title: 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.affiliation: Department of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Ertle
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Behnisch
authors.forename: Naima Ali
authors.initials: NA
authors.lastname: Al Mulla
authors.forename: Mohammed
authors.initials: M
authors.lastname: Bessisso
authors.forename: Dietz
authors.initials: D
authors.lastname: Rating
authors.forename: Gunhild
authors.initials: G
authors.lastname: Mechtersheimer
authors.forename: Barbara
authors.initials: B
authors.lastname: Hero
authors.forename: Andreas E
authors.initials: AE
authors.lastname: Kulozik
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis; D002675:Child, Preschool; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009447:Neuroblastoma / Q000150:complications* / Q000175:diagnosis; D053578:Opsoclonus-Myoclonus Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D010865:Pilot Projects; D011596:Psychomotor Disorders / Q000209:etiology; D012074:Remission Induction; D016879:Salvage Therapy
chemical_list: D007166:Immunosuppressive Agents; D003907:Dexamethasone