• Florian Ertle, Wolfgang Behnisch, Naima Ali Al Mulla, Mohammed Bessisso, Dietz Rating, Gunhild Mechtersheimer, Barbara Hero, and Andreas E Kulozik.
• Department of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, Germany.
• Pediatr Blood Cancer. 2008 Mar 1;50(3):683-7.

AbstractOpsoclonus-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.(c) 2007 Wiley-Liss, Inc.

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