Longitudinal assessment of the Unified Huntington's Disease Rating Scale (UHDRS) and UHDRS–For Advanced Patients (UHDRS‐FAP) in patients with late stage Huntington's disease

• Published in: European journal of neurology Vol. 26; no. 5; pp. 780 - 785
• Main Authors: Winder, J. Y., Achterberg, W. P., Gardiner, S. L., Roos, R. A. C.
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01-05-2019
• Subjects: advanced stage; Change detection; Cognitive ability; Correlation analysis; Dependence; Domains; Huntington's disease; Huntingtons disease; longitudinal analysis; long‐term care facility; Mathematical analysis; Motors; Nursing homes; Patients; Rank tests; rating scales; Signs and symptoms; longitudinal analysis; advanced stage; rating scales; long-term care facility; Huntington's disease
• ISSN: 1351-5101; 1468-1331
• DOI: 10.1111/ene.13889

Background and purpose
Symptoms and signs in patients with Huntington's disease are usually assessed with the Unified Huntington's Disease Rating Scale (UHDRS). Ceiling and floor effects hamper the measurement of disease progression in patients with late stage Huntington's disease and therefore the UHDRS–For Advanced Patients (UHDRS‐FAP) has been developed. The aim of this longitudinal study was to examine if the UHDRS‐FAP and UHDRS are sensitive enough to detect change over time in late stage Huntington's disease.
Methods
Forty nursing home residents and patients receiving day‐care were assessed with the UHDRS, UHDRS‐FAP and Care Dependency Scale (CDS). After 6 months, the assessment scales were completed again in 29 patients. Changes between baseline and follow‐up were calculated using paired t tests. Wilcoxon signed‐rank tests were used to calculate longitudinal changes for middle and late stage patients separately.
Results
The motor and cognitive score of the UHDRS‐FAP deteriorated during 6 months’ follow‐up, whilst the motor and cognitive score of the UHDRS did not show change. Two functional domains of the UHDRS and the CDS also declined. The behavioral score significantly improved with both rating scales in late stage patients.
Conclusions
Our results suggest that the UHDRS‐FAP motor and cognitive score, the functional domains of the UHDRS, and the CDS 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.