儿童抗N-甲基D-门冬氨酸受体脑炎的临床和电生理研究

发布时间：2018-06-07 05:16

本文选题：脑炎 + N-甲基-D-门冬氨酸受体　；参考：《北京协和医学院》2016年博士论文

【摘要】：第一部分儿童抗N-甲基D-门冬氨酸受体脑炎的临床研究研究目的研究儿童抗N-甲基-D-门冬氨酸(NMDA)受体脑炎的临床特点。研究方法对2011年12月-2014年12月于首都儿科研究所神经科病房确诊20例抗NMDA受体脑炎的患者进行临床队列观察,总结其临床表现、对治疗的反应和临床预后特点。研究结果入组20例患者中,男性患儿8例,女性患儿12例,年龄1岁11月～13岁4月。观察时间8月～3年6月,至病情稳定,6月内无改善/进展为止。与成人患者不同,所研究儿童患者均以神经系统症状为首发表现,其中以癫痫发作最为突出,90%的患者均有癫痫发作;其次为语言障碍、认知行为异常和运动异常。偏瘫和小脑共济失调在6岁以下患者中出现频率较高。病情进展具有一定时相性。50%患者一线免疫治疗(IVIG、皮质激素)有效,一线治疗失败者予二线免疫(利妥昔单抗)治疗后,80%患儿有明显好转。部分患儿恢复缓慢,在8～12月的治疗中逐渐好转。未见死亡病例。所有患者均未发现肿瘤。结论抗NMDA受体脑炎在儿童脑炎患者中并不少见,男性及幼儿也可发病,免疫治疗有效。约半数患者一线免疫治疗有效;一线免疫治疗失败后,经二线免疫治疗多数患者可获得满意疗效;部分患者恢复缓慢;本病具有复发可能性。儿童患者肿瘤发生率明显低于成人。第二部分儿童抗N-甲基D-门冬氨酸受体脑炎的电生理研究研究目的研究抗N-甲基-D-门冬氨酸受体脑炎儿童患者不同病程分期的系列脑电图特点。研究对象2011年12月-2014年12月于首都儿科研究所神经科病房确诊的儿童抗N-甲基-D-门冬氨酸受体脑炎的20例患者。研究方法对上述患者在精神症状和/或癫痫发作期、无反应期、恢复期分别进行视频脑电图检查,分析脑电图在病程不同时期的表现,总结脑电图特点、探寻脑电图与临床表现的相关性。研究结果共入组20例,男,8例,女,12例,年龄1岁11月-13岁4月。监测精神症状和/或癫痫发作期脑电图25份,23份异常;在无反应期描记脑电图17份,17份异常;恢复期脑电图13份,7份异常。异常表现以慢波增加为主,可合并双侧导联不对称和快波性异常,局灶性棘波。精神症状和/或癫痫发作期以局灶性慢波增多为主要表现,无反应期以弥漫性慢波增多为主要表现,慢波频率进一步减低。极度σ刷(EDB)少见。恢复期慢波明显减少或消失。结论儿童抗NMDAR脑炎脑电图的显著特征是随病程进展而逐渐加重的慢波性异常,EEG的表现与临床症状有一定相关性。EDB少见。
[Abstract]:The first part: clinical study on N- methyl D-aspartate receptor encephalitis in children objective to study the clinical characteristics of anti-N- methyl-D- aspartate receptor encephalitis in children. Methods from December 2011 to December 2014, 20 patients with NMDA receptor encephalitis confirmed in the neurology ward of Capital Institute of Pediatrics were observed in clinical cohort, and their clinical manifestations, response to treatment and clinical prognosis were summarized. Results among the 20 patients, 8 were male and 12 were female. The observation time was from August to June, until the condition was stable, and no improvement / progress was observed in 6 months. Different from adult children, neurological symptoms were the first manifestation in all children, 90% of whom had epileptic seizures, followed by language disorders, cognitive behavior disorders and motor abnormalities. Hemiplegia and cerebellar ataxia occur more frequently in patients under 6 years of age. The first line immunotherapy (IVIGI, corticosteroid) was effective in 50% of the patients, and 80% of the patients who failed in the first line were treated with second line immunization (Rituximab). Some of the children recovered slowly and gradually improved during 8-12 months of treatment. No cases of death were seen. No tumor was found in all patients. Conclusion Anti- NMDA receptor encephalitis is not uncommon in children with encephalitis. First line immunotherapy was effective in about half of the patients; after the failure of the first line immunotherapy, most of the patients could get satisfactory curative effect through the second line immunotherapy; some patients recovered slowly; the disease had the possibility of recurrence. The incidence of tumor in children was significantly lower than that in adults. The second part: electrophysiological study on the anti-N- methyl D- aspartate receptor encephalitis in children objective to study the characteristics of electroencephalogram (EEG) in different stages of anti-N- methyl-Daspartate receptor encephalitis in children. Participants 20 children with N- methyl-Daspartate receptor encephalitis were diagnosed in neurology ward of Capital Institute of Pediatrics from December 2011 to December 2014. Methods the patients were examined with video electroencephalogram during mental symptoms and / or epileptic seizures, no reaction period, convalescence period, and the manifestations of EEG in different stages of disease course were analyzed, and the characteristics of EEG were summarized. To explore the correlation between EEG and clinical manifestations. Results A total of 20 patients, 8 males and 12 females, aged from 1 year to 13 years old, were enrolled in the study group. Twenty-three cases of EEG were abnormal in mental symptoms and / or epileptic seizures, 17 cases were abnormal in non-response period, 13 cases were abnormal in convalescent EEG, and 7 cases were abnormal in convalescent period. The abnormal features are mainly slow wave increase, which can be combined with bilateral lead asymmetry and fast wave anomaly, and focal spike wave. The main manifestations of mental symptoms and / or epileptic seizures were the increase of local slow wave and the increase of diffuse slow wave in non-response period. The frequency of slow wave decreased further. Extreme 蟽 brush EDB) is rare. The slow waves decreased or disappeared during the recovery period. Conclusion the significant feature of EEG in children with anti NMDAR encephalitis is that the manifestation of slow wave abnormal EEG, which is aggravated gradually with the progression of disease course, is correlated with the clinical symptoms to some extent.
【学位授予单位】：北京协和医学院
【学位级别】：博士
【学位授予年份】：2016
【分类号】：R742.9

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