儿童抗髓鞘少突胶质细胞糖蛋白抗体相关中枢神经系统炎性脱髓鞘病临床特征  

Clinical features of central nerve system inflammatory demyelinating diseases associated with anti-myelin oligodendrocyte glycoprotein antibodies in children

作　　者：张炜华[1] 任晓暾[1] 韩彤立[1] 程华 李久伟[1] 巩帅[1] 冯卫星[1] 朱筱筠[1] 任长虹 周季 丁昌红[1] 任海涛[3] 关鸿志[3] 方方[1] Zhang Weihua;Ren Xiaotun;Han Tongli;Cheng Hua;Li Jiuwei;Gong Shuai;Feng Weixing;Zhu Xiaoyun;Ren Changhong;Zhou Ji;Ding Changhong;Ren Haitao;Guan Hongzhi;Fang Fang(Department of Neurology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Radiology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Neurology,Peking Union Medical College Hospital,Beijing 100730,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院神经内科,北京100045 [2]国家儿童医学中心,首都医科大学附属北京儿童放射科,北京100045 [3]北京协和医院神经科,100730

出　　处：《中华实用儿科临床杂志》2019年第24期1858-1861,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的阐述儿童抗髓鞘少突胶质细胞糖蛋白抗体相关中枢神经系统炎性脱髓鞘病(MOG-IDD)的临床表现,探讨其临床特征。方法对2016年10月至2018年8月首都医科大学附属北京儿童医院确诊的MOG-IDD患儿进行观察性研究,描述患儿的临床及实验室特征,比较单相病程及复发病程患儿的临床数据。结果入组患儿共50例,男女比例为24:26;首发年龄(6.7±3.1)岁(0.4~12.6岁);男女发病年龄差异无统计学意义(t=0.712,P=0.480)。50例中,26例(52.0%)为单相病程,24例(48.0%)为复发病程。主要症状包括发热(31/50例)、脑病(26/50例)和视神经炎(22/50例)等。首次发作时有无脑病和共济失调年龄间差异均有统计学意义[(5.7±2.8)岁比(8.1±3.0)岁,(5.0±2.5)岁比(7.7±3.0)](t=2.746,P=0.009;t=2.837,P=0.007)。复发患儿发作次数(2.1±1.4)次(1~7次),70.8%(17/24例)的患者于12个月内复发,83.3%(20/24例)于2年内复发。复发患儿首次发作和疾病全程中惊厥发生分别为10例和13例,均高于单相病程患儿(均为4例),差异均有统计学意义(χ^2=7.912,P=0.005;χ^2=8.365,P=0.004)。所有患儿对一线免疫治疗敏感,复发患儿中7例应用吗替麦考酚酯胶囊治疗,其他复发患儿重复一线治疗。末次随访时患儿均处于缓解期,2例存在轻度神经功能障碍。结论MOG-IDD可发生于儿童时期各年龄段,脑病及视神经炎是最常见的症状,脑病和共济失调是小年龄儿起病的主要表现,惊厥发作可能提示复发。Objective To describe the clinical manifestations of central nerve system inflammatory demyelinating disease associated with anti-myelin oligodendrocyte glycoprotein antibody(MOG-IDD)in children,and to explore the clinical characteristics of the children.Methods The clinical and laboratory characteristics of the patients diagnosed in Beijing Children′s Hospital,Capital Medical University,from October 2016 to August 2018 were described,and the clinical data of the patients with unipolar and recurrent diseases were compared.Results A total of 50 patients were included,among whom the ratio of male to female was 24:26,and the average age of onset was(6.7±3.1)years old(0.4-12.6 years old).There was no significant difference in the age of onset between boys and girls(t=0.712,P=0.480).The main symptoms included fever(31/50 cases),encephalopathy(26/50 cases)and optic neuritis(22/50 cases),etc.In the last follow-up,26 patients(52.0%)had a monophasic course and 24 patients(48.0%)had a recurrent course.There were age differences in encephalopathy and ataxia in the first episode of[(5.7±2.8)years old vs.(8.1±3.0)years old,(5.0±2.5)years old vs.(7.7±3.0)years old](t=2.746,P=0.009;t=2.837,P=0.007).The average number of recurrence was(2.1±1.4)times(1-7 times),in which 17 cases(70.8%)of recurrence presented within 12 months and 20 cases(83.3%)of recurrence presented within 24 months after onset.Convulsion incidences of recurrent cases were 10 cases and 13 cases respectively in the first episode and recurrent courses,which were significantly higher than those of monophasic cases(4 cases,4 cases)(χ^2=7.912,P=0.005;χ^2=8.365,P=0.004).All patients were sensitive to first-line immunotherapy.Seven patients with recu-rrence were treated with mycophenolatemofetil,and 17 patients with repeated first-line therapy.In the last follow-up,all patients were in remission and 2 patients had mild neurological dysfunction.Conclusions MOG-IDD can occur in childhood.Encephalopathy and optic neuritis are the most common symptoms.Encephalopa

关 键 词：儿童 髓鞘少突胶质细胞糖蛋白 中枢神经系统炎性脱髓鞘病 视神经炎 单相 复发 

分 类 号：R74[医药卫生—神经病学与精神病学]