Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies

Domenico Serino,1 Marta Elena Santarone,2 Davide Caputo,3 Lucia Fusco21Department of Child Neurology, Royal Aberdeen Children’s Hospital, Aberdeen, UK; 2Department of Neuroscience, Bambino Gesù Children’s Hospital, Rome, Italy; 3Neurology Unit, Neuroscience Department,...

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Autores principales:	Serino D, Santarone ME, Caputo D, Fusco L
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2019
Materias:	FIRES epilepsy status epilepticus Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/2185ff0d00c84e38af92bfe6e43362c1
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spelling	oai:doaj.org-article:2185ff0d00c84e38af92bfe6e43362c12021-12-02T04:09:21ZFebrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies1178-2021https://doaj.org/article/2185ff0d00c84e38af92bfe6e43362c12019-07-01T00:00:00Zhttps://www.dovepress.com/febrile-infection-related-epilepsy-syndrome-fires-prevalence-impact-an-peer-reviewed-article-NDThttps://doaj.org/toc/1178-2021Domenico Serino,1 Marta Elena Santarone,2 Davide Caputo,3 Lucia Fusco21Department of Child Neurology, Royal Aberdeen Children’s Hospital, Aberdeen, UK; 2Department of Neuroscience, Bambino Gesù Children’s Hospital, Rome, Italy; 3Neurology Unit, Neuroscience Department, San Paolo Hospital, Milan, ItalyAbstract: 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.Keywords: FIRES, NORSE, epilepsy syndromes, inflammatory epilepsies, status epilepticus, drug-resistanceSerino DSantarone MECaputo DFusco LDove Medical PressarticleFIRESepilepsystatus epilepticusNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeuropsychiatric Disease and Treatment, Vol Volume 15, Pp 1897-1903 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	FIRES epilepsy status epilepticus Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
spellingShingle	FIRES epilepsy status epilepticus Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429 Serino D Santarone ME Caputo D Fusco L Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
description	Domenico Serino,1 Marta Elena Santarone,2 Davide Caputo,3 Lucia Fusco21Department of Child Neurology, Royal Aberdeen Children’s Hospital, Aberdeen, UK; 2Department of Neuroscience, Bambino Gesù Children’s Hospital, Rome, Italy; 3Neurology Unit, Neuroscience Department, San Paolo Hospital, Milan, ItalyAbstract: 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.Keywords: FIRES, NORSE, epilepsy syndromes, inflammatory epilepsies, status epilepticus, drug-resistance
format	article
author	Serino D Santarone ME Caputo D Fusco L
author_facet	Serino D Santarone ME Caputo D Fusco L
author_sort	Serino D
title	Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
title_short	Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
title_full	Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
title_fullStr	Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
title_full_unstemmed	Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies
title_sort	febrile infection-related epilepsy syndrome (fires): prevalence, impact and management strategies
publisher	Dove Medical Press
publishDate	2019
url	https://doaj.org/article/2185ff0d00c84e38af92bfe6e43362c1
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Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies

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