title: FIRES and IHHE: Delineation of the syndromes.
abstract: Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients. In IHHS, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with FIRES develop refractory epilepsy with severe cognitive deficit affecting the temporal and frontal lobe functions. The role of inflammation is hypothesized with a vicious circle involving inflammation and seizure activity facilitated by brain maturation putting them under the concept of "acute encephalopathy with inflammation-mediated status epilepticus."
authors.affiliation: Department of Pediatric Neurology, Referral Center for Rare Epilepsies, Necker Infants Maladies Hospital, APHP, 149 rue de Sévres, Paris, France. rimanabbout@yahoo.com
authors.forename: Rima
authors.initials: R
authors.lastname: Nabbout
mesh_terms: D055423:Diet, Ketogenic; D004660:Encephalitis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D006429:Hemiplegia / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007249:Inflammation / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D003294:Seizures, Febrile / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D016896:Treatment Outcome
keywords: Epilepsy; Fever; Inflammation; Ketogenic diet; Status epilepticus