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儿童髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘病57例临床特征及预后分析 被引量:11

Clinical features and prognosis of myelin oligodendrocyte glycoprotein antibody associated demyelinating disease in 57 children
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摘要 目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关脱髓鞘病的临床特征及预后。方法回顾分析2014年1月至2019年10月住院治疗的57例MOG抗体相关脱髓鞘病患儿的临床资料,并分析其中随访6个月以上患儿的预后。结果57例患儿中,男28例、女29例,中位起病年龄7.3(5.2~10.5)岁,35例(61.4%)起病前有感染或疫苗接种等前驱事件,颅内高压45例(78.9%),发热43例(75.4%),脑病40例(70.2%)。临床表型以急性播散性脑脊髓炎(ADEM,49.1%)最多见,5例(8.8%)MOG抗体与抗N-甲基-D-天冬氨酸受体(NMDAR)抗体同时存在。患儿的血清MOG抗体滴度高于脑脊液,差异有统计学意义(P<0.05)。52例患儿行头颅MRI,以脑白质(40例,76.9%)、基底节(27例,51.9%)、丘脑(22例,42.3%)受累多见。大部分患儿急性期经静脉注射丙种球蛋白(IVIG)和/或大剂量激素冲击治疗后扩展残疾状态量表(EDSS)评分降低,差异有统计学意义(P<0.001)。29例患儿随访超过6个月,其中14例复发,复发患儿初次发作时更多出现发热症状,且IVIG和/或激素冲击治疗时间距离起病超过2周者更多,差异有统计学意义(P<0.05)。血清MOG抗体滴度与急性期患儿EDSS评分无明确相关性,且不能准确评估或预测病情的稳定或复发。结论儿童MOG抗体相关脱髓鞘病以ADEM最为常见,也可与抗NMDAR抗体同时存在。急性期IVIG和/或激素冲击治疗可有效缓解残疾症状。初次发作时发热、IVIG和/或激素治疗时间距起病超过2周可能与复发有关。 Objective To explore the clinical features and prognosis of myelin oligodendrocyte glycoprotein(MOG)antibody-associated demyelinating disease in children.Methods The clinical data of MOG antibody-associated demyelinating disease in 57 children hospitalized from January 2014 to October 2019 were retrospectively analyzed,and the prognosis of the children who were followed up for more than 6 months was analyzed.Results In 57 children(28 boys and 29 girls)with a median onset age of 7.3(5.2-10.5)years,35 children(61.4%)had precursor events such as infection or vaccination before onset.There were 45 cases(78.9%)with intracranial hypertension,43 cases(75.4%)with fever,and 40 cases(70.2%)with encephalopathy.Acute disseminated encephalomyelitis(ADEM,49.1%)was the most common clinical phenotype,and MOG antibody and anti-N-methyl-D-aspartate acid receptor(NMDAR)antibody coexisted in 5 cases(8.8%).The MOG antibody titer in serum was higher than that in cerebrospinal fluid,the difference was statistically significant(P<0.05).Fifty-two children underwent head MRI,and the white matter(40 cases,76.9%),basal ganglia(27 cases,51.9%),and thalamus(22 cases,42.3%)were more commonly affected.The scores of the extended disability status scale(EDSS)decreased after intravenous injection of IVIG and/or high-dose hormone therapy in most children during the acute phase,and the difference was statistically significant(P<0.001).Twenty-nine children were followed up for more than 6 months,and 14 of them had relapsed.The fever at the first episode was more common,and more than 2 week interval between the onset and IVIG and/or hormonal impulse therapy were more commonly observed in relapsed children,the difference was statistically significant(P<0.05).There is no clear correlation between serum MOG antibody titers and EDSS scores of children in the acute phase,and it could not accurately assess or predict the stability or relapse of the disease.Conclusion ADEM is the most common clinical phenotype of MOG antibody associated demyelinating disease in children.MOG antibodies can also co-exist with anti-NMDAR antibodies.IVIG and/or hormonal impulse therapy can effectively relieve the symptoms of disability in acute stage.Fever at first episode and more than 2 week interval between the onset and IVIG and/or hormone treatment may be related to relapse.
作者 孙红 谭建敏 刘静临 李紫妍 蒋艳 袁萍 马建南 吴鹏 钟敏 罗媛媛 蒋莉 李秀娟 SUN Hong;TAN Jianmin;LIU Jinglin;LI Ziyan;JIANG Yan;YUAN Ping;MA Jiannan;WU Peng;ZHONG Min;LUO Yuanyuan;JIANG Li;LI Xiujuan(Department of Neurology,Children’s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation base of Child development and Critical Disorders,Chongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing,400014,China)
出处 《临床儿科杂志》 CAS CSCD 北大核心 2020年第11期824-830,共7页 Journal of Clinical Pediatrics
关键词 MOG抗体相关脱髓鞘病 髓鞘少突胶质细胞糖蛋白 临床特征 预后 儿童 MOG antibody associated demyelinating disease myelin oligodendrocyte glycoprotein clinical feature prognosis child
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