Refractory focal epilepsy following acute encephalopathy with inflammation-mediated status epilepticus

Refractory focal epilepsy following acute febrile encephalopathy, also called fever-induced refractory epileptic encephalopathy in school-aged children or febrile infection-related epilepsy syndrome (FIRES), is a topic of great interest and is a newly described condition in Europe and Asia. In a recent review, Nabbout et al. termed this entity ‘‘acute encephalopathy with inflammation-mediated status epilepticus (AEIMSE)’’. In the last few years, we have diagnosed and treated some patients affected by this condition and showing a similar clinical course. In the present report, we review various literature data and add a few concepts regarding the differential diagnosis and the possible role of inflammation and loss of function of the bloodbrain barrier (BBB) in the pathogenesis of the disease.