本文選題：N-甲基-D-天冬氨酸受體　切入點(diǎn)：腦炎　出處：《臨床兒科雜志》2017年06期 　論文類型：期刊論文

【摘要】：目的探討重癥抗N-甲基-D-天冬氨酸受體(NMDAR)腦炎患兒的臨床特點(diǎn)及治療方案。方法回顧性分析4例重癥抗NMDAR腦炎患兒的臨床資料以及隨訪情況。結(jié)果 4例患兒,男1例,女3例,年齡10~13歲,其中1例合并畸胎瘤�；純壕跃癜Y狀和不自主運(yùn)動(dòng)為主要首發(fā)癥狀,1個(gè)月內(nèi)進(jìn)展為癲癇發(fā)作和意識(shí)障礙及中樞低通氣性呼吸衰竭。腦脊液抗NMDAR抗體均為陽性,腦電圖出現(xiàn)慢波,診斷時(shí)顱腦磁共振無改變。應(yīng)用大劑量丙種球蛋白(IVIG)和甲基潑尼松龍沖擊治療,呼吸機(jī)治療5~95天,其中氣管切開2例,1例因感染死亡。隨訪21~27個(gè)月,1例臨床痊愈;2例持續(xù)應(yīng)用免疫抑制劑和抗癲癇藥物至今,臨床癥狀均明顯改善,合并腫瘤患兒腦電圖和抗NMDAR抗體持續(xù)異常,另1例復(fù)發(fā)。結(jié)論重癥抗NMDAR腦炎多見于較大年齡女童,病程早期即發(fā)生中樞性低通氣性呼吸衰竭,合并畸胎瘤是高危因素,常頻呼吸機(jī)和反復(fù)應(yīng)用激素聯(lián)合IVIG治療有效,病程長(zhǎng),恢復(fù)緩慢,1年后仍可出現(xiàn)復(fù)發(fā)。
[Abstract]:Objective to investigate the clinical characteristics and treatment of severe anti-N- methyl-Daspartic acid receptor (NMDAR) encephalitis in children. Methods the clinical data and follow-up data of 4 children with severe anti NMDAR encephalitis were retrospectively analyzed. Three women, aged 10 to 13, Among them, one case was complicated with teratoma. The main symptoms were mental symptoms and involuntary movement. The children developed epilepsy, disturbance of consciousness and central hypopnea respiratory failure within one month. Cerebrospinal fluid anti NMDAR antibody was positive. There were slow waves in EEG and no change in MRI at the time of diagnosis. The patients were treated with high dose gamma globulin (IVIGG) and methylprednisolone for 5 to 95 days. One case died of infection after tracheotomy, and 1 case was cured from 21 to 27 months, 2 cases were treated with immunosuppressant and antiepileptic drug, the clinical symptoms were improved obviously, EEG and anti NMDAR antibody were abnormal in children with tumor. Conclusion severe anti NMDAR encephalitis is more common in older girls. Central hypopnea respiratory failure occurs in the early stage of the disease. Teratoma is a high risk factor. Frequent ventilator and repeated use of hormone combined with IVIG are effective. The course of disease is long, the recovery is slow, after 1 year still can appear relapse.
【作者單位】： 青島大學(xué)附屬醫(yī)院兒童醫(yī)學(xué)中心;首都醫(yī)科大學(xué)附屬北京兒童醫(yī)院;
【分類號(hào)】：R742.9

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