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

Authors Serino D, Santarone ME, Caputo D, Fusco L

Received 25 February 2019

Accepted for publication 13 June 2019

Published 9 July 2019 Volume 2019:15 Pages 1897—1903

DOI https://doi.org/10.2147/NDT.S177803

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Ms Justinn Cochran

Peer reviewer comments 2

Editor who approved publication: Dr Roger Pinder


Video abstract presented by Domenico Serino.

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Domenico Serino,1 Marta Elena Santarone,2 Davide Caputo,3 Lucia Fusco2

1Department 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, Italy

Abstract: 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

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