{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:55:58Z","timestamp":1791168958504,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2013,10,9]],"date-time":"2013-10-09T00:00:00Z","timestamp":1381276800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>The Unified Huntington's Disease Rating Scale (UHDRS) adequately measures decline in patients at early and moderate stages of Huntington's disease (HD). In patients with advanced HD, floor effects hamper the evaluation, thus calling for an adjusted scale. We designed the UHDRS\u2010For Advanced Patients (UHDRS\u2010FAP) to improve longitudinal assessment of patients at the advanced disease stage. Sixty\u2010nine patients with a Total Functional Capacity score\u2009\u2264\u20095 were recruited in France and the Netherlands. Among them, 45 patients were followed longitudinally (mean\u2009\u00b1\u2009standard deviation, 1.6\u2009\u00b1\u20091.2 years) with the UHDRS\u2010FAP; 30 patients also were assessed with the UHDRS. In cross\u2010sectional analyses, the psychometric properties and inter\u2010rater reliability of the scale were evaluated. Longitudinal analyses were used to evaluate the sensitivity to decline of the UHDRS\u2010FAP compared with the UHDRS. Internal consistency was higher for motor (0.84) and cognitive (0.91) scores than for somatic (0.70) and behavioral (0.49) scores. Inter\u2010rater reliability was\u2009\u2265\u20090.88 for all scores. The somatic score, which was specific to the UHDRS\u2010FAP, declined over time along with motor and cognitive performance on both scales. Although performance with the two scales was correlated, the UHDRS\u2010FAP appeared to be more sensitive to change and was the only scale that detected decline in patients with a Total Functional Capacity score\u2009\u2264\u20091. Neither scale detected a significant decline in behavioral scores. The results indicate that the UHDRS\u2010FAP is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains. It offers items that are relevant for daily care. Behavioral scores tended to decline, but this may reflect the decline in patients' communicative abilities. \u00a9 2013 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.25678","type":"journal-article","created":{"date-parts":[[2013,10,11]],"date-time":"2013-10-11T21:59:26Z","timestamp":1381528766000},"page":"1995-2001","source":"Crossref","is-referenced-by-count":14,"title":["The unified huntington's disease rating scale for advanced patients: Validation and follow\u2010up study"],"prefix":"10.1002","volume":"28","author":[{"given":"Katia","family":"Youssov","sequence":"first","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence Maladie de Huntington Assistance Publique H\u00f4pitaux de Paris (AP\u2010HP), Groupe Hospitalier Henri Mondor\u2010Albert Chenevier Cr\u00e9teil France"},{"name":"Unit\u00e9 INSERM U955 Equipe 01 Neuropsychologie Interventionnelle Cr\u00e9teil France"},{"name":"Institut d'Etudes Cognitives Ecole Normale Sup\u00e9rieure Paris France"},{"name":"Facult\u00e9 de M\u00e9decine Universit\u00e9 Paris Est Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Dolbeau","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence Maladie de Huntington Assistance Publique H\u00f4pitaux de Paris (AP\u2010HP), Groupe Hospitalier Henri Mondor\u2010Albert Chenevier Cr\u00e9teil France"},{"name":"Unit\u00e9 INSERM U955 Equipe 01 Neuropsychologie Interventionnelle Cr\u00e9teil France"},{"name":"Facult\u00e9 de M\u00e9decine Universit\u00e9 Paris Est Cr\u00e9teil France"},{"name":"Unit\u00e9 de Recherche Clinique AP\u2010HP, H\u00f4pital Henri Mondor Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Maison","sequence":"additional","affiliation":[{"name":"Unit\u00e9 INSERM U955 Equipe 01 Neuropsychologie Interventionnelle Cr\u00e9teil France"},{"name":"Institut d'Etudes Cognitives Ecole Normale Sup\u00e9rieure Paris France"},{"name":"Facult\u00e9 de M\u00e9decine Universit\u00e9 Paris Est Cr\u00e9teil France"},{"name":"Pharmacologie Clinique AP\u2010HP, H\u00f4pital Henri Mondor Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Fran\u00e7oise","family":"Boiss\u00e9","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence Maladie de Huntington Assistance Publique H\u00f4pitaux de Paris (AP\u2010HP), Groupe Hospitalier Henri Mondor\u2010Albert Chenevier Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent Cleret","family":"de Langavant","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence Maladie de Huntington Assistance Publique H\u00f4pitaux de Paris (AP\u2010HP), Groupe Hospitalier Henri Mondor\u2010Albert Chenevier Cr\u00e9teil France"},{"name":"Unit\u00e9 INSERM U955 Equipe 01 Neuropsychologie Interventionnelle Cr\u00e9teil France"},{"name":"Institut d'Etudes Cognitives Ecole Normale Sup\u00e9rieure Paris France"},{"name":"Facult\u00e9 de M\u00e9decine Universit\u00e9 Paris Est Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymund A. 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