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MINI REVIEW article
Front. Neurol.
Sec. Epilepsy
Volume 15 - 2024 |
doi: 10.3389/fneur.2024.1426051
This article is part of the Research Topic Immune System Mechanisms Impacting the Onset of Epilepsy and Spontaneous Seizures View all 7 articles
NORSE/FIRES: how can we advance our understanding of this devastating condition?
Provisionally accepted
Dimitrios Champsas
1,2
Xushuo Zhang
1*
Richard Rosch
3,4*
Evangelia Ioannidou
2
Kimberly Gilmour
5,6,7,8
Gerald Cooray
2,9*
Gavin L. Woodhall
1
Suresh PUJAR
2
Marios Kaliakatsos
2*
Sukhvir K. Wright
1,10*- 1 Aston University, Birmingham, United Kingdom
- 2 Department of Neurology, Great Ormond Street Hospital, London, United Kingdom
- 3 Department of Clinical Neurophysiology, King's College Hospital NHS Foundation Trust, London, United Kingdom
- 4 Department of Neurology, Columbia University, New York, New York, United States
- 5 Department of Immunology, Great Ormond Street Hospital, London, United Kingdom
- 6 NIHR Biomedical Research Centre, University College London Hospitals NHS Foundation Trust, London, England, United Kingdom
- 7 University College London, London, England, United Kingdom
- 8 Department of Neuroscience, Karolinska Institutet (KI), Stockholm, Stockholm, Sweden
- 9 Department of Clinical Neuroscience, Karolinska Institutet (KI), Stockholm, Stockholm, Sweden
- 10 Birmingham Women's and Children's Hospital, Birmingham, United Kingdom
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.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 and propose future directions for research.
Keywords: Norse, Fires, Status Epilepticus, Immunomodulation, Autoantibodies, Animal Models
Received: 14 May 2024; Accepted: 29 Jul 2024.
Copyright: © 2024 Champsas, Zhang, Rosch, Ioannidou, Gilmour, Cooray, Woodhall, PUJAR, Kaliakatsos and Wright. This is an open-access article distributed 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) or licensor 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.
* Correspondence:
Xushuo Zhang, Aston University, Birmingham, United Kingdom
Richard Rosch, Department of Clinical Neurophysiology, King's College Hospital NHS Foundation Trust, London, United Kingdom
Gerald Cooray, Department of Neurology, Great Ormond Street Hospital, London, WC1N 3JH, United Kingdom
Marios Kaliakatsos, Department of Neurology, Great Ormond Street Hospital, London, WC1N 3JH, United Kingdom
Sukhvir K. Wright, Aston University, Birmingham, United Kingdom
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