摘要目的 总结抗N-甲基-D-天冬氨酸(NMDA)受体脑炎共患白质脱髓鞘病例的临床和预后特点,探讨其可能的发病机制.方法 分析3例抗NMDA受体脑炎共患白质脱髓鞘患儿的临床和影像学特征,并以"抗NMDA受体脑炎"和"脱髓鞘"及"anti-NMDA receptor encephalitis"和"demyelinating"为检索词分析检查CNKI、万方及PubMed数据库(建库至2017年5月),结合既往文献报道病例总结分析.结果 本组资料3例患儿中2例抗NMDA受体脑炎和白质脱髓鞘同时发生,1例先后发生.1例病例共患AQP4抗体阳性.2例为复发病程,1例单向病程,且预后极差.文献检索共搜集到15篇报道共41例抗NMDA受体脑炎共患白质脱髓鞘的病例,其中起病年龄≤18岁的16例(39.02％).这16例儿童病例中,7例(43.75％)抗NMDA受体脑炎和白质脱髓鞘先后出现,9例(56.25％)同时出现.16例患儿均行血清和/或脑脊液AQP4抗体和MOG抗体检查,其中9例(56.25％)存在一种抗体阳性.结论 抗NMDA受体脑炎和白质脱髓鞘可同时或先后发生.抗NMDA受体脑炎共患白质脱髓鞘较单纯抗NMDA受体脑炎更易呈现复发性的病程,预后可能更差.抗NMDA受体脑炎与白质脱髓鞘二者存在共同免疫途径异常或抗N M D A抗体自身介导脱髓鞘可能是其潜在的发病机制.

abstractsObjective To summarize the clinical and prognostic features of anti - N - methyl - D - aspartate receptor (NMDAR)encephalitis with demyelinated lesions and discuss the possible pathogenesis. Methods The clini-cal and imaging features of 3 pediatric patients diagnosed as anti - NMDAR encephalitis with demyelinated lesions were analyzed. The published papers were browsed by using " anti - NMDA receptor encephalitis" and " demyelinating"as key words into CNKI,Wanfang and PubMed database from starting point to May,2017. Results In 3 cases,anti -NMDAR encephalitis occurred simultaneously with demyelinated episodes in 2 cases,successively in the other case. One case had AQP4 - IgG positive. Two cases had recurrent course,and 1 case had a single course and poor prognosis. A to-tal of 15 articles reported 41 cases,including 16 (39. 02％)pediatric cases. In these pediatric cases,anti - NMDAR encephalitis occurred in 7 cases (43. 75％)successively and demyelinated episodes occurred in 9 cases (56. 25％) simultaneously. AQP4 antibody and MOG antibody in serum and/ or cerebrospinal fluid were detected in all cases,with either of two antibodies positive in 9 cases (56. 25％). Conclusion Anti - NMDAR encephalitis might occur simulta-neously or successively with demyelinated episodes. Compared with typical patients with anti - NMDAR encephalitis, patients with demyelinated lesions are more likely to relapse and have worse outcomes. Anti - NMDAR encephalitis and demyelinated lesions are both based on similar immune dysfunction or demyelinated lesions are also induced by anti -NMDAR antibodies,which is the probable pathogenesis.