儿童眼球阵挛-肌阵挛综合征的临床特点

Clinical features of opsoclonus-myoclonus syndrome in children

目的探讨儿童眼球阵挛-肌阵挛综合征(OMS)的临床特点。方法回顾性分析4例OMS患儿临床资料。结果本组4例OMS患儿临床均伴有眼球阵挛、肌阵挛、共济失调及行为异常,其中3例伴有睡眠障碍,1例伴有语言障碍,1例伴有智力倒退。其中3例患儿胸腹部CT提示胸腹腔占位,病理提示低分化神经母细胞瘤。3例患儿均予肿瘤切除治疗及术后化学治疗,肿瘤均未复发。其中1例患儿在化疗后半年给予免疫治疗,目前OMS症状明显缓解,但遗留语言表达下降及智力倒退;另外未加用免疫治疗的2例患儿目前OMS症状仍持续存在。未合并神经母细胞瘤的患儿在确诊后给予人免疫球蛋白及激素治疗,目前OMS症状好转,无肿瘤新发。结论 OMS是一种罕见的神经系统自身免疫性疾病,儿童常合并神经母细胞瘤,手术后肿瘤预后良好,但多数仍需要进一步接受免疫治疗才能长期改善OMS症状,且OMS已导致的神经系统后遗症预后不良。

Objective To discuss the clinical features of opsoclonus myoclonus syndrome （OMS） in children. Methods Clinical date of 4 patients with OMS were analyzed retrospectively. Results Four cases in this group had opsoclonus, myoclonus, ataxia and abnormal behavior. Three of the patients were with sleep disorder, 1 case with the language barrier, 1 case with intelligence. The chest and abdominal CT revealed that 3 cases had a placeholder in the chest or abdomen. Pathologic examination showed low differentiated neuroblastoma. Three cases confirmed with neuroblastoma were given tumor resection and postoperative chemotherapy; all of them had no recurrence of tumor so far. One case was given immunotherapy half a year after chemotherapy, the OMS symptoms had significantly relieved, but the decline of language expression and mental regression were persisted. Two cases without immunotherapy had OMS symptoms persisting. And 1 case without neuroblastoma was given immunoglobulin and hormone therapy after diagnosed, the symptoms was improved after immunotherapy, and there was no new-onset tumor. Conclusions OMS is a rare autoimmune disease of the nervous system, often associated with neuroblastoma in children. The tumor has good prognosis after surgery, but most of them still need to be given further immunotherapy to improve OMS symptoms. Existing neurological sequelae has poor prognosis.