Anti‐LGI1 encephalitis is strongly associated with HLA‐DR7 and HLA‐DRB4. Issue 2 (27th January 2017) - van Sonderen, Agnes
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 −11 ). HLA‐DRB4 was present in all patients and in 46.5% controls ( p = 1.19 × 10 −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