{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T21:08:02Z","timestamp":1790111282748,"version":"4.0.1"},"reference-count":38,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T00:00:00Z","timestamp":1788480000000},"content-version":"vor","delay-in-days":3,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100020595","name":"National Science and Technology Council","doi-asserted-by":"publisher","award":["NSTC 114-2314-B-002-077"],"award-info":[{"award-number":["NSTC 114-2314-B-002-077"]}],"id":[{"id":"10.13039\/100020595","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["113-S0099"],"award-info":[{"award-number":["113-S0099"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["115-S0178"],"award-info":[{"award-number":["115-S0178"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["110-S4878"],"award-info":[{"award-number":["110-S4878"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["NTUH 105-03"],"award-info":[{"award-number":["NTUH 105-03"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["114-X0014"],"award-info":[{"award-number":["114-X0014"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of the Formosan Medical Association"],"published-print":{"date-parts":[[2026,9]]},"DOI":"10.1016\/j.jfma.2026.09.003","type":"journal-article","created":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T19:52:54Z","timestamp":1788983574000},"update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long-term seizure outcomes and factors associated with response to adjunctive everolimus in TSC-associated epilepsy"],"prefix":"10.1016","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-4097-5988","authenticated-orcid":false,"given":"Tzu-Hsuan","family":"Su","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pei-Lung","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven Shinn-Forng","family":"Peng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6658-7359","authenticated-orcid":false,"given":"Chi-Yung","family":"Shang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6577-0116","authenticated-orcid":false,"given":"Chia-Wei","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6974-971X","authenticated-orcid":false,"given":"Pi-Chuan","family":"Fan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jfma.2026.09.003_bib1","doi-asserted-by":"crossref","DOI":"10.1038\/nrdp.2016.35","article-title":"Tuberous sclerosis complex","volume":"2","author":"Henske","year":"2016","journal-title":"Nat Rev Dis Primers"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib2","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1186\/s13023-018-0870-y","article-title":"Incidence of tuberous sclerosis and age at first diagnosis: new data and emerging trends from a national, prospective surveillance study","volume":"13","author":"Ebrahimi-Fakhari","year":"2018","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.jfma.2026.09.003_bib3","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.pediatrneurol.2021.07.011","article-title":"Updated international Tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations","volume":"123","author":"Northrup","year":"2021","journal-title":"Pediatr Neurol"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib4","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1002\/epi4.12286","article-title":"Epilepsy in Tuberous sclerosis complex: findings from the TOSCA study","volume":"4","author":"Nabbout","year":"2019","journal-title":"Epilepsia Open"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib5","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1212\/01.wnl.0000250330.44291.54","article-title":"Cognitive prognosis of patients with Tuberous sclerosis complex","volume":"68","author":"Winterkorn","year":"2007","journal-title":"Neurology"},{"issue":"7","key":"10.1016\/j.jfma.2026.09.003_bib6","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.1111\/j.1528-1167.2009.02474.x","article-title":"The natural history of epilepsy in Tuberous sclerosis complex","volume":"51","author":"Chu-Shore","year":"2010","journal-title":"Epilepsia"},{"issue":"2","key":"10.1016\/j.jfma.2026.09.003_bib7","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ejpn.2009.03.003","article-title":"Early control of seizures improves long-term outcome in children with Tuberous sclerosis complex","volume":"14","author":"Bombardieri","year":"2010","journal-title":"Eur J Paediatr Neurol"},{"issue":"9","key":"10.1016\/j.jfma.2026.09.003_bib8","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/S1474-4422(22)00213-7","article-title":"Advances in the genetics and neuropathology of Tuberous sclerosis complex: edging closer to targeted therapy","volume":"21","author":"Curatolo","year":"2022","journal-title":"Lancet Neurol"},{"issue":"9861","key":"10.1016\/j.jfma.2026.09.003_bib9","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/S0140-6736(12)61134-9","article-title":"Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with Tuberous sclerosis complex (EXIST-1): a multicentre, randomised, placebo-controlled phase 3 trial","volume":"381","author":"Franz","year":"2013","journal-title":"Lancet"},{"issue":"9869","key":"10.1016\/j.jfma.2026.09.003_bib10","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/S0140-6736(12)61767-X","article-title":"Everolimus for angiomyolipoma associated with Tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial","volume":"381","author":"Bissler","year":"2013","journal-title":"Lancet"},{"issue":"10056","key":"10.1016\/j.jfma.2026.09.003_bib11","doi-asserted-by":"crossref","first-page":"2153","DOI":"10.1016\/S0140-6736(16)31419-2","article-title":"Adjunctive everolimus therapy for treatment-resistant focal-onset seizures associated with tuberous sclerosis (EXIST-3): a phase 3, randomised, double-blind, placebo-controlled study","volume":"388","author":"French","year":"2016","journal-title":"Lancet"},{"issue":"23","key":"10.1016\/j.jfma.2026.09.003_bib12","doi-asserted-by":"crossref","first-page":"2408","DOI":"10.1212\/WNL.0000000000003400","article-title":"Long-term treatment of epilepsy with everolimus in tuberous sclerosis","volume":"87","author":"Krueger","year":"2016","journal-title":"Neurology"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib13","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1186\/s13023-016-0530-z","article-title":"Efficacy and safety of everolimus in children with TSC - associated epilepsy - pilot data from an open single-center prospective study","volume":"11","author":"Samueli","year":"2016","journal-title":"Orphanet J Rare Dis"},{"issue":"3","key":"10.1016\/j.jfma.2026.09.003_bib14","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1111\/epi.16829","article-title":"Everolimus in adult Tuberous sclerosis complex patients with epilepsy: too late for success? A retrospective study","volume":"62","author":"Stockinger","year":"2021","journal-title":"Epilepsia"},{"key":"10.1016\/j.jfma.2026.09.003_bib15","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.seizure.2021.10.011","article-title":"Long-term treatment with everolimus in TSC-associated therapy-resistant epilepsies","volume":"93","author":"Wiegand","year":"2021","journal-title":"Seizure"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib16","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1186\/s13023-023-02982-1","article-title":"Effectiveness and safety of everolimus treatment in patients with Tuberous sclerosis complex in real-world clinical practice","volume":"18","author":"Cockerell","year":"2023","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"10.1016\/j.jfma.2026.09.003_bib17","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1212\/CPJ.0000000000000514","article-title":"Everolimus for treatment-refractory seizures in TSC: extension of a randomized controlled trial","volume":"8","author":"Franz","year":"2018","journal-title":"Neurol, Clin Pract"},{"issue":"5","key":"10.1016\/j.jfma.2026.09.003_bib18","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1002\/ana.23960","article-title":"Everolimus treatment of refractory epilepsy in Tuberous sclerosis complex","volume":"74","author":"Krueger","year":"2013","journal-title":"Ann Neurol"},{"issue":"10","key":"10.1016\/j.jfma.2026.09.003_bib19","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1007\/s40263-021-00839-4","article-title":"Efficacy, retention and tolerability of everolimus in patients with Tuberous Sclerosis complex: a survey-based study on patients' perspectives","volume":"35","author":"Willems","year":"2021","journal-title":"CNS Drugs"},{"issue":"7","key":"10.1016\/j.jfma.2026.09.003_bib20","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1016\/S2352-4642(18)30099-3","article-title":"Adjunctive everolimus for children and adolescents with treatment-refractory seizures associated with Tuberous sclerosis complex: post-hoc analysis of the phase 3 EXIST-3 trial","volume":"2","author":"Curatolo","year":"2018","journal-title":"Lancet Child Adolesc Health"},{"key":"10.1016\/j.jfma.2026.09.003_bib21","article-title":"Common terminology criteria for adverse events","author":"Institute","year":"2017","journal-title":"CTCAE)"},{"issue":"4","key":"10.1016\/j.jfma.2026.09.003_bib22","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1002\/ana.21331","article-title":"Rapamycin prevents epilepsy in a mouse model of Tuberous sclerosis complex","volume":"63","author":"Zeng","year":"2008","journal-title":"Ann Neurol"},{"key":"10.1016\/j.jfma.2026.09.003_bib23","doi-asserted-by":"crossref","first-page":"409","DOI":"10.3389\/fnmol.2018.00409","article-title":"Multiple critical periods for rapamycin treatment to correct structural defects in Tsc-1-Suppressed brain","volume":"11","author":"Cox","year":"2018","journal-title":"Front Mol Neurosci"},{"key":"10.1016\/j.jfma.2026.09.003_bib24","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.pediatrneurol.2018.10.005","article-title":"Longitudinal effects of everolimus on white matter diffusion in Tuberous sclerosis complex","volume":"90","author":"Peters","year":"2019","journal-title":"Pediatr Neurol"},{"issue":"Pt B","key":"10.1016\/j.jfma.2026.09.003_bib25","article-title":"Genetic pathogenesis of the epileptogenic lesions in Tuberous sclerosis complex: therapeutic targeting of the mTOR pathway","volume":"131","author":"Moavero","year":"2022","journal-title":"Epilepsy Behav"},{"issue":"1","key":"10.1016\/j.jfma.2026.09.003_bib26","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1086\/316951","article-title":"Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs","volume":"68","author":"Dabora","year":"2001","journal-title":"Am J Hum Genet"},{"issue":"10","key":"10.1016\/j.jfma.2026.09.003_bib27","doi-asserted-by":"crossref","DOI":"10.1016\/j.ejmg.2022.104573","article-title":"Genotype\/Phenotype correlation in 123 Chinese patients with Tuberous sclerosis complex","volume":"65","author":"Ng","year":"2022","journal-title":"Eur J Med Genet"},{"issue":"12","key":"10.1016\/j.jfma.2026.09.003_bib28","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1212\/01.wnl.0000280578.99900.96","article-title":"Overlapping neurologic and cognitive phenotypes in patients with TSC1 or TSC2 mutations","volume":"70","author":"Jansen","year":"2008","journal-title":"Neurology"},{"issue":"5","key":"10.1016\/j.jfma.2026.09.003_bib29","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1002\/(SICI)1098-1004(199911)14:5<412::AID-HUMU7>3.0.CO;2-K","article-title":"Analysis of both TSC1 and TSC2 for germline mutations in 126 unrelated patients with tuberous sclerosis","volume":"14","author":"Niida","year":"1999","journal-title":"Hum Mutat"},{"issue":"8","key":"10.1016\/j.jfma.2026.09.003_bib30","doi-asserted-by":"crossref","first-page":"2912","DOI":"10.1093\/brain\/awaf072","article-title":"Phenotypic clustering in Tuberous sclerosis complex reveals four distinct disease trajectories","volume":"148","author":"Dhawan","year":"2025","journal-title":"Brain"},{"issue":"56","key":"10.1016\/j.jfma.2026.09.003_bib31","doi-asserted-by":"crossref","first-page":"95516","DOI":"10.18632\/oncotarget.20764","article-title":"Subependymal giant cell astrocytomas in Tuberous sclerosis complex have consistent TSC1\/TSC2 biallelic inactivation, and no BRAF mutations","volume":"8","author":"Bongaarts","year":"2017","journal-title":"Oncotarget"},{"issue":"8","key":"10.1016\/j.jfma.2026.09.003_bib32","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1006242","article-title":"Whole exome sequencing identifies TSC1\/TSC2 biallelic loss as the primary and sufficient driver event for renal angiomyolipoma development","volume":"12","author":"Giannikou","year":"2016","journal-title":"PLoS Genet"},{"issue":"8","key":"10.1016\/j.jfma.2026.09.003_bib33","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0180939","article-title":"Everolimus long-term use in patients with Tuberous sclerosis complex: four-year update of the EXIST-2 study","volume":"12","author":"Bissler","year":"2017","journal-title":"PLoS One"},{"issue":"6","key":"10.1016\/j.jfma.2026.09.003_bib34","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0158476","article-title":"Long-term use of everolimus in patients with Tuberous sclerosis complex: final results from the EXIST-1 study","volume":"11","author":"Franz","year":"2016","journal-title":"PLoS One"},{"issue":"6","key":"10.1016\/j.jfma.2026.09.003_bib35","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1111\/epi.14085","article-title":"Everolimus dosing recommendations for tuberous sclerosis complex-associated refractory seizures","volume":"59","author":"Franz","year":"2018","journal-title":"Epilepsia"},{"issue":"9","key":"10.1016\/j.jfma.2026.09.003_bib36","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1111\/epi.13467","article-title":"Systemic disease manifestations associated with epilepsy in Tuberous sclerosis complex","volume":"57","author":"Jeong","year":"2016","journal-title":"Epilepsia"},{"issue":"12","key":"10.1016\/j.jfma.2026.09.003_bib37","doi-asserted-by":"crossref","first-page":"3827","DOI":"10.1007\/s00381-021-05348-9","article-title":"An Israeli tuberous sclerosis cohort: the efficacy of different anti-epileptic strategies","volume":"37","author":"Shlomovitz","year":"2021","journal-title":"Childs Nerv Syst"},{"key":"10.1016\/j.jfma.2026.09.003_bib38","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.pediatrneurol.2021.12.012","article-title":"Tuber brain proportion determines epilepsy onset in children with Tuberous sclerosis complex","volume":"129","author":"Zhang","year":"2022","journal-title":"Pediatr Neurol"}],"container-title":["Journal of the Formosan Medical Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0929664626008703?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0929664626008703?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T20:34:48Z","timestamp":1790109288000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0929664626008703"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":38,"alternative-id":["S0929664626008703"],"URL":"https:\/\/doi.org\/10.1016\/j.jfma.2026.09.003","relation":{},"ISSN":["0929-6646"],"issn-type":[{"value":"0929-6646","type":"print"}],"subject":[],"published":{"date-parts":[[2026,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Long-term seizure outcomes and factors associated with response to adjunctive everolimus in TSC-associated epilepsy","name":"articletitle","label":"Article Title"},{"value":"Journal of the Formosan Medical Association","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jfma.2026.09.003","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2026, Formosan Medical Association. Published by Elsevier Taiwan LLC. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}