New onset refractory status epilepticus: State of the art

Abstract

NORSE (new onset refractory status epilepticus) has recently been defined as a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. It includes the concept of FIRES described in children with a similar condition but preceded by a 2-14-day febrile illness. NORSE constitutes the acute phase of an entity preceded by a prodromal phase which may be accompanied by numerous manifestations (febrile episode, behavioural changes, headache, …), and followed by a chronic phase marked by long-term neurological sequelae, cognitive impairment, epilepsy and functional disability. There are many causes of NORSE: autoimmune, infectious, genetic, toxic, … but in half of the cases, despite an exhaustive assessment, the cause remains undetermined. Paraneoplastic and non-paraneoplastic autoimmune encephalitis remains by far the leading cause of NORSE. For these reasons, immunotherapy should be considered rapidly in parallel with the treatment of the status epilepticus, including in cryptogenic NORSE. Good communication with the family is important because the management of the acute phase is long and difficult. Although mortality remains high (11-22%), and sequelae can be severe, the majority of survivors can have a good or fair outcome.

Keywords: Autoimmune encephalitis; FIRES; NORSE; Status epilepticus.