ArticleMovement disorders clinical practice
Assessment Scales for Patients with Advanced Huntington's Disease: Comparison of the UHDRS and UHDRS-FAP.
Article in Movement disorders clinical practice. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- The clinical phenotype of carriers of intermediate alleles in the huntingtin gene: A scoping review.Journal of Huntington's disease · 2026Review
- Specific contribution of cognitive and motor impairments with functional capacity and dependence in Huntington's disease.Journal of neurology · 2025Article
- Article
- The effect of Huntington's disease on cognitive and physical motivation.Brain : a journal of neurology · 2024Article
- Predicting clinical scores in Huntington's disease: a lightweight speech test.Journal of neurology · 2022Article
- Psychometric Properties and Validation of the Polish Version of the 12-Item World Health Organization Disability Assessment Schedule 2.0 in Patients with Huntington's Disease.Journal of clinical medicine · 2021Article
- Influence of Age of Onset on Huntington's Disease Phenotype.Tremor and other hyperkinetic movements (New York, N.Y.) · 2020Observational
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Abstract
backgroundThe standard clinical assessment tool in Huntington's disease is the Unified Huntington's Disease Rating Scale (UHDRS). In patients with advanced Huntington's disease ceiling and floor effects of the UHDRS hamper the detection of changes. Therefore, the UHDRS-For Advanced Patients (UHDRS-FAP) has been designed for patients with late-stage Huntington's disease.
objectivesThis cross-sectional study aims to examine if the UHDRS-FAP can differentiate better between patients with advanced Huntington's disease than the UHDRS.
methodsForty patients, who were institutionalized or received day-care, were assessed with the UHDRS, UHDRS-FAP, and Care Dependency Scale (CDS). The severity of Huntington's disease was defined by the Total Functional Capacity (TFC). Comparisons between consecutive TFC stages were performed for all domains of the UHDRS, UHDRS-FAP, and CDS using Mann-Whitney U tests.
resultsThe motor scores of the UHDRS-FAP and UHDRS were the only subscales with significantly worse scores in TFC stage 5 compared to stage 4. In TFC stages 4-5, the range of the UHDRS-FAP motor score was broader, the standard error of measurement was lower, and the effect size r was higher than for the UHDRS motor score. The CDS declined significantly across all TFC stages.
conclusionsOur results suggest that the UHDRS-FAP motor score might differentiate better between patients with severe Huntington's disease than the UHDRS motor score. Therefore, the UHDRS-FAP motor score is potentially a better instrument than the UHDRS motor score to improve disease monitoring and, subsequently, care in patients with advanced Huntington's disease in long-term care facilities.
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