An international team of experts has published standardized definitions of several seizure-related disorders, including new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related disorders.
- NORSE is described as a clinical presentation rather than a specific diagnosis.
- NORSE occurs in a patient who is not experiencing active epilepsy or any other preexisting neurological disorder.
- In NORSE, the patient does not have a clear acute or active structural, toxic or metabolic cause of their condition.
- The expert group defined FIRES as a subtype of NORSE that involves a prior febrile infection that started between 2 weeks and 24 hours before the refractory status epilepticus began.
- The experts also offered standardized definitions for infantile hemiconvulsion-hemiplegia and epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus.
Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59(4):739-744.