【摘要】 目的 探讨重症抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor, NMDAR)脑炎的临床特点和预后。方法 回顾性分析2019年7月至2021年10月作者医院收治的13例重症抗NMDAR脑炎患者的临床资料、治疗及预后特点。结果 13例患者中，女12例(92.3%),男1例。主要临床表现为癫痫发作(100%)、精神行为异常(92.3%)、意识水平下降(92.3%)、运动障碍(69.2%)、自主神经功能障碍(61.5%)、记忆障碍(46.2%)、低通气(38.5%)、语言障碍(30.8%)。视频脑电图以慢波活动最多见，5例出现癫痫样异常放电，3例出现δ刷。头颅磁共振检查显示8例患者额叶、颞叶、顶叶、岛叶、海马、丘脑有异常信号。全部患者均接受一线免疫治疗，其中11例应用静脉人免疫球蛋白(intravenous immunoglobulin, IVIg)+甲泼尼龙琥珀酸钠冲击治疗，2例应用甲泼尼龙琥珀酸钠冲击+血浆置换+IVIg治疗。9例患者接受了重复一线治疗，3例患者行二线免疫治疗，均为利妥昔单抗。平均住院时间(41.0±17.3)d,随访时间中位数(四分位数间距)为11.0(11.9)个月，12例患者预后良好，3例患者复发，无患者死亡。结论 重症抗NMDAR脑炎首发症状以癫痫发作和精神行为异常为主，大多数患者病程可出现多个临床症候群。对于重症抗NMDAR脑炎重复一线免疫治疗方案或一线免疫治疗欠佳后及时启动二线免疫治疗可使患者达到良好结局，新的免疫疗法也可有利于改善患者临床症状。更多还原

【Abstract】 Objective To investigate the clinical characteristics and prognosis of severe anti-NMDAR encephalitis. Methods A retrospective study was conducted on patients with severe anti-NMDAR encephalitis of Second Hospital of Hebei Medical University from July 2019 to October 2021. Clinical features, treatment protocols and prognosis data were collected. Results Thirteen patients(12 females and 1 male)were enrolled in the study. The main clinical symptoms included seizures(100%),mental and behavioral abnormalities(92.3%),consciousness disturbance(92.3%),dyskinesia(69.2%),autonomic dysfunction(61.5%),memory loss(46.2%),central hypoventilation(38.5%),and speech disorders(30.8%). Diffuse slow waves activity were the most common electroencephalogram(EEG)abnormalities, 5 patients showed abnormal discharges and 3 patients showed delta brushes.Abnormal findings in brain magnetic resonance imaging scan were observed in 8 patients, located in frontal lobe, temporal lobe, parietal lobe, insular lobe, hippocampus, and thalamus.All patients received first-line immunotherapy. Eleven patients were treated with intravenous immunoglobulin(IVIg)and methylprednisolone therapy, and two patients were treated with methylprednisolone and therapeutic plasma exchange, and IVIg. Nine patients received repeated first-line treatment. Three patients received second-line immunotherapy, all of which were rituximab. The average hospitalized duration was(41.0±17.3)days and the follow-up time[median(interquartile distance)]was 11.0(11.9)months.Twelve patients had a good prognosis, 3 patients relapsed and no patient died. Conclusions The mainly first clinical symptoms of severe anti-NMDAR encephalitis are seizures and mental and behavioral abnormalities, most patients have multiple clinical syndromes during the course of the disease.Repeated first-line immunotherapy regimens or add second-line therapy in severe patients, resulting in good outcomes, and new immunotherapies maybe improve the clinical presentation.更多还原