抗接触蛋白相关蛋白-2抗体相关脑炎七例临床分析  

Clinical analysis of 7 cases with anti-Caspr2 antibody-associated autoimmune encephalitis

作　　者：郭亚培[1] 李雪元 刘恒方[1] 张敏[1] 石磊[1] 赵幸娟 李建章 刘绪园 崔静[3] Guo Yapei;Li Xueyuan;Liu Hengfang;Zhang Min;Shi Lei;Zhao Xingjuan;Li Jianzhang;Liu Xuyuan;Cui Jing(Department of Neurology,Fifth Affiliated Hospital,Zhengzhou University,Zhengzhou 450052,China;Department of Neurosurgery,First Affiliated Hospital,Zhengzhou University,Zhengzhou 450052,China;Department of Radiology,Henan Provincial People′s Hospital,Zhengzhou 450003,China)

机构地区：[1]郑州大学第五附属医院神经内科,郑州450052 [2]郑州大学第一附属医院神经外科,郑州450052 [3]河南省人民医院影像科,郑州450003

出　　处：《中华医学杂志》2020年第7期513-515,共3页National Medical Journal of China

基　　金：河南省科技发展计划(182102310533)。

摘　　要：目的探讨抗接触蛋白相关蛋白-2(CASPR2)抗体相关脑炎的临床表现、实验室检查、影像特点、脑电图表现、治疗和预后,提高对该疾病的认识。方法回顾性分析2017年1月至2019年1月在郑州大学第五附属医院及郑州大学第一附属医院确诊的7例抗CASPR2抗体相关脑炎患者资料并随访。结果7例患者中男4例,女3例,中位发病年龄为34岁。癫痫发作6(6/7)例,记忆力减退5(5/7)例,精神症状4(4/7)例;CASPR2抗体:脑脊液阳性3(3/7)例,血清阳性7(7/7)例;5(5/7)例有影像学改变,1(1/7)例发现肿瘤。7例均接受免疫治疗,随访1个月至2年,1例患者确诊直肠癌,预后差。其余患者预后好。结论抗CASPR2抗体相关脑炎是一种罕见的自身免疫性脑炎,临床上有以癫痫发作,记忆力减退,精神症状,头晕,睡眠障碍等为主要临床表现的疾病时,需考虑到该疾病。早期识别该疾病,激素和免疫球蛋白治疗该疾病,患者大多预后良好。Objective To investigate the clinical features, laboratory tests, imaging features, electroencephalogram (EEG) manifestations, treatment and prognosis of anti-CASPR2 antibody-associated autoimmune encephalitis with the purpose to improve the understanding of the disease.Methods Seven patients diagnosed with CASPR2 antibody-associated encephalitis in the Fifth and first Affiliated Hospital of Zhengzhou University from January 2017 to January 2019 were selected. The clinical manifestations, course of disease, imaging, treatment plan and prognosis of the patients were retrospectively analyzed.Results Of the 7 patients, 4 were male and 3 were female. The median age of onset was 34 years old (range, 11-66 years). Seizures (6/7), memory decline(5/7), and neuropsychiatric disorders (4/7) were common clinical manifestations. CASPR2 antibody was detected in the CSF of 3 patients (3/7) and the serum of all patients (7/7). Notable imaging changes were observed in 5 patients (5/7). All patients received immunotherapy and were followed up for 1 month to 2 years. One patient was diagnosed with rectal cancer with a poor prognosis;all the others got a good prognosis.Conclusions CASPR2 antibody-related encephalitis is a rare autoimmune disease. It is considered to present with seizures, memory loss, psychiatric symptoms, dizziness and sleep disorders as the main clinical manifestations. Early identification and treatment of the disease can help the patients achieve a good prognosis.

关 键 词：CASPR2抗体 自身免疫性脑炎 癫痫 免疫治疗 

分 类 号：R742[医药卫生—神经病学与精神病学]