{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T20:18:11Z","timestamp":1760300291547,"version":"3.41.2"},"reference-count":37,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,10,2]],"date-time":"2019-10-02T00:00:00Z","timestamp":1569974400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2019,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Clinical manifestations of the hypothalamic hamartoma\u2010epilepsy syndrome (HH\u2010ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and epilepsy who attended our tertiary Epilepsy Unit between 2003 and 2018. We report the clinical and electroencephalographic features of a series of adult patients with HH and related epilepsy seen in our center together with the treatments and seizure outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We describe a series of eight patients. Five males (62.5%), median age at evaluation was 28.5\u00a0years (IQR: 15.5). Clinical manifestations included focal with preserved and impaired awareness emotional seizures (gelastic seizures [GS]) in six patients (75%), focal tonic, atonic with impaired awareness and focal to bilateral tonic\u2013clonic seizures. Mild GS were the only symptom in one patient. Three patients (37.5%) had endocrinological disturbances such as obesity and hypothyroidism. Fifty percent of the patients showed psychiatric comorbidity such as anxiety disorder and aggressiveness, and two patients had psychomotor delay. Seven patients (87.7%) had drug\u2010resistant seizures and three of them were treated with radiosurgery. Out of the treated group, only one (33.3%) became seizure\u2010free 2\u00a0years after surgery but developed psychiatric problems. The other two patients had an Engel IV outcome and received a vagal nerve stimulation (VNS) implant. VNS did not lead to changes either in intensity nor in seizure frequency.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Hypothalamic hamartoma\u2010epilepsy syndrome clinical manifestations in adult patients are as variable as at pediatric age. Outcome of therapeutic options such as radiosurgery or VNS may be poorer at this stage.<\/jats:p><\/jats:sec>","DOI":"10.1002\/brb3.1412","type":"journal-article","created":{"date-parts":[[2019,10,3]],"date-time":"2019-10-03T03:10:36Z","timestamp":1570072236000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome\u2014A unicenter experience"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7436-987X","authenticated-orcid":false,"given":"Estefan\u00eda","family":"Conde Blanco","sequence":"first","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5985-6597","authenticated-orcid":false,"given":"Carla","family":"Anciones Mart\u00edn","sequence":"additional","affiliation":[{"name":"Department of Neurology Hospital Ruber Internacional Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9317-3565","authenticated-orcid":false,"given":"Isabel","family":"Manzanares","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4857-5933","authenticated-orcid":false,"given":"Francisco","family":"Gil L\u00f3pez","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pedro","family":"Rold\u00e1n","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Donaire","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jordi","family":"Rumi\u00e1","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar","family":"Carre\u00f1o","sequence":"additional","affiliation":[{"name":"Epilepsy Unit Department of Neurology Hospital Clinic Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,10,2]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2003.10.003"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.3171\/2016.7.FOCUS16218"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13760"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13758"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.2176\/nmc.43.61"},{"issue":"1","key":"e_1_2_8_7_1","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1684\/j.1950-6945.2000.tb00347.x","article-title":"Hypothalamic hamartoma, precocious puberty and gelastic seizures: A special model of \u201cepileptic\u201d developmental disorder","volume":"2","author":"Deonna T.","year":"2000","journal-title":"Epileptic Disorders"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13751"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1360-0443.2001.961217253.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spen.2007.03.002"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.1.43"},{"issue":"1","key":"e_1_2_8_12_1","first-page":"11","article-title":"Hypothalamic hamartoma: Clinical characteristics. 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