摘要目的 分析中国人抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床及辅助检查特点.方法 对255例临床疑似脑炎患者的血清及脑脊液行抗NMDAR-IgG检测.结合临床表现确诊抗NMDAR脑炎患者,收集其临床及辅助检查资料,并按年龄分为＜12岁,12～17岁以及＞17岁3组.筛查上述患者标本中其他自身免疫及感染相关抗体,并对25例确诊半年以上患者进行随访.结果 自255例中共确诊45例抗NMDAR脑炎患者(占17.6％),男∶女为24∶21,中位年龄10岁,3例患者合并可疑盆腔占位.发病时间多集中在5月、6月、9月及10月.3个年龄组首发症状不同,发病1个月内主要症状亦不相同.26例患者血清和脑脊液抗NMDAR-IgG均阳性,17例患者仅脑脊液IgG阳性,2例患者仅血清IgG阳性.脑脊液抗NMDAR-IgA阳性患者的脑脊液抗NMDAR-IgG滴度高于脑脊液IgA阴性患者.部分患者血清中存在其他自身免疫抗体.6例患者血清风疹病毒IgM阳性,1例血清单核细胞增多性李斯特菌IgM阳性.抗NMDAR脑炎患者脑脊液细胞学演变过程与病毒性脑炎类似.45例患者均行脑电图检查,41例表现异常,主要为广泛慢波,1例出现“极端δ刷”(extreme delta brush).15例患者头颅MRI表现异常.全部患者均接受一线免疫治疗,9例患者接受二线免疫治疗.随访25例确诊半年以上患者,发现脑脊液抗体滴度与患者预后无关.改良Rankin量表(mRS)评分≥5分及mRS评分≤2分患者的中位确诊时间分别为35 d及17 d,差异有统计学意义(Z=-2.096,P=0.036).结论 我国抗NMDAR脑炎发病率被低估.本组抗NMDAR脑炎患者相对西方患者具有男性比例高、年轻患者多、合并肿瘤者少等特点.行为异常、运动障碍、认知缺陷、癫痫发作及自主神经功能障碍为本组患者的五大早期表现.抗NMDAR-IgG可能多合成自鞘内.一些感染因子可能参与本病的发生.本病无特异影像学表现.早期诊断对预后十分重要.

abstractsObjective To analyse the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in Chinese patients.Methods In this study,we tested the presence of antiNMDAR-IgG in serum and cerebrospinal fluid (CSF) of 255 clinical suspected encephalitis patients.AntiNMDAR encephalitis was diagnosed according to the clinical features and anti-NMDAR-IgG positivity.Clinical data of the diagnosed patients were collected.Then patients were divided into three groups according to ages (＜ 12 years old,12-17 years old and ＞ 17 years old).Their samples were sent out for further 10.3760/cma.j.issn.1006-7876.2014.07.009autoimmune and infectious antibodies scanning.Twenty-five patients diagnosed over 6 months were followed up.Results Anti-NMDAR encephalitis was found in 45 of 255 patients (17.6％),with a male:female ratio of 24∶ 21.Median age at disease onset was 10 years.Three patients had suspicious pelvic lesions.The highest incidence of symptoms onset was presented in May,June,September and October.The frequencies of the first symptoms were different among three age groups.During the first month of the disease,the frequencies of symptoms were also different from one another in three age groups.Twenty-six patients were found anti-NMDAR-IgG positive in both sera and CSF,17 patients only CSF IgG positive and 2 patients only sera IgG positive.Titers of CSF anti-NMDAR-IgG were higher in the CSF anti-NMDAR-IgA positive patients than those negative.Some patients were found with other autoimmune antibodies.Serum anti-rubella virus IgM was found positive in 6 patients and serum anti-Listeria monocytogenes IgM positive in 1 patient.Evolution of CSF cytology in patients with anti-NMDAR encephalitis was similar to those with viral encephalitis.Electroencephalograms were found abnormal in 41 patients.Universal slow wave was the most common finding and extreme delta brush was noted in one patient.Head MR images were abnormal in 15 patients.All the patients received first-line immunotherapies,and 9 patients also received second-line immunotherapies.During the follow-up of 25 patients who were diagnosed over 6 months,CSF anti-NMDAR-IgG titers were not relevant with the prognosis.The median diagnostic intervals of mRS score ≥ 5 and mRS score ≤2 were 35 days and 17 days,respectively (Z =-2.096 P =0.036).Conclusions The prevalence of anti-NMDAR encephalitis has been underestimated in China.This cohort has higher male to female ratio,younger age and less proportion of tumor than the western patients.Behavioral changes,movement disorders,cognitive deficits,seizures and autonomic symptoms are the top 5 early clinical features in this cohort.The majority of anti-NMDAR-IgG may be intrathecally synthesized.Some infectious agents may contribute to the disease.Non-specific alterations of cranial magnetic resonance images were found in our research.Early diagnosis is vital for prognosis.