{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T01:52:58Z","timestamp":1777686778088,"version":"3.51.4"},"reference-count":39,"publisher":"SAGE Publications","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JHD"],"published-print":{"date-parts":[[2022,12,16]]},"abstract":"<jats:p>Background: Autonomy describes a psychological state of self-regulation of motivation and action, which is a central characteristic of healthy functioning. In neurodegenerative diseases measures of self-perception have been found to be affected by the disease. However, it has never been investigated whether measures of self-perception, like autonomy, is affected in Huntington\u2019s disease. Objective: We investigated whether autonomy is affected in Huntington\u2019s disease and if the degree of autonomy is associated with motor function, neuropsychiatric symptoms, cognitive impairments, and apathy. Methods: We included 44 premanifest and motor-manifest Huntington\u2019s disease gene expansion carriers and 19 controls. Autonomy was examined using two self-report questionnaires, the Autonomy-Connectedness Scale-30 and the Index of Autonomous Functioning. All participants were examined according to motor function, cognitive impairments, and neuropsychiatric symptoms, including apathy. Results: Statistically significant differences were found between motor-manifest Huntington\u2019s disease gene expansion carriers and premanifest Huntington\u2019s disease gene expansion carriers or controls on two measures of autonomy. Between 25\u201338% of motor-manifest Huntington\u2019s disease gene expansion carriers scored significantly below the normal level on subscales of autonomy as compared to controls. One autonomy subscale was associated with apathy (r\u200a=\u200a\u20130.65), but not with other symptoms of Huntington\u2019s disease. Conclusion: This study provides evidence for impaired autonomy in individuals with Huntington\u2019s disease and an association between autonomy and apathy. The results underline the importance of maintaining patient autonomy and involvement in care throughout the disease.<\/jats:p>","DOI":"10.3233\/jhd-220540","type":"journal-article","created":{"date-parts":[[2022,8,9]],"date-time":"2022-08-09T11:04:12Z","timestamp":1660043052000},"page":"373-381","source":"Crossref","is-referenced-by-count":0,"title":["An Exploratory Study Investigating Autonomy in Huntington\u2019s Disease Gene Expansion Carriers"],"prefix":"10.1177","volume":"11","author":[{"given":"Rebecca K.","family":"Hendel","sequence":"first","affiliation":[{"name":"Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark"},{"name":"Department of Psychology, University of Copenhagen, Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie N.N.","family":"Hellem","sequence":"additional","affiliation":[{"name":"Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lena E.","family":"Hjermind","sequence":"additional","affiliation":[{"name":"Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f8rgen E.","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asmus","family":"Vogel","sequence":"additional","affiliation":[{"name":"Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark"},{"name":"Department of Psychology, University of Copenhagen, Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","reference":[{"issue":"6","key":"10.3233\/JHD-220540_ref1","doi-asserted-by":"crossref","first-page":"1557","DOI":"10.1111\/j.1467-6494.2006.00420.x","article-title":"Self-regulation and the problem of human autonomy: Does psychology need choice, self-determination, and will?","volume":"74","author":"Ryan","year":"2006","journal-title":"J Pers"},{"issue":"1","key":"10.3233\/JHD-220540_ref2","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1037\/0003-066X.55.1.68","article-title":"Self-determination theory and the facilitation of intrinsic motivation, social development, and well-being","volume":"55","author":"Ryan","year":"2000","journal-title":"Am Psychol"},{"issue":"3","key":"10.3233\/JHD-220540_ref3","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1111\/1467-6494.05006","article-title":"The meaning of autonomy: On and beyond the interpersonal circumplex","volume":"70","author":"Hmel","year":"2002","journal-title":"J Pers"},{"issue":"4","key":"10.3233\/JHD-220540_ref4","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1111\/papt.12083","article-title":"Anxiety, depression and autonomy\u2013connectedness: The mediating role of alexithymia and assertiveness","volume":"89","author":"Rutten","year":"2016","journal-title":"Psychol Psychother"},{"key":"10.3233\/JHD-220540_ref5","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1002\/da.20178","article-title":"The role of autonomy-connectedness in depression and anxiety","volume":"23","author":"Bekker","year":"2006","journal-title":"Depress Anxiety"},{"issue":"2","key":"10.3233\/JHD-220540_ref6","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1023\/A:1026491108540","article-title":"Sociotropy, autonomy, and the interpersonal model of depression: An integration","volume":"25","author":"Bieling","year":"2001","journal-title":"Cognit Ther Res"},{"issue":"1","key":"10.3233\/JHD-220540_ref7","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12991-021-00373-z","article-title":"Anaclitic-sociotropic and introjective-autonomic personality dimensions and depressive symptoms: A systematic review","volume":"20","author":"Marfoli","year":"2021","journal-title":"Ann Gen Psychiatry"},{"key":"10.3233\/JHD-220540_ref8","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","article-title":"Huntington\u2019s disease","volume":"369","author":"Walker","year":"2007","journal-title":"Lancet"},{"issue":"1","key":"10.3233\/JHD-220540_ref9","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1111\/ene.13413","article-title":"Huntington\u2019s disease: A clinical review","volume":"25","author":"McColgan","year":"2018","journal-title":"Eur J Neurol"},{"issue":"6","key":"10.3233\/JHD-220540_ref10","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/brainsci7060067","article-title":"Neuropsychiatric burden in huntington\u2019s disease","volume":"7","author":"Paoli","year":"2017","journal-title":"Brain Sci"},{"issue":"7","key":"10.3233\/JHD-220540_ref11","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1093\/arclin\/acx086","article-title":"The neuropsychology of Huntington\u2019s disease","volume":"32","author":"Snowden","year":"2017","journal-title":"Arch Clin Neuropsychol"},{"issue":"1","key":"10.3233\/JHD-220540_ref12","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.cpr.2009.10.003","article-title":"The impact of dementia on self and identity: A systematic review","volume":"30","author":"Caddell","year":"2010","journal-title":"Clin Psychol Rev"},{"key":"10.3233\/JHD-220540_ref13","doi-asserted-by":"crossref","unstructured":"Caddell LS , Clare L , How does identity relate to cognition and functional abilities in early-stage dementia? 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