A Newly Emerging Disease Entity: Anti-LGI1 Encephalitis

Antibodies to leucine-rich glioma-inactivated 1 (LGI1) were discovered in 2010, predominantly within the hippocampus and temporal cortex. Patients were sometimes classified under voltage-gated potassium channel (VGKC) encephalitis.

The reference laboratory for the Netherlands conducted a review of antibody-positive patients from 2007 through 2015. They identified 39, of whom 66% were men, with median onset of 64 years. Annual incidence was <1 per million.

Patients commonly presented with limbic encephalitis, which included seizures and a cognitive disorder. Frequent manifestations included behavior disturbance, spatial disorientation, insomnia, hyponatremia, and autonomic dysfunction. Faciobrachial dystonic seizures (FBDS) are fairly specific…