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

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 et al. · Ann Neurol · 2017

case_control · Level III

Where this comes from

Abstract

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<sup>-11</sup> ). HLA-DRB4 was present in all patients and in 46.5% controls (p = 1.19 × 10<sup>-7</sup> ). 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.

Medical subject headings