Department of Neurology, Erasmus University Medical Center, Rotterdam.
Department of Neurology, Haga Hospital, the Hague.
Ann Neurol. 2017 Feb;81(2):193-198. doi: 10.1002/ana.24858. Epub 2017 Jan 27.
Leucine-rich glioma-inactivated1 (LGI1) encephalitis is an antibody-associated inflammation of the limbic area. An autoimmune etiology is suspected but not yet proven. We performed human leukocyte antigen (HLA) analysis in 25 nontumor anti-LGI1 patients and discovered a remarkably strong HLA association. HLA-DR7 was present in 88% compared to 19.6% in healthy controls (p = 4.1 × 10 ). HLA-DRB4 was present in all patients and in 46.5% controls (p = 1.19 × 10 ). These findings support the autoimmune hypothesis. An exploratory analysis was performed in a small group of 4 tumor-LGI1 patients. The strong HLA association seems not applicable in these patients. Therefore, the absence of HLA-DR7 or HLA-DRB4 could raise tumor suspicion in anti-LGI1 patients. Ann Neurol 2017;81:193-198.
富含亮氨酸胶质瘤失活 1 型（LGI1）脑炎是一种边缘区域的抗体相关炎症。疑似自身免疫病因，但尚未得到证实。我们对 25 例非肿瘤抗 LGI1 患者进行了人类白细胞抗原（HLA）分析，发现了一种非常强的 HLA 相关性。与健康对照组的 19.6%相比，HLA-DR7 存在于 88%的患者中（p=4.1×10）。HLA-DRB4 存在于所有患者和 46.5%的对照组中（p=1.19×10）。这些发现支持自身免疫假说。对一小组 4 例肿瘤 LGI1 患者进行了探索性分析。在这些患者中，强烈的 HLA 相关性似乎不适用。因此，抗 LGI1 患者中 HLA-DR7 或 HLA-DRB4 的缺失可能会增加肿瘤的怀疑。神经病学年鉴 2017；81:193-198.
