本文關鍵詞： 抗N-甲基-D-天冬氨酸受體 腦炎 兒童 臨床特征　出處：《中國實用兒科雜志》2017年07期 　論文類型：期刊論文

【摘要】：目的探討兒童抗N-甲基-D-天冬氨酸受體(NMDAR)腦炎的臨床特點。方法對2015年4月至12月復旦大學附屬兒科醫(yī)院神經(jīng)內(nèi)科收治的15例確診為抗NMDAR腦炎患兒資料進行回顧性分析。結果 15例中男8例、女7例,年齡8個月至12歲,中位發(fā)病年齡7歲。臨床癥狀:首發(fā)癥狀意識障礙伴驚厥3例(20.0%),精神行為異常4例(26.7%)。15例均有驚厥發(fā)作,睡眠障礙5例(33.3%);發(fā)生驚厥持續(xù)狀態(tài)3例;意識障礙10例(66.7%),昏迷3例;肢體或顏面部不自主運動12例(80.0%);尿潴留7例(46.7%)。中樞性低通氣3例(20.0%)。繼發(fā)于感染后2例(13.3%)。輔助檢查:均行腦脊液檢查,5例(33.3%)白細胞輕度升高,2例(13.3%)蛋白明顯升高;腦脊液抗NMDAR抗體均陽性;其中10例行IgG指數(shù)檢查,4例(40.0%)Ig G指數(shù)升高。10例(66.7%)頭顱磁共振成像(MRI)異常。腦電圖均為背景活動變慢,9例(60.0%)癇樣放電,未發(fā)現(xiàn)δ刷。僅1例(6.7%)發(fā)現(xiàn)卵巢占位性病變。治療和隨訪:均應用靜脈甲潑尼龍加丙種球蛋白免疫治療,1例(6.7%)給予血漿置換。隨訪1~6個月,9例改良Rankin量表評級為0~2級。結論抗NMDAR腦炎臨床表現(xiàn)和實驗室檢查缺乏特異性,初期診斷困難。腦脊液特異性抗NMDAR抗體陽性可確診該病。兒童抗NMDAR腦炎患者腫瘤發(fā)生率低,腦電圖δ刷少見。早期診斷和及時應用免疫抑制劑治療,預后良好。
[Abstract]:Objective to investigate the anti N- methyl -D- aspartate receptor (NMDAR) encephalitis clinical features. Methods 15 cases from April 2015 to December in Paediatrics Hospital Affiliated to Fudan University from the Department of Neurology for anti NMDAR encephalitis were retrospectively analyzed. Results among the 15 cases, male 8 cases, female 7 cases, aged 8 months to 12 years in the median age was 7 years old. The clinical symptoms: symptoms of disturbance of consciousness with convulsion in 3 cases (20%), 4 cases of abnormal mental behavior (26.7%).15 cases were seizure onset, sleep disorders in 5 cases (33.3%) occurred in 3 cases; status epilepticus; 10 cases of disturbance of consciousness (66.7%), 3 cases of coma or limb; the face of involuntary movement in 12 cases (80%); 7 cases of urinary retention (46.7%). 3 cases of central hypoventilation (20%). Secondary to infection in 2 cases (13.3%). Auxiliary examination: 5 cases underwent the examination of cerebrospinal fluid, (33.3%) white blood cells increased slightly, 2 cases (13.3%) was significantly elevated CSF; anti NMDAR antibody Positive; 10 cases underwent IgG examination index, 4 cases (40% cases).10 Ig G index was significantly increased (66.7%) brain magnetic resonance imaging (MRI) abnormalities. EEG background activity was slow, 9 cases (60%) of epileptic discharge, did not find the delta brush only 1 cases (6.7%) found that ovarian lesions lesions. Treatment and follow-up: intravenous injection of methylprednisolone plus gamma globulin immune therapy, 1 cases (6.7%) received plasma exchange. The follow-up of 1~6 months, 9 cases of modified Rankin scale rating of 0~2. Conclusion anti NMDAR encephalitis clinical manifestation and laboratory examination of lack of specificity, the early diagnosis of cerebral spinal fluid. The specificity of anti NMDAR antibody positive can be diagnosed the disease in children. The incidence of tumors in patients with anti NMDAR encephalitis is low, EEG delta brush rare. Early diagnosis and timely application of immunosuppressive therapy, the prognosis is good.

【作者單位】： 復旦大學附屬兒科醫(yī)院神經(jīng)內(nèi)科;揚州大學附屬醫(yī)院(原揚州市第一人民醫(yī)院)兒科;
【分類號】：R742.9

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