7例儿童抗N-甲基-D-天冬氨酸受体脑炎的临床研究

A retrospective study on 7 cases of pediatric anti-N-methyl-D-aspartic receptor encephalitis

目的:探讨儿童抗N-甲基-D-天冬氨酸受体脑炎(抗NMDAR脑炎)的临床特点及预后。方法:回顾性分析2016年1月1日-2018年9月30日在武汉大学人民医院儿科住院治疗且诊断为抗NMDAR脑炎的患儿,分析患儿的基本资料、临床特点、实验室检查、治疗和预后,并结合文献分析儿童抗NMDAR脑炎的特点。结果:筛选出抗NMDAR抗体阳性患儿共7例,其中男性患儿6例,女性患儿1例。所有患儿在病程中均出现胡言乱语、易激惹、烦吵、恐惧、惊厥、缄默、肢体乏力、不自主运动、刻板运动及夜间突发坐起等异常表现中的一项或多项观察指标。6例患儿脑电图出现异常,3例患儿颅脑核磁共振出现异常。7例患儿均予以一线免疫治疗,同时6例患儿根据其特异性临床表现给予精神药物和(或)抗癫痫治疗后,患儿症状均明显好转。1例患儿有再次入院记录,其余电话随访家属诉患儿未再发明显异常。结论:抗NMDAR脑炎患儿多急性或亚急性起病;抗NMDAR脑炎患儿的临床表现与既往文献报道类似,病程中多出现精神行为异常、惊厥、运动障碍、语言障碍及睡眠障碍等一种或多种症状;儿童抗NMDAR脑炎一线免疫治疗的效果一般较好。

Objective:To explore the clinical characteristics and prognosis of pediatric anti-N-methyl-D-aspartic receptor(NMDAR)encephalitis.Methods:A retrospective analysis was made on the children who were diagnosed with anti NMDAR encephalitis in Pediatrics Department of Renmin Hospital of Wuhan University from January 1,2016 to September 30,2018.The basic data,clinical characteristics,laboratory examinations,treatment and prognosis of children with anti NMDAR encephalitis were respectively analyzed.The specific conditions of children were described by frequency or percentage,and the characteristics of children with AE were analyzed in combination with literature.Results:Seven children with positive anti NMDAR antibodies were screened out,including 6 male and 1 female.In the progression of disease,all the children showed one or more abnormal manifestations such as gibberishness,irritability,noiseness,fear,convulsion,mutism,weakness,involuntary movement,rigid movement,sudden sitting at night and so on.Abnormal EEG was found in 6 cases,and unusual MRI signals was found in 3 cases.All the 7 children were treated with first-line immunotherapy.At the same time,psychotropic drugs and antiepileptic drugs were given according to the specific clinical manifestations.The symptoms of the children were significantly improved.Only one child had two admission records,and the rest had no significant abnormalities.Conclusion:Children with anti NMDAR encephalitis usually have an acute or sub-acute onset.The clinical characteristics of anti NMDAR encephalitis in children are consistent with those reported in previous literature on the whole.One or more symptoms,such as abnormal mental behavior,convulsions,motor disorders,language disorders and sleep disorders,often occur in the course of AE.The efficacy of first-line immunotherapy of anti NMDAR encephalitis is generally favorable.