伴癫痫发作的髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病患者临床特点及危险因素分析  

Clinical features and risk factors analysis of myelin oligodendrocyte glycoprotein immunoglobulin G-antibody associated disease with epilepsy

作　　者：王瑶瑶 孙一迪 李怡萌 马蕴青 张海峰[1] 程璇[1] Wang Yaoyao;Sun Yidi;Li Yimeng;Ma Yunqing;Zhang Haifeng;Cheng Xuan(Department of Neurology,the 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：《中华神经科杂志》2024年第6期616-624,共9页Chinese Journal of Neurology

摘　　要：目的:分析伴癫痫发作的髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(MOGAD)患者的临床特征及其对免疫治疗的反应性,并探讨影响MOGAD伴癫痫发作的危险因素。方法:回顾性分析2019年10月至2023年5月郑州大学第一附属医院神经内科收治的89例MOGAD患者的临床资料及实验室检查数据,根据是否伴癫痫发作将患者分为伴癫痫发作组( n=29)与不伴癫痫发作组( n=60),分别用扩展残疾状况量表(EDSS)、自身免疫性脑炎临床评估量表(CASE)评估不同时期患者的病情严重程度,以一线免疫治疗30 d时EDSS或CASE评分低于入院时评分作为免疫治疗反应良好。比较两组患者在一般资料、临床特征、脑脊液与外周血生化检查结果、免疫治疗反应良好率之间的差异,并用单因素及多因素Logistic回归分析方法分析影响癫痫发作的危险因素。 结果:与不伴癫痫发作组相比,伴癫痫发作组患者发病年龄偏低[24.5(10.3,34.0)岁比11.0(6.5,20.0)岁, Z=-2.348, P=0.019],男性患者比例偏高[43.3%(26/60)比75.9%(22/29),χ^( 2)=8.326, P=0.004],病毒感染率[28.3%(17/60)比51.7%(15/29),χ^( 2)=4.645, P=0.031]、前驱症状发生率[11.7%(7/60)比34.5%(10/29),χ^( 2)=6.586, P=0.010]、血脑屏障损伤率[35.0%(21/60)比58.6%(17/29),χ^( 2)=4.458, P=0.035]、脑脊液白蛋白>450 mg/L者比例[48.3%(29/60)比75.9%(22/29),χ^( 2)=6.056, P=0.014]、肌酸激酶水平[45.50(28.50,69.75)U/L比57.50(41.75,97.25)U/L, Z=-2.349, P=0.019]较高;临床表现以癫痫发作[0(0)比29/29(100.0%),χ^( 2)=89.000, P<0.001]、意识障碍[0(0)比6/29(20.7%),χ^( 2)=10.224, P=0.001]为主,影像学示病灶主要在大脑皮质[30/60(50.0%)比25/29(86.2%),χ^( 2)=10.856, P=0.001]。而不伴癫痫发作组临床表现则以视物模糊[23/60(38.3%)比3/29(10.3%),χ^( 2)=7.406, P=0.007]、肢体无力[18/60(30.0%)比1/29(3.4%),χ^( 2)=8.209, P=0.004]、感觉障碍[15/60(25.0%)比0(0),Fisher精确概率法检验, P=0.002]为主,病灶累及�Objective To analyze the clinical features and immunotherapy responsiveness of patients with myelin oligodendrocyte glycoprotein immunoglobulin G-antibody associated disease(MOGAD)with epilepsy,and display the risk factors of epilepsy in MOGAD.Methods Eighty-nine patients with MOGAD diagnosed at the First Affiliated Hospital of Zhengzhou University between October 2019 and May 2023 were enrolled and classified into 2 groups upon MOGAD with(n=29)or without epilepsy(n=60).The Expanded Disability Status Scale(EDSS)and Clinical Assessment Scale for Autoimmune Encephalitis(CASE)were used for evaluation of severity,and EDSS or CASE scores on the 30th day after first-line immunotherapy initiation lower than that on admission were defined as well treatment responsiveness.The differences of general data,clinical manifestations,cerebrospinal fluid(CSF)and peripheral blood biochemical examination results,and immunotherapy reactivity between the 2 groups were thoroughly explicated.In addition,the risk factors of epilepsy in MOGAD were analyzed by univariate and multivariate Logistic regression analysis.Results Compared with patients with MOGAD without epilepsy,patients with MOGAD with epilepsy were characterized by lower age of onset[24.5(10.3,34.0)years vs 11.0(6.5,20.0)years,Z=-2.348,P=0.019],higher percentage of male patients[43.3%(26/60)vs 75.9%(22/29),χ2=8.326,P=0.004],higher virus infection rate[28.3%(17/60)vs 51.7%(15/29),χ2=4.645,P=0.031],higher incidence of prodromal symptoms[11.7%(7/60)vs 34.5%(10/29),χ2=6.586,P=0.010],higher blood-brain barrier breakdown rate[35.0%(21/60)vs 58.6%(17/29),χ2=4.458,P=0.035],higher percentage of CSF albumin level>450 mg/L[48.3%(29/60)vs 75.9%(22/29),χ2=6.056,P=0.014]and higher creatine kinase level[45.50(28.50,69.75)U/L vs 57.50(41.75,97.25)U/L,Z=-2.349,P=0.019];more epilepsy[0(0)vs 29/29(100.0%),χ2=89.000,P<0.001]and disturbance of consciousness[0(0)vs 6/29(20.7%),χ2=10.224,P=0.001]as clinical manifestations,and more cerebral cortex lesions[30/60(50.0%)vs 25/29(86.2%),χ2=10.8

关 键 词：神经系统自身免疫疾病 癫痫 发作 危险因素 髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病 临床特点 

分 类 号：R741[医药卫生—神经病学与精神病学]