Annual change in Friedreich's ataxia evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is independent of disease severity.
Identifieur interne : 000F44 ( PubMed/Checkpoint ); précédent : 000F43; suivant : 000F45Annual change in Friedreich's ataxia evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is independent of disease severity.
Auteurs : Cecilia Marelli [France] ; Julie Figoni ; Perrine Charles ; Mathieu Anheim ; Maya Tchikviladze ; Carlo-Maria Vincitorio ; Sophie Tezenas Du Montcel ; Alexis Brice ; Jean Louis Golmard ; Alexandra DürrSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2012.
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- KwdEn :
- MESH :
Abstract
The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia.
DOI: 10.1002/mds.23879
PubMed: 22076850
Affiliations:
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<affiliation wicri:level="1"><nlm:affiliation>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere, UMR-S975, Paris, France.</nlm:affiliation>
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<author><name sortKey="Vincitorio, Carlo Maria" sort="Vincitorio, Carlo Maria" uniqKey="Vincitorio C" first="Carlo-Maria" last="Vincitorio">Carlo-Maria Vincitorio</name>
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<author><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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<author><name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
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<author><name sortKey="Golmard, Jean Louis" sort="Golmard, Jean Louis" uniqKey="Golmard J" first="Jean Louis" last="Golmard">Jean Louis Golmard</name>
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<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
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<term>Disability Evaluation</term>
<term>Female</term>
<term>Follow-Up Studies</term>
<term>Friedreich Ataxia (diagnosis)</term>
<term>Humans</term>
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<front><div type="abstract" xml:lang="en">The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia.</div>
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<Title>Movement disorders : official journal of the Movement Disorder Society</Title>
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<ArticleTitle>Annual change in Friedreich's ataxia evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is independent of disease severity.</ArticleTitle>
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<Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia.</AbstractText>
<AbstractText Label="METHODS" NlmCategory="METHODS">This was a follow-up study in adult patients with genetically confirmed Friedreich's ataxia evaluated at least twice (minimum interval, 6 months). Participants were outpatients at the Center for Neurogenetics of the Pitie-Salpêtrière Hospital in Paris.</AbstractText>
<AbstractText Label="RESULTS" NlmCategory="RESULTS">We included 84 patients; 60% had 3 or more evaluations. The mean score on first assessment was 22.7 ± 9, and the mean follow-up was 1.84 ± 1.10 years. The mean increase was 1.36 ± 2.3 points/year; this variation was not significantly linked to factors known to influence disease severity such as age at onset, disease duration, GAA expansion length, and wheelchair use.</AbstractText>
<AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">In adult Friedreich's ataxia patients the Scale for the Assessment and Rating of Ataxia can detect annual changes independently of disease severity. In future therapeutic trials no patient stratification is globally required.</AbstractText>
<CopyrightInformation>Copyright © 2011 Movement Disorder Society.</CopyrightInformation>
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