抗体阳性儿童自身免疫性脑炎32例临床分析  

Clinica analysis of 32 autoimmune encephalitis children with autoantibody

作　　者：谭自明 张静[1] 罗琼[1] 王君[1] 张美[1] Tan Ziming;Zhang Jing;Luo Qiong;Wang Jun;Zhang Mei(Department of Nurology,the First Affiliated Hospital of Xinjiang Medical University,Urumqi 830054,China)

机构地区：[1]新疆医科大学第一附属医院儿童神经内科,乌鲁木齐830054

出　　处：《脑与神经疾病杂志》2022年第10期595-601,共7页Journal of Brain and Nervous Diseases

基　　金：新疆维吾尔自治区自然科学基金(2021D01C313)。

摘　　要：目的 通过对神经元抗体阳性的32例儿童自身免疫性脑炎(AE)临床特点的分析,为临床医师提供参考依据。方法收集2017年1月至2020年10月新疆医科大学第一附属医院儿童神经内科确诊的儿童AE32例,分析其临床表现,总结其临床特点。结果32例患者,年龄11个月~14岁,以抗N-甲基-D-天冬氨酸受体(NMDAR)抗体阳性脑炎为主,占75%(24/32)。临床主要表现有癫痫发作且对抗癫痫药物治疗欠佳、认知障碍、精神症状、语言障碍及运动障碍等,脑脊液检查细胞数升高占50%(16/32),蛋白定量升高占68.8%(23/32);有1例颅咽管瘤患儿,手术切除2年后出现头痛症状,诊断抗NMDAR抗体脑炎;56.2%(18/32)的患儿头颅磁共振(MRI)异常,87.5%(28/32),脑电图(EEG)异常,选择同期确诊抗体阴性的AE患儿22例作为对照组,抗体阳性组在癫痫发作、脑脊液蛋白定量两方面比例高于抗体阴性组,差异有统计学意义(P<0.05)。治疗方案方面,5例(15.6%)患儿单用大剂量激素冲击治疗,18例(56.3%)患儿大剂量激素冲击+丙种球蛋白治疗,9例(28.1%)患儿大剂量激素冲击+丙种球蛋白治疗+吗替麦考酚酯口服治疗,治疗预后分别进行两两比较,差异均无统计学意义(P>0.05)。结论儿童短时间内出现新发症状,包括药物难以控制的癫痫发作,运动、语言、认知障碍,需警惕AE;副肿瘤综合征在儿童中发生率相对低,需要长期随访;早期免疫治疗可以减少使用抗癫痫药物的种类、剂量。Objective The clinical characteristics of 32 children with autoimmune encephalitis(AE) with positive neuron antibody were analyzed to provide reliable reference for clinical practice.Methods 32 children with AE diagnosed in our hospital from January 2017 to October 2020 were collected,their clinical manifestations were analyzed and their clinical characteristics were summarized.Results 32 children,aged from 11 months to 14 years,were mainly positive for anti N-methyl-D-aspartica receptor(NMDAR) antibody,accounting for 75%(24/32),The main clinical manifestations were epileptic seizures and poor treatment of antiepileptic drugs,cognitive impairment,mental symptoms,language impairment and motor impairment.The increase of cell number in cerebrospinal fluid accounted for 50%(16/32),and the increase of protein quantity accounted for 68.8%(23/32),one child with craniopharyngioma developed headache 2 years after surgical resection and was diagnosed as anti NMDAR antibody encephalitis,56.2%(18/32) of the children had abnormal cranial MRI and 87.5%(28/32) had abnormal EEG,22 children with AE diagnosed as antibody negative in the same period were selected as the control group.The proportion of epilepsyand cerebrospinal fluid protein quantification in the antibody positive group was higher than that in the antibody negative group,and the difference was statistically significant(P<0.05),in terms of treatment plan,5 cases(15.6%) were treated with high-dose hormone,18 cases(56.3%) were treated with high-dose hormone+gamma globulin,and 9 cases(28.1%) were treated with high-dose hormone+gamma globulin+oral mycophenolate mofetil,there was no significant difference between the two groups(P> 0.05).Conclusion Children have new symptoms in a short time,including seizures that are difficult to control with drugs,motor,language and cognitive impairment.They should be vigilant against autoimmune encephalitis;Paraneoplastic syndrome has a relatively low incidence in children and requires long-term follow-up;Early immunotherapy can reduce

关 键 词：自身免疫性脑炎 儿童 临床特点 治疗 预后 

分 类 号：R512.3[医药卫生—内科学]