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

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Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.

Identifieur interne : 000584 ( Main/Exploration ); précédent : 000583; suivant : 000585

Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.

Auteurs : Heike Jacobi [Allemagne] ; Sophie Tezenas Du Montcel [France] ; Peter Bauer [Allemagne] ; Paola Giunti [Royaume-Uni] ; Arron Cook [Royaume-Uni] ; Robyn Labrum [Royaume-Uni] ; Michael H. Parkinson [Royaume-Uni] ; Alexandra Durr [France] ; Alexis Brice [France] ; Perrine Charles [France] ; Cecilia Marelli [France] ; Caterina Mariotti [Italie] ; Lorenzo Nanetti [Italie] ; Marta Panzeri [Italie] ; Maria Rakowicz [Pologne] ; Anna Sulek [Pologne] ; Anna Sobanska [Pologne] ; Tanja Schmitz-Hübsch [Allemagne] ; Ludger Schöls [Allemagne] ; Holger Hengel [Allemagne] ; Laszlo Baliko [Hongrie] ; Bela Melegh [Hongrie] ; Alessandro Filla [Italie] ; Antonella Antenora [Italie] ; Jon Infante [Espagne] ; José Berciano [Espagne] ; Bart P. Van De Warrenburg [Pays-Bas] ; Dagmar Timmann [Allemagne] ; Sandra Szymanski [Allemagne] ; Sylvia Boesch [Autriche] ; Jun-Suk Kang [Allemagne] ; Massimo Pandolfo [Belgique] ; Jörg B. Schulz [Allemagne] ; Sonia Molho [France] ; Alhassane Diallo [France] ; Thomas Klockgether [Allemagne]

Source :

RBID : pubmed:26377379

English descriptors

Abstract

Spinocerebellar ataxias are dominantly inherited neurodegenerative diseases. As potential treatments for these diseases are being developed, precise knowledge of their natural history is needed. We aimed to study the long-term disease progression of the most common spinocerebellar ataxias: SCA1, SCA2, SCA3, and SCA6. Furthermore, we aimed to establish the order and occurrence of non-ataxia symptoms, and identify predictors of disease progression.

DOI: 10.1016/S1474-4422(15)00202-1
PubMed: 26377379


Affiliations:


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Le document en format XML

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<name sortKey="Parkinson, Michael H" sort="Parkinson, Michael H" uniqKey="Parkinson M" first="Michael H" last="Parkinson">Michael H. Parkinson</name>
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<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name>
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<name sortKey="Nanetti, Lorenzo" sort="Nanetti, Lorenzo" uniqKey="Nanetti L" first="Lorenzo" last="Nanetti">Lorenzo Nanetti</name>
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<name sortKey="Sulek, Anna" sort="Sulek, Anna" uniqKey="Sulek A" first="Anna" last="Sulek">Anna Sulek</name>
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<name sortKey="Sobanska, Anna" sort="Sobanska, Anna" uniqKey="Sobanska A" first="Anna" last="Sobanska">Anna Sobanska</name>
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<country xml:lang="fr">Pologne</country>
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<author>
<name sortKey="Schmitz Hubsch, Tanja" sort="Schmitz Hubsch, Tanja" uniqKey="Schmitz Hubsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name>
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<name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name>
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<nlm:affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen</wicri:regionArea>
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<name sortKey="Hengel, Holger" sort="Hengel, Holger" uniqKey="Hengel H" first="Holger" last="Hengel">Holger Hengel</name>
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<nlm:affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen</wicri:regionArea>
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<region type="land" nuts="1">Bade-Wurtemberg</region>
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<name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name>
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<nlm:affiliation>Department of Neurology, Zala County Hospital, Zalaegerszeg, Hungary.</nlm:affiliation>
<country xml:lang="fr">Hongrie</country>
<wicri:regionArea>Department of Neurology, Zala County Hospital, Zalaegerszeg</wicri:regionArea>
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<name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name>
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<nlm:affiliation>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs, Hungary.</nlm:affiliation>
<country xml:lang="fr">Hongrie</country>
<wicri:regionArea>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs</wicri:regionArea>
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<author>
<name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name>
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<nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples</wicri:regionArea>
<wicri:noRegion>Federico II University Naples</wicri:noRegion>
</affiliation>
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<author>
<name sortKey="Antenora, Antonella" sort="Antenora, Antonella" uniqKey="Antenora A" first="Antonella" last="Antenora">Antonella Antenora</name>
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<nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples</wicri:regionArea>
<wicri:noRegion>Federico II University Naples</wicri:noRegion>
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</author>
<author>
<name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name>
<affiliation wicri:level="1">
<nlm:affiliation>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander, Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander</wicri:regionArea>
<wicri:noRegion>Santander</wicri:noRegion>
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<author>
<name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name>
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<nlm:affiliation>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander, Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander</wicri:regionArea>
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</author>
<author>
<name sortKey="Van De Warrenburg, Bart P" sort="Van De Warrenburg, Bart P" uniqKey="Van De Warrenburg B" first="Bart P" last="Van De Warrenburg">Bart P. Van De Warrenburg</name>
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<nlm:affiliation>Radboud University Medical Center, Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, Netherlands.</nlm:affiliation>
<country xml:lang="fr">Pays-Bas</country>
<wicri:regionArea>Radboud University Medical Center, Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen</wicri:regionArea>
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<settlement type="city">Nimègue</settlement>
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<name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name>
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<nlm:affiliation>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen</wicri:regionArea>
<wicri:noRegion>Essen</wicri:noRegion>
<wicri:noRegion>Essen</wicri:noRegion>
<wicri:noRegion>Essen</wicri:noRegion>
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<name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name>
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<nlm:affiliation>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum</wicri:regionArea>
<wicri:noRegion>Bochum</wicri:noRegion>
<wicri:noRegion>Bochum</wicri:noRegion>
<wicri:noRegion>Bochum</wicri:noRegion>
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</author>
<author>
<name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.</nlm:affiliation>
<country xml:lang="fr">Autriche</country>
<wicri:regionArea>Department of Neurology, Medical University Innsbruck, Innsbruck</wicri:regionArea>
<wicri:noRegion>Innsbruck</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
<affiliation wicri:level="3">
<nlm:affiliation>Department of Neurology, University of Frankfurt, Frankfurt am Main, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, University of Frankfurt, Frankfurt am Main</wicri:regionArea>
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<region type="land" nuts="1">Hesse (Land)</region>
<region type="district" nuts="2">District de Darmstadt</region>
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<country xml:lang="fr">Allemagne</country>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Sorbonne Universités, Université Pierre et Marie Curie (UPMC) Univ Paris 06, UMR S 1136, INSERM U 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris</wicri:regionArea>
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<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, University Hospital of Bonn, Bonn, Germany; German Center for Neurodegenerative Diseases</wicri:regionArea>
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<title xml:lang="en">Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.</title>
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<name sortKey="Jacobi, Heike" sort="Jacobi, Heike" uniqKey="Jacobi H" first="Heike" last="Jacobi">Heike Jacobi</name>
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<nlm:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
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<nlm:affiliation>Sorbonne Universités, Université Pierre et Marie Curie (UPMC) Univ Paris 06, UMR S 1136, INSERM U 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris, France; AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.</nlm:affiliation>
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<country xml:lang="fr">Allemagne</country>
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<nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
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<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Molecular Neuroscience, UCL, Institute of Neurology, London</wicri:regionArea>
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<nlm:affiliation>Neurogenetic Laboratory, National Hospital of Neurology and Neurosurgery, UCLH, London, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Neurogenetic Laboratory, National Hospital of Neurology and Neurosurgery, UCLH, London</wicri:regionArea>
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<affiliation wicri:level="3">
<nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Molecular Neuroscience, UCL, Institute of Neurology, London</wicri:regionArea>
<placeName>
<settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
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<affiliation wicri:level="3">
<nlm:affiliation>INSERM, U 1127, F-75013, Paris, France, CNRS, Paris, France; Sorbonne Universités, UPMC Univ Paris 06, Paris, France; Institut du Cerveau et de la Moelle Epinière, ICM, Paris, France; AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U 1127, F-75013, Paris, France, CNRS, Paris, France; Sorbonne Universités, UPMC Univ Paris 06, Paris, France; Institut du Cerveau et de la Moelle Epinière, ICM, Paris, France; AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris</wicri:regionArea>
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<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
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<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<affiliation wicri:level="3">
<nlm:affiliation>INSERM, U 1127, F-75013, Paris, France, CNRS, Paris, France; Sorbonne Universités, UPMC Univ Paris 06, Paris, France; Institut du Cerveau et de la Moelle Epinière, ICM, Paris, France; AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U 1127, F-75013, Paris, France, CNRS, Paris, France; Sorbonne Universités, UPMC Univ Paris 06, Paris, France; Institut du Cerveau et de la Moelle Epinière, ICM, Paris, France; AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris</wicri:regionArea>
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<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
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<author>
<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<affiliation wicri:level="3">
<nlm:affiliation>AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris</wicri:regionArea>
<placeName>
<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
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<author>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
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<nlm:affiliation>Service de Neurologie, CMRR, CHRU Gui de Chauliac, Montpellier, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie, CMRR, CHRU Gui de Chauliac, Montpellier</wicri:regionArea>
<placeName>
<region type="region">Occitanie (région administrative)</region>
<region type="old region">Languedoc-Roussillon</region>
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<name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name>
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<nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan</wicri:regionArea>
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<settlement type="city">Milan</settlement>
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<affiliation wicri:level="3">
<nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan</wicri:regionArea>
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<settlement type="city">Milan</settlement>
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<author>
<name sortKey="Panzeri, Marta" sort="Panzeri, Marta" uniqKey="Panzeri M" first="Marta" last="Panzeri">Marta Panzeri</name>
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<nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan</wicri:regionArea>
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<settlement type="city">Milan</settlement>
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<nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation>
<country xml:lang="fr">Pologne</country>
<wicri:regionArea>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw</wicri:regionArea>
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</author>
<author>
<name sortKey="Sulek, Anna" sort="Sulek, Anna" uniqKey="Sulek A" first="Anna" last="Sulek">Anna Sulek</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation>
<country xml:lang="fr">Pologne</country>
<wicri:regionArea>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw</wicri:regionArea>
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</author>
<author>
<name sortKey="Sobanska, Anna" sort="Sobanska, Anna" uniqKey="Sobanska A" first="Anna" last="Sobanska">Anna Sobanska</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation>
<country xml:lang="fr">Pologne</country>
<wicri:regionArea>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw</wicri:regionArea>
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</affiliation>
</author>
<author>
<name sortKey="Schmitz Hubsch, Tanja" sort="Schmitz Hubsch, Tanja" uniqKey="Schmitz Hubsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name>
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<nlm:affiliation>Charité Universitätsmedizin Berlin, Klinik für Neurologie, Berlin, Germany; NeuroCure Clinical Research Center, Charité- Universitätsmedizin Berlin, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Charité Universitätsmedizin Berlin, Klinik für Neurologie, Berlin, Germany; NeuroCure Clinical Research Center, Charité- Universitätsmedizin Berlin</wicri:regionArea>
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<wicri:noRegion>Charité- Universitätsmedizin Berlin</wicri:noRegion>
<wicri:noRegion>Charité- Universitätsmedizin Berlin</wicri:noRegion>
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<author>
<name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name>
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<nlm:affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen</wicri:regionArea>
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<region type="land" nuts="1">Bade-Wurtemberg</region>
<region type="district" nuts="2">District de Tübingen</region>
<settlement type="city">Tübingen</settlement>
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<name sortKey="Hengel, Holger" sort="Hengel, Holger" uniqKey="Hengel H" first="Holger" last="Hengel">Holger Hengel</name>
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<nlm:affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen</wicri:regionArea>
<placeName>
<region type="land" nuts="1">Bade-Wurtemberg</region>
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<author>
<name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Neurology, Zala County Hospital, Zalaegerszeg, Hungary.</nlm:affiliation>
<country xml:lang="fr">Hongrie</country>
<wicri:regionArea>Department of Neurology, Zala County Hospital, Zalaegerszeg</wicri:regionArea>
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</affiliation>
</author>
<author>
<name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs, Hungary.</nlm:affiliation>
<country xml:lang="fr">Hongrie</country>
<wicri:regionArea>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs</wicri:regionArea>
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</author>
<author>
<name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name>
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<nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples</wicri:regionArea>
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<author>
<name sortKey="Antenora, Antonella" sort="Antenora, Antonella" uniqKey="Antenora A" first="Antonella" last="Antenora">Antonella Antenora</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples</wicri:regionArea>
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</affiliation>
</author>
<author>
<name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name>
<affiliation wicri:level="1">
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<country xml:lang="fr">Espagne</country>
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<author>
<name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name>
<affiliation wicri:level="1">
<nlm:affiliation>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander, Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Santander</wicri:regionArea>
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<author>
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<wicri:regionArea>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen</wicri:regionArea>
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<wicri:regionArea>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum</wicri:regionArea>
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<nlm:affiliation>Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.</nlm:affiliation>
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<wicri:regionArea>Department of Neurology, Medical University Innsbruck, Innsbruck</wicri:regionArea>
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<name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
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<nlm:affiliation>Department of Neurology, University of Frankfurt, Frankfurt am Main, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, University of Frankfurt, Frankfurt am Main</wicri:regionArea>
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<region type="land" nuts="1">Hesse (Land)</region>
<region type="district" nuts="2">District de Darmstadt</region>
<settlement type="city">Francfort-sur-le-Main</settlement>
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</affiliation>
</author>
<author>
<name sortKey="Pandolfo, Massimo" sort="Pandolfo, Massimo" uniqKey="Pandolfo M" first="Massimo" last="Pandolfo">Massimo Pandolfo</name>
<affiliation wicri:level="3">
<nlm:affiliation>Neurology Service, Hôpital Erasme-Université Libre de Bruxelles (ULB), Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Neurology Service, Hôpital Erasme-Université Libre de Bruxelles (ULB), Brussels</wicri:regionArea>
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<region nuts="2">Région de Bruxelles-Capitale</region>
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</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">
<nlm:affiliation>Department of Neurology, RWTH Aachen University, Aachen, Germany; JARA-Translational Brain Medicine, Aachen-Jülich, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, RWTH Aachen University, Aachen, Germany; JARA-Translational Brain Medicine, Aachen-Jülich</wicri:regionArea>
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<wicri:noRegion>Aachen-Jülich</wicri:noRegion>
<wicri:noRegion>Aachen-Jülich</wicri:noRegion>
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<name sortKey="Molho, Sonia" sort="Molho, Sonia" uniqKey="Molho S" first="Sonia" last="Molho">Sonia Molho</name>
<affiliation wicri:level="3">
<nlm:affiliation>AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris</wicri:regionArea>
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<region type="region">Île-de-France</region>
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<name sortKey="Diallo, Alhassane" sort="Diallo, Alhassane" uniqKey="Diallo A" first="Alhassane" last="Diallo">Alhassane Diallo</name>
<affiliation wicri:level="3">
<nlm:affiliation>Sorbonne Universités, Université Pierre et Marie Curie (UPMC) Univ Paris 06, UMR S 1136, INSERM U 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
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<placeName>
<region type="region">Île-de-France</region>
<region type="old region">Île-de-France</region>
<settlement type="city">Paris</settlement>
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</author>
<author>
<name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<affiliation wicri:level="1">
<nlm:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany; German Center for Neurodegenerative Diseases, Germany. Electronic address: klockgether@uni-bonn.de.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, University Hospital of Bonn, Bonn, Germany; German Center for Neurodegenerative Diseases</wicri:regionArea>
<wicri:noRegion>Germany; German Center for Neurodegenerative Diseases</wicri:noRegion>
<wicri:noRegion>Germany; German Center for Neurodegenerative Diseases</wicri:noRegion>
<wicri:noRegion>Germany; German Center for Neurodegenerative Diseases</wicri:noRegion>
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</author>
</analytic>
<series>
<title level="j">The Lancet. Neurology</title>
<idno type="eISSN">1474-4465</idno>
<imprint>
<date when="2015" type="published">2015</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc>
<textClass>
<keywords scheme="KwdEn" xml:lang="en">
<term>Adult</term>
<term>Ataxin-1 (genetics)</term>
<term>Ataxin-2 (genetics)</term>
<term>Ataxin-3 (genetics)</term>
<term>Calcium Channels (genetics)</term>
<term>Cohort Studies</term>
<term>Disease Progression</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>PubMed (statistics & numerical data)</term>
<term>Repressor Proteins (genetics)</term>
<term>Spinocerebellar Ataxias (classification)</term>
<term>Spinocerebellar Ataxias (genetics)</term>
<term>Spinocerebellar Ataxias (physiopathology)</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en">
<term>Ataxin-1</term>
<term>Ataxin-2</term>
<term>Ataxin-3</term>
<term>Calcium Channels</term>
<term>Repressor Proteins</term>
</keywords>
<keywords scheme="MESH" qualifier="classification" xml:lang="en">
<term>Spinocerebellar Ataxias</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en">
<term>Spinocerebellar Ataxias</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en">
<term>Spinocerebellar Ataxias</term>
</keywords>
<keywords scheme="MESH" qualifier="statistics & numerical data" xml:lang="en">
<term>PubMed</term>
</keywords>
<keywords scheme="MESH" xml:lang="en">
<term>Adult</term>
<term>Cohort Studies</term>
<term>Disease Progression</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Young Adult</term>
</keywords>
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</teiHeader>
<front>
<div type="abstract" xml:lang="en">Spinocerebellar ataxias are dominantly inherited neurodegenerative diseases. As potential treatments for these diseases are being developed, precise knowledge of their natural history is needed. We aimed to study the long-term disease progression of the most common spinocerebellar ataxias: SCA1, SCA2, SCA3, and SCA6. Furthermore, we aimed to establish the order and occurrence of non-ataxia symptoms, and identify predictors of disease progression.</div>
</front>
</TEI>
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<li>Allemagne</li>
<li>Autriche</li>
<li>Belgique</li>
<li>Espagne</li>
<li>France</li>
<li>Hongrie</li>
<li>Italie</li>
<li>Pays-Bas</li>
<li>Pologne</li>
<li>Royaume-Uni</li>
</country>
<region>
<li>Angleterre</li>
<li>Bade-Wurtemberg</li>
<li>District de Cologne</li>
<li>District de Darmstadt</li>
<li>District de Tübingen</li>
<li>Grand Londres</li>
<li>Gueldre</li>
<li>Hesse (Land)</li>
<li>Languedoc-Roussillon</li>
<li>Lombardie</li>
<li>Occitanie (région administrative)</li>
<li>Rhénanie-du-Nord-Westphalie</li>
<li>Région de Bruxelles-Capitale</li>
<li>Île-de-France</li>
</region>
<settlement>
<li>Bonn</li>
<li>Bruxelles</li>
<li>Francfort-sur-le-Main</li>
<li>Londres</li>
<li>Milan</li>
<li>Montpellier</li>
<li>Nimègue</li>
<li>Paris</li>
<li>Tübingen</li>
</settlement>
</list>
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<region name="Rhénanie-du-Nord-Westphalie">
<name sortKey="Jacobi, Heike" sort="Jacobi, Heike" uniqKey="Jacobi H" first="Heike" last="Jacobi">Heike Jacobi</name>
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<name sortKey="Bauer, Peter" sort="Bauer, Peter" uniqKey="Bauer P" first="Peter" last="Bauer">Peter Bauer</name>
<name sortKey="Hengel, Holger" sort="Hengel, Holger" uniqKey="Hengel H" first="Holger" last="Hengel">Holger Hengel</name>
<name sortKey="Kang, Jun Suk" sort="Kang, Jun Suk" uniqKey="Kang J" first="Jun-Suk" last="Kang">Jun-Suk Kang</name>
<name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name>
<name sortKey="Schmitz Hubsch, Tanja" sort="Schmitz Hubsch, Tanja" uniqKey="Schmitz Hubsch T" first="Tanja" last="Schmitz-Hübsch">Tanja Schmitz-Hübsch</name>
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<name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name>
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<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
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<name sortKey="Molho, Sonia" sort="Molho, Sonia" uniqKey="Molho S" first="Sonia" last="Molho">Sonia Molho</name>
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