探讨儿童自身免疫性脑炎(AE)的临床特点、治疗及其预后。

选择2014年10月至2016年11月，首都儿科研究所附属儿童医院神经内科收治的12例AE患儿为研究对象，包括11例抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎及1例抗富亮氨酸胶质瘤失活蛋白1(LGI1)抗体相关脑炎患儿。对12例AE患儿的临床病例资料进行回顾性分析，包括临床表现、辅助检查、诊断、治疗及随访等，总结儿童AE的临床特点、治疗及预后。本研究遵循的程序符合2013年修订的《世界医学协会赫尔辛基宣言》要求，与所有受试儿的监护人签署临床研究知情同意书。

①一般临床资料：本组12例AE患儿的平均年龄为7岁5个月，入院时平均病程为20.5 d，男、女患儿比例为5∶7。②临床表现：多为急性或亚急性起病，首发症状可见情绪及行为异常、发热伴头疼、惊厥、言语障碍、乏力、感觉异常等。11例抗NMDAR脑炎患儿主要临床表现为，90.9%(10/11)有精神症状，81.8%(9/11)有睡眠障碍，72.7%(8/11)有语言、认知障碍，或者运动障碍，54.5%(6/11)有惊厥发作，45.5%(5/11)有意识障碍，36.3%(4/11)出现自主神经功能异常。1例抗LGI1抗体相关脑炎患儿，临床以睡眠障碍为主要表现。③实验室检查结果：对12例AE患儿脑脊液进行常规检查的结果均显示基本正常，并且影像学筛查亦未发现肿瘤。12例AE患儿中，6例头颅MRI检查可见颅内异常信号，8例视频脑电图检查可见不同程度慢波背景。11例抗NMDAR脑炎患儿的脑脊液抗NMDAR抗体检测，均呈阳性或强阳性；1例抗LGI1抗体相关脑炎患儿的脑脊液抗LGI1抗体检测呈阳性。④治疗及随访结果：对12例AE患儿均采用甲泼尼龙和(或)静脉注射免疫球蛋白(IVIG)进行免疫治疗，对其随访时间为12～27个月。其中，9例患儿临床痊愈，2例存在不同程度神经系统后遗症，1例出院后即失访。⑤特殊表现：患儿1临床仅表现为视力障碍；患儿12临床表现主要为睡眠障碍；3例年龄<6岁患儿的临床症状以反复惊厥发作、意识障碍或运动障碍为主，病情均严重，并且一线免疫治疗的疗效差，均需联合应用二线免疫治疗。

儿童AE以抗NMDAR脑炎为主，抗LGI1抗体相关脑炎亦可见于儿童；临床以神经、精神症状为主，并且特异性不明显，免疫治疗的疗效确切。对危重症AE患儿，应早期联合二线免疫治疗，而且较为安全有效。

To explore the clinical features, treatments and prognosis of autoimmune encephalitis (AE) in children.

A total of 12 cases of children with AE who were admitted to Department of Neurology in Children′s Hospital, Capital Institute of Pediatrics from October 2014 to November 2016 were selected as research subjects. Among 12 cases of children with AE, there were 11 cases of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and 1 case of child with anti-leucine-rich glioma inactivated protein 1 (LGI1) antibody-associated encephalitis. Clinical cases data of 12 children with AE were retrospectively analyzed, which included clinical manifestations, auxiliary examinations, diagnosis, treatment and follow-up, to summarize the clinical characteristics, treatment and prognosis of AE in children. This study was in line with World Medical Association Declaration of Helsinki revised in 2013 and informed contents were obtained from the guardians of all patients.

①General clinical data: the average age of 12 children with AE was 7 years and 5 months, the average course of disease on admission was 20.5 d, and the ratio of male to female children was 5∶7. ②Clinical manifestations: most of patients were acute or subacute onset, the first symptoms could be abnormal emotions and behavior, fever along with headache, convulsion, speech disorder, fatigue and abnormal sensation, etc.. The main clinical manifestations of 11 children with anti-NMDAR encephalitis included psychiatric symptoms (90.9%, 10/11), sleep disorders (81.8%, 9/11), language, cognitive disorders or dyscinesia (72.7%, 8/11), seizures (54.5%, 6/11), disturbance of consciousness (45.5%, 5/11), dysautonomia (36.3%, 4/11). The main clinical manifestation of one child with anti-LGI1 antibody-associated encephalitis was sleep disorder. ③Results of laboratory examinations: routine examination results of cerebrospinal fluid were basically normal, and no tumors were found in 12 children by imaging screening. Among 12 children with AE, the cranial MRI examination of 6 children showed abnormal intracranial signals. Video-electroencephalogram examination of 8 children showed different degrees of slow waves background. Anti-NMDAR antibodies in cerebrospinal fluid of 11 children with anti-NMDAR encephalitis were positive or strongly positive, and anti-LGI1 antibody in cerebrospinal fluid of that child with anti-LGI1 antibody-associated encephalitis was positive. ④Results of treatment and followed-up: methylprednisolone and/or intravenous immunoglobulin (IVIG) were used for immunotherapy in 12 children with AE. The followed-up period was 12 to 27 months of 12 children, and among them, 9 cases were clinically cured, 2 cases had neurological sequelae of varying degrees, 1 case lost to follow-up just after discharge. ⑤Special manifestations: the clinical manifestation of case 1 was only visual impairment and case 12 was mainly sleep disorder. Three children under 6 years old were mainly characterized by recurrent convulsions, disturbance of consciousness or motor disturbance, and their conditions were serious, while the efficacy of the first-line immunotherapy was poor, so they all needed to carry out the second-line immunotherapy.

Anti-NMDAR encephalitis is the main type of AE in children, and anti-LGI1 antibody-associated encephalitis is also found in children. Neurological and psychiatric symptoms are the main clinical manifestations of children with AE but without specificity. Immunotherapy for AE is effective. Severe cases of AE should be combined with the second-line immunotherapy at early stage, and it is safer and more effective.