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中国循证儿科杂志

中国循证儿科杂志 ›› 2016, Vol. 11 ›› Issue (3): 182-186.

• 论著 • 上一篇    下一篇

抗N-甲基-D-天冬氨酸受体脑炎102例临床特征及随访

张炜华1，王晓慧1，方方1，吕俊兰1，丁昌红1，陈春红1，王旭1，韩彤立1，伍妘1，李久伟1，王红梅1，杨欣英1，张珅1，孙欣1，邓劼1，代丽芳1，任海涛2，崔丽英2   

  1. 1 首都医科大学附属北京儿童医院 北京，100045；2 中国医学科学院中国协和医科大学 北京，100730
  • 收稿日期:2016-03-30 修回日期:2016-06-25 出版日期:2016-06-25 发布日期:2016-06-25
  • 通讯作者: 王晓慧

Clinical characteristics and follow-up of anti-N-methyl-D-aspartate receptor encephalitis in 102 children

ZHANG Wei-hua1, WANG Xiao-hui1, FANG Fang1, LV Jun-lan1, DING Chang-hong1, CHEN Chun-hong1, WANG Xu1, HAN Tong-li1, WU Yun1, LI Jiu-wei1, WANG Hong-mei1, YANG Xin-ying1, ZHANG Shen1, SUN Xin1, DENG Jie1, DAI Li-fang1, REN Hai-tao2, CUI Li-ying2   

  1. 1 Beijing Children′s Hospital, Capital Medical University, Beijing 100045, China; 2 Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
  • Received:2016-03-30 Revised:2016-06-25 Online:2016-06-25 Published:2016-06-25
  • Contact: WANG Xiao-hui

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摘要：

目的 分析抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患儿的临床特征及预后。方法 对首都医科大学北京儿童医院神经内科2012年1月至2014年12月收治的抗NMDA受体脑炎患儿的确诊前情况、临床发现、实验室检查和治疗情况进行回顾性分析，并对患儿进行随访，采用改良Rankin量表（MRS）在末次随访时评估预后。结果 102例抗NMDA受体脑炎患儿纳入分析，男38例（37.2％），发病年龄6月龄至15.8岁，平均（7.5±4.0）岁。男女发病年龄分别为（6.2±3.8）岁和（8.3±3.9）岁（P=0.009）。①17例（16.7%）在确诊前诊断为病毒性脑炎。27例（26.5%）起病1个月内有感染史。合并卵巢畸胎瘤2例。②102例确诊时中位病程24.5 d。40例（39.2%）以精神症状首发，62例（61.8%）以神经系统症状首发。急性期症状包括精神行为异常（99例，97.1%）、运动障碍（98例，96.1%）、语言障碍（91例，89.2%）、睡眠障碍（79例，77.5%）、意识障碍（77例，75.5％）、惊厥（56例，54.9％）、植物神经功能紊乱（35例，34.3％）和中枢性低通气（7例，6.9%），86例（84.3%）在达病情高峰时出现≥4种症状。③102例CSF抗NMDA受体抗体均阳性，76例（74.5%）同期血清抗NMDA受体抗体阳性。头颅MRI检查异常30/102例（29.4%），主要表现为额叶、颞叶、丘脑等不同程度的限局性脑软化、坏死。④102例均接受IVIG和甲泼尼龙治疗，18例（17.3%）予利妥昔单抗治疗。⑤88例随访1~4年，其中74例（84.1%）预后良好 ，14例（15.9%）预后不良。复发4例（3.9％），死亡3例（3.4%）。结论 抗NMDA受体脑炎可发生于儿童各年龄段，临床特征与成人相似，总体预后良好，但小年龄及继发于病毒性脑炎患儿预后不良。

Abstract:

Objective To describe the clinical manifestations and prognosis of children with anti-N-methyl-D-aspartate receptor encephalitis.Methods Clinical manifestations, lab examination, management and prognosis of children with anti-N-methyl-D-aspartate receptor encephalitis hospitalized in Neurology Department of Beijing Children′s Hospital from January 2012 to December 2014 were retrospectively analyzed and the effect at the last follow-up was assessed by Modified Rankin Scale (MRS).Results A total of 102 cases diagnosed as anti-N-methyl-D-aspartate receptor encephalitis with the average onset age of (7.5±4.0) years ranged from 6 months to 15.8 years were enrolled into the analysis including 38 males and 64 females. The average onset age of females was elder than that of males (P=0.009). Psychiatric disturbances was found in 40 cases and neurology disorder was found in 62 cases as the initial symptoms. The symptoms in acute stage included mental and behavior disorder (99/102，97.1%), dyskinesia (98/102，96.1%), speech disorder (91/102，89.2%), sleep disorder (79/102，77.5%) and central hypoventilation (7/102，6.9%), and there was no significant difference among different age groups. All cases received the first line therapy, 18(17.3%) cases received the second line therapy, 14 cases(15.9%) had sequelae (MRS≥3), 3 cases died and 4 cases relapsed. Conclusion Anti-N-methyl-D-aspartate receptor encephalitis happened in different age groups, clinical manifestations were similar to adult, most of cases had favourable prognosis, but young children and those secondary to virus encephalitis had poor prognosis.