Department of Pediatrics, University of Chicago, Chicago, Illinois.
Department of Pathology, University of Chicago, Chicago, Illinois.
Pediatr Blood Cancer. 2020 Aug;67(8):e28218. doi: 10.1002/pbc.28218. Epub 2020 May 30.
Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8-month follow-up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.
眼震-肌阵挛综合征（OMS）是一种罕见的神经综合征，由影响神经母细胞瘤患儿的副肿瘤自身免疫过程引起。治疗包括皮质类固醇、静脉注射免疫球蛋白（IVIG）、利妥昔单抗和其他免疫抑制疗法。在这里，我们描述了一名诊断为与局部炎症性肌纤维母细胞瘤相关的 OMS 的患者。该患者在手术切除后 8 个月随访时无肿瘤复发证据。神经症状用皮质类固醇和 IVIG 缓解。该病例表明，在儿童中，神经母细胞瘤以外的肿瘤也可能与这种副肿瘤综合征相关，并强调了评估 OMS 患者是否存在潜在恶性肿瘤的重要性。
