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Abstract
New-onset refractory status epilepticus (NORSE) is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurologic disorder, with new onset of refractory status epilepticus (RSE) that does not resolve after 2 or more rescue medications, without a clear acute or active structural, toxic, or metabolic cause. Febrile infection-related epilepsy syndrome is a subset of NORSE in which fever began at least 24 hours prior to the RSE. Both terms apply to all age groups. Until recently, NORSE was a poorly recognized entity without a consistent definition or approach to care. We review the current state of knowledge in NORSE and propose a roadmap for future collaborative research. Research investigating NORSE should prioritize the following 4 domains: (1) clinical features, etiology, and pathophysiology; (2) treatment; (3) adult and pediatric evaluation and management approaches; and (4) public advocacy, professional education, and family support. We consider international collaboration and multicenter research crucial in achieving these goals.
Glossary
- FIRES=
- febrile infection-related epilepsy syndrome;
- GCSE=
- generalized convulsive status epilepticus;
- IVIg=
- IV immunoglobulin;
- NCSE=
- nonconvulsive status epilepticus;
- NORSE=
- new-onset refractory status epilepticus;
- RSE=
- refractory status epilepticus;
- SE=
- status epilepticus;
- SRSE=
- superrefractory status epilepticus
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
- Received July 18, 2018.
- Accepted in final form January 17, 2019.
- © 2019 American Academy of Neurology
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