Autoimmune-associated epilepsy (AAE) denotes epilepsy in which adaptive immune mechanisms, often antibodies against neuronal surface proteins such as N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), CASPR2, or Gamma-Aminobutyric Acid-B, play a causative role; seizures in these disorders frequently respond incompletely to ASMs but improve after immunotherapy (e.g., corticosteroids, intravenous immunoglobulin, plasma exchange, and, when needed, rituximab or cyclophosphamide). Here, LGI1 is linked to acquired angioedema.