儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病26例临床特点及脑电图分析  

Analysis of clinical characteristics and electroencephalogram of myelin oligodendrocyte glycoprotein antibody related diseases in 26 children

作　　者：刘亮[1] 贾珊珊[1] 汪东[1] 李霞[1] 李东景[1] 赵斯钰[1] LIU Liang;JIA Shanshan;WANG Dong;LI Xia;LI Dongjing;ZHAO Siyu(Neurology Department,Xi'an Children's Hospital,Xi'an 710003,China)

机构地区：[1]西安市儿童医院神经内科,陕西西安710003

出　　处：《临床医学研究与实践》2022年第34期24-28,32,共6页Clinical Research and Practice

基　　金：陕西省重点研发计划项目(No.2020SF-006);陕西省西安市卫生和计划生育委员会科研项目(No.J201903061);西安市科技计划项目[No.2019115113YX006SF039(7)]。

摘　　要：目的分析儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病26例的临床特点及脑电图。方法回顾性收集、分析2019年1月至2020年12月就诊于西安市儿童医院的26例MOG抗体病患儿的临床资料。结果26例MOG抗体病患儿中,男13例,女13例,男女比例1:1,首发症状表现多样,以意识障碍及癫痫发作等症状多见(占比42.31%)。26例MOG抗体病患儿行头颅磁共振成像(MRI)平扫,其中25例在颅内发现异常病灶,主要表现为多发片状或斑片状长/稍长T_(1)及长/稍长T_(2)信号,T_(2)Flair高/稍高信号影,最常见为双侧大脑半球受累,其次为脑干、丘脑、小脑受累。26例MOG抗体病患儿均于病程急性期行视频脑电图(VEEG)监测,其中20例患儿脑电图出现异常,主要表现为背景活动减慢,或出现多灶、弥漫或广泛性慢波。26例MOG抗体病患儿血清MOG抗体均阳性,4例患儿脑脊液MOG抗体阳性。26例MOG抗体病患儿均使用激素治疗,17例患儿联合应用免疫球蛋白冲击治疗,出院时急性期患儿临床症状均有不同程度好转;随访期间3例患儿病情复发,复发患儿应用吗替麦考酚酯治疗后临床症状缓解。结论儿童MOG抗体相关疾病的男女发病比例差异较小,首发症状以意识障碍、癫痫发作多见,颅内病灶往往多发;血清MOG抗体检测较脑脊液更有价值,脑电图未见特征性改变;急性期以激素及丙种球蛋白冲击治疗为主,患儿临床预后大多良好,但部分有复发。Objective To analyze the clinical characteristics and electroencephalogram of myelin oligodendrocyte glycoprotein(MOG)antibody related diseases in 26 children.Methods The clinical data of 26 children with MOG antibody diseases who were admitted in Xi'an Children's Hospital from January 2019 to December 2020 were retrospectively collected and analyzed.Results Among the 26 children with MOG antibody diseases,there were 13 boys and 13 girls,with a boy to girl ratio of 1:1;the first symptoms were diverse,with consciousness disorder and seizure as the common symptoms(accounted for 42.31%).Magnetic resonance imaging(MRI)plain scan was performed in 26 children with MOG antibody diseases,and abnormal lesions in the brain were found in 25 cases;the lesions were mainly manifested as multiple schistose or patchy long/slightly long T_(1) and long/slightly long T_(2) signals,T_(2)Flair high/slightly high signal intensity;the most common was bilateral cerebral hemisphere involvement,followed by brainstem,thalamus and cerebellum.All the 26 children with MOG antibody diseases were monitored by video electroencephalogram(VEEG)during the acute stage of the disease,and 20 cases showed abnormal electroencephalogram,which was mainly characterized by slowing down of background activity,or multifocal,diffuse or extensive slow waves.The serum MOG antibody was positive in 26 children with MOG antibody diseases,and the cerebrospinal fluid MOG antibody was positive in 4 children.All the 26 children with MOG antibody diseases were treated with hormone,17 children were treated with immunoglobulin pulse therapy,and the clinical symptoms at discharge of the children in the acute stage improved in varying degrees;during the follow-up period,3 children relapsed,and the clinical symptoms of relapsed children relieved after treatment with mycophenolate mofetil.Conclusion There is little difference between boy and girl in the incidence of MOG antibody related diseases in children,the first symptoms are consciousness disorder and seizure,and the int

关 键 词：髓鞘少突胶质细胞糖蛋白 儿童 临床特点 脑电图 

分 类 号：R682.1[医药卫生—骨科学]