Febrile acute epileptic encephalopathies in children Volume 22, issue 4, Décembre 2010


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APHP, Service de neurologie et métabolisme, Hôpital Necker, Paris, Inserm, U663, Paris ; Université Paris-Descartes, Faculté de médecine, Paris, Service hospitalier Frédéric-Joliot, I2BM, CEA, Orsay

Fever induced refractory epileptic encephalopathy in school aged children (FIRES) and idiopathic hemiconvulsion-hemiplegia-epilepsy syndrome (IHHES) are two main pediatric conditions triggered by fever with neither any micro-organism nor any evidence of an autoimmune phenomenon. FIRES begins with status epilepticus lasting several weeks and involving perisylvian areas including mesial temporal structures and followed by pharmacoresistant epilepsy with major cognitive deterioration. IHHES begins in infancy with unilateral clonic status epilepticus and leaves hemiplegia with pharmacoresistant homolateral epilepsy. Ketogenic diet seems to be useful for early seizure control. Aetiology of FIRES and IHHES remains unknown although clinical features and experimental models point to a likely vicious circle involving inflammation and seizure activity facilitated by brain maturation.