The unified Huntington's Disease Rating Scale for advanced patients: validation and follow-up study.
Identifieur interne : 000055 ( France/Analysis ); précédent : 000054; suivant : 000056The unified Huntington's Disease Rating Scale for advanced patients: validation and follow-up study.
Auteurs : Katia Youssov [France] ; Guillaume Dolbeau ; Patrick Maison ; Marie-Françoise Boissé ; Laurent Cleret De Langavant ; Raymund A C. Roos ; Anne-Catherine Bachoud-LéviSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2013.
English descriptors
- KwdEn :
- MESH :
- complications : Huntington Disease.
- diagnosis : Huntington Disease.
- psychology : Huntington Disease.
- Adult, Aged, Cross-Sectional Studies, Disability Evaluation, Female, Humans, Longitudinal Studies, Male, Middle Aged, Reproducibility of Results, Severity of Illness Index.
Abstract
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.
DOI: 10.1002/mds.25678
PubMed: 24123464
Affiliations:
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pubmed:24123464Le document en format XML
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Roos</name></author><author><name sortKey="Bachoud Levi, Anne Catherine" sort="Bachoud Levi, Anne Catherine" uniqKey="Bachoud Levi A" first="Anne-Catherine" last="Bachoud-Lévi">Anne-Catherine Bachoud-Lévi</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2013" type="published">2013</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Cross-Sectional Studies</term><term>Disability Evaluation</term><term>Female</term><term>Humans</term><term>Huntington Disease (complications)</term><term>Huntington Disease (diagnosis)</term><term>Huntington Disease (psychology)</term><term>Longitudinal Studies</term><term>Male</term><term>Middle Aged</term><term>Reproducibility of Results</term><term>Severity of Illness Index</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Cross-Sectional Studies</term><term>Disability Evaluation</term><term>Female</term><term>Humans</term><term>Longitudinal Studies</term><term>Male</term><term>Middle Aged</term><term>Reproducibility of Results</term><term>Severity of Illness Index</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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.</div></front></TEI><affiliations><list><country><li>France</li></country><settlement><li>Créteil</li></settlement></list><tree><noCountry><name sortKey="Bachoud Levi, Anne Catherine" sort="Bachoud Levi, Anne Catherine" uniqKey="Bachoud Levi A" first="Anne-Catherine" last="Bachoud-Lévi">Anne-Catherine Bachoud-Lévi</name><name sortKey="Boisse, Marie Francoise" sort="Boisse, Marie Francoise" uniqKey="Boisse M" first="Marie-Françoise" last="Boissé">Marie-Françoise Boissé</name><name sortKey="Cleret De Langavant, Laurent" sort="Cleret De Langavant, Laurent" uniqKey="Cleret De Langavant L" first="Laurent" last="Cleret De Langavant">Laurent Cleret De Langavant</name><name sortKey="Dolbeau, Guillaume" sort="Dolbeau, Guillaume" uniqKey="Dolbeau G" first="Guillaume" last="Dolbeau">Guillaume Dolbeau</name><name sortKey="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</name><name sortKey="Roos, Raymund A C" sort="Roos, Raymund A C" uniqKey="Roos R" first="Raymund A C" last="Roos">Raymund A C. Roos</name></noCountry><country name="France"><noRegion><name sortKey="Youssov, Katia" sort="Youssov, Katia" uniqKey="Youssov K" first="Katia" last="Youssov">Katia Youssov</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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