Consensus definition and development of new-onset refractory status epilepticus and the related syndromes

Abstract

New-onset refractory status epilepticus(NORSE) is a clinical syndrome, not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue, toxic or metabolic cause.Febrile infection-related epilepsy syndrome(FIRES) is a subcategory of NORSE, applicable for all ages, occurring in a prior febrile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus(RSE) with fever or not.Proposed consensus definitions are also applicable for infantile hemiconvulsion-hemiplegia and epilepsy syndrome, RSE, super-RSE, prolonged RSE and prolonged super-RSE.These consensus definitions will promote much more effective communication and multicenter research, and ultimately improve recognition and treatment of these conditions. Key words: New-onset refractory status epilepticus; Febrile infection-related epilepsy syndrome; Infantile hemiconvulsion-hemiplegia and epilepsy syndrome; Super-refractory status epilepticus; Consensus definition; Clinical characteristics; Autoimmune encephalitis