Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies.
FIRES
NORSE
drug-resistance
epilepsy syndromes
inflammatory epilepsies
status epilepticus
Journal
Neuropsychiatric disease and treatment
ISSN: 1176-6328
Titre abrégé: Neuropsychiatr Dis Treat
Pays: New Zealand
ID NLM: 101240304
Informations de publication
Date de publication:
2019
2019
Historique:
received:
25
02
2019
accepted:
13
06
2019
entrez:
3
8
2019
pubmed:
3
8
2019
medline:
3
8
2019
Statut:
epublish
Résumé
Febrile infection-related epilepsy syndrome (FIRES) is a rare catastrophic epileptic encephalopathy with a yet undefined etiology, affecting healthy children. It is characterized by acute manifestation of recurrent seizures or refractory status epilepticus preceded by febrile illness, but without evidence of infectious encephalitis. To date, the absence of specific biomarkers poses a significant diagnostic challenge; nonetheless, early diagnosis is very important for optimal management. FIRES is mostly irreversible and its sequelae include drug-resistant epilepsy and neuropsychological impairments. The treatment of FIRES represents a significant challenge for clinicians and is associated with low success rates. Early introduction of ketogenic diet seems to represent the most effective and promising treatment. This review aims to highlight the most recent insights on clinical features, terminology, epidemiology, pathogenesis, diagnostic challenges and therapeutic options.
Identifiants
pubmed: 31371963
doi: 10.2147/NDT.S177803
pii: 177803
pmc: PMC6635824
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1897-1903Déclaration de conflit d'intérêts
All of the authors declare that they have no conflicts of interest in this work.
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