Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses.

Abstract

Opsoclonus-myoclonus-ataxia-syndrome (OMS) represents a rare neuroblastoma-associated paraneoplastic syndrome that commonly results in neurologic deficits despite tumor resection and immunosuppressive therapy. We describe the response of five such children to high-dose dexamethasone pulses including two patients in whom previous glucocorticoids, rituximab, and cytostatic drugs were not successful. All patients had MYCN non-amplified tumors that were detected 1 to 7 months after the onset of the OMS or ataxia. This treatment resulted in a good partial response in three and in complete remission in two patients. Our results show that dexamethasone pulses are likely to be useful for both, first-line- and salvage-therapy for OMS-patients.

• Authors: Florian Ertle, Wolfgang Behnisch, Naima Ali Al Mulla, Mohammed Bessisso, Dietz Rating, Gunhild Mechtersheimer, Barbara Hero, Andreas E Kulozik
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 50 Issue 3 Pg. 683-7 (Mar 2008) ISSN: 1545-5017 [Electronic] United States
• PMID: 17226843 (Publication Type: Case Reports, Clinical Trial, Journal Article, Multicenter Study)
• Copyright: (c) 2007 Wiley-Liss, Inc.

Chemical References

• Immunosuppressive Agents
• Dexamethasone

Topics

• Adrenal Gland Neoplasms (complications, diagnosis)
• Child, Preschool
• Dexamethasone (administration & dosage, therapeutic use)
• Drug Administration Schedule
• Female
• Humans
• Immunosuppressive Agents (administration & dosage, therapeutic use)
• Infant
• Male
• Neuroblastoma (complications, diagnosis)
• Opsoclonus-Myoclonus Syndrome (diagnosis, drug therapy, etiology)
• Pilot Projects
• Psychomotor Disorders (etiology)
• Remission Induction
• Salvage Therapy