Zhang Y X, Yang H L, Wu Y Y, Wang C C, Gao X Y, Shi Y Y, Liu H Q, Huang Y, Zhang J W
Department of Neurology, People's Hospital of Zhengzhou University, Zhengzhou 450003, China.
Zhonghua Yi Xue Za Zhi. 2017 May 9;97(17):1295-1298. doi: 10.3760/cma.j.issn.0376-2491.2017.17.004.
This study was to describe the clinical characteristics of Anti-leucine-rich glioma inactivated 1 protein(LGI1) antibody associated limbic encephalitis. Clinical data including clinical features, laboratory and radiological findings, treatment and prognosis of the 9 patients were analyzed. 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. 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.
本研究旨在描述抗富含亮氨酸胶质瘤失活1蛋白（LGI1）抗体相关边缘性脑炎的临床特征。分析了9例患者的临床资料，包括临床特征、实验室及影像学检查结果、治疗及预后情况。9例患者中，6例出现癫痫发作，5例有精神病症状，7例有记忆障碍，4例出现面臂肌张力障碍性发作，2例有难治性低钠血症。1例表现为典型的急性吉兰-巴雷综合征（GBS）。脑脊液（CSF）样本中6例检测到抗LGI1抗体，血清样本中9例检测到。9例中有7例存在脑影像学异常。9例均未发现肿瘤证据。8例接受了免疫治疗。在1至16个月的随访期间，1例完全康复，5例有不同程度的后遗症，其中4例有记忆障碍，1例有性格改变。2例失访。边缘性脑炎是抗LGI1抗体相关脑炎最常见的表现形式。面臂肌张力障碍性发作（FBDS）是一种有助于早期诊断的特异性症状。肿瘤少见。该病预后相对良好，记忆障碍是常见的后遗症。
