本文選題：腦炎 + N-甲基-D-門冬氨酸受體　； 參考：《北京協(xié)和醫(yī)學(xué)院》2016年博士論文

【摘要】：第一部分 兒童抗N-甲基D-門冬氨酸受體腦炎的臨床研究研究目的研究兒童抗N-甲基-D-門冬氨酸(NMDA)受體腦炎的臨床特點(diǎn)。研究方法對2011年12月-2014年12月于首都兒科研究所神經(jīng)科病房確診20例抗NMDA受體腦炎的患者進(jìn)行臨床隊(duì)列觀察,總結(jié)其臨床表現(xiàn)、對治療的反應(yīng)和臨床預(yù)后特點(diǎn)。研究結(jié)果入組20例患者中,男性患兒8例,女性患兒12例,年齡1歲11月～13歲4月。觀察時(shí)間8月～3年6月,至病情穩(wěn)定,6月內(nèi)無改善/進(jìn)展為止。與成人患者不同,所研究兒童患者均以神經(jīng)系統(tǒng)癥狀為首發(fā)表現(xiàn),其中以癲癇發(fā)作最為突出,90%的患者均有癲癇發(fā)作;其次為語言障礙、認(rèn)知行為異常和運(yùn)動(dòng)異常。偏癱和小腦共濟(jì)失調(diào)在6歲以下患者中出現(xiàn)頻率較高。病情進(jìn)展具有一定時(shí)相性。50%患者一線免疫治療(IVIG、皮質(zhì)激素)有效,一線治療失敗者予二線免疫(利妥昔單抗)治療后,80%患兒有明顯好轉(zhuǎn)。部分患兒恢復(fù)緩慢,在8～12月的治療中逐漸好轉(zhuǎn)。未見死亡病例。所有患者均未發(fā)現(xiàn)腫瘤。結(jié)論抗NMDA受體腦炎在兒童腦炎患者中并不少見,男性及幼兒也可發(fā)病,免疫治療有效。約半數(shù)患者一線免疫治療有效;一線免疫治療失敗后,經(jīng)二線免疫治療多數(shù)患者可獲得滿意療效;部分患者恢復(fù)緩慢;本病具有復(fù)發(fā)可能性。兒童患者腫瘤發(fā)生率明顯低于成人。第二部分 兒童抗N-甲基D-門冬氨酸受體腦炎的電生理研究研究目的研究抗N-甲基-D-門冬氨酸受體腦炎兒童患者不同病程分期的系列腦電圖特點(diǎn)。研究對象2011年12月-2014年12月于首都兒科研究所神經(jīng)科病房確診的兒童抗N-甲基-D-門冬氨酸受體腦炎的20例患者。研究方法對上述患者在精神癥狀和/或癲癇發(fā)作期、無反應(yīng)期、恢復(fù)期分別進(jìn)行視頻腦電圖檢查,分析腦電圖在病程不同時(shí)期的表現(xiàn),總結(jié)腦電圖特點(diǎn)、探尋腦電圖與臨床表現(xiàn)的相關(guān)性。研究結(jié)果共入組20例,男,8例,女,12例,年齡1歲11月-13歲4月。監(jiān)測精神癥狀和/或癲癇發(fā)作期腦電圖25份,23份異常;在無反應(yīng)期描記腦電圖17份,17份異常;恢復(fù)期腦電圖13份,7份異常。異常表現(xiàn)以慢波增加為主,可合并雙側(cè)導(dǎo)聯(lián)不對稱和快波性異常,局灶性棘波。精神癥狀和/或癲癇發(fā)作期以局灶性慢波增多為主要表現(xiàn),無反應(yīng)期以彌漫性慢波增多為主要表現(xiàn),慢波頻率進(jìn)一步減低。極度σ刷(EDB)少見�；謴�(fù)期慢波明顯減少或消失。結(jié)論兒童抗NMDAR腦炎腦電圖的顯著特征是隨病程進(jìn)展而逐漸加重的慢波性異常,EEG的表現(xiàn)與臨床癥狀有一定相關(guān)性。EDB少見。
[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.
【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級別】：博士
【學(xué)位授予年份】：2016
【分類號】：R742.9

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