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VGKC 复合物/LGI1 抗体脑炎:临床表现和免疫治疗反应。

VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy.

机构信息

Department of Neurology, Laboratory for Neurotherapeutics, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul, South Korea; Program in Neuroscience, Seoul National University College of Medicine, Seoul, South Korea.

出版信息

J Neuroimmunol. 2013 Dec 15;265(1-2):75-81. doi: 10.1016/j.jneuroim.2013.10.005. Epub 2013 Oct 17.

Abstract

Leucine-rich glioma inactivated 1 (LGI1) was recently identified as a target protein in autoimmune synaptic encephalitis, a rare condition associated with autoantibodies against structures in the neuronal synapse. Studies dealing with LGI1 are small in number and the various outcomes of different therapeutic regimens are not well studied. Here, we analyzed clinical characteristics of 14 patients with LGI1 antibodies, and outcomes according to therapeutic strategies. Most patients exhibited abnormal brain positron emission tomography and that patients treated with steroids alone were more likely to relapse and had less favorable outcomes than those treated with steroids and intravenous immunoglobulins.

摘要

富含亮氨酸胶质瘤失活 1 蛋白(LGI1)最近被确定为自身免疫性突触脑炎的一种靶蛋白,这种罕见的疾病与神经元突触结构的自身抗体有关。涉及 LGI1 的研究数量较少,不同治疗方案的各种结果也没有得到很好的研究。在这里,我们分析了 14 例 LGI1 抗体患者的临床特征,以及根据治疗策略的结果。大多数患者表现出异常的脑正电子发射断层扫描,单独使用类固醇治疗的患者比使用类固醇和静脉注射免疫球蛋白治疗的患者更有可能复发,并且结果更差。

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