髓鞘少突胶质细胞糖蛋白抗体相关疾病患儿的临床特征  

Clinical features of children with myelin oligodendrocyte glycoprotein antibodyassociated disorders

作　　者：郑萍[1] 张建昭[1] 孙静[1] 冯硕[1] 仪晓立[2] 毛莹莹[1] 李冬青[1] 陈倩[1] ZHENG Ping;ZHANG Jian-Zhao;SUN Jing;FENG Shuo;YI Xiao-Li;MAO Ying-Ying;LI Dong-Qing;CHEN Qian(Department of Neurology,Children's Hospital,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院神经内科,北京100020 [2]首都儿科研究所附属儿童医院放射科,北京100020

出　　处：《中国当代儿科杂志》2020年第4期368-373,共6页Chinese Journal of Contemporary Pediatrics

摘　　要：目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)患儿的临床特点及治疗转归。方法回顾性分析28例MOGAD患儿(38例次脱髓鞘发作)的临床资料。结果28例MOGAD患儿的疾病谱中,视神经炎占比最高(12例,43%),其次为急性播散性脑脊髓炎(9例,32%)。28例患儿(38例次脱髓鞘发作)急性期头颅MRI显示病变者共29例次(76%),大部分表现为广泛性或孤立性皮质下白质病灶。共记录24例次急性发作期脊髓MRI结果,其中脊髓病变者11例次(46%)。18例次视神经炎急性期MRI显示均伴视神经病变。20例(71%)患儿脑脊液白细胞升高,细胞分类以淋巴细胞为主,蛋白升高者3例。28例患儿血清MOG抗体滴度1:10~1:320。28例患儿均给予了糖皮质激素治疗,18例同时予以免疫球蛋白治疗。随访中26例(93%)治疗后症状基本缓解,仅2例遗留视觉能区的神经系统后遗症。结论MOGAD儿童的临床表现呈多样性;免疫治疗有效,大部分预后良好。Objective To study the clinical features and treatment outcome of children with myelin oligodendrocyte glycoprotein(MOG)antibody-associated disorders(MOGAD).Methods A retrospective analysis was performed for the clinical data of 28 children with MOGAD(with 38 demyelinating episodes).Results Among the disease spectrums of 28 children with MOGAD,optic neuritis was the most common(12 cases,43%),followed by acute disseminated encephalomyelitis(9 cases,32%).Among the 38 demyelinating episodes in the 28 children,there were 29 cases(76%)of lesions in the acute stage on head magnetic resonance imaging(MRI),and most of these lesions were extensive or isolated subcortical white matter lesions.A total of 24 cases of spinal MRI results in the acute stage were recorded,among which there were 11 cases(46%)of spinal lesions.MRI abnormalities of the optic nerve were found in 18 cases of optic neuritis in the acute stage.Of the 28 children,20(71%)had an increase in white blood cell count in cerebrospinal fluid,with lymphocytes as the most common type of cells,and 3 children had an increase in protein.The titer of serum MOG antibody was 1:10-1:320 in the 28 children.All 28 children were administered with glucocorticoids,along with immunoglobulin in 18 children.The symptoms of 26 children(93%)were alleviated during follow-up,and only 2 children had neurological sequela of the optic function.Conclusions The clinical manifestations are diverse in children with MOGAD.Immunotherapy is effective and most children have a good prognosis.

关 键 词：髓鞘少突胶质细胞糖蛋白抗体相关疾病 脱髓鞘发作 儿童 

分 类 号：R744.5[医药卫生—神经病学与精神病学]