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

Neuropsychiatric Disease and Treatment. 2019;Volume 15:1897-1903

 

Journal Homepage

Journal Title: Neuropsychiatric Disease and Treatment

ISSN: 1176-6328 (Print); 1178-2021 (Online)

Publisher: Dove Medical Press

LCC Subject Category: Medicine: Internal medicine: Neurosciences. Biological psychiatry. Neuropsychiatry: Neurology. Diseases of the nervous system

Country of publisher: United Kingdom

Language of fulltext: English

Full-text formats available: PDF, HTML

 

AUTHORS


Serino D

Santarone ME

Caputo D

Fusco L

EDITORIAL INFORMATION

Blind peer review

Editorial Board

Instructions for authors

Time From Submission to Publication: 16 weeks

 

Abstract | Full Text

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