神经母细胞瘤相关的眼阵挛-肌阵挛综合征研究进展

Research Progress of Opsoclonus-Myoclonnus Syndrome Associated Neuroblastoma in Children

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。

Opsoclonus-myoclonus syndrome（OMS） is a rare neurological disease,which is characterized by rapid,involuntary and irregular eye-movements,myoclonus and ataxia,and usually along with the sequela like developmental regression and behavior disorder.OMS is often associated with malignancies,in children,about half of the cases are associates with neuroblastoma,which is the most common malignant solid tumor of childhood derived from the sympathetic nervous system.Most of the NB children with OMS can have long-term survival.The pathogenesis of this syndrome is still undefined but is suspected to be the result of an autoimmune response.Regarding OMS′s treatment,there is no unified therapy now,most are treated with a number of immunosuppressive and immunomodulator agents.Because tumor resection and immunosuppressive therapies can not prevent the nervous system developmental regression and behavior disorder sequelae,how to improve the prognosis through effective treatment is still discussed.