抗LGI1抗体相关脑炎患者的临床、脑电图及影像学特点  

Clinical,electroencephalogram and imaging characteristics of patients with anti-LGI 1 antibody encephalitis

作　　者：高盼盼 马灿灿 朱琳 殷梦媚 吕翔 崔营营 段立晖 GAO Panpan;MA Cancan;ZHU Lin(Department of Neurological Function Examination,Northern Jiangsu People's Hospital Affiliated to Yangzhou University,Yangzhou(225001),Jiangsu,China)

机构地区：[1]扬州大学附属苏北人民医院神经功能检查科,江苏扬州225001 [2]扬州大学附属苏北人民医院神经内科 [3]江苏省人民医院神经内科

出　　处：《癫痫与神经电生理学杂志》2022年第6期333-340,共8页Journal of Epileptology and Electroneurophysiology(China)

基　　金：总后勤部卫生部专项课题(15BJZ10)。

摘　　要：目的探讨抗富亮氨酸胶质瘤失活蛋白1(LGI1)抗体相关脑炎患者的临床、脑电图及影像学特点,为临床诊断治疗提供参考。方法选取2020年6月至2022年4月江苏省苏北人民医院神经内科收治的10例抗LGI1抗体相关脑炎患者,总结其临床表现、脑电图、影像学特点、治疗及预后。结果10例抗LGI1抗体相关脑炎患者表现为近记忆障碍4例、精神行为异常2例、面-臂肌张力障碍(FBDS)3例、全面性强直阵挛发作4例,局灶性发作5例。脑电图异常6例,主要为癫痫样放电、弥漫性及阵发性慢波。头颅MRI异常4例,主要累及海马、颞叶或基底节区。头颅动脉自旋标记(ASL)异常6例,主要为海马、颞叶或全脑灌注增高。所有患者均接受免疫治疗,其中9例行抗癫痫发作治疗。除1例患者拒绝后续治疗后死亡,9例预后良好。结论抗LGI1抗体相关脑炎的临床表现为癫痫发作、FBDS、近记忆障碍、精神行为异常及低钠血症等,可伴甲状腺功能抗体升高,很少合并肿瘤,易出现颞叶及海马等局灶性影像学以及脑电图异常,免疫治疗疗效较好。Objective To investigate the clinical,electroencephalogram(EEG)and imaging characteristics of patients with anti-leucine rich glioma inactivated protein 1(LGI1)antibody encephalitis and provide a reference for clinical diagnosis and treatment.Methods Ten patients with anti LGI1-antibody encephalitis admitted to the Department of Neurology of Northern Jiangsu People's Hospital in Jiangsu Province were collected from June 2020 to April 2022.Their clinical manifestations,EEG,imaging characteristics,treatment and prognosis were summarized.Results Among the 10 patients with anti LGI1 encephalitis,there were 4 patients with recent memory impairment,2 patients with abnormal mental behavior,3 patients with faciobrachial dystonic seizures(FBDS),4 patients with generalized tonic-clonic seizures(GTCS)and 5 patients with focal seizures.There were 6 patients with abnormal EEG,mainly exhibiting epileptiform discharges,diffuse and paroxysmal slow waves.Four patients exhibited brain magnetic resonance image(MRI)abnormality,mainly in hippocampus,temporal lobe or basal ganglia.Six patients had abnormality in cranial arterial spin labeling(ASL),showing increased perfusion in hippocampal,temporal or whole brain.All patients received immunotherapy,and 9 of them received anti-epileptic treatment.One patient died after refusing follow-up treatment,and 9 patients had a good prognosis.Conclusion Clinical manifestations of the patients with anti-LGI1 antibody encephalitis include seizures,FBDS,recent memory impairment,psychobehavioral abnormalities and hyponatremia,which may be accompanied by elevated thyroid autoantibodies which were measured using thyroid function tests,but rarely accompanied by tumor.They often have focal imaging abnormalities in temporal lobe and hippocampus,EEG abnormalities and good immunotherapy therapeutic response.

关 键 词：抗富亮氨酸胶质瘤失活蛋白1 自身免疫性脑炎 面-臂肌张力障碍 癫痫发作 

分 类 号：R512.3[医药卫生—内科学] R741.044[医药卫生—临床医学]