Early rituximab therapy for paraneoplastic opsoclonus-myoclonus syndrome

Abstract

  Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder presenting with opsoclonus, myoclonus, and ataxia. Though several autoantibodies have been reported to be involved in OMS, the pathogenetic roles of these autoantibodies remain largely unknown. The prognosis of OMS remains unsatisfactory despite standard immune therapies. Herein, we report the case of an infant with OMS and the presence of anti-glutamate receptor δ2 (GluD2) and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies. Rituximab therapy at an early phase led to good prognosis. This case highlights the pathogenetic role of autoantibodies in OMS and the efficacy of early phase rituximab therapy.