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Treatment of neuroblastoma‐related opsoclonus–myoclonus–ataxia syndrome with high‐dose dexamethasone pulses
Author(s) -
Ertle Florian,
Behnisch Wolfgang,
Al Mulla Naima Ali,
Bessisso Mohammed,
Rating Dietz,
Mechtersheimer Gunhild,
Hero Barbara,
Kulozik Andreas E.
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21107
Subject(s) - medicine , dexamethasone , ataxia , neuroblastoma , myoclonus , blood cancer , rituximab , cerebellar ataxia , oncology , pediatrics , cancer , anesthesia , lymphoma , psychiatry , biology , genetics , cell culture
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. Pediatr Blood Cancer 2008;50:683–687. © 2007 Wiley‐Liss, Inc.