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。

abstractsObjective: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.