{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T22:03:26Z","timestamp":1790287406101,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,9,26]],"date-time":"2018-09-26T00:00:00Z","timestamp":1537920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R37\u2010NS31146"],"award-info":[{"award-number":["R37\u2010NS31146"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2018,11]]},"abstract":"Summary<\/jats:title>\n \n Objectives<\/jats:title>\n \n Developmental encephalopathic epilepsies (\n DEEs<\/jats:styled-content>\n ) are characterized by refractory seizures, disability, and early death. Opportunities to improve care and outcomes focus on West syndrome\/infantile spasms (\n WS<\/jats:styled-content>\n \/\n IS<\/jats:styled-content>\n ). Lennox\u2010Gastaut syndrome (\n LGS<\/jats:styled-content>\n ) is almost as common but receives little attention. We examined initial presentations of\n DEE<\/jats:styled-content>\n s and their evolution over time to identify risk and indicators of developing\n LGS<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Data are from the Connecticut Study of Epilepsy, a prospective, longitudinal study of 613 children with newly diagnosed epilepsy recruited in 1993\u20101997. Central review of medical records permitted classification of epilepsy syndromes at diagnosis and at reclassification 2, 5, and 9\u00a0years later.\n DEE<\/jats:styled-content>\n s were compared to other epilepsies for seizure and cognitive outcomes and mortality. Analyses examined the evolution of\n DEE<\/jats:styled-content>\n syndromes after initial presentation, with specific comparisons made between\n WS<\/jats:styled-content>\n \/\n IS<\/jats:styled-content>\n and\n LGS<\/jats:styled-content>\n . Statistical analyses were performed with\n t<\/jats:italic>\n tests and chi\u2010square tests.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Fifty\u2010eight children (9.4%) had\n DEE<\/jats:styled-content>\n s, median onset age\u00a0=\u00a01.1\u00a0years (interquartile range (\n [IQR<\/jats:styled-content>\n ]\u00a00.3\u20101.3) in\n DEE<\/jats:styled-content>\n s and 6.0\u00a0years (\n IQR<\/jats:styled-content>\n \u00a03.0\u20109.0) in other epilepsies (\n P<\/jats:italic>\n \u00a0<\u00a00.001).\n DEE<\/jats:styled-content>\n s vs other epilepsies had more pharmacoresistance (71% vs 18%), intellectual disability (84% vs 11%), and mortality (21% vs <1%; all\n P<\/jats:italic>\n \u00a0<\u00a00.001). During follow\u2010up, the form of epilepsy evolved in 33 children.\n WS<\/jats:styled-content>\n \/\n IS<\/jats:styled-content>\n was the most common initial diagnosis (N\u00a0=\u00a023) and in 5 children WS\/IS evolved later. LGS was diagnosed initially in 4 children (1 later revised) and in 22 by the end of follow\u2010up, including 7 evolving from\n WS<\/jats:styled-content>\n \/\n IS<\/jats:styled-content>\n and 12 from nonsyndromic generalized, focal, or undetermined epilepsies. Evolution to\n LGS<\/jats:styled-content>\n took a median of 1.9\u00a0years.\n LGS<\/jats:styled-content>\n developed in 13% of infants, including 9% of those who did not present initially with\n WS<\/jats:styled-content>\n \/\n IS<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Significance<\/jats:title>\n \n DEEs account for disproportionate amounts of pharmacoresistance, disability, and early mortality.\n LGS<\/jats:styled-content>\n often has a window between initial presentation and full expression. LGS should become targeted for early detection and prevention.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/epi.14569","type":"journal-article","created":{"date-parts":[[2018,9,26]],"date-time":"2018-09-26T06:15:02Z","timestamp":1537942502000},"page":"2096-2105","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":63,"title":["Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox\u2010Gastaut syndrome"],"prefix":"10.1111","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0298-5523","authenticated-orcid":false,"given":"Anne T.","family":"Berg","sequence":"first","affiliation":[{"name":"Ann & Robert H. Lurie Children\u2019s Hospital of Chicago Chicago Illinois"},{"name":"Department of Pediatrics Northwestern\u2010Feinberg School of Medicine Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan R.","family":"Levy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Yale School of Medicine New Haven Connecticut"},{"name":"Department of Neurology Yale School of Medicine New Haven Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francine M.","family":"Testa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Yale School of Medicine New Haven Connecticut"},{"name":"Department of Neurology Yale School of Medicine New Haven Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,9,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2016-3908"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1996.tb00506.x"},{"key":"e_1_2_8_4_1","unstructured":"Preventing epilepsy using vigabatrin in infants with tuberous sclerosis complex (PREVeNT Trial) a randomized double\u2010blind placebo\u2010controlled seizure prevention clinical trial for infants with TSC[online]. Available fromhttps:\/\/clinicaltrials.gov\/ct2\/show\/NCT02849457. 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Available fromhttp:\/\/www.childneurologyfoundation.org\/programs\/infantilespasms\/. Accessed July 16 2018."},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-1807"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12030"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004284"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXG.0000000000000096"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12984"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1712649"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0920-1211(99)00021-2"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70292-8"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12567"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2017.00505"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1714631"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000303813.95800.0d"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2010.01334.x"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2017.05.021"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2011.03179.x"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fepi.14569","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.14569","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/epi.14569","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.14569","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,24]],"date-time":"2023-08-24T06:07:37Z","timestamp":1692857257000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.14569"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,26]]},"references-count":50,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["10.1111\/epi.14569"],"URL":"https:\/\/doi.org\/10.1111\/epi.14569","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,26]]},"assertion":[{"value":"2018-08-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-08-30","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-09-26","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}