Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses.
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.
Ertle
Florian
F
Department of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, Germany.
Behnisch
Wolfgang
W
Al Mulla
Naima Ali
NA
Bessisso
Mohammed
M
Rating
Dietz
D
Mechtersheimer
Gunhild
G
Hero
Barbara
B
Kulozik
Andreas E
AE
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
D007166:Immunosuppressive Agents; D003907:Dexamethasone