Anti-LGI1 encephalitis is strongly associated with HLA-DR7 and HLA-DRB4

作者：van Sonderen Agnes; Roelen Dave L; Stoop Johannes A; Verdijk Robert M; Haasnoot Geert W; Thijs Roland D; Wirtz Paul W; Schreurs Marco W J; Claas Frans H J; Smitt Peter A E Sillevis; Titulaer Maarten J^*

来源：Annals of Neurology, 2017, 81(2): 193-198.

DOI：10.1002/ana.24858

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=4.1x10(-11)). HLA-DRB4 was present in all patients and in 46.5% controls (p=1.19x10(-7)). 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

• 出版日期2017-2