Proposal to optimize evaluation and treatment of Febrile infection‐related epilepsy syndrome (FIRES): A Report from FIRES workshop Article Swipe
Sookyong Koh
,
Elaine Wirrell
,
Annamaria Vezzani
,
Rima Nabbout
,
Eyal Muscal
,
Marios Kaliakatsos
,
Ronny Wickström
,
James J. Riviello
,
Andreas Brunklaus
,
Eric T. Payne
,
Antonio Valentı́n
,
Elizabeth Wells
,
Jessica L. Carpenter
,
Kihyeong Lee
,
Yi‐Chen Lai
,
Krista Eschbach
,
Craig A. Press
,
Mark Gorman
,
Coral M. Stredny
,
William Roche
,
Tara Mangum
·
YOU?
·
· 2020
· Open Access
·
· DOI: https://doi.org/10.1002/epi4.12447
YOU?
·
· 2020
· Open Access
·
· DOI: https://doi.org/10.1002/epi4.12447
Febrile infection‐related epilepsy syndrome (FIRES) is a rare catastrophic epileptic encephalopathy that presents suddenly in otherwise normal children and young adults causing significant neurological disability, chronic epilepsy, and high rates of mortality. To suggest a therapy protocol to improve outcome of FIRES, workshops were held in conjunction with American Epilepsy Society annual meeting between 2017 and 2019. An international group of pediatric epileptologists, pediatric neurointensivists, rheumatologists and basic scientists with interest and expertise in FIRES convened to propose an algorithm for a standardized approach to the diagnosis and treatment of FIRES. The broad differential for refractory status epilepticus (RSE) should include FIRES, to allow empiric therapies to be started early in the clinical course. FIRES should be considered in all previously healthy patients older than two years of age who present with explosive onset of seizures rapidly progressing to RSE, following a febrile illness in the preceding two weeks. Once FIRES is suspected, early administrations of ketogenic diet and anakinra (the IL‐1 receptor antagonist that blocks biologic activity of IL‐1β) are recommended.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1002/epi4.12447
- OA Status
- gold
- Cited By
- 56
- References
- 69
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W3105209831
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W3105209831Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.1002/epi4.12447Digital Object Identifier
- Title
-
Proposal to optimize evaluation and treatment of Febrile infection‐related epilepsy syndrome (FIRES): A Report from FIRES workshopWork title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2020Year of publication
- Publication date
-
2020-11-20Full publication date if available
- Authors
-
Sookyong Koh, Elaine Wirrell, Annamaria Vezzani, Rima Nabbout, Eyal Muscal, Marios Kaliakatsos, Ronny Wickström, James J. Riviello, Andreas Brunklaus, Eric T. Payne, Antonio Valentı́n, Elizabeth Wells, Jessica L. Carpenter, Kihyeong Lee, Yi‐Chen Lai, Krista Eschbach, Craig A. Press, Mark Gorman, Coral M. Stredny, William Roche, Tara MangumList of authors in order
- Landing page
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https://doi.org/10.1002/epi4.12447Publisher landing page
- Open access
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
-
https://doi.org/10.1002/epi4.12447Direct OA link when available
- Concepts
-
Epilepsy, Medicine, Status epilepticus, Pediatrics, Perampanel, Ketogenic diet, Epilepsy syndromes, Dravet syndrome, Intensive care medicine, Anakinra, Clobazam, Empiric treatment, Psychiatry, Internal medicine, Disease, Biology, Microbiology, AntibioticsTop concepts (fields/topics) attached by OpenAlex
- Cited by
-
56Total citation count in OpenAlex
- Citations by year (recent)
-
2025: 8, 2024: 14, 2023: 14, 2022: 15, 2021: 4Per-year citation counts (last 5 years)
- References (count)
-
69Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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| primary_location.source.host_organization_name | Wiley |
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| primary_location.is_published | True |
| primary_location.raw_source_name | Epilepsia Open |
| primary_location.landing_page_url | https://doi.org/10.1002/epi4.12447 |
| publication_date | 2020-11-20 |
| publication_year | 2020 |
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