Febrile infection–related epilepsy syndrome (FIRES) in four patients

Abstract

FIRES is an epileptic syndrome that presents with multifocal refractory status epilepticus (SE) in previously normal children following a nonspecific febrile illness and evolves into a chronic, refractory, focal epilepsy with associated cognitive and behavioral difficulties. Objective: To describe the course of FIRES in 4 patients. Methods: Two boys and two girls with FIRES were analyzed. The duration of follow-up was 1–5 years. Results: Onset of the disease was 4–14 years (median 8.75 years). All patients were with a normal development. The duration of fever prior SE was 5–10 days (median 6.75 days). In all cases, the fever was higher than 39 degrees without obvious signs of infection. Seizures manifested with head and eyes version in all patients, one patient had facial twitching and hemiclonic jerking. All patients required intensive care treatment using burst-suppression coma and ventilatory support. CSF, MRI were no significant deviations during the acute period in all patients. In one case, HSV 6 type was detected by PCR in the serum and lymphocytes. All patients were treated with AEDs, antiviral and antimicrobial drugs, steroids and IVIG. The duration of SE was 3–27 days (median 12.25 days). After SE drug-resistant focal epilepsy developed in all children. Two patients had mild cognitive impairment, the other two – heavy (especially in case with SE lasting 27 days). Light motor disturbances occurred in two children, one child had severe spastic tetraparesis. Interictal EEG revealed the rhythm slowing and independent bilateral focal IEDs, predominantly over the frontotemporal regions in all patients. Follow-up MRI was performed in 3 cases: two were normal and one had mild diffuse cortical atrophy. For epilepsy treatment have been consistently applied 3–7 AEDs without a significant effect. Conclusion: FIRES resulted in the development of drug-resistant epilepsy and cognitive impairment in all cases.