Movement Disorders (revue)

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Self-rated health status in spinocerebellar ataxia--results from a European multicenter study.

Identifieur interne : 002A36 ( Ncbi/Curation ); précédent : 002A35; suivant : 002A37

Self-rated health status in spinocerebellar ataxia--results from a European multicenter study.

Auteurs : Tanja Schmitz-Hübsch [Allemagne] ; Mathieu Coudert ; Paola Giunti ; Christoph Globas ; Laszlo Baliko ; Roberto Fancellu ; Caterina Mariotti ; Alessandro Filla ; Maryla Rakowicz ; Perrine Charles ; Pascale Ribai ; Sandra Szymanski ; Jon Infante ; Bart P C. Van De Warrenburg ; Alexandra Dürr ; Dagmar Timmann ; Sylvia Boesch ; Rafal Rola ; Chantal Depondt ; Ludger Schöls ; Elszbieta Zdzienicka ; Jun-Suk Kang ; Susanne Ratzka ; Berry Kremer ; Jörg B. Schulz ; Thomas Klopstock ; Bela Melegh ; Sophie Tezenas Du Montcel ; Thomas Klockgether

Source :

RBID : pubmed:20175183

English descriptors

Abstract

Patient-based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ-5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient-reported health status was compromised in patients of all genotypes (EQ-5D visual analogue scale (EQ-VAS) mean 61.45 +/- 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ-VAS variance in the whole sample and might be extrapolated to other SCA genotypes.

DOI: 10.1002/mds.22740
PubMed: 20175183

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<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
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<name sortKey="Ribai, Pascale" sort="Ribai, Pascale" uniqKey="Ribai P" first="Pascale" last="Ribai">Pascale Ribai</name>
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<name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name>
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<name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name>
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<name sortKey="Van De Warrenburg, Bart P C" sort="Van De Warrenburg, Bart P C" uniqKey="Van De Warrenburg B" first="Bart P C" last="Van De Warrenburg">Bart P C. Van De Warrenburg</name>
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<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
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<name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name>
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<name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
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<name sortKey="Rola, Rafal" sort="Rola, Rafal" uniqKey="Rola R" first="Rafal" last="Rola">Rafal Rola</name>
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<name sortKey="Depondt, Chantal" sort="Depondt, Chantal" uniqKey="Depondt C" first="Chantal" last="Depondt">Chantal Depondt</name>
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<name sortKey="Zdzienicka, Elszbieta" sort="Zdzienicka, Elszbieta" uniqKey="Zdzienicka E" first="Elszbieta" last="Zdzienicka">Elszbieta Zdzienicka</name>
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<name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
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<name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name>
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<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
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<term>Adult</term>
<term>Aged</term>
<term>Anxiety (diagnosis)</term>
<term>Anxiety (etiology)</term>
<term>Emotions (physiology)</term>
<term>Europe (epidemiology)</term>
<term>Female</term>
<term>Health Status</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neurologic Examination</term>
<term>Pain Measurement</term>
<term>Quality of Life</term>
<term>Questionnaires</term>
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<term>Spinocerebellar Ataxias (classification)</term>
<term>Spinocerebellar Ataxias (complications)</term>
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<term>Statistics, Nonparametric</term>
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<term>Europe</term>
</keywords>
<keywords scheme="MESH" qualifier="classification" xml:lang="en">
<term>Spinocerebellar Ataxias</term>
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<keywords scheme="MESH" qualifier="complications" xml:lang="en">
<term>Spinocerebellar Ataxias</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en">
<term>Anxiety</term>
<term>Spinocerebellar Ataxias</term>
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<term>Anxiety</term>
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<keywords scheme="MESH" qualifier="physiology" xml:lang="en">
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<term>Spinocerebellar Ataxias</term>
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<term>Adult</term>
<term>Aged</term>
<term>Female</term>
<term>Health Status</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neurologic Examination</term>
<term>Pain Measurement</term>
<term>Quality of Life</term>
<term>Questionnaires</term>
<term>Severity of Illness Index</term>
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<front>
<div type="abstract" xml:lang="en">Patient-based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ-5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient-reported health status was compromised in patients of all genotypes (EQ-5D visual analogue scale (EQ-VAS) mean 61.45 +/- 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ-VAS variance in the whole sample and might be extrapolated to other SCA genotypes.</div>
</front>
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