{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T04:33:29Z","timestamp":1785818009890,"version":"3.56.0"},"reference-count":14,"publisher":"Wiley","issue":"s6","license":[{"start":{"date-parts":[[2013,9,3]],"date-time":"2013-09-03T00:00:00Z","timestamp":1378166400000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2013,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (<jats:styled-content style=\"fixed-case\">IHHE<\/jats:styled-content>) and febrile infection\u2013related epilepsy syndrome (<jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content>) 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 <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content>, except for a response to ketogenic diet (<jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>) in half of patients. In <jats:styled-content style=\"fixed-case\">IHHS<\/jats:styled-content>, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content> 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 \u201cacute encephalopathy with inflammation\u2010mediated status epilepticus.\u201d<\/jats:p>","DOI":"10.1111\/epi.12278","type":"journal-article","created":{"date-parts":[[2013,9,3]],"date-time":"2013-09-03T16:37:20Z","timestamp":1378226240000},"page":"54-56","source":"Crossref","is-referenced-by-count":41,"title":["<scp>FIRES<\/scp> and <scp>IHHE<\/scp>:<scp> D<\/scp>elineation of the syndromes"],"prefix":"10.1111","volume":"54","author":[{"given":"Rima","family":"Nabbout","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology Referral Center for Rare Epilepsies Necker Infants Maladies Hospital APHP Paris France"},{"name":"Inserm U663 Paris France"},{"name":"University Paris Descartes; CEA Gif sur Y vete France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,9,3]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2012.04435.x"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02522.x"},{"key":"e_1_2_5_4_1","first-page":"247","volume-title":"Epileptic syndrome in infancy, childhood and adolescence","author":"Chauvel P","year":"2005"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1959.tb04278.x"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2011.03250.x"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.110.077214"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2006.01.002"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2012.03614.x"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02703.x"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70214-3"},{"key":"e_1_2_5_12_1","first-page":"203","volume-title":"Henri Gastaut and the Marseille school's contribution to neurosciences (EEG S35)","author":"Roger J","year":"1982"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e318266fcc5"},{"key":"e_1_2_5_14_1","first-page":"92","article-title":"FIRES: febrile infection responsive epileptic (FIRE) encephalopathies of school age","volume":"31","author":"Baalen A","year":"2009","journal-title":"Brain Dev"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02535.x"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fepi.12278","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.12278","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,13]],"date-time":"2023-09-13T22:39:53Z","timestamp":1694644793000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.12278"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,9]]},"references-count":14,"journal-issue":{"issue":"s6","published-print":{"date-parts":[[2013,9]]}},"alternative-id":["10.1111\/epi.12278"],"URL":"https:\/\/doi.org\/10.1111\/epi.12278","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,9]]}}}