{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T13:11:30Z","timestamp":1760015490650,"version":"3.40.5"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2019,1,27]],"date-time":"2019-01-27T00:00:00Z","timestamp":1548547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2019,5]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Symptoms and signs in patients with Huntington's disease are usually assessed with the Unified Huntington's Disease Rating Scale (<jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>). Ceiling and floor effects hamper the measurement of disease progression in patients with late stage Huntington's disease and therefore the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>\u2013For Advanced Patients (<jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">FAP<\/jats:styled-content>) has been developed. The aim of this longitudinal study was to examine if the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">FAP<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content> are sensitive enough to detect change over time in late stage Huntington's disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Forty nursing home residents and patients receiving day\u2010care were assessed with the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> UHDRS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">FAP<\/jats:styled-content> and Care Dependency Scale (<jats:styled-content style=\"fixed-case\">CDS<\/jats:styled-content>). After 6\u00a0months, the assessment scales were completed again in 29 patients. Changes between baseline and follow\u2010up were calculated using paired <jats:italic>t<\/jats:italic> tests. Wilcoxon signed\u2010rank tests were used to calculate longitudinal changes for middle and late stage patients separately.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The motor and cognitive score of the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">FAP<\/jats:styled-content> deteriorated during 6\u00a0months\u2019 follow\u2010up, whilst the motor and cognitive score of the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content> did not show change. Two functional domains of the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content> and the <jats:styled-content style=\"fixed-case\">CDS<\/jats:styled-content> also declined. The behavioral score significantly improved with both rating scales in late stage patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our results suggest that the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">FAP<\/jats:styled-content> motor and cognitive score, the functional domains of the <jats:styled-content style=\"fixed-case\">UHDRS<\/jats:styled-content>, and the <jats:styled-content style=\"fixed-case\">CDS<\/jats:styled-content> can detect disease progression in late stage Huntington's disease. Therefore, the use of these scores in nursing homes is recommended to optimize care by monitoring disease progression and by evaluating the effect of interventions in clinical care. Psychiatric symptoms seem to fade away as the disease progresses.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13889","type":"journal-article","created":{"date-parts":[[2018,12,21]],"date-time":"2018-12-21T21:48:08Z","timestamp":1545428888000},"page":"780-785","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Longitudinal assessment of the Unified Huntington's Disease Rating Scale (UHDRS) and UHDRS\u2013For Advanced Patients (UHDRS\u2010FAP) in patients with late stage Huntington's disease"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4637-086X","authenticated-orcid":false,"given":"J. Y.","family":"Winder","sequence":"first","affiliation":[{"name":"Department of Neurology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W. 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