儿童抗N-甲基-D-天门冬氨酸受体脑炎17例临床研究  

Clinical study of anti-N-methyl-D-aspartate receptor encephalitis in children

作　　者：季涛云[1] 张月华[1] 吴晔[1] 张欣[1] 王爽[1] 常杏芝[1] 熊晖[1] 姜玉武[1] 秦炯[1] 包新华[1] 

机构地区：[1]北京大学第一医院儿科,北京100034

出　　处：《中国实用儿科杂志》2014年第7期515-519,共5页Chinese Journal of Practical Pediatrics

摘　　要：目的总结儿童抗N-甲基-D-天门冬氨酸受体（NMDAR）脑炎临床特点和预后,提高对该病的认识。方法对2012年5月至2013年6月北京大学第一医院儿科17例临床确诊为抗NMDAR脑炎患者进行回顾及随访研究,总结其临床特点、治疗方案及预后。结果本组17例中男12例、女5例;年龄2～12岁。首发症状以惊厥和精神行为异常最常见,各7例。病程中常见症状依次为精神行为异常17例、睡眠障碍16例、锥体外系症状14例、惊厥11例等,仅4例有自主神经症状。所有患者脑脊液抗NMDAR抗体（＋）,16例脑电图为弥漫性慢波,均未筛查出肿瘤。14例给予静脉注射免疫球蛋白（IVIG）及甲基泼尼松龙治疗,2例仅给予IVIG,1例放弃,无缓解3例中2例给予利妥昔单抗、1例给予环磷酰胺治疗。随访3～15个月,12例恢复正常,2例死亡,2例遗留癫痫发作,1例依旧存在言语障碍。结论精神行为异常、睡眠障碍、锥体外系症状与惊厥是儿童抗NMDAR脑炎的常见临床表现,而肿瘤及自主神经功能紊乱发生率较低,免疫治疗疗效显著,预后较好。Objective To identify the clinical features and the prognosis of anti-NMDAR encephalitis in children,so as to improve the pediatrician’s awareness about this disease.Methods The clinical features,therapeutic regimen and prognosis of 17 children with anti-NMDAR encephalitis were summarized by a retrospective and follow-up study. Results Among the 17 patients with anti-NMDAR encephalitis,12 were males and 5 were females.Their age varied from 2 to 12 years,and the average age was 5 years 8 months.The most common initial symptoms were convulsions（7 cases）and psychiatric symptoms（7 cases）.The common symptoms in the course of the disease were psychiatric symptoms（17 cases）,sleep disorders（16 cases）,extrapyramidal symptoms（14 cases）,and convulsion（11 cases）.Only 4 patients had autonomic symptoms.The anti-NMDAR antibody were found in all the patients’CSF.The EEG test of 16 patients showed diffused slow background.Tumors were not found in any patient. Fourteen cases were treated with IVIg and methylprednisolone,2 cases only received IVIg therapy and 1 case gave up.Rituximab（2 cases）and cyclophosphamide（1 cases）were administrated in 3 cases with no improvement after the above treatment.The patients had been followed up for 3 to 15 months.Twelve of them recovered completely,2 cases died,2 cases had epilepsy,and 1 case had speech disorder. Conclusion Psychiatric symptoms,sleep disorders,extrapyramidal symptoms and convulsion are the common symptoms of anti-NMDAR encephalitis in children.Tumors and autonomic nervous system dysfunction rarely happen. Immunotherapy is effective in the majority of the patients.The prognosis of anti-NMDAR encephalitis in children is optimistic.

关 键 词：抗NMDAR脑炎 精神症状 肿瘤 免疫治疗 儿童 

分 类 号：R72[医药卫生—儿科]