{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T06:13:18Z","timestamp":1790662398512,"version":"4.1.0"},"reference-count":34,"publisher":"Wiley","issue":"s8","license":[{"start":{"date-parts":[[2013,11,13]],"date-time":"2013-11-13T00:00:00Z","timestamp":1384300800000},"content-version":"vor","delay-in-days":12,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2013,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Epileptic encephalopathies (<jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content>s) are electroclinical entities with a peculiar course of disease; seizures and electroencephalographic (<jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content>) epileptiform abnormalities, ictal and interictal, contribute to progressive disturbance of cerebral functions. Frequently<jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content>s are drug resistant, and consequences may be catastrophic. The main goal of treatment is to stop the peculiar course of epilepsy, operating on three parameters: seizure control, reduction of<jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content>abnormalities, and developmental outcome. For a correct therapeutic approach it is mandatory to have an as accurate as possible syndromic and etiologic diagnosis. Given the poor efficacy of conventional antiepileptic drugs (<jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s), the use of specific drugs for<jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content>s, such as adrenocorticotropic hormone (<jats:styled-content style=\"fixed-case\">ACTH<\/jats:styled-content>) and corticosteroids or stiripentol is suggested. In some cases the choice of treatment is strictly related to the etiology: vigabatrin in tuberous sclerosis, ketogenic diet in glucose transporter type 1 (<jats:styled-content style=\"fixed-case\">GLUT<\/jats:styled-content>\u20101) deficiency, and pyridoxine in pyridoxine deficiency. Some AEDs combinations, such as sodium valproate with lamotrigine, have also provided interesting results, for example, in Lennox\u2010Gastaut syndrome, although controlled studies are lacking. Finally, early surgery can be an option in children with focal structural abnormalities responsible for<jats:styled-content style=\"fixed-case\">EEs<\/jats:styled-content>preferably before irreversible damage on developmental outcome. Multispecialist support is recommended in<jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content>. Management should be global from the onset, integrating not only seizure control but also all issues related to comorbidities, particularly neuropsychological and psychiatric.<\/jats:p>","DOI":"10.1111\/epi.12423","type":"journal-article","created":{"date-parts":[[2013,11,13]],"date-time":"2013-11-13T15:44:25Z","timestamp":1384357465000},"page":"45-50","source":"Crossref","is-referenced-by-count":41,"title":["Therapeutic approach to epileptic encephalopathies"],"prefix":"10.1111","volume":"54","author":[{"given":"Federico","family":"Vigevano","sequence":"first","affiliation":[{"name":"Department of Neuroscience Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis","family":"Arzimanoglou","sequence":"additional","affiliation":[{"name":"Epilepsy, Sleep and Pediatric Neurophysiology Department University Hospitals of Lyon (HCL) and Lyon's Neuroscience Research Centre (CRNL) INSERM U1028 CNRS 5292 UCB Lyon 1 Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Perrine","family":"Plouin","sequence":"additional","affiliation":[{"name":"Clinical Neurophysiology Hospital Necker Enfants Malades et INSERM U663 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Specchio","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,11,13]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70292-8"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02522.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2009.02224.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2011.03009.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1684\/j.1950-6945.2006.tb00174.x","article-title":"Ketogenic diet in patients with myoclonic\u2010astatic epilepsy","volume":"8","author":"Caraballo RH","year":"2006","journal-title":"Epileptic Disord"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2011.03007.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0920-1211(96)01006-6"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)03157-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2009.160606"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2012.10.006"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2001.10401.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2005.10.003"},{"issue":"1","key":"e_1_2_8_14_1","first-page":"CD005222","article-title":"Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms","volume":"24","author":"Gayatri NA","year":"2007","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000303813.95800.0d"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2012.11.002"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(83)80606-4"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1991.tb05246.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2001.tb00198.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2012.09.014"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-013-0223-z"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2012.04233.x"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0032-1307454"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(05)70199-X"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000127773.72699.C8"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40263-013-0041-6"},{"key":"e_1_2_8_27_1","unstructured":"National Institute for Clinical Excellence. 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