Progression of ataxia in patients with Machado-Joseph disease.
Identifieur interne : 001C97 ( PubMed/Corpus ); précédent : 001C96; suivant : 001C98Progression of ataxia in patients with Machado-Joseph disease.
Auteurs : Marcondes C. França ; Anelyssa D'Abreu ; Anamarli Nucci ; Fernando Cendes ; Iscia Lopes-CendesSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2009.
English descriptors
- KwdEn :
- MESH :
- geographic : Brazil.
- complications : Machado-Joseph Disease.
- etiology : Ataxia.
- genetics : Machado-Joseph Disease, Trinucleotide Repeat Expansion.
- Adult, Aged, Disability Evaluation, Disease Progression, Female, Humans, Male, Middle Aged, Prospective Studies, Severity of Illness Index, Young Adult.
Abstract
Although ataxia is the most distressing manifestation of Machado-Joseph disease (MJD), little is known about its natural history. Therefore, we prospectively followed a cohort of patients with MJD for 13 months to characterize the progression of ataxia and identify its contributory factors. The international cooperative ataxia rating scale (ICARS) was used to estimate severity of ataxia at baseline and at follow-up. Thirty-four patients were enrolled in the study, 22 of whom were men. Mean age at onset of the disease was 34.7 years and length of expanded CAG repeat was 66. Mean ICARS scores at baseline was 37.6 and at follow-up was 42.7 (P < 0.001). Multivariate analysis did not find significant association of progression of disease and age at disease onset, length of expanded (CAG), or duration of disease.
DOI: 10.1002/mds.22627
PubMed: 19441132
Links to Exploration step
pubmed:19441132Le document en format XML
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<author><name sortKey="Franca, Marcondes C" sort="Franca, Marcondes C" uniqKey="Franca M" first="Marcondes C" last="França">Marcondes C. França</name>
<affiliation><nlm:affiliation>Department of Neurology, University of Campinas, São Paulo, Brazil.</nlm:affiliation>
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<author><name sortKey="D Abreu, Anelyssa" sort="D Abreu, Anelyssa" uniqKey="D Abreu A" first="Anelyssa" last="D'Abreu">Anelyssa D'Abreu</name>
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<author><name sortKey="Nucci, Anamarli" sort="Nucci, Anamarli" uniqKey="Nucci A" first="Anamarli" last="Nucci">Anamarli Nucci</name>
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<author><name sortKey="Cendes, Fernando" sort="Cendes, Fernando" uniqKey="Cendes F" first="Fernando" last="Cendes">Fernando Cendes</name>
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<author><name sortKey="Lopes Cendes, Iscia" sort="Lopes Cendes, Iscia" uniqKey="Lopes Cendes I" first="Iscia" last="Lopes-Cendes">Iscia Lopes-Cendes</name>
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<author><name sortKey="Lopes Cendes, Iscia" sort="Lopes Cendes, Iscia" uniqKey="Lopes Cendes I" first="Iscia" last="Lopes-Cendes">Iscia Lopes-Cendes</name>
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<term>Disability Evaluation</term>
<term>Disease Progression</term>
<term>Female</term>
<term>Humans</term>
<term>Machado-Joseph Disease (complications)</term>
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<term>Severity of Illness Index</term>
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<term>Female</term>
<term>Humans</term>
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<front><div type="abstract" xml:lang="en">Although ataxia is the most distressing manifestation of Machado-Joseph disease (MJD), little is known about its natural history. Therefore, we prospectively followed a cohort of patients with MJD for 13 months to characterize the progression of ataxia and identify its contributory factors. The international cooperative ataxia rating scale (ICARS) was used to estimate severity of ataxia at baseline and at follow-up. Thirty-four patients were enrolled in the study, 22 of whom were men. Mean age at onset of the disease was 34.7 years and length of expanded CAG repeat was 66. Mean ICARS scores at baseline was 37.6 and at follow-up was 42.7 (P < 0.001). Multivariate analysis did not find significant association of progression of disease and age at disease onset, length of expanded (CAG), or duration of disease.</div>
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<Abstract><AbstractText>Although ataxia is the most distressing manifestation of Machado-Joseph disease (MJD), little is known about its natural history. Therefore, we prospectively followed a cohort of patients with MJD for 13 months to characterize the progression of ataxia and identify its contributory factors. The international cooperative ataxia rating scale (ICARS) was used to estimate severity of ataxia at baseline and at follow-up. Thirty-four patients were enrolled in the study, 22 of whom were men. Mean age at onset of the disease was 34.7 years and length of expanded CAG repeat was 66. Mean ICARS scores at baseline was 37.6 and at follow-up was 42.7 (P < 0.001). Multivariate analysis did not find significant association of progression of disease and age at disease onset, length of expanded (CAG), or duration of disease.</AbstractText>
<CopyrightInformation>2009 Movement Disorder Society.</CopyrightInformation>
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