Febrile infection-related epilepsy syndrome (FIRES): what the radiologist should know and recognize

Febrile infection-related epilepsy syndrome (FIRES): what the radiologist should know and recognize

Adriana García 1, Germán D. López 2, Roy López-Grove 2, Ma. Magdalena Vaccarezza 3, Manuel Pérez-Akly 4, Cristina H. Besada 2

1 Servicio de Diagnóstico por Imágenes, Hospital Británico de Buenos Aires, Buenos Aires, Argentina; 2 Servicio de Diagnóstico por Imágenes, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina; 3 Servicio de Neurología Pediátrica, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina; 4 Diagnóstico por Imágenes, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

*Correspondence: Germán D. López. Email: german.lopez@hospitalitaliano.org.ar

Abstract

FIRES (febrile infection-related epilepsy syndrome) is a rare epileptic syndrome occurring at any age in previously healthy
patients. Clinically it presents with febrile episodes within 24 hours to two weeks prior to the onset of status epilepticus. Two phases or stages of the disease are considered: in the acute phase there is refractoriness in the treatment of seizures for several weeks, followed by a chronic phase in which epilepsy is intractable, accompanied by varying degrees of cognitive impairment. The acute phase is characterized by a normal brain magnetic resonance imaging (MRI) study in most cases. The temporal lobe including the temporo-mesial structures and hippocampus may be affected. In the chronic phase of the disease diffuse atrophy and/or uni- or bilateral temporo-mesial sclerosis may be found, a course without sequelae lesions is also possible. It is necessary to understand these abnormalities on MRI to support the diagnosis of FIRES as it is a diagnosis of exclusion.

Keywords: Status epilepticus. Fever. Pediatrics.

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