Febrile infection-related epilepsy syndrome: long-term consequences (own observations)

Febrile infection-related epilepsy syndrome (FIRES) is an exceedingly rare disorder that affects 1 in 1.000.000 children. However, we believe that FIRES is more common, since many cases remain undiagnosed. The exact pathogenesis of this clinical syndrome is still poorly understood. There are several theories of its development, including immune, genetic, and inflammatory-mediated ones. FIRES is known to have dismal outcomes with a death rate of up to 30 % in the acute phase and subsequent development (often immediately after the acute phase) of refractory epilepsy and mental retardation in 66–100 % of survivors. Despite the increasing number of publications, the problems of timely diagnosis and treatment of such patients have not yet been addressed. We describe 6 patients presumed to have had FIRES. The most common outcomes included drug-resistant epilepsy, as well as cognitive and behavioral disorders. Continuing seizures and epileptiform activity on the electroencephalogram trigger cognitive and behavioral disorders. The analysis of treatment outcomes indicates that combinations of carbamazepine / oxcarbazepine with other antiepileptic drugs are most effective.