儿童眼球阵挛-肌阵挛综合征的临床特点

发布时间：2018-11-21 20:09

【摘要】：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。
[Abstract]:Objective to investigate the clinical features of (OMS) in children with ocular clonus-myoclonic syndrome. Methods the clinical data of 4 children with OMS were analyzed retrospectively. Results all the 4 cases of OMS were accompanied with ocular clonus myoclonic ataxia and behavioral abnormality including 3 with sleep disorder 1 with language disorder and 1 with mental retrogression. Among them, 3 cases showed chest and abdominal space occupying with CT and 3 cases with poorly differentiated neuroblastoma by pathology, and 3 cases received tumor resection and postoperative chemotherapeutic therapy, and no tumor recurred. One of them received immunotherapy half a year after chemotherapy. The symptoms of OMS were obviously relieved, but the expression of residual language was decreased and the mental retardation was decreased. In the other two cases without immunotherapy, the symptoms of OMS were still present. Children without neuroblastoma were treated with human immunoglobulin and hormone after diagnosis. The symptoms of OMS were improved and no tumor was found. Conclusion OMS is a rare autoimmune disease of the nervous system. Children often have neuroblastoma. The prognosis of the tumor after operation is good, but most of them still need further immunotherapy to improve the symptoms of OMS for a long time. The prognosis of neurological sequelae caused by OMS is poor.
【作者单位】：成都市妇女儿童中心医院神经内科;
【分类号】：R741

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