儿童抗N-甲基-D-天门冬氨酸受体脑炎10例临床分析

Clinical analysis of anti-N-methyl-D-aspartate receptor encephalitis in 10 children

目的分析儿童抗N-甲基-D-天门冬氨酸受体(anti N-methyl-D-aspartate receptor,NMDAR)脑炎的临床特征、治疗方法及预后,提高对该病的认识。方法对2015年1月~2016年9月哈尔滨市儿童医院神经内科收治的10例临床确诊为抗NMDAR脑炎患儿进行回顾分析及随访。结果 10例患儿中男2例,女8例,年龄1~15岁。首发症状均以惊厥就诊,逐渐出现睡眠障碍、认知障碍、精神行为异常、锥体外系症状及自主神经功能紊乱症状。10例脑脊液抗NMDAR抗体均阳性;8例血液抗NMDAR抗体阳性,2例阴性。10例脑电图均呈现弥漫性慢波改变,1例可见δ刷,1例早期局灶性痫性放电。头颅核磁共振检查2例边缘叶异常信号,8例正常。卵巢畸胎瘤1例。10例患儿均给予静脉注射免疫球蛋白(intravenous immunoglobulin,IVIG)及甲基泼尼松龙冲击治疗。随访3月~1年,8例治愈,2例继发癫痫。结论惊厥、精神行为异常、睡眠障碍、认知障碍、锥体外系症状是儿童抗NMDAR脑炎的常见临床症状。对于以精神行为异常、惊厥起病,且抗精神病及抗癫痫治疗效果较差的患儿要尤其注意本病,尽早完善脑脊液及血自身免疫抗体检测,做到早诊断,早治疗。

Objective To analyse the clinical features, treatment and prognosis of children with anti N-methyl-D-aspartate receptor （NMDAR） encephalitis. Methods Ten children with anti-NMDAR encephalitis were summarized by a retrospective and followed-up study, in the Department of Neurology, Harbin Children＇ s Hospital from Jan. 2015 to Sep. 2016. Results Among the 10 cases of anti-NMDAR encephalitis, 2 were males and 8 were females. Their age varied from 1 to 15 years. The most common initial symptoms were convulsions, gradual emer- gence of psychiatric symptoms, sleep disorders, cognitive disorders, extrapyramidal symptoms and autonomic symptoms. The anti-NMDAR antibody were found in all the patients＇ CSF and 8 patients＇ blood. The EEG test of 10 cases showed diffused slow background, 1 case with delta brush, 1 case with early focal electro-discharge. Two cases had abnormal signal of limbic lobe with head MRI examination, others were normal. One case had ovary tumour. All cases were treated with IVIG and methylprednisolone. The patients were followed up for 3 to 12 months. Eight cases of them were recovered completely, 2 case had epilepsy. Conclusion Convul-sions, psychiatric symptoms, sleep disorders, cognitive disorder, extrapyramidal symptoms, au- tonomic symptoms are the common symptoms of anti-NMDAR encephalitis in children. In the onsets of psychiatric symptoms, seizures, less effective antipsychotic and antiepileptic treatment must especially pay attention to the disease and could elevate the diagnostic rate of the disease.