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FIRES and IHHE : D elineation of the syndromes
Author(s) -
Nabbout Rima
Publication year - 2013
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/epi.12278
Subject(s) - status epilepticus , epilepsy , ketogenic diet , etiology , medicine , epilepsy syndromes , atrophy , inflammation , encephalitis , encephalopathy , pediatrics , neuroscience , psychology , immunology , virus
Summary 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.”