Severe, Refractory Seizures: New-Onset Refractory Status

Med. Clin. North Am. · Jan 2024

Review

Severe, Refractory Seizures: New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome.

NORSE (new-onset refractory status epilepticus) and FIRES (febrile infection-related epilepsy syndrome) represent presentations of new-onset status epilepticus without apparent underlying structural, metabolic, or toxic etiology. The cause of NORSE/FIRES remains cryptogenic in up to half of cases, and an abnormal response of the innate immune system has been implicated. Consensus guidelines recommend broad diagnostic investigation and empiric treatment with immunotherapy. NORSE/FIRES is associated with poor outcomes including cognitive impairment and epilepsy, but early recognition and treatment may be important for improving outcomes.

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- Ross Carson and Coral M Stredny.
- Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
- Med. Clin. North Am. 2024 Jan 1; 108 (1): 201213201-213.
AbstractNORSE (new-onset refractory status epilepticus) and FIRES (febrile infection-related epilepsy syndrome) represent presentations of new-onset status epilepticus without apparent underlying structural, metabolic, or toxic etiology. The cause of NORSE/FIRES remains cryptogenic in up to half of cases, and an abnormal response of the innate immune system has been implicated. Consensus guidelines recommend broad diagnostic investigation and empiric treatment with immunotherapy. NORSE/FIRES is associated with poor outcomes including cognitive impairment and epilepsy, but early recognition and treatment may be important for improving outcomes.Copyright © 2023 Elsevier Inc. All rights reserved.

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Severe, Refractory Seizures: New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome.

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