富亮氨酸胶质瘤失活1蛋白抗体相关自身免疫性脑炎10例临床分析  

Clinical analysis of 10 patients with LGI-1 antibody associated autoimmune encephalitis

作　　者：赵则燕 韩玮欣 李青[1] 高瑞平 李晓庆 崔君昭 赵辉[1] 何俊瑛[1] 卜晖[1] ZHAO Ze-yan;HAN Wei-xin;LI Qing(the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China)

机构地区：[1]河北医科大学第二医院神经内科,石家庄050000

出　　处：《临床神经病学杂志》2018年第6期441-444,共4页Journal of Clinical Neurology

摘　　要：目的总结富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关自身免疫性脑炎临床特点及治疗转归,以加强对本病的认识。方法分析2012年11月至2017年6月在河北医科大学第二医院神经内科就诊的10例LGI1抗体阳性相关自身免疫性脑炎患者的临床、实验室检查、影像学检查等资料,并跟踪随访。结果10例患者中男性6例,女性4例,平均年龄55岁,临床表现有记忆力下降、精神行为异常、癫痫、面臂肌张力障碍发作等。9例患者行CSF检查,其中7例患者常规、生化均正常,2例蛋白升高。6例患者出现低钠血症(<135 mmol/L)。1例患者肿瘤标志物升高,胸部CT发现左侧肺部占位; 9例行EEG检查均异常,表现为癫痫样放电或慢波; 10例患者行MRI检查,5例示海马、颞叶出现异常信号。10例患者中9例经免疫治疗预后良好,1例死亡。结论 LGI1抗体相关自身免疫性脑炎患者临床多表现为记忆力下降、精神行为异常、癫痫发作,面臂肌张力障碍发作为其特征性表现,可伴有肿瘤,免疫治疗疗效较好。Objective To summarize the clinical features,diagnosis and prognosis of Leucine-rich glioma inactivated-1 (LGI1 )antibody associated autoimmune encephalitis. Methods The clinical features,results of laboratory and imagine examination results of patients with LGI1 antibodies who were admited to the second hospital of Hebei medical university from November 2012 to June 2017 were analyzed. The prognosis of the patients was followed up.Results Ten patients were enrolled,including 6 male and 4 femal. The average age of ten patients was 55 years old. Patients mainly presented with memory loss,psychiatric symptoms,epilepsy and faciobrachial dystonic seizures. Nine patients underwent CSF examination,7 were found to have normal tests while 2 had increased protein concentrations. The serum sodium concentration was <135 mmol/L in 6 patients. One patient had elevated serum tumor markers and lung mass was found by chest CT. Nine patients exhibited abnormalities in their EEGs,including slowed wave or epileptiform discharges. MRI scans were performed in all the patients,with 5 patients showing increased signals on MRI fluid-attenuated inversion recovery or T2sequences. Nine patients treated with immunotherapy had favorable outcomes,1 was dead. Conclusions LGI1-ab associated autoimmune encephalitis should be considered among patient with memory loss,hyponatremia,epilepsy. Faciobrachial dystonic seizures must be a special symptom. Patients with positive antibody of LGI1 receptor can be associated with tumor. The respond to immunotherapy is good.

关 键 词：富亮氨酸胶质瘤失活1蛋白 记忆力下降 面臂肌张力障碍 自身免疫性脑炎 

分 类 号：R512.3[医药卫生—内科学]