Learning objectives
Review and illustrate the neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES).
Demonstrate the usefulness of the the magnetic resonance imaging (MRI) in its characterization.
Background
FIRES is a rare severe epileptic syndrome occurring in previously healthy children which is characterized by the onset of fever between 24 hours and 2 weeks before the onset of status epilepticus [1]. The term was first used by van Baalen and colleagues in 2010 [2], although the first reports fulfilling the clinical criteria were from 1965 [3].
It is a biphasic disease. In the acute phase, status epilepticus is particularly refractory to treatment and can last for many weeks. This is followed by a...
Findings and procedure details
Acute findings
Brain imaging findings in patients with FIRES are often normal in the initial phase of the disease, up to 61% of cases [6]. The primary role of radiology is to exclude encephalitis or any other structural cause of seizures such as cortical malformation, stroke, metabolic disorders, or neoplasm [6].
In the acute phase imaging, the abnormal MRI findings include signal abnormalities in the temporal lobes, basal ganglia, thalami, or brainstem and diffuse cerebral edema (Fig. 1&Fig. 2). Less frequently described findings include multifocal...
Conclusion
FIRES is a severe and extremely rare epileptic syndrome with an unknown etiology. A broad spectrum of MRI imaging findings can be seen, including normal to extensive brain changes. Understanding the MRI characteristics can support the clinical diagnosis and help in early initiation of the treatment.
Personal information and conflict of interest
G. D. López; Capital Federal, CA/AR - nothing to disclose M. M. Vaccarezza; Capital Federal/AR - nothing to disclose R. Lopez Grove; Buenos Aires/AR - nothing to disclose M. S. Perez Akly; Buenos Aires/AR - nothing to disclose C. H. Besada; Buenos Aires/AR - nothing to disclose
References
Lam S-K, Lu W-Y, Weng W-C, et al. The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children. Epilepsy Behav. 2019;95:117–23.
van Baalen A, Häusler M, Boor R, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia. 2010;51:1323–8.
Lyon G, Dodge PR, Adams RD. The acute encephalopathies of obscure origin in infants and children. Brain. 1961;84:680–708.
Lee H-F, Chi C-S. Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up. Seizure. 2018;56:53–9.
Caraballo RH, Reyes G, Avaria...