MovDisordV3, Main, Exploration, bibRecord, 002501

Progression of Ataxia in Patients with Machado-Joseph Disease

Identifieur interne : 002501 ( Main/Exploration ); précédent : 002500; suivant : 002502

Progression of Ataxia in Patients with Machado-Joseph Disease

Auteurs : Marcondes C. Jr Franca [Brésil] ; Anelyssa D'Abreu [Brésil] ; Anamarli Nucci [Brésil] ; Fernando Cendes [Brésil] ; Iscia Lopes-Cendes [Brésil]

Source :

Movement disorders [ 0885-3185 ] ; 2009.

RBID : Pascal:09-0349976

Descripteurs français

Pascal (Inist)
- Maladie de Joseph, Pathologie du système nerveux, Ataxie spinocérébelleuse, Homme.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Human, Joseph disease, Nervous system diseases, Spinocerebellar ataxia.

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.

Affiliations:

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Le document en format XML

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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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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Progression of Ataxia in Patients with Machado-Joseph Disease</title>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Human</term>
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<term>Nervous system diseases</term>
<term>Spinocerebellar ataxia</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Maladie de Joseph</term>
<term>Pathologie du   système nerveux</term>
<term>Ataxie spinocérébelleuse</term>
<term>Homme</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>
</front>
</TEI>
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<name sortKey="Nucci, Anamarli" sort="Nucci, Anamarli" uniqKey="Nucci A" first="Anamarli" last="Nucci">Anamarli Nucci</name>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Progression of Ataxia in Patients with Machado-Joseph Disease

Progression of Ataxia in Patients with Machado-Joseph Disease

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri