MovDisordV3, Main, Merge, bibRecord, 004C82

Characterization of nigrostriatal dysfunction in spinocerebellar ataxia 17

Identifieur interne : 004C82 ( Main/Merge ); précédent : 004C81; suivant : 004C83

Characterization of nigrostriatal dysfunction in spinocerebellar ataxia 17

Auteurs : Elena Salvatore [Italie] ; Andrea Varrone [Italie] ; Valeria Sansone [Italie] ; Maria Nolano [Italie] ; Amalia C. Bruni [Italie] ; Anna De Rosa [Italie] ; Lucio Santoro [Italie] ; Sabina Pappata [Italie] ; Alessandro Filla [Italie] ; Giuseppe De Michele [Italie]

Source :

Movement disorders [ 0885-3185 ] ; 2006.

RBID : Pascal:06-0352389

Descripteurs français

Pascal (Inist)
- Système nerveux pathologie, Trouble fonctionnel, Ataxie spinocérébelleuse, Tomoscintigraphie émission monophotonique, Photon, Voie nigrostriatale, Voie dopaminergique.

English descriptors

KwdEn :
- Dopaminergic pathway, Dysfunction, Nervous system diseases, Nigrostriatal pathway, Photon, Single photon emission tomography, Spinocerebellar ataxia.

Abstract

Extrapyramidal signs are a main feature of spinocerebellar ataxia 17 (SCA17). However, the extent of dopaminergic dysfunction and its correlation with parkinsonian signs are not fully understood. In order to define this, we investigated five subjects from three different families with a pathological CAG/CAA expansion in the TATA-binding protein gene (SCA17), ranging from asymptomatic carrier to patient with advanced disease, by FP-CIT SPECT. Nigrostriatal dysfunction was present in patients manifesting a fully developed phenotype but not in preclinical and early stages. Dopamine transporter reduction was symmetrical and uniform in caudate and putamen and it correlated with the clinical severity of ataxia.

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Pascal:06-0352389

Le document en format XML

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<series><title level="j" type="main">Movement disorders</title>
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<front><div type="abstract" xml:lang="en">Extrapyramidal signs are a main feature of spinocerebellar ataxia 17 (SCA17). However, the extent of dopaminergic dysfunction and its correlation with parkinsonian signs are not fully understood. In order to define this, we investigated five subjects from three different families with a pathological CAG/CAA expansion in the TATA-binding protein gene (SCA17), ranging from asymptomatic carrier to patient with advanced disease, by FP-CIT SPECT. Nigrostriatal dysfunction was present in patients manifesting a fully developed phenotype but not in preclinical and early stages. Dopamine transporter reduction was symmetrical and uniform in caudate and putamen and it correlated with the clinical severity of ataxia.</div>
</front>
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<affiliations><list><country><li>Italie</li>
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<tree><country name="Italie"><noRegion><name sortKey="Salvatore, Elena" sort="Salvatore, Elena" uniqKey="Salvatore E" first="Elena" last="Salvatore">Elena Salvatore</name>
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<name sortKey="Bruni, Amalia C" sort="Bruni, Amalia C" uniqKey="Bruni A" first="Amalia C." last="Bruni">Amalia C. Bruni</name>
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<name sortKey="Nolano, Maria" sort="Nolano, Maria" uniqKey="Nolano M" first="Maria" last="Nolano">Maria Nolano</name>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Characterization of nigrostriatal dysfunction in spinocerebellar ataxia 17

Characterization of nigrostriatal dysfunction in spinocerebellar ataxia 17

Source :

Descripteurs français

English descriptors

Abstract

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

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri