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Over the past few years, some epilepsies have been found to be associated with immunomediated disorders. The presence of antibodies against intracellular and extracellular proteins especially in refractory epilepsy provides additional evidence of immunomediated diseases in the brain. In refractory epilepsy, autoantibodies against NMDAR, GAD and the VGKC-complex can be found in 2 to 16% of patients. The definition of autoinflammatory diseases was first introduced by Kastner et al in 2009 after the discovery of a homozygous mutation in the gene MEV1, responsible for Mediterranean Fever. After that, the recognition of inflammassome and interleukin 1 as the main actors in the innate immune system responsible for inflammation in these diseases has changed the treatment for some rare disorders.

Refractory epilepsy responsive to nonspecific immunossupression: autoimmune or auto inflammatory disease? A case report and review of literature