Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition which follows a prolonged refractory status epilepticus (SE) triggered by fever of unknown cause. The intractable perisylvian status epilepticus might persist for more than a month and results in severe cognitive impairment.
History
Several authors[1][2][3][4][5] have reported a presumed post encephalitic catastrophic epileptic encephalopathy resulting in refractory repetitive seizures associated with severe cognitive impairment.
Mikaeloff et al[3] in 2006 described this condition in a retrospective study of normally developing 14 children (mean age 7.5yrs) admitted with prolonged status epilepticus following a nonspecific febrile illness and called it Devastating Epileptic encephalopathy in School-age Children (DESC).
The current term febrile infection-related epilepsy syndrome (FIRES) was proposed by Van Baalen et al[6] in 2010 who published a retrospective multicenter case series of 22 previously healthy children aged 3-15yrs (largest of the series) who developed prolonged and recurrent refractory seizures occurring 2-14 days (median 5 days) after fever onset.