Qiao Shan, Wu Huai-Kuan, Liu Ling-Ling, Wang Mei-Ling, Zhang Ran-Ran, Han Tao, Liu Xue-Wu
Department of Neurology, The First Affiliated Hospital of Shandong First Medical University, Jinan, Shandong Province 250014, People's Republic of China.
Department of Neurology, Liaocheng People's Hospital, Liaocheng, Shandong Province 252000, People's Republic of China.
Neuropsychiatr Dis Treat. 2021 Jan 25;17:203-212. doi: 10.2147/NDT.S292343. eCollection 2021.
To describe the clinical manifestation, immunotherapy, and long-term outcomes of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis.
This study was a retrospective analysis of 117 patients with a diagnosis of anti-LGI1 encephalitis identified from the databases of multiple clinical centers between September 2014 and December 2019. The clinical features, ancillary test results, and details of long-term outcomes were evaluated.
Among the 117 patients with anti-LGI1 encephalitis, 69.2% (81/117) were male and 30.8% (36/117) were female. The median age of all patients at the onset of the disease was 57 years (interquartile range [IQR], 52-67). The median time from symptom onset to diagnosis was 8.7 weeks (IQR, 4.2-25). The main clinical features identified were seizures, cognitive impairment, and mental and behavioral abnormalities. Of the 117 patients, 109 were treated with immunotherapy. Symptoms including memory, mental ability, and behavior improved in all 109 patients after 3-5 days of treatment. The median time of follow-up for the treated patients was 33 months (IQR, 17-42). Of the treated patients, 16.2% (19/117) experienced a relapse, with a median delay of 5 months (IQR, 2.1-17) between onset and the first relapse. There were no mortalities over the follow-up period.
The long-term outcome of patients with anti-LGI1 encephalitis was mostly favorable, although some patients continued to experience cognitive dysfunction. Early recognition is important for prompt initiation of immunotherapy that can improve clinical symptoms of anti-LGI1 encephalitis.
描述抗富含亮氨酸胶质瘤失活1蛋白（LGI1）脑炎的临床表现、免疫治疗及长期预后。
本研究对2014年9月至2019年12月期间多个临床中心数据库中确诊的117例抗LGI1脑炎患者进行回顾性分析。评估其临床特征、辅助检查结果及长期预后细节。
117例抗LGI1脑炎患者中，男性占69.2%（81/117），女性占30.8%（36/117）。所有患者发病时的中位年龄为57岁（四分位间距[IQR]，52 - 67岁）。从症状出现到确诊的中位时间为8.7周（IQR，4.2 - 25周）。主要临床特征为癫痫发作、认知障碍以及精神和行为异常。117例患者中，109例接受了免疫治疗。治疗3 - 5天后，所有109例患者的记忆、智力和行为等症状均有改善。接受治疗患者的中位随访时间为33个月（IQR，17 - 42个月）。在接受治疗的患者中，16.2%（19/117）出现复发，发病至首次复发的中位延迟时间为5个月（IQR，2.1 - 17个月）。随访期间无死亡病例。
抗LGI1脑炎患者的长期预后大多良好，尽管部分患者仍存在认知功能障碍。早期识别对于及时启动免疫治疗以改善抗LGI1脑炎的临床症状很重要。
