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

Abstract

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

Keywords

• FIRES
• epilepsy
• status epilepticus