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
© 2018 EAN..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:26 |
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| Enthalten in: |
European journal of neurology - 26(2019), 5 vom: 27. Mai, Seite 780-785 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Winder, J Y [VerfasserIn] |
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| Links: |
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| Themen: |
Advanced stage |
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| Anmerkungen: |
Date Completed 28.07.2020 Date Revised 28.07.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/ene.13889 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM292054696 |
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| 100 | 1 | |a Winder, J Y |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a 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 |
| 264 | 1 | |c 2019 | |
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| 500 | |a Date Completed 28.07.2020 | ||
| 500 | |a Date Revised 28.07.2020 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2018 EAN. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Validation Study | |
| 650 | 4 | |a Huntington's disease | |
| 650 | 4 | |a advanced stage | |
| 650 | 4 | |a long-term care facility | |
| 650 | 4 | |a longitudinal analysis | |
| 650 | 4 | |a rating scales | |
| 700 | 1 | |a Achterberg, W P |e verfasserin |4 aut | |
| 700 | 1 | |a Gardiner, S L |e verfasserin |4 aut | |
| 700 | 1 | |a Roos, R A C |e verfasserin |4 aut | |
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