Objective: This study was to describe the clinical characteristics of Anti-leucine-rich glioma inactivated 1 protein(LGI1) antibody associated limbic encephalitis. Methods: Clinical data including clinical features, laboratory and radiological findings, treatment and prognosis of the 9 patients were analyzed. Results: In all 9 cases, 6 cases experienced epileptic seizure, 5 cases had psychosis, 7 cases presented with memory impairment, 4 cases showed faciobrachial dystonic seizure, 2 had refractory hyponatremia. One case presented with typically acute Guillain-Barre syndrome (GBS). Anti-LGI1 antibody was detected in 6 cases in cerebrospinal fluid (CSF) samples and 9 in serum samples. Seven cases out of 9 had brain imaging abnormalities. All 9 cases found no evidence of tumors. Eight cases were given immune therapy. During a 1-16 months follow-up, 1 case had complete recovery, 5cases had various degree of sequelae , among whom 4 had memory disturbance and 1 case had changed personality. 2cases were lost to follow-up. Conclusions: Limbic encephalitis is the most common manifestation of anti-LGI1 antibody associated encephalitis. Faciobrachial dystonic seizure (FBDS) is a specific symptom which favors an early diagnosis. Tumor is uncommon to see. The disease has a relatively favorable prognosis while impaired memory can be seen as a common sequelae.
目的： 探讨抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关边缘性脑炎的临床特点，以利于早期诊治及预后评估。 方法： 对2015年3月至2016年9月郑州大学人民医院神经内科确诊的9例LGI1抗体相关边缘性脑炎患者资料进行分析并随访。 结果： 临床表现为癫痫发作6例，精神行为异常5例，记忆损害7例，面臂肌张力障碍发作4例，顽固性低钠血症2例，急性吉兰－巴林综合征1例；LGI1抗体：脑脊液阳性6例，血清阳性9例；7例有影像学改变；9例均未发现肿瘤。8例在本院接受免疫治疗，好转出院。随访1～16个月，1例完全恢复，4例有记忆力障碍，1例有性格改变，1例无边缘性脑炎表现，2例失访。 结论： 抗LGI1抗体相关边缘性脑炎主要表现为边缘性脑炎，面臂肌张力障碍发作为特异性表现，可协助早期诊断，很少伴有肿瘤，预后较好，但可遗留记忆力障碍。.
Keywords: Autoimmune diseases of the nervous system; Cognition disorders; Limbic encephalitis; Proteins.