摘要：

目的: 总结抗富亮氨酸胶质瘤失活蛋白1（LGI1）抗体相关脑炎临床特点。方法与结果: 江苏省淮安市第三人民医院2018年3月至2022年10月诊断与治疗13例抗LGI1抗体相关脑炎患者，首发症状分别为局灶性癫痫发作6例、精神症状3例、近事记忆减退2例、面-臂肌张力障碍发作2例；10例血清抗LGI1抗体阳性，2例脑脊液抗LGI1抗体阳性；4例伴低钠血症；9例MRI异常，表现为海马FLAIR成像高信号3例、腔隙性梗死4例、皮质及皮质下轻度萎缩1例、额叶皮质下白质缺血性改变1例；10例脑电图异常，表现为局灶性痫样放电5例，慢波增多5例；12例行免疫治疗，6例同时行抗癫痫发作治疗；10例预后良好，3例预后不良。结论: 抗LGI1抗体相关脑炎临床主要表现为癫痫发作、精神障碍、近事记忆减退，面-臂肌张力障碍发作为其特征性表现，可伴低钠血症，应尽早启动免疫治疗。

关键词: 脑炎, 神经系统自身免疫疾病, 张力障碍, 低钠血症, 免疫疗法

Abstract:

Objective: To summarize the clinical features of anti-leucine-rich-glioma-inactivation 1 (LGI1) antibody associated encephalitis. Methods and Results: A total of 13 patients with anti - LGI1 antibody associated encephalitis were admitted to Huai'an No. 3 People's Hospital from March 2018 to October 2022. The first symptoms were focal seizure in 6 cases, psychiatric symptom in 3 cases, recent memory loss in 2 cases and faciobrachial dystonic seizures (FBDS) in 2 cases. Serum anti-LGI1 antibody was positive in 10 cases, cerebrospinal fluid (CSF) anti-LGI1 antibody was positive in 2 cases. And 4 cases had hyponatremia. MRI showed abnormal findings in 9 cases, including 3 cases of hippocampal FLAIR hyperintensity, 4 cases of lacunar infarction, one case of cortical and subcortical mild cerebral atrophy, and one case of subcortical white matter ischemic changes. EEG abnormalities were observed in 10 cases, including focal epileptoid discharge in 5 cases and slow wave increase in 5 cases. Twelve cases received immunotherapy and 6 cases received antiepileptic seizure medicine. The prognosis was good in 10 cases and poor in 3 cases. Conclusions: The main clinical manifestations of anti - LGI1 antibody associated encephalitis include seizures, mental disorders and recent memory loss, FBDS as its characteristic manifestations, may be accompanied by hyponatremia, immunotherapy should be started as soon as possible.

Key words: Encephalitis, Autoimmune diseases of the nervous system, Dystonic disorders, Hyponateramia, Immunotherapy