{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:33:05Z","timestamp":1790767985505,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,5,26]],"date-time":"2017-05-26T00:00:00Z","timestamp":1495756800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Funda\u00e7\u00e3o AstraZeneca"},{"DOI":"10.13039\/100015672","name":"European Huntington's Disease Network","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100015672","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Huntington's disease (<jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>) is a rare and fatal inherited genetic disorder characterized by progressive motor, cognitive, and behavioral impairment. It leads to premature death, but data regarding advanced\u2010stage disease are scarce. We sought to determine <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\u2010associated survival, mortality, and causes and places of death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data from the European <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> Network prospective study (<jats:styled-content style=\"fixed-case\">REGISTRY<\/jats:styled-content>) collected from 2001 through 2013 were used, including the Unified Huntington's Disease Rating Scale and death report forms. Group comparisons were performed using the <jats:italic>t<\/jats:italic> test or the \u03c7<jats:sup>2<\/jats:sup> test. Survival analyses were computed through Kaplan\u2010Meier estimates of median survival. All tests were 2\u2010sided with a significance level of <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.05.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 5164 participants were analyzed. The mean age at diagnosis was 49\u00a0years, and the mean age at death was 58\u00a0years. At the end of the study period, there were 533 deaths (10.3% of patients). Median survival was 24\u00a0years from diagnosis and 35\u00a0years from symptom onset. The most frequent causes of death were pneumonia (19.5%), other infections (6.9%), and suicide (6.6%). The most frequent places of death were the hospital (29.8%), the home (23.9%), and nursing houses (19.8%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> tend to die from the same conditions as patients with other neurodegenerative diseases. However, compared with nonhereditary Parkinson's disease and Alzheimer's disease, the median time from onset to death is longer, and the places of death are distinctive.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mdc3.12502","type":"journal-article","created":{"date-parts":[[2017,4,23]],"date-time":"2017-04-23T07:16:44Z","timestamp":1492931804000},"page":"737-742","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":85,"title":["Survival, Mortality, Causes and Places of Death in a European Huntington's Disease Prospective Cohort"],"prefix":"10.1002","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5567-7938","authenticated-orcid":false,"given":"Filipe Brogueira","family":"Rodrigues","sequence":"first","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisy","family":"Abreu","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joana","family":"Dam\u00e1sio","sequence":"additional","affiliation":[{"name":"Neurology Department Hospital de Santo Ant\u00f3nio\u2010Centro Hospitalar do Porto Porto Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nilza","family":"Goncalves","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonor","family":"Correia\u2010Guedes","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"},{"name":"Department of Neurosciences Neurology Hospital de Santa Maria Centro Hospitalar de Lisboa Norte Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Coelho","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"},{"name":"Department of Neurosciences Neurology Hospital de Santa Maria Centro Hospitalar de Lisboa Norte Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joaquim J.","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Unit Instituto de Medicina Molecular Lisbon Portugal"},{"name":"Laboratory of Clinical Pharmacology and Therapeutics Faculty of Medicine University of Lisbon Lisbon Portugal"},{"name":"Campus Neurol\u00f3gico Senior Torres Vedras Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"REGISTRY Investigators of the European Huntington's Disease Network","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,5,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/306234a0"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"e_1_2_8_4_1","first-page":"CD006455","article-title":"Therapeutic interventions for disease progression in Huntington's disease","volume":"3","author":"Mestre T","year":"2009","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.12968\/ijpn.2007.13.3.23274"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-2018.2004.00183.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1891\/1521-0987.9.2.75"},{"key":"e_1_2_8_8_1","unstructured":"European Huntington's Disease Network (EHDN). 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