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June 12, 2006

Scale for the assessment and rating of ataxia
Development of a new clinical scale

T. Schmitz-Hübsch, MD, S. Tezenas du Montcel, MD, PhD, L. Baliko, MD, J. Berciano, MD, S. Boesch, MD, C. Depondt, MD, P. Giunti, MD, … Show All … , C. Globas, MD, J. Infante, MD, J. -S. Kang, MD, B. Kremer, MD, C. Mariotti, MD, B. Melegh, MD, PhD, M. Pandolfo, MD, M. Rakowicz, MD, P. Ribai, MD, R. Rola, MD, L. Schöls, MD, S. Szymanski, MD, B. P. van de Warrenburg, MD, A. Dürr, MD, and T. Klockgether, MDAuthors Info & Affiliations
June 13, 2006 issue
66 (11) 1717-1720
https://doi.org/10.1212/01.wnl.0000219042.60538.92
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Abstract

Objective: To develop a reliable and valid clinical scale measuring the severity of ataxia.
Methods: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia.
Results: The mean time to administer SARA in patients was 14.2 ± 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's α of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p < 0.0001, r2 = 0.98). SARA score increased with the disease stage (p < 0.001) and was closely correlated with the Barthel Index (r = −0.80, p < 0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = −0.89, p < 0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p < 0.0002)
Conclusions: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

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Information & Authors

Information

Published In

Neurology®
Volume 66 • Number 11 • June 13, 2006
Pages: 1717-1720
PubMed: 16769946

Copyright

© 2006.

Publication History

Published online: June 12, 2006
Published in print: June 13, 2006

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Authors

Affiliations & Disclosures

T. Schmitz-Hübsch, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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S. Tezenas du Montcel, MD, PhD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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L. Baliko, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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J. Berciano, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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S. Boesch, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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C. Depondt, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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P. Giunti, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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C. Globas, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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J. Infante, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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J. -S. Kang, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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B. Kremer, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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C. Mariotti, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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B. Melegh, MD, PhD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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M. Pandolfo, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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M. Rakowicz, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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P. Ribai, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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R. Rola, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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L. Schöls, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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S. Szymanski, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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B. P. van de Warrenburg, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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A. Dürr, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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T. Klockgether, MD
From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.
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Notes

Address correspondence and reprint requests to Dr. Thomas Klockgether, Department of Neurology, University Hospital of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany; e-mail: [email protected]

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