How autoimmunity changed our diagnostic practice in epileptology?

Since few years, a new etiology of epilepsy emerges with the labelling of new autoantibodies against the central nervous system. In 2017, the International League Against Epilepsy (ILAE) concluded that autoimmunity is one of six etiologies contributing to epilepsy and that autoimmune epilepsy is directly caused by immune disorders in which seizures constitute a core symptom. Epileptic disorders of immune origin are now distinguished in two different entities: acute symptomatic seizures secondary to autoimmune (ASS) and autoimmune-associated epilepsy (AAE) with different expected clinical outcome under immunotherapy. If acute encephalitis is usually related to ASS with a classic good control of the disease under immunotherapy, clinical phenotype characterized by isolated seizures (new onset seizures and chronic focal epilepsy patients) may be due to either ASS or to AAE. Decision of Abs testing and early immunotherapy initiation needs the development of clinical scores able to select patients with high risk of positive Abs testings. If this selection is now included in the usual medical care of encephalitic patients, specifically with NORSE, the actual bigger challenge is in patients with non or only mild encephalitic symptoms followed for new onset seizures or chronic focal epilepsy patients of unknown origin. The emergence of this new entity provides new therapeutic strategies with specific etiologic and probably anti epileptogenic medication rather than the usual and nonspecific ASM. In the world of the epileptology, this new autoimmune entity appears as a big challenge with an exciting chance to improve or even definitely cure patients of their epilepsy. However, the detection of these patients has to be done in the early phase of the disease to offer the best outcome.