LAUSR

Interest in autoimmune encephalitis has been growing since the discovery of various autoimmune antibodies, such as N‐methyl D‐aspartate receptors antibody and leucine‐rich glioma‐inactivated 1 antibody. However, in contrast to autoimmune encephalitis associated with dysregulated adaptive immunity in the brain, the question of whether innate immunity‐mediated autoinflammatory diseases exist in the brain has drawn much attention. Herein, we report a patient with microglia‐dominant acute autoinflammatory encephalitis successfully treated with anakinra, an including interleukin‐1 receptor blocker. In comparison to systemic autoinflammatory disease, we term this encephalitis cerebral autoinflammatory disease. Cerebral autoinflammatory disease could suggest new conceptual approaches to patients previously diagnosed with an unspecified encephalitis.