抗富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎九例临床分析

Clinical analysis of 9 cases with Anti-leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis

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。

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. Faeiobrachial 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.