Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor : A unusual clinical benefit
Identifieur interne : 004035 ( Main/Merge ); précédent : 004034; suivant : 004036Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor : A unusual clinical benefit
Auteurs : Hans-Joachim Freund [Allemagne] ; Utako B. Barnikol [Allemagne] ; Dagmar Nolte [Allemagne] ; Harald Treuer [Allemagne] ; Georg Auburger [Allemagne] ; Peter A. Tass [Allemagne] ; Madjid Samii [Allemagne] ; Volker Sturm [Allemagne]Source :
- Movement disorders [ 0885-3185 ] ; 2007.
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Abstract
This is a single case report of a patient with spinocerebellar ataxia type 2 (SCA2) and severe tremor. Whereas disease progression with prevailing ataxia and dysmetria was slow over the first symptomatic 6 years, 6 months prior to operation were characterized by the development of a severe, debilitating postural tremor rendering the patient unable to independently sit, stand, speak, or swallow. Deep brain stimulation (DBS) at a subthalamic-thalamic electrode position almost completely arrested her tremor. The patient regained the functional state prior to her rapid disease progression allowing a restricted range of daily activities. Her condition has remained approximately stable over the two postoperative years to date. In addition to the efficacy of DBS on cerebellar tremor, the results illustrate a remarkable improvement of the patient's general condition and independence.
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor : A unusual clinical benefit</title><author><name sortKey="Freund, Hans Joachim" sort="Freund, Hans Joachim" uniqKey="Freund H" first="Hans-Joachim" last="Freund">Hans-Joachim Freund</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Institute of Neuroscience and Biophysics 3, Research Center Jülich</s1><s2>Julich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Julich</wicri:noRegion><wicri:noRegion>Research Center Jülich</wicri:noRegion><wicri:noRegion>Julich</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Virtual Institute of Neuromodulation, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>International Neuroscience Institute</s1><s2>Hannover</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation></author><author><name sortKey="Barnikol, Utako B" sort="Barnikol, Utako B" uniqKey="Barnikol U" first="Utako B." last="Barnikol">Utako B. 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when="2007">2007</date><idno type="stanalyst">PASCAL 07-0263046 INIST</idno><idno type="RBID">Pascal:07-0263046</idno><idno type="wicri:Area/PascalFrancis/Corpus">001711</idno><idno type="wicri:Area/PascalFrancis/Curation">001610</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">001445</idno><idno type="wicri:doubleKey">0885-3185:2007:Freund H:subthalamic:thalamic:dbs</idno><idno type="wicri:Area/Main/Merge">004035</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor : A unusual clinical benefit</title><author><name sortKey="Freund, Hans Joachim" sort="Freund, Hans Joachim" uniqKey="Freund H" first="Hans-Joachim" last="Freund">Hans-Joachim Freund</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Institute of Neuroscience and Biophysics 3, Research Center Jülich</s1><s2>Julich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Julich</wicri:noRegion><wicri:noRegion>Research Center Jülich</wicri:noRegion><wicri:noRegion>Julich</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Virtual Institute of Neuromodulation, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>International Neuroscience Institute</s1><s2>Hannover</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation></author><author><name sortKey="Barnikol, Utako B" sort="Barnikol, Utako B" uniqKey="Barnikol U" first="Utako B." last="Barnikol">Utako B. Barnikol</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Virtual Institute of Neuromodulation, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Stereotactic and Functional Neurosurgery, University of Cologne</s1><s2>Cologne</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Cologne</wicri:noRegion><wicri:noRegion>University of Cologne</wicri:noRegion><wicri:noRegion>Cologne</wicri:noRegion></affiliation></author><author><name sortKey="Nolte, Dagmar" sort="Nolte, Dagmar" uniqKey="Nolte D" first="Dagmar" last="Nolte">Dagmar Nolte</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Institute of Human Genetics, University of Giessen</s1><s2>Giessen</s2><s3>DEU</s3><sZ>3 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Giessen</wicri:noRegion><wicri:noRegion>University of Giessen</wicri:noRegion><wicri:noRegion>Giessen</wicri:noRegion></affiliation></author><author><name sortKey="Treuer, Harald" sort="Treuer, Harald" uniqKey="Treuer H" first="Harald" last="Treuer">Harald Treuer</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Stereotactic and Functional Neurosurgery, University of Cologne</s1><s2>Cologne</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Cologne</wicri:noRegion><wicri:noRegion>University of Cologne</wicri:noRegion><wicri:noRegion>Cologne</wicri:noRegion></affiliation></author><author><name sortKey="Auburger, Georg" sort="Auburger, Georg" uniqKey="Auburger G" first="Georg" last="Auburger">Georg Auburger</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Section of Molecular Neurogenetics, Department of Neurology, Johann Wolfgang Goethe University</s1><s2>Frankfurt/ Main</s2><s3>DEU</s3><sZ>5 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Frankfurt/ Main</wicri:noRegion><wicri:noRegion>Johann Wolfgang Goethe University</wicri:noRegion><wicri:noRegion>Frankfurt/ Main</wicri:noRegion></affiliation></author><author><name sortKey="Tass, Peter A" sort="Tass, Peter A" uniqKey="Tass P" first="Peter A." last="Tass">Peter A. Tass</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Institute of Neuroscience and Biophysics 3, Research Center Jülich</s1><s2>Julich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Julich</wicri:noRegion><wicri:noRegion>Research Center Jülich</wicri:noRegion><wicri:noRegion>Julich</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Virtual Institute of Neuromodulation, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Stereotactic and Functional Neurosurgery, University of Cologne</s1><s2>Cologne</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Cologne</wicri:noRegion><wicri:noRegion>University of Cologne</wicri:noRegion><wicri:noRegion>Cologne</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Brain Imaging Center West, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation></author><author><name sortKey="Samii, Madjid" sort="Samii, Madjid" uniqKey="Samii M" first="Madjid" last="Samii">Madjid Samii</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>International Neuroscience Institute</s1><s2>Hannover</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation></author><author><name sortKey="Sturm, Volker" sort="Sturm, Volker" uniqKey="Sturm V" first="Volker" last="Sturm">Volker Sturm</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Virtual Institute of Neuromodulation, Research Center Julich</s1><s2>Jülich</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Rhénanie-du-Nord-Westphalie</region><region type="district" nuts="2">District de Cologne</region><settlement type="city">Juliers</settlement></placeName></affiliation><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>International Neuroscience Institute</s1><s2>Hannover</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Basse-Saxe</region><settlement type="city">Hanovre</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Stereotactic and Functional Neurosurgery, University of Cologne</s1><s2>Cologne</s2><s3>DEU</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>6 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Cologne</wicri:noRegion><wicri:noRegion>University of Cologne</wicri:noRegion><wicri:noRegion>Cologne</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="08"><s1>Leibniz Institute for Neurobiology, Center for Learning and Memory Research</s1><s2>Magdeburg</s2><s3>DEU</s3><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Saxe-Anhalt</region><settlement type="city">Magdebourg</settlement></placeName></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2007">2007</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Cerebellum</term><term>Deep brain stimulation</term><term>Long lasting</term><term>Nervous system diseases</term><term>Spinocerebellar ataxia</term><term>Tremor</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Système nerveux pathologie</term><term>Tremblement</term><term>Ataxie spinocérébelleuse</term><term>Cervelet</term><term>Longue durée</term><term>Stimulation cérébrale profonde</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">This is a single case report of a patient with spinocerebellar ataxia type 2 (SCA2) and severe tremor. Whereas disease progression with prevailing ataxia and dysmetria was slow over the first symptomatic 6 years, 6 months prior to operation were characterized by the development of a severe, debilitating postural tremor rendering the patient unable to independently sit, stand, speak, or swallow. Deep brain stimulation (DBS) at a subthalamic-thalamic electrode position almost completely arrested her tremor. The patient regained the functional state prior to her rapid disease progression allowing a restricted range of daily activities. Her condition has remained approximately stable over the two postoperative years to date. In addition to the efficacy of DBS on cerebellar tremor, the results illustrate a remarkable improvement of the patient's general condition and independence.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>Basse-Saxe</li><li>District de Cologne</li><li>Rhénanie-du-Nord-Westphalie</li><li>Saxe-Anhalt</li></region><settlement><li>Hanovre</li><li>Juliers</li><li>Magdebourg</li></settlement></list><tree><country name="Allemagne"><noRegion><name sortKey="Freund, Hans Joachim" sort="Freund, Hans Joachim" uniqKey="Freund H" first="Hans-Joachim" last="Freund">Hans-Joachim Freund</name></noRegion><name sortKey="Auburger, Georg" sort="Auburger, Georg" uniqKey="Auburger G" first="Georg" last="Auburger">Georg Auburger</name><name sortKey="Barnikol, Utako B" sort="Barnikol, Utako B" uniqKey="Barnikol U" first="Utako B." last="Barnikol">Utako B. Barnikol</name><name sortKey="Barnikol, Utako B" sort="Barnikol, Utako B" uniqKey="Barnikol U" first="Utako B." last="Barnikol">Utako B. Barnikol</name><name sortKey="Freund, Hans Joachim" sort="Freund, Hans Joachim" uniqKey="Freund H" first="Hans-Joachim" last="Freund">Hans-Joachim Freund</name><name sortKey="Freund, Hans Joachim" sort="Freund, Hans Joachim" uniqKey="Freund H" first="Hans-Joachim" last="Freund">Hans-Joachim Freund</name><name sortKey="Nolte, Dagmar" sort="Nolte, Dagmar" uniqKey="Nolte D" first="Dagmar" last="Nolte">Dagmar Nolte</name><name sortKey="Samii, Madjid" sort="Samii, Madjid" uniqKey="Samii M" first="Madjid" last="Samii">Madjid Samii</name><name sortKey="Sturm, Volker" sort="Sturm, Volker" uniqKey="Sturm V" first="Volker" last="Sturm">Volker Sturm</name><name sortKey="Sturm, Volker" sort="Sturm, Volker" uniqKey="Sturm V" first="Volker" last="Sturm">Volker Sturm</name><name sortKey="Sturm, Volker" sort="Sturm, Volker" uniqKey="Sturm V" first="Volker" last="Sturm">Volker Sturm</name><name sortKey="Sturm, Volker" sort="Sturm, Volker" uniqKey="Sturm V" first="Volker" last="Sturm">Volker Sturm</name><name sortKey="Tass, Peter A" sort="Tass, Peter A" uniqKey="Tass P" first="Peter A." last="Tass">Peter A. Tass</name><name sortKey="Tass, Peter A" sort="Tass, Peter A" uniqKey="Tass P" first="Peter A." last="Tass">Peter A. Tass</name><name sortKey="Tass, Peter A" sort="Tass, Peter A" uniqKey="Tass P" first="Peter A." last="Tass">Peter A. Tass</name><name sortKey="Tass, Peter A" sort="Tass, Peter A" uniqKey="Tass P" first="Peter A." last="Tass">Peter A. Tass</name><name sortKey="Treuer, Harald" sort="Treuer, Harald" uniqKey="Treuer H" first="Harald" last="Treuer">Harald Treuer</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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