RESPONSE TO RITUXIMAB AND PREDNISOLONE FOR OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME IN A CHILD WITH GANGLIONEUROBLASTOMA

Corapcioglu F., Mutlu H., KARA B., Inan N., AKANSEL G., Gurbuz Y., ...More

PEDIATRIC HEMATOLOGY AND ONCOLOGY, vol.25, no.8, pp.756-761, 2008 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 25 Issue: 8
  • Publication Date: 2008
  • Doi Number: 10.1080/08880010802341690
  • Journal Name: PEDIATRIC HEMATOLOGY AND ONCOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.756-761
  • Keywords: children, ganglioneuroblastoma, opsoclonus-myoclonus-ataxia syndrome, rituximab, NEUROBLASTOMA, THERAPY
  • Kocaeli University Affiliated: Yes

Abstract

Opsoclonus-myoclonus-ataxia (OMA) syndrome is a rare neurobehavioral paraneoplastic disorder in children with neuroblastic tumors. The neurologic symptoms are generally treated with a number of immunosupressive and immunomodulating agents. A 4-year-old previously healthy male patient was admitted to the authors' center with progressive ataxia, gait disturbance, diffuculty of speech, and opsoclonus. He had a diagnosis of ganglionueroblastoma at the thoracal paraspinal region. Following surgey, the patient received IVIG and prednisolone but his cerebellar symptoms progressed. Rituximab therapy was started and continued for total 8 weeks without any side effect. The authors observed excellent neurologic response in the patient at the 4th week of treatment. Rituximab is a new, promising, and safe therapy for OMA syndrome in children with neuroblastoma.