Immunotherapies for NORSE and FIRES.

New onset refractory status epilepticus (NORSE) and its subtype febrile infection-related epilepsy syndrome (FIRES) are severe presentations of epilepsy syndromes that are characterized by treatment refractory seizures in an individual without prior epilepsy. Some cases have identified etiologies, such as specific autoantibodies or viral infections, but many cases are classified as cryptogenic. There is increasing evidence suggesting that both adaptive and innate immune dysregulatory mechanisms play a central role in the pathogenesis of these disorders, particularly in cryptogenic cases, and with this, increased use of immunotherapy as a key component of treatment of NORSE and FIRES.