Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)
Identifieur interne : 001F52 ( PascalFrancis/Curation ); précédent : 001F51; suivant : 001F53Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)
Auteurs : Katrin Bürk [Allemagne] ; Ulrike M Lzig [Allemagne] ; Stefanie Wolf [Allemagne] ; Suzette Heck [Allemagne] ; Konstantinos Dimitriadis [Allemagne] ; Tanja Sehmitz-Hübseh [Allemagne] ; Sascha Hering [Autriche] ; Tobias M. Lindig [Allemagne] ; Verena Haug [Allemagne] ; Dagmar Timmann [Allemagne] ; Ingrid Degen [Allemagne] ; Bernd Kruse [Allemagne] ; Jan-Markus Dörr [Allemagne] ; Susanne Ratzka [Allemagne] ; Anja Ivo [Allemagne] ; Ludger Schöls [Allemagne] ; Sylvia Boesch [Autriche] ; Thomas Klockgether [Allemagne] ; Thomas Klopstock [Allemagne] ; Jörg B. Schulz [Allemagne]Source :
- Movement disorders [ 0885-3185 ] ; 2009.
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- Pascal (Inist)
English descriptors
Abstract
To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure, SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited for clinical trials of FRDA.
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)</title><author><name sortKey="Burk, Katrin" sort="Burk, Katrin" uniqKey="Burk K" first="Katrin" last="Bürk">Katrin Bürk</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Neurology, University of Marburg</s1><s2>Marburg</s2><s3>DEU</s3><sZ>1 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="M Lzig, Ulrike" sort="M Lzig, Ulrike" uniqKey="M Lzig U" first="Ulrike" last="M Lzig">Ulrike M Lzig</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Wolf, Stefanie" sort="Wolf, Stefanie" uniqKey="Wolf S" first="Stefanie" last="Wolf">Stefanie Wolf</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Heck, Suzette" sort="Heck, Suzette" uniqKey="Heck S" first="Suzette" last="Heck">Suzette Heck</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Dimitriadis, Konstantinos" sort="Dimitriadis, Konstantinos" uniqKey="Dimitriadis K" first="Konstantinos" last="Dimitriadis">Konstantinos Dimitriadis</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Sehmitz Hubseh, Tanja" sort="Sehmitz Hubseh, Tanja" uniqKey="Sehmitz Hubseh T" first="Tanja" last="Sehmitz-Hübseh">Tanja Sehmitz-Hübseh</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Hering, Sascha" sort="Hering, Sascha" uniqKey="Hering S" first="Sascha" last="Hering">Sascha Hering</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, University of Innsbruck</s1><s2>Innsbruck</s2><s3>AUT</s3><sZ>7 aut.</sZ><sZ>17 aut.</sZ></inist:fA14><country>Autriche</country></affiliation></author><author><name sortKey="Lindig, Tobias M" sort="Lindig, Tobias M" uniqKey="Lindig T" first="Tobias M." last="Lindig">Tobias M. Lindig</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Neurodegeneration and Hertie- Institute for Clinical Brain Research, University of Tübingen</s1><s2>Tübingen</s2><s3>DEU</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Haug, Verena" sort="Haug, Verena" uniqKey="Haug V" first="Verena" last="Haug">Verena Haug</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Neuropediatrics, University of Freiburg</s1><s2>Freiburg</s2><s3>DEU</s3><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Neurology, University of Essen</s1><s2>Essen</s2><s3>DEU</s3><sZ>10 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Degen, Ingrid" sort="Degen, Ingrid" uniqKey="Degen I" first="Ingrid" last="Degen">Ingrid Degen</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Department of Neuropediatrics, Sankt Elisabeth Krankenhaus</s1><s2>Neuwied</s2><s3>DEU</s3><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Kruse, Bernd" sort="Kruse, Bernd" uniqKey="Kruse B" first="Bernd" last="Kruse">Bernd Kruse</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Department of Neuropediatrics, University of Hamburg</s1><s2>Hamburg</s2><s3>DEU</s3><sZ>12 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Dorr, Jan Markus" sort="Dorr, Jan Markus" uniqKey="Dorr J" first="Jan-Markus" last="Dörr">Jan-Markus Dörr</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of Neurology, Helios-Klinikum</s1><s2>Berlin</s2><s3>DEU</s3><sZ>13 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Ivo, Anja" sort="Ivo, Anja" uniqKey="Ivo A" first="Anja" last="Ivo">Anja Ivo</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Neurodegeneration and Hertie- Institute for Clinical Brain Research, University of Tübingen</s1><s2>Tübingen</s2><s3>DEU</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, University of Innsbruck</s1><s2>Innsbruck</s2><s3>AUT</s3><sZ>7 aut.</sZ><sZ>17 aut.</sZ></inist:fA14><country>Autriche</country></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Schulz, Jorg B" sort="Schulz, Jorg B" uniqKey="Schulz J" first="Jörg B." last="Schulz">Jörg B. Schulz</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">09-0431487</idno><date when="2009">2009</date><idno type="stanalyst">PASCAL 09-0431487 INIST</idno><idno type="RBID">Pascal:09-0431487</idno><idno type="wicri:Area/PascalFrancis/Corpus">000D67</idno><idno type="wicri:Area/PascalFrancis/Curation">001F52</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)</title><author><name sortKey="Burk, Katrin" sort="Burk, Katrin" uniqKey="Burk K" first="Katrin" last="Bürk">Katrin Bürk</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Neurology, University of Marburg</s1><s2>Marburg</s2><s3>DEU</s3><sZ>1 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="M Lzig, Ulrike" sort="M Lzig, Ulrike" uniqKey="M Lzig U" first="Ulrike" last="M Lzig">Ulrike M Lzig</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Wolf, Stefanie" sort="Wolf, Stefanie" uniqKey="Wolf S" first="Stefanie" last="Wolf">Stefanie Wolf</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Heck, Suzette" sort="Heck, Suzette" uniqKey="Heck S" first="Suzette" last="Heck">Suzette Heck</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Dimitriadis, Konstantinos" sort="Dimitriadis, Konstantinos" uniqKey="Dimitriadis K" first="Konstantinos" last="Dimitriadis">Konstantinos Dimitriadis</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Sehmitz Hubseh, Tanja" sort="Sehmitz Hubseh, Tanja" uniqKey="Sehmitz Hubseh T" first="Tanja" last="Sehmitz-Hübseh">Tanja Sehmitz-Hübseh</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Hering, Sascha" sort="Hering, Sascha" uniqKey="Hering S" first="Sascha" last="Hering">Sascha Hering</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, University of Innsbruck</s1><s2>Innsbruck</s2><s3>AUT</s3><sZ>7 aut.</sZ><sZ>17 aut.</sZ></inist:fA14><country>Autriche</country></affiliation></author><author><name sortKey="Lindig, Tobias M" sort="Lindig, Tobias M" uniqKey="Lindig T" first="Tobias M." last="Lindig">Tobias M. Lindig</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Neurodegeneration and Hertie- Institute for Clinical Brain Research, University of Tübingen</s1><s2>Tübingen</s2><s3>DEU</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Haug, Verena" sort="Haug, Verena" uniqKey="Haug V" first="Verena" last="Haug">Verena Haug</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Neuropediatrics, University of Freiburg</s1><s2>Freiburg</s2><s3>DEU</s3><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Neurology, University of Essen</s1><s2>Essen</s2><s3>DEU</s3><sZ>10 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Degen, Ingrid" sort="Degen, Ingrid" uniqKey="Degen I" first="Ingrid" last="Degen">Ingrid Degen</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Department of Neuropediatrics, Sankt Elisabeth Krankenhaus</s1><s2>Neuwied</s2><s3>DEU</s3><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Kruse, Bernd" sort="Kruse, Bernd" uniqKey="Kruse B" first="Bernd" last="Kruse">Bernd Kruse</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Department of Neuropediatrics, University of Hamburg</s1><s2>Hamburg</s2><s3>DEU</s3><sZ>12 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Dorr, Jan Markus" sort="Dorr, Jan Markus" uniqKey="Dorr J" first="Jan-Markus" last="Dörr">Jan-Markus Dörr</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of Neurology, Helios-Klinikum</s1><s2>Berlin</s2><s3>DEU</s3><sZ>13 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Ratzka, Susanne" sort="Ratzka, Susanne" uniqKey="Ratzka S" first="Susanne" last="Ratzka">Susanne Ratzka</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Ivo, Anja" sort="Ivo, Anja" uniqKey="Ivo A" first="Anja" last="Ivo">Anja Ivo</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Neurodegeneration and Hertie- Institute for Clinical Brain Research, University of Tübingen</s1><s2>Tübingen</s2><s3>DEU</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, University of Innsbruck</s1><s2>Innsbruck</s2><s3>AUT</s3><sZ>7 aut.</sZ><sZ>17 aut.</sZ></inist:fA14><country>Autriche</country></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Klopstock, Thomas" sort="Klopstock, Thomas" uniqKey="Klopstock T" first="Thomas" last="Klopstock">Thomas Klopstock</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Allemagne</country></affiliation></author><author><name sortKey="Schulz, Jorg B" sort="Schulz, Jorg B" uniqKey="Schulz J" first="Jörg B." last="Schulz">Jörg B. Schulz</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Allemagne</country></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="2009">2009</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>Comparative study</term><term>Friedreich ataxia</term><term>Nervous system diseases</term><term>Validation</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Hérédodégénérescence spinocérébelleuse de Friedreich</term><term>Pathologie du système nerveux</term><term>Etude comparative</term><term>Validation</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure, SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited for clinical trials of FRDA.</div></front></TEI><inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>0885-3185</s0></fA01><fA03 i2="1"><s0>Mov. disord.</s0></fA03><fA05><s2>24</s2></fA05><fA06><s2>12</s2></fA06><fA08 i1="01" i2="1" l="ENG"><s1>Comparison of Three Clinical Rating Scales In Friedreich Ataxia (FRDA)</s1></fA08><fA11 i1="01" i2="1"><s1>BÜRK (Katrin)</s1></fA11><fA11 i1="02" i2="1"><s1>MÄLZIG (Ulrike)</s1></fA11><fA11 i1="03" i2="1"><s1>WOLF (Stefanie)</s1></fA11><fA11 i1="04" i2="1"><s1>HECK (Suzette)</s1></fA11><fA11 i1="05" i2="1"><s1>DIMITRIADIS (Konstantinos)</s1></fA11><fA11 i1="06" i2="1"><s1>SEHMITZ-HÜBSEH (Tanja)</s1></fA11><fA11 i1="07" i2="1"><s1>HERING (Sascha)</s1></fA11><fA11 i1="08" i2="1"><s1>LINDIG (Tobias M.)</s1></fA11><fA11 i1="09" i2="1"><s1>HAUG (Verena)</s1></fA11><fA11 i1="10" i2="1"><s1>TIMMANN (Dagmar)</s1></fA11><fA11 i1="11" i2="1"><s1>DEGEN (Ingrid)</s1></fA11><fA11 i1="12" i2="1"><s1>KRUSE (Bernd)</s1></fA11><fA11 i1="13" i2="1"><s1>DÖRR (Jan-Markus)</s1></fA11><fA11 i1="14" i2="1"><s1>RATZKA (Susanne)</s1></fA11><fA11 i1="15" i2="1"><s1>IVO (Anja)</s1></fA11><fA11 i1="16" i2="1"><s1>SCHÖLS (Ludger)</s1></fA11><fA11 i1="17" i2="1"><s1>BOESCH (Sylvia)</s1></fA11><fA11 i1="18" i2="1"><s1>KLOCKGETHER (Thomas)</s1></fA11><fA11 i1="19" i2="1"><s1>KLOPSTOCK (Thomas)</s1></fA11><fA11 i1="20" i2="1"><s1>SCHULZ (Jörg B.)</s1></fA11><fA14 i1="01"><s1>Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen</s1><s2>Göttingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>14 aut.</sZ><sZ>20 aut.</sZ></fA14><fA14 i1="02"><s1>Department of Neurology, University of Marburg</s1><s2>Marburg</s2><s3>DEU</s3><sZ>1 aut.</sZ></fA14><fA14 i1="03"><s1>Department of Neurology, University of Munich</s1><s2>Munich</s2><s3>DEU</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>19 aut.</sZ></fA14><fA14 i1="04"><s1>Department of Neurology, University of Bonn</s1><s2>Bonn</s2><s3>DEU</s3><sZ>6 aut.</sZ><sZ>15 aut.</sZ><sZ>18 aut.</sZ></fA14><fA14 i1="05"><s1>Department of Neurology, University of Innsbruck</s1><s2>Innsbruck</s2><s3>AUT</s3><sZ>7 aut.</sZ><sZ>17 aut.</sZ></fA14><fA14 i1="06"><s1>Department of Neurodegeneration and Hertie- Institute for Clinical Brain Research, University of Tübingen</s1><s2>Tübingen</s2><s3>DEU</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ></fA14><fA14 i1="07"><s1>Department of Neuropediatrics, University of Freiburg</s1><s2>Freiburg</s2><s3>DEU</s3><sZ>9 aut.</sZ></fA14><fA14 i1="08"><s1>Department of Neurology, University of Essen</s1><s2>Essen</s2><s3>DEU</s3><sZ>10 aut.</sZ></fA14><fA14 i1="09"><s1>Department of Neuropediatrics, Sankt Elisabeth Krankenhaus</s1><s2>Neuwied</s2><s3>DEU</s3><sZ>11 aut.</sZ></fA14><fA14 i1="10"><s1>Department of Neuropediatrics, University of Hamburg</s1><s2>Hamburg</s2><s3>DEU</s3><sZ>12 aut.</sZ></fA14><fA14 i1="11"><s1>Department of Neurology, Helios-Klinikum</s1><s2>Berlin</s2><s3>DEU</s3><sZ>13 aut.</sZ></fA14><fA20><s1>1779-1784</s1></fA20><fA21><s1>2009</s1></fA21><fA23 i1="01"><s0>ENG</s0></fA23><fA43 i1="01"><s1>INIST</s1><s2>20953</s2><s5>354000170057120090</s5></fA43><fA44><s0>0000</s0><s1>© 2009 INIST-CNRS. All rights reserved.</s1></fA44><fA45><s0>16 ref.</s0></fA45><fA47 i1="01" i2="1"><s0>09-0431487</s0></fA47><fA60><s1>P</s1></fA60><fA61><s0>A</s0></fA61><fA64 i1="01" i2="1"><s0>Movement disorders</s0></fA64><fA66 i1="01"><s0>USA</s0></fA66><fC01 i1="01" l="ENG"><s0>To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure, SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited for clinical trials of FRDA.</s0></fC01><fC02 i1="01" i2="X"><s0>002B17</s0></fC02><fC02 i1="02" i2="X"><s0>002B17G</s0></fC02><fC03 i1="01" i2="X" l="FRE"><s0>Hérédodégénérescence spinocérébelleuse de Friedreich</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="ENG"><s0>Friedreich ataxia</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="SPA"><s0>Heredodegeneración espinocerebelosa Friedreich</s0><s5>01</s5></fC03><fC03 i1="02" i2="X" l="FRE"><s0>Pathologie du système nerveux</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="ENG"><s0>Nervous system diseases</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="SPA"><s0>Sistema nervioso patología</s0><s5>02</s5></fC03><fC03 i1="03" i2="X" l="FRE"><s0>Etude comparative</s0><s5>09</s5></fC03><fC03 i1="03" i2="X" l="ENG"><s0>Comparative study</s0><s5>09</s5></fC03><fC03 i1="03" i2="X" l="SPA"><s0>Estudio comparativo</s0><s5>09</s5></fC03><fC03 i1="04" i2="X" l="FRE"><s0>Validation</s0><s5>10</s5></fC03><fC03 i1="04" i2="X" l="ENG"><s0>Validation</s0><s5>10</s5></fC03><fC03 i1="04" i2="X" l="SPA"><s0>Validación</s0><s5>10</s5></fC03><fC07 i1="01" i2="X" l="FRE"><s0>Pathologie de l'encéphale</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="ENG"><s0>Cerebral disorder</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="SPA"><s0>Encéfalo patología</s0><s5>37</s5></fC07><fC07 i1="02" i2="X" l="FRE"><s0>Maladie dégénérative</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="ENG"><s0>Degenerative disease</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0><s5>38</s5></fC07><fC07 i1="03" i2="X" l="FRE"><s0>Maladie héréditaire</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="ENG"><s0>Genetic disease</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0><s5>39</s5></fC07><fC07 i1="04" i2="X" l="FRE"><s0>Pathologie de la moelle épinière</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="ENG"><s0>Spinal cord disease</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="SPA"><s0>Médula espinal patología</s0><s5>40</s5></fC07><fC07 i1="05" i2="X" l="FRE"><s0>Pathologie du système nerveux central</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="ENG"><s0>Central nervous system disease</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0><s5>41</s5></fC07><fN21><s1>313</s1></fN21><fN44 i1="01"><s1>OTO</s1></fN44><fN82><s1>OTO</s1></fN82></pA></standard></inist></record>Pour manipuler ce document sous Unix (Dilib)
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