LGI1抗体相关自身免疫性脑炎的临床及影像学特点分析  

Clinical and imaging features of leucine-rich glioma inactivated 1 antibody associated autoimmune encephalitis

作　　者：苏柳迪 张婷婷[2] 王康[3] 梁辉[3] 王锦程 陈涛 楼海燕[2] Su Liudi;Zhang Tingting;Wang Kang;Liang Hui;Wang Jincheng;Chen Tao;Lou Haiyan(Department of Radiology,Eighth People's Hospital of Nanning,Nanning 530003,China;Department of Radiology,First Affiliated Hospital,School of Medicine,Zhejiang University,Hangzhou 310006,China;Department of Neurology,First Affiliated Hospital,School of Medicine,Zhejiang University,Hangzhou 310006,China)

机构地区：[1]南宁市第八人民医院放射科,南宁530003 [2]浙江大学医学院附属第一医院放射科,杭州310006 [3]浙江大学医学院附属第一医院神经内科,杭州310006

出　　处：《中华神经医学杂志》2022年第12期1215-1219,共5页Chinese Journal of Neuromedicine

摘　　要：目的:探讨富亮氨酸胶质瘤失活1蛋白抗体相关自身免疫性脑炎(LGI1-AE)的临床及影像学特征。方法:选择浙江大学医学院附属第一医院神经内科自2016年1月至2021年12月确诊的9例LGI1-AE患者进入研究。回顾性分析所有患者临床表现、MRI、PET-CT特征及临床转归。结果:9例患者中1例因突发意识障碍急性起病;3例患者呈亚急性病程,病程持续10~15 d,表现为头晕、记忆力减退、意识不清伴肢体抽搐;5例患者呈慢性起病,病程半年至2年,起病前期有遗忘、记忆力下降、头晕、头痛症状,其中2例伴情绪紧张、性格改变的精神症状。8例患者平均随访8个月,急性起病患者进展为器质性精神障碍(痴呆状态);3例患者发展为难治性癫痫(1例亚急性及2例慢性起病患者);4例患者症状缓解或消失。9例患者中8例呈典型影像学表现,病变位于边缘系统,形态肿胀伴T2WI信号增高,PET代谢降低或增高,预后较好;急性起病的1例患者影像学表现不典型,基底节呈非对称性高信号,临床症状较位于边缘系统者重且进展迅速,预后差。结论:本研究结果初步显示,影像学表现不典型的LGI1-AE患者预后相对较差。Objective To explore the clinical and imaging features of leucine-rich glioma inactivated 1 antibody associated autoimmune encephalitis(LGI1-AE).Methods Nine LGI1-AE patients had conformed diagnosis in Department of Neurology,First Affiliated Hospital,School of Medicine,Zhejiang University from January 2016 to December 2021 were enrolled.A retrospective analysis was performed on the clinical manifestations,MRI and PET-CT features,and clinical outcomes of these patients.Results One patient had acute onset due to sudden disturbance of consciousness;3 had subacute onset with a duration of 10-15 d,manifested as dizziness,memory loss,unconsciousness and limb convulsions;5 had chronic onset with a duration of 6 months-2 years,manifested as amnesia,memory loss,dizziness,and headache in the early stage of the disease,including 2 accompanied by emotional stress and personality changes.Eight patients were followed up for an average of 8 months:1 with acute onset had organic mental disorder(dementia state),3 had recurrent seizures(1 with subacute onset and 2 with chronic onset),and the remaining 4 had symptom relief or disappearance.In these 9 patients,8 patients,enjoying good prognosis,showed typical imaging findings:lesions were located at the limbic system,morphological swelling was accompanied by increased T2WI signal,and PET showed reduced or increased metabolism;1 patient,enjoying poor prognosis,had atypical imaging finding:asymmetric hyperintensity in the basal ganglia,the clinical symptoms were more severe than the 8 patients having lesions at the limbic system,and rapid disease progress was noted.Conclusion The prognosis of LGI1-AE patients with atypical imaging manifestations might be relatively poor.

关 键 词：富亮氨酸胶质瘤失活1蛋白抗体相关自身免疫性脑炎 基底节 边缘系统 

分 类 号：R742.9[医药卫生—神经病学与精神病学]