【摘要】 目的:分析抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)脑炎患儿的临床特点及预后,为临床早期识别本病提供依据。方法:回顾性分析2015年1月至2018年3月在中南大学湘雅二医院儿科住院的42例抗NMDAR脑炎患儿的临床资料及随访情况,总结其临床特点、治疗效果及预后情况。结果:42例患儿中男15例(35.7%),女27例(64.3%),男女比例1:1.8,年龄(9.20±4.66)岁(最小4个月,最大17岁)。首发症状以癫痫发作(47.6%,20/42)和精神行为异常(35.7%,15/42)最为常见;患儿在病程中累积出现精神行为异常(36/42,85.7%)、癫痫发作(36/42,85.7%)、言语障碍(32/42,76.2%)、运动障碍(28/42,66.7%)、意识障碍(28/42,66.7%)、自主神经功能紊乱(26/42,61.9%)及睡眠障碍(24/42,57.1%)等症状。42例患儿脑脊液抗NMDAR抗体均为阳性;50.0%(21/42)的患儿头颅MRI检查结果异常,病变累及顶叶、额叶、颞叶、枕叶、基底节及视神经等部位,1例患儿病变累及视神经合并髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein,MOG)抗体阳性;40例患儿行脑电图检查,92.5%(37/40)出现异常,以弥漫性慢波为主要表现,1例可见δ刷;合并肠系膜畸胎瘤1例,占2.4%(1/42)。出院时mRS评分为2.14±1.46,较住院期间最高mRS评分(3.88±1.38)显著降低(P<0.05),疗效显著;随访3～39个月,出院后3个月时mRS评分仅为0.81±1.29,与出院时mRS评分比较仍有显著改善,其中76.2%(32/42)mRS评分≤2,预后良好;4.8%(2/42)的患儿复发,无死亡病例。开始免疫治疗时间和病程中最高m RS评分是影响预后的因素,开始免疫治疗时间越早、病程中mRS评分越低,预后越好。结论:癫痫发作、精神行为异常、运动障碍、言语障碍及自主神经功能紊乱是儿童抗NMDAR脑炎的常见临床表现;免疫治疗效果显著,开始免疫治疗的时间和病情严重程度是影响预后的重要因素;抗NMDAR脑炎可合并其他自身抗体,其临床意义及机制有待深入研究。更多还原

【Abstract】 Objective: To analyze the clinical characteristics and prognosis of children with anti-Nmethyl-D-aspartate receptor(NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.Methods: The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.Results: There were 15 cases(35.7%) of males and 27 cases(64.3%) of females in 42 children, with a ratio of 1:1.8. They were aged from 4 months to 17 years, with an average of(9.20±4.66) years. The most common initial symptoms were seizures(47.6%,20/42) and mental behavior disorder(35.7%,15/42). During the course of the disease,85.7% patients(36/42) had mental and behavior disorder, 85.7% patients(36/42) had epilepsy,76.2%(32/42) had speech disorder, 66.7% patients(28/42) had dyskinesia,66.7% patients(28/42) had the decreased level of consciousness,61.9% patients(26/42) had autonomic instability,and57.1%(24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0%(21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve.There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein(MOG) antibody positive. Forty cases were examined by electroencephalogram(EEG), 92.5% cases(37/40) were abnormal, mainly showing diffuse slow waves, and δbrushes could be seen in severe cases. And there was 1 patient(2.4%) complicated with mesenteric teratoma. The m RS score(2.14±1.46) at discharge was significantly lower than the highest m RS score(3.88±1.38) during hospitalization(P<0.05). After 3-39 months of follow-up, m RS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases(32/42) experienced complete or near-complete recovery(m RS score≤2), and 4.8%(2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest m RS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower m RS score in the course of the disease were, the better the prognosis was.Conclusion: Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.更多还原