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Case Reports in Oncological Medicine
Volume 2012, Article ID 164082, 4 pages
Case Report

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

1Pediatric Hematology and Oncology Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran 15468-15514, Iran
2Division of Gastroenterology and Hepatology, Johns Hopkins University, Baltimore, MD 21218, USA

Received 22 August 2012; Accepted 15 October 2012

Academic Editors: S. Aksoy, K. Aogi, and F. A. Mauri

Copyright © 2012 Samin Alavi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.