Human Leukocyte Antigen Association Study...
In addition to the established primary DRB1*07:01 association in anti-LGI1 encephalitis, we observe a secondary effect of DRB1*04:02 with lower age at onset. Our study provides evidence for secondary effects within HLA locus that correlate with clinical phenotypes in anti-LGI1 encephalitis. Anti-leucine–rich glioma-inactivated 1 (LGI1) encephalitis is one of the most common autoimmune encephalitis with approximate incidence of 0.83/million/year in Europe. The classic presentation is in men (66%) of around 65 years. Faciobrachial dystonic seizures (FBDSs) are a characteristic and often presenting clinical feature of the disease, with other focal seizures in addition to amnesia, disorientation, and psychiatry features following. Despite an initial good response to most immunotherapies cognitive sequelae after the disease are common and disabling.
