儿童髓鞘少突胶质细胞糖蛋白抗体相关急性播散性脑脊髓炎的临床特点  

Clinical features of children with myelin oligodendrocyte glycoprotein antibody associated acute disseminated encephalomyelitis

作　　者：吴小慧[1] 庄嘉鑫 林学锋[1] WU Xiao-hui;ZHUANG Jia-xin;LIN Xue-feng(Department of Pediatric Neurology,Quanzhou Children's Hospital,Quanzhou 362000,Fujian,CHINA)

机构地区：[1]泉州市儿童医院小儿神经内科,福建泉州362000

出　　处：《海南医学》2022年第8期1041-1045,共5页Hainan Medical Journal

摘　　要：目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关急性播散性脑脊髓炎(ADEM)的临床特点。方法回顾性分析2019年5月至2020年12月在泉州市儿童医院小儿神经内科住院的18例MOG抗体相关的ADEM患儿的临床表现、影像学检查、治疗及随访情况。结果MOG抗体相关ADEM患儿发病年龄为2.5~8岁,中位年龄4.6岁,其中5岁以下占66.7%;55%以上有前驱感染史或疫苗接种史,临床表现以脑病最多见(100%),其次为抽搐(44.4%);所有患儿头颅MRI均异常,大部分表现为皮质下白质、深部白质广泛、边界不清的大片状病灶;脊髓病变1例,表现为长节段脊髓炎;所有患儿急性期均予大剂量静脉甲泼尼龙(IVMP)冲击联合静脉丙种球蛋白(IVIG)治疗,病情均改善,约11.1%复发,缓解期使用小剂量激素维持配合每月IVIG未再复发。结论儿童MOG抗体相关ADEM以5岁以下多见,临床表现以脑病及抽搐最常见,急性期IVMP联合IVIG治疗反应好,部分可复发,缓解期小剂量激素维持配合每月IVIG可能可减少复发。Objective To study the clinical features of children with myelin oligodendrocyte glycoprotein(MOG)antibody-associated acute disseminated encephalomyelitis(ADEM).Methods The clinical manifestations,im-aging examination,treatment,and follow-up of 18 children with MOG antibody-associated related ADEM hospitalized in the Department of Pediatric Neurology,Quanzhou Children's Hospital from May 2019 to December 2020 were ana-lyzed retrospectively.Results The onset age of children with MOG antibody-related ADEM was 2.5 to 8 years old,with a median age of 4.6 years old,of which 66.7%were under 5 years old.More than 55%of these children had a histo-ry of precede infection or vaccination before onset.The most common clinical manifestations was encephalopathy(100%),followed by seizures(44.4%).All patients had abnormal brain magnetic resonance imaging(MRI),and most of them showed large flake lesions with subcortical white matter,extensive deep white matter,and unclear boundary.One case of myelopathy showed long segmental myelitis.All patients were treated with high dose intravenous methylpred-nisolone(IVMP)combined with intravenous gamma globulin(IVIG)in the acute stage.The condition was improved and about 11.1%of the patients recurred.Low dose hormone was used to maintain the remission stage and IVIG did not recur every month.Conclusion The onset age of MOG antibody-associated ADEM is most under 5 years old.The most common clinical manifestations were encephalopathy and seizures.Most children have a good response to IVMP combined IVIG in the acute stage,and some can relapse.Low dose of corticosteroid combined monthly IVIG may re-duce recurrent rate in the remission stage.

关 键 词：儿童 髓鞘少突胶质细胞糖蛋白抗体 急性播散性脑脊髓炎 中枢神经系统炎性脱髓鞘病 临床特点 复发 

分 类 号：R729[医药卫生—儿科]