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眼阵挛-肌阵挛综合征(OMS)是一种罕见的神经系统疾病,临床以不自主、快速、无规律眼球运动,肌阵挛,共济失调为特征,常有发育和行为异常后遗症。OMS与恶性肿瘤相关,在儿童,约50%的OMS并神经母细胞瘤(NB),NB是儿童常见的一种颅外恶性实体肿瘤。伴有OMS的NB患儿大多数能长期存活。其确切发病机制尚不十分清楚,多数学者认为是一种自身免疫性疾病。关于OMS的治疗,目前尚无统一的治疗方法,多应用免疫抑制剂和免疫调节剂。由于切除肿瘤、免疫抑制治疗未能有效阻止神经系统发育与行为异常后遗症的发生,如何通过有效的治疗改变预后,其观点仍有争议。
Myoclonus - myoclonus syndrome (OMS) is a rare neurological disease that is characterized by involuntary, rapid, irregular eye movement, myoclonus, ataxia, and often with developmental and behavioral abnormalities . OMS is associated with malignancies. In children, about 50% of OMS and neuroblastoma (NB) are common extracranial solid tumors in children. Most children with NB with OMS survive long term. The exact pathogenesis is not yet clear, most scholars think it is an autoimmune disease. There is currently no single treatment for OMS treatment, multiple use of immunosuppressive agents and immunomodulators. Because resection of tumors, immunosuppressive therapy failed to effectively prevent the development of nervous system and behavioral abnormalities sequelae, how to change the prognosis through effective treatment is still controversial.