Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures
Identifieur interne : 001160 ( Main/Exploration ); précédent : 001159; suivant : 001161Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures
Auteurs : Martin Südmeyer [Allemagne] ; Andreas Saleh [Allemagne] ; Lars Wojtecki [Allemagne] ; Mathias Cohnen [Allemagne] ; Joachim Gross [Allemagne] ; Markus Ploner [Allemagne] ; Harald Hefter [Allemagne] ; Lars Timmermann [Allemagne] ; Alfons Schnitzler [Allemagne]Source :
- Movement Disorders [ 0885-3185 ] ; 2006-12.
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Abstract
Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long‐term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS‐III). MRI findings in symptomatic WD patients revealed significant symmetric T2*‐weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra. © 2006 Movement Disorder Society
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DOI: 10.1002/mds.21136
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2006-12">2006-12</date><biblScope unit="volume">21</biblScope><biblScope unit="issue">12</biblScope><biblScope unit="page" from="2134">2134</biblScope><biblScope unit="page" to="2139">2139</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">7A3B8958CC1316D338BB01661291F3EF2BC24C98</idno><idno type="DOI">10.1002/mds.21136</idno><idno type="ArticleID">MDS21136</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>MRI</term><term>Wilson's disease</term><term>basal‐ganglia</term><term>tremor</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long‐term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS‐III). MRI findings in symptomatic WD patients revealed significant symmetric T2*‐weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra. © 2006 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Allemagne</li></country></list><tree><country name="Allemagne"><noRegion><name sortKey="Sudmeyer, Martin" sort="Sudmeyer, Martin" uniqKey="Sudmeyer M" first="Martin" last="Südmeyer">Martin Südmeyer</name></noRegion><name sortKey="Cohnen, Mathias" sort="Cohnen, Mathias" uniqKey="Cohnen M" first="Mathias" last="Cohnen">Mathias Cohnen</name><name sortKey="Gross, Joachim" sort="Gross, Joachim" uniqKey="Gross J" first="Joachim" last="Gross">Joachim Gross</name><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name><name sortKey="Ploner, Markus" sort="Ploner, Markus" uniqKey="Ploner M" first="Markus" last="Ploner">Markus Ploner</name><name sortKey="Saleh, Andreas" sort="Saleh, Andreas" uniqKey="Saleh A" first="Andreas" last="Saleh">Andreas Saleh</name><name sortKey="Schnitzler, Alfons" sort="Schnitzler, Alfons" uniqKey="Schnitzler A" first="Alfons" last="Schnitzler">Alfons Schnitzler</name><name sortKey="Timmermann, Lars" sort="Timmermann, Lars" uniqKey="Timmermann L" first="Lars" last="Timmermann">Lars Timmermann</name><name sortKey="Wojtecki, Lars" sort="Wojtecki, Lars" uniqKey="Wojtecki L" first="Lars" last="Wojtecki">Lars Wojtecki</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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