以癫痫发作为首发症状的自身免疫性脑炎47例临床分析

Clinical analysis of 47 cases of autoimmune encephalitis with epilepsy as the first symptom

目的分析以癫痫发作为首发症状的自身免疫性脑炎(AE)的临床特点.方法分析以癫痫发作为首发症状的AE的患者47例的自然信息、临床特点、影像学检查、实验室检测结果、免疫治疗药物等.结果47例患者中,男性29例,女性18例;发病年龄18~79岁,年龄中位值52岁;抗富亮氨酸胶质瘤失活基因-1(leucinie-richgliomainactivated1,LGI1)脑炎21例,抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartic acid recepto,NMDAR)脑炎16例,抗γ-氨基丁酸B型受体(γ-aminobutyric acid-Breceptor,GABABR)脑炎7例,抗接触蛋白相关蛋白2脑炎(contactin-associated protein 2,CASPR2)2例,抗N-甲基-D-天冬氨酸受体及抗γ-氨基丁酸B型受体双抗体脑炎1例;均以癫痫发作为首发症状,多伴有精神症状21例、记忆力减退24例等,接受免疫治疗患者42例,单独使用糖皮质激素冲击治疗20例,同时使用同时使用糖皮质激素和静脉注射免疫球蛋白治疗22例.结论AE临床表现异质性大,对于癫痫发作的患者需及时考虑到AE的可能,并给予相关检验检查尽早做出诊断,早期启动糖皮质激素及静脉注射免疫球蛋白等免疫治疗.

Objective The clinical characteristics of autoimmune encephalitis(AE)with epilepsy as the first symptom were analyzed Methods Natural information,clinical features,imaging examination,laboratory test results,immunotherapy drugs,etc.of 47 cases of AE patients with epilepsy as the first symptom were analyzed.Results Among the 47 patients,29 were males and 18 were females.The age of onset ranged from 18 to 79 years,and the median age was M=52 years.There were 21 cases of anti-LGI1 encephalitis,16 cases of anti-NMDAR encephalitis,7 cases of antiGABABR encephalitis,2 cases of anti-CASPR2 encephalitis,1 case of anti-NMDAR and anti-GABABR double antibody encephalitis.All patients had epilepsy as the first symptom,most of which were accompanied by mental symptoms in 21 cases and memory loss in 24 cases.42 patients received immunotherapy,20 patients received glucocorticoid shock therapy alone,and 22 patients were treated with simultaneous glucocorticoid and intravenous immunoglobulin.Conclusion The clinical manifestations of AE have a large heterogeneity,so the possibility of AE should be considered in time for patients with epilepsy seizures,and relevant tests should be given to make diagnosis as soon as possible,glucocorticoid and intravenous immunoglobulin immunotherapy should be initiated early.