Funding agencies: This work was supported by the National Reference Center for Huntington's Disease (Ministry of Health, France) and by a Contrat d'Interface INSERM (A.-C.B.-L.). Additional support was provided through the NeurATRIS (European Advanced Translational Research Infrastructure in Neurosciences) Project, the Groupement d'Intérêt Scientifique (GIS)-Institut des Maladies Rares, and the “Biomarker Program” AP-HP.
The unified huntington's disease rating scale for advanced patients: Validation and follow-up study
Article first published online: 9 OCT 2013
© 2013 Movement Disorder Society
Volume 28, Issue 14, pages 1995–2001, December 2013
How to Cite
Youssov, K., Dolbeau, G., Maison, P., Boissé, M.-F., de Langavant, L. C., Roos, R. A. C. and Bachoud-Lévi, A.-C. (2013), The unified huntington's disease rating scale for advanced patients: Validation and follow-up study. Mov. Disord., 28: 1995–2001. doi: 10.1002/mds.25678
Relevant conflicts of interest/financial disclosures: Nothing to report.
Full financial disclosures and author roles may be found in the online version of this article.
- Issue published online: 9 DEC 2013
- Article first published online: 9 OCT 2013
- Manuscript Accepted: 16 AUG 2013
- Manuscript Received: 13 JUL 2013
- Huntington's disease;
- outcome research;
- neuropsychological assessment
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-For Advanced Patients (UHDRS-FAP) to improve longitudinal assessment of patients at the advanced disease stage. Sixty-nine patients with a Total Functional Capacity score ≤ 5 were recruited in France and the Netherlands. Among them, 45 patients were followed longitudinally (mean ± standard deviation, 1.6 ± 1.2 years) with the UHDRS-FAP; 30 patients also were assessed with the UHDRS. In cross-sectional analyses, the psychometric properties and inter-rater reliability of the scale were evaluated. Longitudinal analyses were used to evaluate the sensitivity to decline of the UHDRS-FAP 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-rater reliability was ≥ 0.88 for all scores. The somatic score, which was specific to the UHDRS-FAP, declined over time along with motor and cognitive performance on both scales. Although performance with the two scales was correlated, the UHDRS-FAP appeared to be more sensitive to change and was the only scale that detected decline in patients with a Total Functional Capacity score ≤ 1. Neither scale detected a significant decline in behavioral scores. The results indicate that the UHDRS-FAP 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. © 2013 International Parkinson and Movement Disorder Society