抗富亮氨酸胶质瘤失活1蛋白抗体相关脑炎85例特点和临床转归分析  

Clinical characteristics and prognosis of anti-Leucine-rich glioma inactivated 1 antibody-associated encephalitis:an analysis of 85 cases

作　　者：于莎莎 吕晶 张玉婧 祖煜 冯雪丹[1] Yu Shasha;Lyu Jing;Zhang Yujing;Zu Yu;Feng Xuedan(Department of Neurology,the Being Fengtai Youanmen Hospital,Beijing 100069,China)

机构地区：[1]北京丰台右安门医院神经内科,北京100069

出　　处：《脑与神经疾病杂志》2024年第10期599-603,共5页Journal of Brain and Nervous Diseases

基　　金：北京丰台区卫生健康系统科研项目(2023-73)。

摘　　要：目的探讨抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关脑炎患者的临床特点、治疗和预后。方法分析北京丰台右安门医院神经内科2014年8月至2023年6月连续收治的85例抗LGI1抗体相关脑炎患者的临床资料。结果本组中位起病年龄为60岁(24~84岁),其中男性51例,3例(4%)合并肿瘤。临床表现为出现癫痫发作(85%)、精神症状(60%)、认知障碍(88%)、意识障碍(15%)。68例(91%)脑电图(EEG)异常,72例(90%)头颅MRI有异常信号,其中61%颞叶、海马异常信号。所有患者均给予一线免疫治疗,1例使用利妥昔单抗,12例加用吗替麦考酚酯。为期21.5个月(1~82个月)随访,74例(91%)预后良好,改良Rakin量表(mRS评分0~2分),7例(9%)预后不良(mRS评分3~6分),11例(14%)复发。结论抗LGI1抗体相关脑炎多见于中老年人,男性居多,肿瘤合并率低。以癫痫发作、认知障碍、精神行为异常、血及脑脊液(CSF)抗LGI1受体抗体阳性为其主要临床特点。大部分患者对免疫治疗反应良好,预后佳。复发的主要原因为合并恶性肿瘤或减停免疫抑制剂。肺部感染和合并肿瘤患者是预后不良的危险因素。Objective To explore the clinical characteristics,treatment,and prognosis of patients with antileucine-rich glioma inactivated 1(LGI1)antibody-associated encephalitis.Methods A retrospective analysis was conducted on the clinical data of 85 consecutive patients with anti-LGI1 antibody-associated encephalitis treated at the Neurology Department of Beijing Fengtai You'anmen Hospital from August 2014 to June 2023.Results The median age of onset in our cohort was 60 years(range:24-84 years),with 51 male patients and 3 cases(4%)of concomitant tumors.Clinical manifestations included seizures(85%),psychiatric symptoms(60%),cognitive impairment(88%),and altered consciousness(15%).Abnormalities were observed in 68 cases(91%)on electroencephalogram(EEG)and in 72 cases(90%)on cranial MRI,with abnormal signals mainly in the temporal lobes and hippocampus(61%).All patients received first-line immunotherapy,with 1 case treated with rituximab and 12 cases receiving additional methylprednisolone.The median follow-up period was 21.5 months(range:1-82 months),with 74 patients(91%)showing good prognosis(modified Rankin Scale score 0-2),7 patients(9%)with poor prognosis(modified Rankin Scale score 3-6),and 11 patients(14%)experiencing relapse.Conclusion Anti-LGI1 antibody-associated encephalitis is more common in middle-aged and elderly males,with a low incidence of concomitant tumors.Key clinical features include seizures,cognitive impairment,psychiatric symptoms,and positivity for blood and cerebrospinal fluid LGI1 receptor antibodies.Most patients respond well to immunotherapy with a favorable prognosis.The main causes of relapse are concomitant malignant tumors or reduction/cessation of immunosuppressive agents.Pulmonary infections and concomitant tumors are risk factors for poor prognosis.

关 键 词：抗LGI1抗体相关脑炎 临床表现 脑脊液 头颅MRI 预后 

分 类 号：R512.3[医药卫生—内科学]