Abstract
Introduction: New onset refractory status epilepticus (NORSE) is a rare and devastating condition characterised by the sudden onset of refractory status epilepticus (RSE) without an identifiable acute or active structural, toxic, or metabolic cause in an individual without a pre-existing diagnosis of epilepsy. Febrile infection-related epilepsy syndrome (FIRES) is considered a subcategory of NORSE and presents following a febrile illness prior to seizure onset. NORSE/FIRES is associated with high morbidity and mortality in children and adults. Methods and results: In this review we first briefly summarise the reported clinical, paraclinical, treatment and outcome data in the literature. We then report on existing knowledge of the underlying pathophysiology in relation to in vitro and in vivo pre-clinical seizure and epilepsy models of potential relevance to NORSE/FIRES. Discussion: We highlight how pre-clinical models can enhance our understanding of FIRES/NORSE and propose future directions for research.
| Original language | English |
|---|---|
| Article number | 1426051 |
| Number of pages | 9 |
| Journal | Frontiers in Neurology |
| Volume | 15 |
| Early online date | 8 Aug 2024 |
| DOIs | |
| Publication status | Published - 8 Aug 2024 |
Bibliographical note
Copyright © 2024 Champsas, Zhang, Rosch, Ioannidou, Gilmour, Cooray, Woodhall, Pujar, Kaliakatsos and Wright. This is an open-access articledistributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Keywords
- status epilepticus
- immunomodulation
- NORSE
- autoantibodies
- FIRES
- animal models