Purpose:

Febrile infection-related epilepsy syndrome (FIRES) is a rare severe epileptic syndrome occurring in previously healthy children. It is characterised by refractory status epilepticus following febrile illness. The purpose of this educational presentation is to provide the delegate with an overview and understanding of the spectrum of imaging findings in this condition and to aid the neuroradiologist in excluding mimics and to support the diagnosis of FIRES.

Materials and Methods:

We reviewed all the patients in our specialist epilepsy service over the last 5 years. We describe the specific MRI imaging features of 10 patients with a clinical diagnosis of FIRES.

Results:

4 patients had normal initial scan. 6 patients had abnormalities in the temporal lobes (2), basal ganglia (3), thalami (2) and brain stem (1) and diffuse cerebral oedema (1). In the chronic phase of the disease, when the child had recovered from status epilepticus the imaging features consisted of brain atrophy and mesial temporal lobe sclerosis in 6/10 subjects in the areas showing previous acute abnormalities.

Conclusion:

There is a wide spectrum of imaging findings in children with FIRES. These can be separated into acute and chronic phases of the disease. Understanding the MRI features can aid the neuroradiologist in supporting the clinical diagnosis and thereby facilitating the early initiation of appropriate therapy.

Spectrum of Neuroimaging Findings in Febrile Infection-related Epilepsy Syndrome (FIRES) - a National Specialist Center Study

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American Society of Neuroradiology (ASNR) 58th Annual Meeting 2020: Educational Exhibits