31例抗富含亮氨酸胶质瘤失活蛋白1抗体相关脑炎患者的临床特点  

Clinical characteristics of 31 patients with anti-leucine-rich glioma inactivated-1 antibody-associated encephalitis

作　　者：徐淑兰[1] 李薇[1] 陈道文[1] XU Shulan;LI Wei;CHEN Daowen(Department of Geriatric,Brain Hospital Affiliated to Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学附属脑科医院老年医学科,210029

出　　处：《临床神经病学杂志》2023年第6期447-451,共5页Journal of Clinical Neurology

摘　　要：目的分析抗富含亮氨酸胶质瘤失活蛋白1(LGI1)抗体相关脑炎的临床特点,以提高对其的临床识别,提高诊断率、改善预后。方法收集2020年1月至2022年3月在南京脑科医院连续住院的31例抗LGI1抗体相关脑炎患者的临床资料。按照初始免疫治疗方案是否添加吗替麦考酚酯(MMF)分为一线免疫治疗组、MMF组。采用单因素及多因素COX回归分析抗LGI1抗体相关脑炎患者复发的相关因素。用mRS量表评价患者预后。结果31例抗LGI1抗体相关脑炎患者中,临床症状以痫性发作(93.5%)、记忆力下降(80.6%)及精神症状(58.1%)最为常见。14例患者出现低钠血症,1例患者出现CSF白细胞增高,17例患者出现CSF蛋白增高。血清和CSF抗体阳性率分别为90.3%、93.1%。14例患者头颅MR异常。所有患者均接受一线免疫治疗,另有13例患者后续接受MMF治疗。随访发现11例患者出现复发,单因素COX回归发现与复发相关的危险因素为女性(HR=3.85,95%CI:1.12~13.24,P=0.032)、添加MMF治疗(HR=4.03,95%CI:1.07~15.22,P=0.04)。多因素COX回归分析没有发现独立相关危险因素。随访发现22例患者遗留后遗症,以记忆力下降最常见;所有患者预后良好。结论抗LGI1抗体相关脑炎总体预后良好,部分患者会复发。大部分患者会遗留有后遗症,以记忆障碍及精神症状多见。MMF对抗LGI1抗体相关脑炎可能无预防复发的作用。Objective To improve the clinical recognition of anti-leucine-rich glioma inactivated-1(LGI1)antibody associated encephalitis through investigating the clinical characteristics of the disease,so as to improve diagnosis and prognosis.Methods A total of 31 consecutive patients with anti-LGI1 antibody associated encephalitis were recruited during January 2020 to March 2022 in Brain Hospital Affiliated to Nanjing Medical University including their clinical manifestations.Patients were divided into first-line immunotherapy group and mycophenolate mofetil(MMF)adding-on group according to the initial treatment regimen.Univariate and multivariate COX regression analysis were used to analyze the factors of the recurrence of anti-LGI1 antibody encephalitis.mRS was used to evaluate the prognosis.Results Seizure(93.5%),memory defect(80.6%)and psychosis(58.1%)were the most common manifestations in 31 patients.Fourteen patients suffered with hyponatremia,1 patients with elevated white cells and 17 patients with elevated proteins in CSF.The corresponding positive rates of anti-LGI1 antibody were 90.3%and 93.1%in serum and CSF.Fourteen patients had abnormal cranial MR.All patients received first-line immunotherapy,with 13 patients followed by MMF.Eleven patients were recurred in the follow-up.Univariate COX regression analysis found female(HR=3.85,95%CI:1.12-13.24,P=0.032),MMF adding-on therapy(HR=4.03,95%CI:1.07-15.22,P=0.04)were associated with relapse.However no independent associations were found after multivariate COX regression analysis.Twenty-two patients had sequeleas during follow-up,with memory decline as the most common symptom.All patients achieved good prognosis.Conclusions Anti-LGI1 antibody associated encephalitis may have good prognosis,but relapse is not rare.The majority patients have sequelaes,with memory defect and psychosis being the most common symptoms.MMF might have no prevention effect on anti-LGI1 antibody encephalitis recurrence.

关 键 词：抗富含亮氨酸胶质瘤失活蛋白1 自身免疫性脑炎 复发 预后 

分 类 号：R512.3[医药卫生—内科学]