma and a constitutional cytogenetic abnormality t(5;12)(q11.2;q15). The patient's OM was recalcitrant to conventional therapies, but showed significant improvement following treatment with rituximab.