title: Anti-LGI1 encephalitis is strongly associated with HLA-DR7 and HLA-DRB4.
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.
authors.affiliation: Department of Neurology, Erasmus University Medical Center, Rotterdam.|Department of Neurology, Haga Hospital, the Hague.
authors.forename: Agnes
authors.initials: A
authors.lastname: van Sonderen
authors.affiliation: Department of Immunogenetics and Transplantation Immunology, Leiden University Medical Center, Leiden.
authors.forename: Dave L
authors.initials: DL
authors.lastname: Roelen
authors.affiliation: Department of Pathology, Erasmus University Medical Center, Rotterdam.
authors.forename: Johannes A
authors.initials: JA
authors.lastname: Stoop
authors.affiliation: Department of Pathology, Erasmus University Medical Center, Rotterdam.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Verdijk
authors.affiliation: Department of Immunogenetics and Transplantation Immunology, Leiden University Medical Center, Leiden.
authors.forename: Geert W
authors.initials: GW
authors.lastname: Haasnoot
authors.affiliation: Foundation Epilepsy Institutes Netherlands, Heemstede.|Department of Neurology, Leiden University Medical Center, Leiden.
authors.forename: Roland D
authors.initials: RD
authors.lastname: Thijs
authors.affiliation: Department of Neurology, Haga Hospital, the Hague.
authors.forename: Paul W
authors.initials: PW
authors.lastname: Wirtz
authors.affiliation: Department of Immunology, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: Marco W J
authors.initials: MW
authors.lastname: Schreurs
authors.affiliation: Department of Immunogenetics and Transplantation Immunology, Leiden University Medical Center, Leiden.
authors.forename: Frans H J
authors.initials: FH
authors.lastname: Claas
authors.affiliation: Department of Neurology, Erasmus University Medical Center, Rotterdam.
authors.forename: Peter A E
authors.initials: PA
authors.lastname: Sillevis Smitt
authors.affiliation: Department of Neurology, Erasmus University Medical Center, Rotterdam.
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Titulaer
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies; D004660:Encephalitis / Q000235:genetics* / Q000276:immunology*; D005260:Female; D015809:HLA-DR7 Antigen / Q000235:genetics*; D059845:HLA-DRB4 Chains / Q000235:genetics*; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008875:Middle Aged; D011506:Proteins / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D015809:HLA-DR7 Antigen; D059845:HLA-DRB4 Chains; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins