{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T01:26:48Z","timestamp":1777685208048,"version":"3.51.4"},"reference-count":12,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2020,10,8]],"date-time":"2020-10-08T00:00:00Z","timestamp":1602115200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JHD"],"published-print":{"date-parts":[[2020,10,8]]},"abstract":"<jats:p>Background: Huntington\u2019s disease (HD) is an autosomal dominant neurodegenerative disorder that typically manifests between the ages of 30 and 50 years. However, the disease can present at any age, and phenotypic differences between younger and later-onset patients have received limited attention. Objective: To compare clinical features of late- (&gt;70 years of age) and younger-onset (&lt;30 years of age) HD patients. Methods: Patients presenting to our regional NHS HD clinic with new-onset manifest HD diagnosed over the age of 70 years (LoHD) (n\u200a=\u200a18) were compared with a younger cohort who developed disease under the age of 30 years (YoHD) (n\u200a=\u200a12). Rate of progression over time on standard cognitive and motor measures was compared. Results: At first clinic presentation, both groups had the same total UHDRS scores. However, the LoHD group had higher chorea scores (F (1,28)\u200a=\u200a6.52, p\u200a=\u200a0.016), while the YoHD group had more dystonia (F (1,28)\u200a=\u200a8.69, p\u200a=\u200a0.006) and eye movement abnormalities (F (1,28)\u200a=\u200a16.991, p\u200a&lt;\u200a0.001). The YoHD group also had a greater rate of motor progression, especially for bulbar measures (F (1, 28)\u200a=\u200a6.96, p\u200a=\u200a0.013) and bradykinesia (F (1, 28)\u200a=\u200a7.99, p\u200a=\u200a0.009). No differences were found in the rate of cognitive change (F (1,21)\u200a=\u200a1.727, p\u200a=\u200a0.203) nor functional capacity (F (1,28)\u200a=\u200a1.388, p\u200a=\u200a0.249) between the groups. Conclusion: Phenotypic differences between YoHD and LoHD patients were found in terms of initial presentation and rate of motor progression. This has implications for therapeutic trials involving HD patients of different ages, given their different clinical features and progression.<\/jats:p>","DOI":"10.3233\/jhd-200404","type":"journal-article","created":{"date-parts":[[2020,7,16]],"date-time":"2020-07-16T10:54:13Z","timestamp":1594896853000},"page":"275-282","source":"Crossref","is-referenced-by-count":21,"title":["The Clinical Features and Progression of Late-Onset Versus Younger-Onset in an Adult Cohort of Huntington\u2019s Disease Patients"],"prefix":"10.1177","volume":"9","author":[{"given":"Megha","family":"Anil","sequence":"first","affiliation":[{"name":"Department of Clinical Neuroscience, John van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah L.","family":"Mason","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience, John van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roger A.","family":"Barker","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience, John van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","reference":[{"issue":"6","key":"10.3233\/JHD-200404_ref1","first-page":"971","article-title":"The Huntington\u2019s Disease Collaborative Research Group","volume":"72","author":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes","year":"1993","journal-title":"Cell"},{"key":"10.3233\/JHD-200404_ref2","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/1750-1172-5-40","article-title":"Huntington\u2019s disease: A clinical review","volume":"5","author":"Roos","year":"2010","journal-title":"Orphanet J Rare Dis"},{"issue":"10","key":"10.3233\/JHD-200404_ref3","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1212\/WNL.0b013e318249f683","article-title":"CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion","volume":"78","author":"Lee","year":"2012","journal-title":"Neurology"},{"key":"10.3233\/JHD-200404_ref4","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1016\/j.ebiom.2019.09.020","article-title":"A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomes","volume":"48","author":"Ciosi","year":"2019","journal-title":"EBioMedicine"},{"issue":"2","key":"10.3233\/JHD-200404_ref5","doi-asserted-by":"crossref","first-page":"95","DOI":"10.3233\/JHD-170247","article-title":"What do we know about late onset Huntington\u2019s disease?","volume":"6","author":"Chaganti","year":"2017","journal-title":"J Huntingtons Dis"},{"issue":"6","key":"10.3233\/JHD-200404_ref6","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1093\/ageing\/23.6.445","article-title":"Late-onset Huntington\u2019s disease: A clinical and molecular study","volume":"23","author":"James","year":"1994","journal-title":"Age Ageing"},{"issue":"1","key":"10.3233\/JHD-200404_ref7","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1111\/j.1447-0594.2007.00372.x","article-title":"Huntington\u2019s disease with onset ages greater than 60 years","volume":"7","author":"Yoshida","year":"2007","journal-title":"Geriatr Gerontol Int"},{"issue":"1-2","key":"10.3233\/JHD-200404_ref8","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.jns.2008.09.029","article-title":"Late onset Huntington disease: Clinical and genetic characteristics of 34 cases","volume":"276","author":"Lipe","year":"2009","journal-title":"J Neurol Sci"},{"issue":"7","key":"10.3233\/JHD-200404_ref9","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1016\/j.parkreldis.2014.03.017","article-title":"Late-onset Huntington\u2019s disease: Diagnostic and prognostic considerations","volume":"20","author":"Koutsis","year":"2014","journal-title":"Parkinsonism Relat Disord"},{"issue":"1","key":"10.3233\/JHD-200404_ref10","doi-asserted-by":"crossref","first-page":"99","DOI":"10.3233\/JHD-140119","article-title":"Clinical and molecular features of late onset Huntington disease in a Peruvian cohort","author":"Cornejo-Olivas","year":"2015","journal-title":"J Huntingtons Dis"},{"issue":"3","key":"10.3233\/JHD-200404_ref11","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1212\/WNL.45.3.443","article-title":"Hereditary late-onset chorea without significant dementia: Genetic evidence for substantial phenotypic variation in Huntington\u2019s disease","volume":"45","author":"Britton","year":"1995","journal-title":"Neurology"},{"issue":"5","key":"10.3233\/JHD-200404_ref13","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1007\/s11910-011-0215-x","article-title":"Cognitive impairment in Huntington disease: Diagnosis and treatment","volume":"11","author":"Paulsen","year":"2011","journal-title":"Curr Neurol Neurosci Re"}],"container-title":["Journal of Huntington's Disease"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/JHD-200404","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:51:04Z","timestamp":1777452664000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/JHD-200404"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,8]]},"references-count":12,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3233\/jhd-200404","relation":{},"ISSN":["1879-6397","1879-6400"],"issn-type":[{"value":"1879-6397","type":"print"},{"value":"1879-6400","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,8]]}}}