自身免疫性脑炎患者的临床特征、治疗和预后的回顾性研究

目的了解自身免疫性脑炎(autoimmune encephalitis, AE)患者的临床特征和预后。方法采用2013–2019年四川大学华西医院病历资料,对AE患者的临床特征、实验室检查、治疗及预后进行回顾性研究。患者出院时采用改良Rankin量表(mRS)评估临床预后,预后分为良好(mRS评分0～2分)和不良(mRS评分3～6分),logistic多因素回归分析患者预后不良的危险因素。结果本研究共纳入121例患者,男女比例为1∶1.1,确诊时年龄段集中在中青年。抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者占64.5%(78例)。有33.1%(40例)的AE患者首诊于精神科。有62.8%(76例)的患者存在前驱症状。AE患者入院时常见临床表现为认知功能障碍(104例,86.0%)、思维障碍(90例,74.4%)、行为改变(88例,72.7%)。NMDAR抗体在脑脊液中滴度高于血清中的滴度。双阳性抗体在6例患者中检出。42例患者(34.7%)的头部磁共振无异常。71例患者(58.7%)脑电图异常。111例患者(91.7%)接受了一线免疫治疗,61例患者(50.4%)出院后仍存在神...

Disease Characteristics,Treatment, and Prognosis of Chinese Patients with Autoimmune Encephalitis: A Retrospective Study

  Objective  To investigate the clinical features and prognosis of autoimmune encephalitis (AE).  Methods  Accessing data from the electronic medical records of patients receiving care at West China Hospital, Sichuan University, China between 2013 and 2019, we conducted a retrospective study of the disease characteristics, laboratory examinations, treatment, and prognosis of AE patients. Before they were discharged, modified Rankin Scale (mRS) was used to assess the prognosis of AE patients and their mRS scores were then used to categorize patients as having good prognosis (mRS scores of 0-2) or poor prognosis (mRS scores of 3-6). Multivariate logistic regression was used to analyze risk factors associated with poor prognosis.   Results  A total of 121 patients were included in the study. The male-to-female ratio was 1∶1.1. At the time of diagnosis, patients were mainly from the age group of young to middle-aged individuals. Among the 121 patients, 64.5% (78 patients) had anti-N-methyl D-aspartate receptor (NMDAR) encephalitis, accounting for the largest proportion. 33.1% (40 patients) first visited the Department of Psychiatry for medical assistance. 62.8% (76 patients ) showed prodromal symptoms. The common clinical manifestations of the AE patients at the time of admission included cognitive dysfunction (104 patients, 86.0%), thought disorder (90 patients, 74.4%), and altered behaviors (88 patients, 72.7%). The NMDAR antibody titers were higher in the cerebrospinal fluid (CSF) than those in the serum. Double-positive antibodies were detected in 6 patients. No abnormality was observed in the brain magnetic resonance imaging (MRI) of 42 patients (34.7%) . Electroencephalography abnormalities were observed in 71 patients (58.7%). 111 patients (91.7%) received first-line immunotherapy and 61 patients (50.4%) still had neuropsychiatric deficits when they were discharged. Multivariate logistic regression revealed that consciousness disorder (odds ratio [OR]=4.230, 95% confidence interval [CI]: 1.540-11.617; P=0.005), altered behavior (OR=2.997, 95% CI: 1.068-8.406; P=0.037) and movement disorder (OR=7.753, 95% CI: 1.446-41.578; P=0.017) were risk factors for poor clinical prognosis of AE patients.  Conclusions  Patients with AE mainly manifest cognitive damage. Half of the patients left neuropsychiatric deficits. The relationship between CSF titers and serum titers is unparalleled. The options of immunotherapy show no difference in their influence on prognosis. Cognitive dysfunction, altered behavior and movement disorder are independent risk factors for a poor prognosis at discharge.