自身免疫性脑炎合并血脑屏障损伤患者的临床特点及免疫治疗反应性分析  

Clinical characteristics of patients with autoimmune encephalitis combined with blood-brain barrier damage and their response to immunotherapy

作　　者：李怡萌 王瑶瑶 孙一迪 连亚军[1] 程璇[1] Li Yimeng;Wang Yaoyao;Sun Yidi;Lian Yajun;Cheng Xuan(Department of Neurology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院神经内科,郑州450052

出　　处：《中华神经医学杂志》2023年第2期157-164,共8页Chinese Journal of Neuromedicine

摘　　要：目的探讨自身免疫性脑炎(AE)患者的血脑屏障(BBB)完整性与其临床特点、免疫治疗反应性间的关系。方法回顾性收集自2015年8月至2021年12月于郑州大学第一附属医院神经内科确诊的147例AE患者的临床资料及实验室检查数据,以脑脊液白蛋白/血清白蛋白(Qalb)值7.00为界将患者分为BBB正常组(n=101,Qalb值≤7.00)与BBB损伤组(n=46,Qalb值>7.00),分别用改良Rankin量表(mRS)评分及AE临床评估量表(CASE)评分评估患者入院时和一线免疫治疗30 d时的疾病严重程度,并以一线免疫治疗30 d时的CASE评分或mRS评分低于入院时评分定义为免疫治疗反应性良好。对比分析2组患者间一般资料、临床表现、脑脊液及外周血生化检查结果、免疫治疗反应性等的差异。结果BBB损伤组患者中意识水平下降比例(58.7%vs.37.6%),脑脊液蛋白水平增高、IgG增高、24 h鞘内IgG合成率增高比例以及外周血中纤维蛋白原水平均明显高于BBB正常组,差异均有统计学意义(P<0.05)。BBB损伤组患者治疗30 d时mRS评分明显高于BBB正常组,治疗前后CASE评分及mRS评分差值均明显低于BBB正常组(0.50±0.46 vs.3.24±2.93、0.70±0.62 vs.1.15±1.04),差异均有统计学意义(P<0.05)。BBB损伤组患者中接受单一免疫治疗患者的免疫治疗反应性良好率明显低于接受两联、三联免疫治疗患者,差异均有统计学意义(P<0.05)。结论BBB完整性与AE患者的临床特点及免疫治疗反应性密切相关,建议对BBB损伤的AE患者积极采用联合一线免疫治疗方式。Objective To analyze the relations of blood-brain barrier(BBB)integrity with clinical features and their response to immunotherapy in patients with autoimmune encephalitis(AE).Methods One hundred and forty-seven AE patients confirmed in Department of Neurology,First Affiliated Hospital of Zhengzhou University from August 2015 to December 2021 were chosen;their clinical and laboratory data were collected retrospectively.In accordance with cerebrospinal fluid(CSF)albumin/serum albumin(QAlb)value of 7,patients were classified into normal BBB group(n=101,QAlb value≤7.00)and damaged BBB group(n=46,QAlb value>7.00).Modified Rankin Scale(mRS)and Clinical Assessment Scale for Autoimmune Encephalitis(CASE)were used to evaluate the severity on admission and 30 d after first-line immunotherapy;and good immunotherapy responsiveness was defined as CASE or mRS scores 30 d after first-line immunotherapy lower than those at admission.Differences of general data,clinical manifestations,CSF and peripheral blood biochemical results,and response to immunotherapy between the two groups were compared and analyzed.Results The damaged BBB group had significantly higher proportions of patients with decreased consciousness level(58.7%vs.37.6%),increased CSF protein,increased immunoglobulin G(IgG)and increased 24 h intramural IgG synthesis rate,and significantly higher fibrinogen in peripheral blood than normal BBB group(P<0.05).On 30th d of treatment,mRS scores of patients in damaged BBB group were significantly higher than those of patients in normal BBB group(P<0.05),and the differences of CASE scores and mRS scores before and after treatment in damaged BBB group were significantly lower than those in normal BBB group(0.50±0.46 vs.3.24±2.93,0.70±0.62 vs.1.15±1.04,P<0.05).In damaged BBB group,good immunotherapy response rate in patients receiving single immunotherapy was significantly lower than that in patients receiving two or three combinations of first-line immunotherapy(P<0.05).Conclusion BBB integrity is closely related to cl

关 键 词：血脑屏障 自身免疫性脑炎 免疫治疗反应性 临床特点 

分 类 号：R742[医药卫生—神经病学与精神病学]