以皮质脑炎为表型的儿童抗髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床特点  

Clinical features of anti-myelin oligodendrocyte glycoprotein antibody-associated diseases in children with cortical encephalitis

作　　者：周季 丁昌红[1] 张炜华[1] 卓秀伟[1] 李久伟[1] 巩帅[1] 关鸿志[2] 方方[1] 朱筱筠[1] 程华[3] 任晓暾[1] Zhou Ji;Ding Changhong;Zhang Weihua;Zhuo Xiuwei;Li Jiuwei;Gong Shuai;Guan Hongzhi;Fang Fang;Zhu Xiaoyun;Cheng Hua;Ren Xiaotun(Department of Neurology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Medical Imaging,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院神经内科,北京100045 [2]中国医学科学院北京协和医院神经科,北京100730 [3]首都医科大学附属北京儿童医院影像科,北京100045

出　　处：《中华医学杂志》2020年第25期1952-1955,共4页National Medical Journal of China

摘　　要：目的描述儿童抗髓鞘少突细胞糖蛋白(MOG)抗体阳性皮质脑炎临床特点。方法回顾性分析2018年6月至2019年10月于北京儿童医院神经内科住院,血清抗MOG抗体检测阳性,且病程中存在皮质脑炎表型患儿的临床特点。抗MOG抗体检测使用基于转染细胞(CBA)的间接免疫荧光法。结果共5例患儿病程中存在1次及以上皮质脑炎发作,女3例,男2例。起病年龄8岁至12岁1个月。至随访末3例为单相病程,2例为复发缓解病程。8次以皮质脑炎为表型的发作中,6次有癫痫发作,其中3次为癫痫持续状态,缓解期患儿无遗留反复癫痫发作。其他症状包括:发热(7/8),头痛、呕吐(4/8),嗜睡(3/8),偏瘫(1/8)。头颅MRI均存在单侧皮质肿胀,病灶无出血坏死。脑脊液白细胞存在不同程度升高(8×10^6/L^186×10^6/L)。所有患儿急性期使用丙种球蛋白及糖皮质激素治疗后症状均消失,其中2例有复发,加用吗替麦考酚酯口服。结论抗MOG抗体可介导皮质脑炎表型,临床以发热、头痛、癫痫发作常见,严重意识障碍及局灶神经功能受累少见;头颅MRI常表现为单侧皮质肿胀,无出血坏死;免疫治疗大多效果好,缓解期无遗留癫痫发作,部分患者可复发。Objective To describe the clinical features of anti-myelin oligodendrocyte glycoprotein(MOG)antibody-positive cortical encephalitis in children.Methods Patients who were hospitalized in Beijing Children′s Hospital from June 2018 to October 2019,with positive MOG antibodies and phenotype of cortical encephalitis were retrospectively analyzed.Cell-based assays(CBAs)were used to test MOG antibodies.Results Five patients had the phenotype of cortical encephalitis during follow-up,with 3 females and 2 males.The age of onset ranged from 8 years to 12 years and 1 month.At the last follow-up,3 cases exhibited a monophasic course and 2 cases were with relapse and remission courses.Six out of 8 episodes which had the phenotype of cortical encephalitis presented with seizures,among which 3 episodes had status epilepticus.None had recurrent seizures during remission.Other symptoms included fever(7/8),headache and vomiting(4/8),somnolence(3/8)and hemiplegia(1/8).Unilateral cortical swelling was observed in cerebral magnetic resonance imaging(MRI)of all patients,without any hemorrhage and necrosis.White blood cell(WBC)counts of cerebrospinal fluid increased,ranging from8×10^6/L to 186×10^6/L.All patients recovered well after treatment with intravenous immunogloblin and glucocorticoid.Two patients had relapses during follow-up and were additionally treated with mycophenolate mofetil.Conclusions Anti-MOG antibodies can induce cortical encephalitis.In clinical setting,fever,headache and seizures are common,however,severe consciousness disturbance and local neurological deficits are rare in these patients.Cerebral MRI shows unilateral cortical swelling without any hemorrhage and necrosis.Usually,immunotherapy works well.No patients exist repeated seizures in remission,but some patients may have relapses.

关 键 词：脑炎 抗髓鞘少突胶质细胞糖蛋白抗体 

分 类 号：R744.5[医药卫生—神经病学与精神病学]