{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T10:18:12Z","timestamp":1784197092227,"version":"3.55.0"},"reference-count":50,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,10,27]],"date-time":"2019-10-27T00:00:00Z","timestamp":1572134400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008272","name":"Novartis Pharmaceuticals Corporation","doi-asserted-by":"publisher","award":["Not applicable"],"award-info":[{"award-number":["Not applicable"]}],"id":[{"id":"10.13039\/100008272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To estimate health\u2010related quality of life (HRQoL) in patients with tuberous sclerosis complex (TSC) and associated manifestations and to identify potential factors associated with HRQoL in this population of patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a retrospective chart review of adults with TSC who attended the outpatient clinic of the University Medical Center Utrecht in the Netherlands from 1990 to 2015 (N\u00a0=\u00a0363; on average 33.6\u00a0years of follow\u2010up). HRQoL data were assessed in 2012 using the Health Utility Index version 3 (HUI\u20103) questionnaire completed by patients or caregivers (N\u00a0=\u00a0214 with HUI score and \u22651 TSC manifestation, including renal angiomyolipomas [rAMLs], subependymal giant cell astrocytoma [SEGA], or epilepsy).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 214 patients in the study sample, 171 had TSC\u2010associated epilepsy (with or without rAML\/SEGA), 37 had TSC and rAML (without epilepsy or SEGA), and 6 had other combinations of manifestations. The median HUI score for the 214 patients with \u22651 TSC manifestation was 0.51 (\u22120.371 to 1 scale, 1\u00a0=\u00a0perfect health, 0\u00a0=\u00a0death, &lt;0\u00a0=\u00a0worse than death). Among all components used to build the overall HUI score, the cognition component had the lowest score (mean\u00a0=\u00a00.47; 0\u20101 scale). Patients with TSC\u2010epilepsy had significantly lower overall HUI than patients with TSC and rAML only (median HUI\u00a0=\u00a00.31 vs 0.95, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.05), especially those who were in refractory state for prolonged periods of time (median HUI\u00a0=\u00a0\u22120.11 among patients with seizures during the entire duration of their follow\u2010up time). In multivariate analyses, severe impairment of daily functioning was the strongest predictor of HRQoL decrement (adjusted HUI difference between patients with severe vs. no impairment\u00a0=\u00a0\u22120.55, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.05).<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>This study showed that TSC\u2010related epilepsy is associated with lower HUI, especially for patients who have refractory seizures for prolonged periods of time. Early and effective interventions to control or reduce seizures and preserve patients\u2019 cognitive functions may help to improve patients\u2019 quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1002\/epi4.12365","type":"journal-article","created":{"date-parts":[[2019,10,17]],"date-time":"2019-10-17T17:33:43Z","timestamp":1571333623000},"page":"581-592","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Epilepsy, impaired functioning, and quality of life in patients with tuberous sclerosis complex"],"prefix":"10.1002","volume":"4","author":[{"given":"Menno","family":"Vergeer","sequence":"first","affiliation":[{"name":"University Medical Center Utrecht Utrecht Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendela L.","family":"de Ranitz\u2010Greven","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht Utrecht Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen P.","family":"Neary","sequence":"additional","affiliation":[{"name":"Novartis Pharmaceuticals Corporation East Hanover NJ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raluca","family":"Ionescu\u2010Ittu","sequence":"additional","affiliation":[{"name":"Analysis Group, Inc. Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0060-765X","authenticated-orcid":false,"given":"Bruno","family":"Emond","sequence":"additional","affiliation":[{"name":"Analysis Group, Inc. Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mei","family":"Sheng Duh","sequence":"additional","affiliation":[{"name":"Analysis Group, Inc. 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