11例抗神经元抗体阳性的自身免疫性脑炎的临床特征

Clinical Features of 11 Autoimmune Encephalitis Patients with Positive Anti-Neuronal Antibodies

目的:探讨自身免疫性脑炎(AE)的临床特点。方法:回顾性研究2018年1月~2020年3月在我科住院治疗的AE患者,分析人口统计学特点、临床特征、治疗和结局。结果:共纳入11例抗神经元抗体阳性的AE患者,平均年龄44.45±17.90岁。5例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,其中1例合并卵巢畸胎瘤;5例为抗γ-氨基丁酸B受体(GABA_(B)R)脑炎,其中1例合并小细胞肺癌;1例为抗富含亮氨酸胶质瘤失活蛋白1(LGI1)脑炎。在整个病程中,有9例患者出现完全性脑病表现(精神异常+癫痫发作+认知障碍)。9例患者全部接受了一线免疫治疗,5例患者接受了二线免疫治疗和/或长程免疫治疗。2例患者死亡,6例患者预后良好[改良Rankin量表(mRS)≤2分],3例患者中度残疾(mRS=3分)。结论:AE是一类可治疗性很强的疾病,尽早识别及确诊,及时采用适当的治疗干预,可改善患者预后。

Objective:To investigate the clinical features of autoimmune encephalitis(AE).Methods:The demographic characteristics,clinical features,treatment and outcomes were retrospectively analyzed in patients with AE in our hospital between January 2018 and March 2020.Results:A total of 11 AE patients with positive anti-neuronal antibodies were enrolled,with average age at 44.45±17.90 years.There were 5 cases of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis,one of which was complicated with ovarian teratoma.There were 5 cases of anti-gamma-aminobutyric acid type B receptor(GABA_(B)R)encephalitis,one of which was complicated with small cell lung cancer.There was one case of anti-leucine-rich glioma inactivated protein 1(LGI1)encephalitis.Throughout the course of the disease,a total of 9 patients presented with complete encephalopathy,including mental disorder,epileptic seizure,and cognitive impairment.All 11 patients received first-line immunotherapy,and 5 patients received second-line immunotherapy or long-term immunotherapy.Finally,2 patients died,6 patients had a good prognosis with the modified Rankin Scale(mRS)score less than or equal to 2,and 3 patients had moderate disability with mRS score equal to 3.Conclusion:AE is a type of highly treatable disease.Early diagnosis and timely application of appropriate therapeutic intervention can improve the prognosis.