La maladie de Parkinson en France (serveur d'exploration)

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Unified Wilson's Disease Rating Scale - a proposal for the neurological scoring of Wilson's disease patients.

Identifieur interne : 000D79 ( PubMed/Curation ); précédent : 000D78; suivant : 000D80

Unified Wilson's Disease Rating Scale - a proposal for the neurological scoring of Wilson's disease patients.

Auteurs : Anna Członkowska [Pologne] ; Beata Tarnacka ; Jens Carsten Möller ; Barbara Leinweber ; Oliver Bandmann ; France Woimant ; Wolfgang H. Oertel

Source :

RBID : pubmed:17330175

English descriptors

Abstract

The clinical forms of Wilson's disease (WD) neurological manifestations can be divided into three movement disorder syndromes: a) dystonic, b) ataxic, c) parkinsonian syndrome. These syndromes in WD seldom occur in isolation. Clinical rating scales such as the Unified Parkinson;s Disease Rating Scale (UPDRS), the International Cooperative Ataxia Rating Scale (ICARS) and the Rating Scale for Dystonia (RSD), focusing on either parkinsonism or ataxia or dystonia alone, are not sufficient to reflect accurately the motor impairment of WD patients. The aim of the study was to develop a novel rating scale for WD, because as far as we know no scale for the clinical rating in WD has been designed before.

PubMed: 17330175

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pubmed:17330175

Le document en format XML

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<title xml:lang="en">Unified Wilson's Disease Rating Scale - a proposal for the neurological scoring of Wilson's disease patients.</title>
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<name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Członkowska">Anna Członkowska</name>
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<nlm:affiliation>2nd Neurological Department, Institute of Psychiatry and Neurology, Warsaw, Poland. czlonkow@ipin.edu.pl</nlm:affiliation>
<country xml:lang="fr">Pologne</country>
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<name sortKey="Tarnacka, Beata" sort="Tarnacka, Beata" uniqKey="Tarnacka B" first="Beata" last="Tarnacka">Beata Tarnacka</name>
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<name sortKey="Moller, Jens Carsten" sort="Moller, Jens Carsten" uniqKey="Moller J" first="Jens Carsten" last="Möller">Jens Carsten Möller</name>
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<name sortKey="Leinweber, Barbara" sort="Leinweber, Barbara" uniqKey="Leinweber B" first="Barbara" last="Leinweber">Barbara Leinweber</name>
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<name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name>
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<name sortKey="Woimant, France" sort="Woimant, France" uniqKey="Woimant F" first="France" last="Woimant">France Woimant</name>
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<name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name>
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<term>Hepatolenticular Degeneration (diagnosis)</term>
<term>Humans</term>
<term>Male</term>
<term>Neurologic Examination</term>
<term>Reproducibility of Results</term>
<term>Severity of Illness Index</term>
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<div type="abstract" xml:lang="en">The clinical forms of Wilson's disease (WD) neurological manifestations can be divided into three movement disorder syndromes: a) dystonic, b) ataxic, c) parkinsonian syndrome. These syndromes in WD seldom occur in isolation. Clinical rating scales such as the Unified Parkinson;s Disease Rating Scale (UPDRS), the International Cooperative Ataxia Rating Scale (ICARS) and the Rating Scale for Dystonia (RSD), focusing on either parkinsonism or ataxia or dystonia alone, are not sufficient to reflect accurately the motor impairment of WD patients. The aim of the study was to develop a novel rating scale for WD, because as far as we know no scale for the clinical rating in WD has been designed before.</div>
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<Title>Neurologia i neurochirurgia polska</Title>
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<ArticleTitle>Unified Wilson's Disease Rating Scale - a proposal for the neurological scoring of Wilson's disease patients.</ArticleTitle>
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<AbstractText Label="BACKGROUND AND PURPOSE" NlmCategory="OBJECTIVE">The clinical forms of Wilson's disease (WD) neurological manifestations can be divided into three movement disorder syndromes: a) dystonic, b) ataxic, c) parkinsonian syndrome. These syndromes in WD seldom occur in isolation. Clinical rating scales such as the Unified Parkinson;s Disease Rating Scale (UPDRS), the International Cooperative Ataxia Rating Scale (ICARS) and the Rating Scale for Dystonia (RSD), focusing on either parkinsonism or ataxia or dystonia alone, are not sufficient to reflect accurately the motor impairment of WD patients. The aim of the study was to develop a novel rating scale for WD, because as far as we know no scale for the clinical rating in WD has been designed before.</AbstractText>
<AbstractText Label="MATERIAL AND METHODS" NlmCategory="METHODS">In 2004 the EuroWilson consortium was founded, to create a European WD database. Members of the consortium from Poland, Germany, and France prepared a new scale using clinical rating scales as the UPDRS, ICARS, and RSD. Prepared drafts were discussed several times in detail at the first international neurological EuroWilson meeting in September 2004 in Paris and in November in Warsaw.</AbstractText>
<AbstractText Label="RESULTS AND CONCLUSIONS" NlmCategory="CONCLUSIONS">The novel scale for WD consists of 3 parts, including: consciousness, a historical review based on the Barthel scale (2-11 items), and neurological examination (12-35, items). The maximum score for the first part is 3, for the second 39 points, and for the last 143 points. The initial reliability of the scale on the basis of 6 patients (on DVD) and 8 investigators was assessed. Inter-rater agreement was high. Now the scale is used by the EuroWilson and GeNeMove consortia.</AbstractText>
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