抗富亮氨酸胶质瘤失活1蛋白抗体脑炎7例临床特点分析  

Analysis of 7 Patients of Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis

作　　者：赵静[1] 韩柏林 杨静[1] ZHAO Jing;HAN Bai-lin;YANG Jing(Department of Neurology,Aero Space Center Hospital,Beijing 100049,China)

机构地区：[1]航天中心医院神经内科,北京100049

出　　处：《神经损伤与功能重建》2020年第10期571-574,609,共5页Neural Injury and Functional Reconstruction

摘　　要：目的:分析抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体脑炎患者的临床表现、辅助检查特点、治疗和预后。方法:回顾性分析我院2016年8月至2018年12月确诊的7例LGI1抗体脑炎患者的临床资料。结果:7例患者中男5例,女2例,平均68岁;6例有近记忆力及认知障碍,6例出现精神行为异常,其中2例有幻觉,1例饮食过度,4例有面臂肌张力障碍(FBDS),5例出现睡眠障碍,3例癫痫发作。7例均行脑脊液检查,LGI1抗体均为阳性。5例血清抗体阳性。4例出现低钠血症。3例头颅MRI异常,主要累及颞叶、海马,底节区。5例脑电图异常。所有患者行肿瘤筛查未发现异常。所有患者均接受一线免疫治疗,延误诊治的1例患者对治疗反应差并出现复发。结论:LGI1抗体脑炎是可治性疾病,出现快速进展的认知障碍、精神异常、FBDS伴有低钠血症,头颅MRI以边缘系统受累为主时需考虑LGI1抗体脑炎可能,及早识别和诊治十分关键,预后良好。Objective:To analyze the clinical presentation,therapeutic intervention,and prognosis of anti-leucine-rich glioma-inactivated 1(LGI1)encephalitis.Methods:We retrospectively analyzed the clinical data of 7 patients diagnosed with LGI1 encephalitis in our department between August 2016 and December 2018.Re⁃sults:Of the 7 patients,5 were male and 2 female.The average age was 68 years old.Six patients experienced memory loss and cognitive deficits;6 had mental and behavioral abnormalites,2 of which experienced hallucination and 1 overeating;4 presented faciobrachial dystonic seizures(FBDS);5 showed sleep dysfunction;and 3 experienced seizures.All patients underwent cerebrospinal fluid examination and tested positive for LGI1 antibodies;among these,5 patients were also positive for serum LGI1 antibodies.Four patients showed hyponatremia.Three patients presented with abnormal lesions in brain magnetic resonance imaging(MRI)located in the temporal lobe,hippocampus,and basal ganglia.Five patients exhibited abnormalities in their electroencephalograms(EEGs).No patients showed malignant tumors.All patients received first-line immunotherapy;1 patient delayed diagnosis which resulted in poor response to treatment and recurrence.Conclusion:Anti-LGI1 encephalitis is a treatable disease and should be suspected in patients who develop rapidly progressive cognitive impairment,mental and behavioral abnormalites,FBDS,hyponatremia,and brain MRI showing lesions located mostly in the limbic system.Timely diagnosis and treatment are critical to yielding a favorable prognosis.

关 键 词：富亮氨酸胶质瘤失活1蛋白 自身免疫性脑炎 面臂肌张力障碍 记忆障碍 低钠血症 

分 类 号：R741[医药卫生—神经病学与精神病学] R742[医药卫生—临床医学]