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

Ross Carson; Coral M Stredny

doi:10.1016/j.mcna.2023.05.020

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

Med Clin North Am. 2024 Jan;108(1):201-213. doi: 10.1016/j.mcna.2023.05.020. Epub 2023 Jul 26.

Authors

Ross Carson¹, Coral M Stredny²

Affiliations

¹ Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
² Division of Epilepsy and Clinical Neurophysiology, Program in Neuroimmunology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address: coral.stredny@childrens.harvard.edu.

PMID: 37951651
DOI: 10.1016/j.mcna.2023.05.020

Abstract

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.

Keywords: Epilepsy; FIRES; NORSE; Refractory status epilepticus; Seizure.

Publication types

Review

MeSH terms

Encephalitis*
Epilepsy* / diagnosis
Epilepsy* / etiology
Epileptic Syndromes* / diagnosis
Epileptic Syndromes* / etiology
Epileptic Syndromes* / therapy
Humans
Seizures
Status Epilepticus* / diagnosis
Status Epilepticus* / drug therapy
Status Epilepticus* / etiology