Clinical Responses to Rituximab in a Case of Neuroblastoma with Refractory Opsoclonus Myoclonus Ataxia Syndrome

Samin Alavi,Mohammad Taghi Arzanian,Borhan Moradveisi,Ali Kord Valeshabad,Ali Aminasnafi

doi:10.1155/2012/164082

Abstract

Opsoclonus myoclonus ataxia syndrome (OMS) is a rare neurologic syndrome. In a high proportion of children, it is associated with neuroblastoma. The etiology of this condition is thought to be immune mediated. In children, immunotherapy with conventional treatments such as corticosteroids, intravenous immunoglobulin, adrenocorticotropic hormone, and even antiepileptic drugs has been tried. Recently rituximab has been used safely for refractory OMS in children with neuroblastoma. Our patient was a 3.5-year-old girl referred for ataxia and dancing eye movements starting since 1.5 years ago. She was diagnosed with neuroblastoma on imaging studies on admission. The OMS was refractory to surgical resection, chemotherapy, corticosteroids, and intravenous immunoglobulin. Patient received rituximab simultaneously with chemotherapy. The total severity score decreased by 61.1% after rituximab. Patient's ataxia markedly improved that she was able to walk independently after 6 months. Our case confirmed the clinical efficacy and safety of rituximab in a refractory case of OMS.

Highlights

Opsoclonus myoclonus ataxia syndrome (OMS) is a serious, but rare immune-mediated paraneoplastic syndrome with an incidence rate of 0.18 cases per million population per year which occurs in 1.8% to 3% of patients with neuroblastoma [1, 2]
Rituximab was approved for treatment of B cell non-Hodgkin lymphoma [13], it has been used for other types of autoimmune disorders [14,15,16]
We investigate the clinical responses to rituximab in a case of refractory OMS who did not respond to surgical resection of tumor and standard chemotherapy

Summary

Introduction

Opsoclonus myoclonus ataxia syndrome (OMS) is a serious, but rare immune-mediated paraneoplastic syndrome with an incidence rate of 0.18 cases per million population per year which occurs in 1.8% to 3% of patients with neuroblastoma [1, 2]. A monoclonal anti-CD20 antibody, has been reported to have favorable effects to subset refractory signs of OMS [8,9,10,11,12]. We investigate the clinical responses to rituximab in a case of refractory OMS who did not respond to surgical resection of tumor and standard chemotherapy. It was decided to continue rituximab at least for 4 doses while patient was under close observation for clinical improvement.

Results

Conclusion