{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T02:24:07Z","timestamp":1791339847674,"version":"4.3.1"},"reference-count":22,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,1,24]],"date-time":"2024-01-24T00:00:00Z","timestamp":1706054400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100011096","name":"Jazz Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011096","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Lennox\u2010Gastaut syndrome (LGS) is an archetypal developmental and epileptic encephalopathy, for which novel treatments are emerging. Diagnostic criteria for LGS have recently been defined by the International League Against Epilepsy (ILAE). We aimed to apply these criteria in a real\u2010world setting.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We applied ILAE diagnostic criteria to a cohort of patients diagnosed with LGS by epileptologists following inpatient video\u2010EEG monitoring (VEM) at tertiary comprehensive epilepsy centers between 1995 and 2015. We also assessed mortality in this cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty patients diagnosed with LGS and had complete records available for review were identified. Among them, 29 (48%) patients met ILAE diagnostic criteria for LGS (ILAE\u2010DC group). Thirty\u2010one did not meet criteria (non\u2010ILAE\u2010DC) due to the absence of documented tonic seizures (<jats:italic>n<\/jats:italic>\u2009=\u20097), EEG features (<jats:italic>n<\/jats:italic>\u2009=\u200912), or both tonic seizures and EEG features (<jats:italic>n<\/jats:italic>\u2009=\u200910), intellectual disability (<jats:italic>n<\/jats:italic>\u2009=\u20091), or drug resistance (<jats:italic>n<\/jats:italic>\u2009=\u20091). The ILAE\u2010DC group had a shorter duration of epilepsy at VEM than the non\u2010ILAE\u2010DC group (median\u2009=\u200912.0\u2009years vs. 23.7\u2009years, respectively; <jats:italic>p<\/jats:italic>\u2009=\u20090.015). The proportions of patients with multiple seizure types (100% vs. 96.7%), \u22642.5\u2009Hz slow spike\u2010and\u2010wave EEG activity (100% vs. 90%), seizure\u2010related injuries (27.6% vs. 25.8%), and mortality (standardized mortality ratio 4.60 vs. 5.12) were similar between the groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Up to 52% of patients diagnosed with LGS following VEM may not meet recently accepted ILAE criteria for LGS diagnosis. This may reflect both the limitations of retrospective medical record review and a historical tendency of applying the LGS diagnosis to a broad spectrum of severe, early\u2010onset drug\u2010resistant epilepsies with drop attacks. The ILAE criteria allow the delineation of LGS based on distinct electroclinical features, potentiating accurate diagnosis, prognostication, and management formulation. Nonetheless, mortality outcomes between those who did and did not meet ILAE diagnostic criteria for LGS were similarly poor, and both groups suffered high rates of seizure\u2010related injury.<\/jats:p><\/jats:sec><jats:sec><jats:title>Plain Language Summary<\/jats:title><jats:p>More than half of patients diagnosed with Lennox\u2010Gastaut Syndrome (LGS) at three Australian epilepsy monitoring units between 1995 and 2015 did not meet the recently devised International League Against Epilepsy (ILAE) diagnostic criteria for LGS. Mortality was equally high in those who did and did not meet the ILAE diagnostic criteria, and seizure\u2010related injury was common. The ILAE diagnostic criteria will guide accurate diagnosis, management, prognostication, and research in patients with LGS, however may be limited in their practical application to patients with a longer duration of epilepsy, or to those for whom detailed assessment is difficult.<\/jats:p><\/jats:sec>","DOI":"10.1002\/epi4.12894","type":"journal-article","created":{"date-parts":[[2023,12,23]],"date-time":"2023-12-23T00:34:00Z","timestamp":1703291640000},"page":"602-612","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Applying the ILAE diagnostic criteria for Lennox\u2010Gastaut syndrome in the real\u2010world setting: A multicenter retrospective cohort study"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9108-207X","authenticated-orcid":false,"given":"Russell","family":"Nightscales","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Central Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Department of Neurology Alfred Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1888-6917","authenticated-orcid":false,"given":"Zhibin","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Central Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Clinical Epidemiology, School of Public Health and Preventive Medicine Monash University  Melbourne Victoria Australia"},{"name":"Department of Medicine (The Royal Melbourne Hospital) The University of Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0517-2533","authenticated-orcid":false,"given":"Sarah","family":"Barnard","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Central Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Department of Neurology Alfred Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clarissa","family":"Auvrez","sequence":"additional","affiliation":[{"name":"NorthWestern Mental Health Melbourne Health  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerard","family":"Tao","sequence":"additional","affiliation":[{"name":"Department of Medicine (The Royal Melbourne Hospital) The University of Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4638-9579","authenticated-orcid":false,"given":"Shobi","family":"Sivathamboo","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Central Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Department of Neurology Alfred Health  Melbourne Victoria Australia"},{"name":"Department of Medicine (The Royal Melbourne Hospital) The University of Melbourne  Melbourne Victoria Australia"},{"name":"Department of Neurology The Royal Melbourne Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caitlin","family":"Bennett","sequence":"additional","affiliation":[{"name":"Epilepsy Research Centre, Department of Medicine (Austin Health) The University of Melbourne  Heidelberg Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Rychkova","sequence":"additional","affiliation":[{"name":"Department of Medicine (The Royal Melbourne Hospital) The University of Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendyl","family":"D'Souza","sequence":"additional","affiliation":[{"name":"Department of Medicine, St. Vincent's Hospital The University of Melbourne  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4580-841X","authenticated-orcid":false,"given":"Samuel F.","family":"Berkovic","sequence":"additional","affiliation":[{"name":"Epilepsy Research Centre, Department of Medicine (Austin Health) The University of Melbourne  Heidelberg Victoria Australia"},{"name":"Bladin\u2010Berkovic Comprehensive Epilepsy Program, Department of Neurology Austin Health  Heidelberg Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0473-6475","authenticated-orcid":false,"given":"John\u2010Paul","family":"Nicolo","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Central Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Department of Neurology Alfred Health  Melbourne Victoria Australia"},{"name":"Department of Neurology The Royal Melbourne Hospital  Melbourne Victoria 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