Abstract
Close to 200 unusual causes of status epilepticus have been reported in the literature. These include inflammatory and autoimmune disorders, uncommon cerebral infections, drug toxicity, and rare genetic disorders. For almost half of these disorders, a specific treatment is required beyond anti-seizure medications. A correct etiologic diagnosis is thus of great importance for both treatment and prognosis. An organized clinical approach is necessary to optimize work-up and ensure an accurate diagnosis. Despite extensive investigations, a substantial proportion of cases of new-onset status epilepticus remain cryptogenic. These entities are known as new-onset refractory status epilepticus (NORSE) and febrile illness-related epilepsy syndrome, febrile illness-related epilepsy syndrome (FIRES) and possibly result from an inflammatory process, but further research is required to determine their etiology and define appropriate therapies.
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Gaspard, N. (2018). Unusual Causes of Status Epilepticus. In: Drislane, F., Kaplan MBBS, P. (eds) Status Epilepticus. Current Clinical Neurology. Springer, Cham. https://doi.org/10.1007/978-3-319-58200-9_8
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