Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.
Identifieur interne : 000263 ( PubMed/Corpus ); précédent : 000262; suivant : 000264Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.
Auteurs : Heike Jacobi ; Sophie Tezenas Du Montcel ; Peter Bauer ; Paola Giunti ; Arron Cook ; Robyn Labrum ; Michael H. Parkinson ; Alexandra Durr ; Alexis Brice ; Perrine Charles ; Cecilia Marelli ; Caterina Mariotti ; Lorenzo Nanetti ; Marta Panzeri ; Maria Rakowicz ; Anna Sulek ; Anna Sobanska ; Tanja Schmitz-Hübsch ; Ludger Schöls ; Holger Hengel ; Laszlo Baliko ; Bela Melegh ; Alessandro Filla ; Antonella Antenora ; Jon Infante ; José Berciano ; Bart P. Van De Warrenburg ; Dagmar Timmann ; Sandra Szymanski ; Sylvia Boesch ; Jun-Suk Kang ; Massimo Pandolfo ; Jörg B. Schulz ; Sonia Molho ; Alhassane Diallo ; Thomas KlockgetherSource :
- The Lancet. Neurology [ 1474-4465 ] ; 2015.
English descriptors
- KwdEn :
- Adult, Ataxin-1 (genetics), Ataxin-2 (genetics), Ataxin-3 (genetics), Calcium Channels (genetics), Cohort Studies, Disease Progression, Female, Humans, Male, Middle Aged, PubMed (statistics & numerical data), Repressor Proteins (genetics), Spinocerebellar Ataxias (classification), Spinocerebellar Ataxias (genetics), Spinocerebellar Ataxias (physiopathology), Young Adult.
- MESH :
- chemical , genetics : Ataxin-1, Ataxin-2, Ataxin-3, Calcium Channels, Repressor Proteins.
- classification : Spinocerebellar Ataxias.
- genetics : Spinocerebellar Ataxias.
- physiopathology : Spinocerebellar Ataxias.
- statistics & numerical data : PubMed.
- Adult, Cohort Studies, Disease Progression, Female, Humans, Male, Middle Aged, Young Adult.
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
Links to Exploration step
pubmed:26377379Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.</title><author><name sortKey="Jacobi, Heike" sort="Jacobi, Heike" uniqKey="Jacobi H" first="Heike" last="Jacobi">Heike Jacobi</name><affiliation><nlm:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany.</nlm:affiliation></affiliation></author><author><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><affiliation><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></affiliation></author><author><name sortKey="Bauer, Peter" sort="Bauer, Peter" uniqKey="Bauer P" first="Peter" last="Bauer">Peter Bauer</name><affiliation><nlm:affiliation>Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Cook, Arron" sort="Cook, Arron" uniqKey="Cook A" first="Arron" last="Cook">Arron Cook</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Labrum, Robyn" sort="Labrum, Robyn" uniqKey="Labrum R" first="Robyn" last="Labrum">Robyn Labrum</name><affiliation><nlm:affiliation>Neurogenetic Laboratory, National Hospital of Neurology and Neurosurgery, UCLH, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Parkinson, Michael H" sort="Parkinson, Michael H" uniqKey="Parkinson M" first="Michael H" last="Parkinson">Michael H. Parkinson</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name><affiliation><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></affiliation></author><author><name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name><affiliation><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></affiliation></author><author><name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name><affiliation><nlm:affiliation>AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</nlm:affiliation></affiliation></author><author><name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name><affiliation><nlm:affiliation>Service de Neurologie, CMRR, CHRU Gui de Chauliac, Montpellier, France.</nlm:affiliation></affiliation></author><author><name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Nanetti, Lorenzo" sort="Nanetti, Lorenzo" uniqKey="Nanetti L" first="Lorenzo" last="Nanetti">Lorenzo Nanetti</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Panzeri, Marta" sort="Panzeri, Marta" uniqKey="Panzeri M" first="Marta" last="Panzeri">Marta Panzeri</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Rakowicz, Maria" sort="Rakowicz, Maria" uniqKey="Rakowicz M" first="Maria" last="Rakowicz">Maria Rakowicz</name><affiliation><nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></affiliation></author><author><name sortKey="Sulek, Anna" sort="Sulek, Anna" uniqKey="Sulek A" first="Anna" last="Sulek">Anna Sulek</name><affiliation><nlm:affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></affiliation></author><author><name sortKey="Sobanska, Anna" sort="Sobanska, Anna" uniqKey="Sobanska A" first="Anna" last="Sobanska">Anna Sobanska</name><affiliation><nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></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><affiliation><nlm:affiliation>Charité Universitätsmedizin Berlin, Klinik für Neurologie, Berlin, Germany; NeuroCure Clinical Research Center, Charité- Universitätsmedizin Berlin, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation><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></affiliation></author><author><name sortKey="Hengel, Holger" sort="Hengel, Holger" uniqKey="Hengel H" first="Holger" last="Hengel">Holger Hengel</name><affiliation><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></affiliation></author><author><name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name><affiliation><nlm:affiliation>Department of Neurology, Zala County Hospital, Zalaegerszeg, Hungary.</nlm:affiliation></affiliation></author><author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name><affiliation><nlm:affiliation>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs, Hungary.</nlm:affiliation></affiliation></author><author><name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name><affiliation><nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Antenora, Antonella" sort="Antenora, Antonella" uniqKey="Antenora A" first="Antonella" last="Antenora">Antonella Antenora</name><affiliation><nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name><affiliation><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></affiliation></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name><affiliation><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></affiliation></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><affiliation><nlm:affiliation>Radboud University Medical Center, Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, Netherlands.</nlm:affiliation></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation><nlm:affiliation>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name><affiliation><nlm:affiliation>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation><nlm:affiliation>Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.</nlm:affiliation></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><nlm:affiliation>Department of Neurology, University of Frankfurt, Frankfurt am Main, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Pandolfo, Massimo" sort="Pandolfo, Massimo" uniqKey="Pandolfo M" first="Massimo" last="Pandolfo">Massimo Pandolfo</name><affiliation><nlm:affiliation>Neurology Service, Hôpital Erasme-Université Libre de Bruxelles (ULB), Brussels, Belgium.</nlm:affiliation></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><nlm:affiliation>Department of Neurology, RWTH Aachen University, Aachen, Germany; JARA-Translational Brain Medicine, Aachen-Jülich, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Molho, Sonia" sort="Molho, Sonia" uniqKey="Molho S" first="Sonia" last="Molho">Sonia Molho</name><affiliation><nlm:affiliation>AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.</nlm:affiliation></affiliation></author><author><name sortKey="Diallo, Alhassane" sort="Diallo, Alhassane" uniqKey="Diallo A" first="Alhassane" last="Diallo">Alhassane Diallo</name><affiliation><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></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation><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></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2015">2015</date><idno type="RBID">pubmed:26377379</idno><idno type="pmid">26377379</idno><idno type="doi">10.1016/S1474-4422(15)00202-1</idno><idno type="wicri:Area/PubMed/Corpus">000263</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000263</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.</title><author><name sortKey="Jacobi, Heike" sort="Jacobi, Heike" uniqKey="Jacobi H" first="Heike" last="Jacobi">Heike Jacobi</name><affiliation><nlm:affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany.</nlm:affiliation></affiliation></author><author><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><affiliation><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></affiliation></author><author><name sortKey="Bauer, Peter" sort="Bauer, Peter" uniqKey="Bauer P" first="Peter" last="Bauer">Peter Bauer</name><affiliation><nlm:affiliation>Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Cook, Arron" sort="Cook, Arron" uniqKey="Cook A" first="Arron" last="Cook">Arron Cook</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Labrum, Robyn" sort="Labrum, Robyn" uniqKey="Labrum R" first="Robyn" last="Labrum">Robyn Labrum</name><affiliation><nlm:affiliation>Neurogenetic Laboratory, National Hospital of Neurology and Neurosurgery, UCLH, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Parkinson, Michael H" sort="Parkinson, Michael H" uniqKey="Parkinson M" first="Michael H" last="Parkinson">Michael H. Parkinson</name><affiliation><nlm:affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Durr">Alexandra Durr</name><affiliation><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></affiliation></author><author><name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name><affiliation><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></affiliation></author><author><name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name><affiliation><nlm:affiliation>AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</nlm:affiliation></affiliation></author><author><name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name><affiliation><nlm:affiliation>Service de Neurologie, CMRR, CHRU Gui de Chauliac, Montpellier, France.</nlm:affiliation></affiliation></author><author><name sortKey="Mariotti, Caterina" sort="Mariotti, Caterina" uniqKey="Mariotti C" first="Caterina" last="Mariotti">Caterina Mariotti</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Nanetti, Lorenzo" sort="Nanetti, Lorenzo" uniqKey="Nanetti L" first="Lorenzo" last="Nanetti">Lorenzo Nanetti</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Panzeri, Marta" sort="Panzeri, Marta" uniqKey="Panzeri M" first="Marta" last="Panzeri">Marta Panzeri</name><affiliation><nlm:affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Rakowicz, Maria" sort="Rakowicz, Maria" uniqKey="Rakowicz M" first="Maria" last="Rakowicz">Maria Rakowicz</name><affiliation><nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></affiliation></author><author><name sortKey="Sulek, Anna" sort="Sulek, Anna" uniqKey="Sulek A" first="Anna" last="Sulek">Anna Sulek</name><affiliation><nlm:affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></affiliation></author><author><name sortKey="Sobanska, Anna" sort="Sobanska, Anna" uniqKey="Sobanska A" first="Anna" last="Sobanska">Anna Sobanska</name><affiliation><nlm:affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</nlm:affiliation></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><affiliation><nlm:affiliation>Charité Universitätsmedizin Berlin, Klinik für Neurologie, Berlin, Germany; NeuroCure Clinical Research Center, Charité- Universitätsmedizin Berlin, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Schols, Ludger" sort="Schols, Ludger" uniqKey="Schols L" first="Ludger" last="Schöls">Ludger Schöls</name><affiliation><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></affiliation></author><author><name sortKey="Hengel, Holger" sort="Hengel, Holger" uniqKey="Hengel H" first="Holger" last="Hengel">Holger Hengel</name><affiliation><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></affiliation></author><author><name sortKey="Baliko, Laszlo" sort="Baliko, Laszlo" uniqKey="Baliko L" first="Laszlo" last="Baliko">Laszlo Baliko</name><affiliation><nlm:affiliation>Department of Neurology, Zala County Hospital, Zalaegerszeg, Hungary.</nlm:affiliation></affiliation></author><author><name sortKey="Melegh, Bela" sort="Melegh, Bela" uniqKey="Melegh B" first="Bela" last="Melegh">Bela Melegh</name><affiliation><nlm:affiliation>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs, Hungary.</nlm:affiliation></affiliation></author><author><name sortKey="Filla, Alessandro" sort="Filla, Alessandro" uniqKey="Filla A" first="Alessandro" last="Filla">Alessandro Filla</name><affiliation><nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Antenora, Antonella" sort="Antenora, Antonella" uniqKey="Antenora A" first="Antonella" last="Antenora">Antonella Antenora</name><affiliation><nlm:affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</nlm:affiliation></affiliation></author><author><name sortKey="Infante, Jon" sort="Infante, Jon" uniqKey="Infante J" first="Jon" last="Infante">Jon Infante</name><affiliation><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></affiliation></author><author><name sortKey="Berciano, Jose" sort="Berciano, Jose" uniqKey="Berciano J" first="José" last="Berciano">José Berciano</name><affiliation><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></affiliation></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><affiliation><nlm:affiliation>Radboud University Medical Center, Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, Netherlands.</nlm:affiliation></affiliation></author><author><name sortKey="Timmann, Dagmar" sort="Timmann, Dagmar" uniqKey="Timmann D" first="Dagmar" last="Timmann">Dagmar Timmann</name><affiliation><nlm:affiliation>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Szymanski, Sandra" sort="Szymanski, Sandra" uniqKey="Szymanski S" first="Sandra" last="Szymanski">Sandra Szymanski</name><affiliation><nlm:affiliation>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Boesch, Sylvia" sort="Boesch, Sylvia" uniqKey="Boesch S" first="Sylvia" last="Boesch">Sylvia Boesch</name><affiliation><nlm:affiliation>Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.</nlm:affiliation></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><nlm:affiliation>Department of Neurology, University of Frankfurt, Frankfurt am Main, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Pandolfo, Massimo" sort="Pandolfo, Massimo" uniqKey="Pandolfo M" first="Massimo" last="Pandolfo">Massimo Pandolfo</name><affiliation><nlm:affiliation>Neurology Service, Hôpital Erasme-Université Libre de Bruxelles (ULB), Brussels, Belgium.</nlm:affiliation></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><nlm:affiliation>Department of Neurology, RWTH Aachen University, Aachen, Germany; JARA-Translational Brain Medicine, Aachen-Jülich, Germany.</nlm:affiliation></affiliation></author><author><name sortKey="Molho, Sonia" sort="Molho, Sonia" uniqKey="Molho S" first="Sonia" last="Molho">Sonia Molho</name><affiliation><nlm:affiliation>AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.</nlm:affiliation></affiliation></author><author><name sortKey="Diallo, Alhassane" sort="Diallo, Alhassane" uniqKey="Diallo A" first="Alhassane" last="Diallo">Alhassane Diallo</name><affiliation><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></affiliation></author><author><name sortKey="Klockgether, Thomas" sort="Klockgether, Thomas" uniqKey="Klockgether T" first="Thomas" last="Klockgether">Thomas Klockgether</name><affiliation><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></affiliation></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></textClass></profileDesc></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><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">26377379</PMID><DateCreated><Year>2015</Year><Month>10</Month><Day>15</Day></DateCreated><DateCompleted><Year>2016</Year><Month>01</Month><Day>26</Day></DateCompleted><DateRevised><Year>2016</Year><Month>11</Month><Day>25</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1474-4465</ISSN><JournalIssue CitedMedium="Internet"><Volume>14</Volume><Issue>11</Issue><PubDate><Year>2015</Year><Month>Nov</Month></PubDate></JournalIssue><Title>The Lancet. Neurology</Title><ISOAbbreviation>Lancet Neurol</ISOAbbreviation></Journal><ArticleTitle>Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study.</ArticleTitle><Pagination><MedlinePgn>1101-8</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1016/S1474-4422(15)00202-1</ELocationID><ELocationID EIdType="pii" ValidYN="Y">S1474-4422(15)00202-1</ELocationID><Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">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.</AbstractText><AbstractText Label="METHODS" NlmCategory="METHODS">In this longitudinal cohort study (EUROSCA), we enrolled men and women with positive genetic testing for SCA1, SCA2, SCA3, or SCA6 and with progressive, otherwise unexplained ataxia who were aged 18 years or older from 17 ataxia referral centres in ten European countries. Patients were seen every year for 3 years, and at irregular intervals thereafter. The primary outcome was the scale for the assessment and rating of ataxia (SARA), and the inventory of non-ataxia signs (INAS). We used linear mixed models to analyse progression. To account for dropouts, we applied a pattern-mixture model. This study is registered with ClinicalTrials.gov, number NCT02440763.</AbstractText><AbstractText Label="FINDINGS" NlmCategory="RESULTS">Between July 1, 2005, and Aug 31, 2006, 526 patients with SCA1, SCA2, SCA3, or SCA6 were enrolled. We analysed data for 462 patients with at least one follow-up visit. Median observation time was 49 months (IQR 35-72). SARA progression data were best fitted with a linear model in all genotypes. Annual SARA score increase was 2.11 (SE 0.12) in patients with SCA1, 1.49 (0.07) in patients with SCA2, 1.56 (0.08) in patients with SCA3, and 0.80 (0.09) in patients with SCA6. The increase of the number of non-ataxia signs reached a plateau in SCA1, SCA2, and SCA3. In patients with SCA6, the number of non-ataxia symptoms increased linearly, but more slowly than in patients with SCA1, SCA2, and SCA3 (p<0.0001). Factors that were associated with faster progression of the SARA score were short duration of follow-up (p=0.0179), older age at inclusion (0.04 [SE 0.02] per additional year; p=0.0476), and longer repeat expansions (0.06 [SE 0.02] per additional repeat unit; p=0.0128) in SCA1, short duration of follow-up (p<0.0001), lower age at onset (-0.02 [SE 0.01] per additional year; p=0.0014), and lower baseline SARA score (-0.02 [SE 0.01] per additional SARA point; p=0.0083) in SCA2, and lower baseline SARA score (-0.03 [SE 0.01] per additional SARA point; p=0·0195) in SCA6. In SCA3, we did not identify factors that affected progression of the SARA score.</AbstractText><AbstractText Label="INTERPRETATION" NlmCategory="CONCLUSIONS">Our study provides quantitative data on the progression of the most common spinocerebellar ataxias based on a follow-up period that exceeds those of previous studies. Our data could prove useful for sample size calculation and patient stratification in interventional trials.</AbstractText><AbstractText Label="FUNDING" NlmCategory="BACKGROUND">EU FP6 (EUROSCA), German Ministry of Education and Research (BMBF; GeneMove), Polish Ministry of Science, EU FP7 (NEUROMICS).</AbstractText><CopyrightInformation>Copyright © 2015 Elsevier Ltd. All rights reserved.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Jacobi</LastName><ForeName>Heike</ForeName><Initials>H</Initials><AffiliationInfo><Affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>du Montcel</LastName><ForeName>Sophie Tezenas</ForeName><Initials>ST</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Bauer</LastName><ForeName>Peter</ForeName><Initials>P</Initials><AffiliationInfo><Affiliation>Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Giunti</LastName><ForeName>Paola</ForeName><Initials>P</Initials><AffiliationInfo><Affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Cook</LastName><ForeName>Arron</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Labrum</LastName><ForeName>Robyn</ForeName><Initials>R</Initials><AffiliationInfo><Affiliation>Neurogenetic Laboratory, National Hospital of Neurology and Neurosurgery, UCLH, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Parkinson</LastName><ForeName>Michael H</ForeName><Initials>MH</Initials><AffiliationInfo><Affiliation>Department of Molecular Neuroscience, UCL, Institute of Neurology, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Durr</LastName><ForeName>Alexandra</ForeName><Initials>A</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Brice</LastName><ForeName>Alexis</ForeName><Initials>A</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Charles</LastName><ForeName>Perrine</ForeName><Initials>P</Initials><AffiliationInfo><Affiliation>AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Génétique, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Marelli</LastName><ForeName>Cecilia</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Service de Neurologie, CMRR, CHRU Gui de Chauliac, Montpellier, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Mariotti</LastName><ForeName>Caterina</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Nanetti</LastName><ForeName>Lorenzo</ForeName><Initials>L</Initials><AffiliationInfo><Affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Panzeri</LastName><ForeName>Marta</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>SOSD Genetics of Neurodegenerative and Metabolic Diseases, Fondazione-IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Rakowicz</LastName><ForeName>Maria</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sulek</LastName><ForeName>Anna</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sobanska</LastName><ForeName>Anna</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Schmitz-Hübsch</LastName><ForeName>Tanja</ForeName><Initials>T</Initials><AffiliationInfo><Affiliation>Charité Universitätsmedizin Berlin, Klinik für Neurologie, Berlin, Germany; NeuroCure Clinical Research Center, Charité- Universitätsmedizin Berlin, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Schöls</LastName><ForeName>Ludger</ForeName><Initials>L</Initials><AffiliationInfo><Affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Hengel</LastName><ForeName>Holger</ForeName><Initials>H</Initials><AffiliationInfo><Affiliation>German Center for Neurodegenerative Diseases, Germany; Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Baliko</LastName><ForeName>Laszlo</ForeName><Initials>L</Initials><AffiliationInfo><Affiliation>Department of Neurology, Zala County Hospital, Zalaegerszeg, Hungary.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Melegh</LastName><ForeName>Bela</ForeName><Initials>B</Initials><AffiliationInfo><Affiliation>Department of Medical Genetics, and Szentagothai Research Center, University of Pécs, Pécs, Hungary.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Filla</LastName><ForeName>Alessandro</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Antenora</LastName><ForeName>Antonella</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Department of Neuroscience, and Reproductive and Odontostomatological Sciences, Federico II University Naples, Italy.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Infante</LastName><ForeName>Jon</ForeName><Initials>J</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Berciano</LastName><ForeName>José</ForeName><Initials>J</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>van de Warrenburg</LastName><ForeName>Bart P</ForeName><Initials>BP</Initials><AffiliationInfo><Affiliation>Radboud University Medical Center, Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, Netherlands.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Timmann</LastName><ForeName>Dagmar</ForeName><Initials>D</Initials><AffiliationInfo><Affiliation>Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Szymanski</LastName><ForeName>Sandra</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Neurology, St Josef Hospital, University Hospital of Bochum, Bochum, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Boesch</LastName><ForeName>Sylvia</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Kang</LastName><ForeName>Jun-Suk</ForeName><Initials>JS</Initials><AffiliationInfo><Affiliation>Department of Neurology, University of Frankfurt, Frankfurt am Main, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Pandolfo</LastName><ForeName>Massimo</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>Neurology Service, Hôpital Erasme-Université Libre de Bruxelles (ULB), Brussels, Belgium.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Schulz</LastName><ForeName>Jörg B</ForeName><Initials>JB</Initials><AffiliationInfo><Affiliation>Department of Neurology, RWTH Aachen University, Aachen, Germany; JARA-Translational Brain Medicine, Aachen-Jülich, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Molho</LastName><ForeName>Sonia</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>AP-HP, Biostatistics Unit, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Diallo</LastName><ForeName>Alhassane</ForeName><Initials>A</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Klockgether</LastName><ForeName>Thomas</ForeName><Initials>T</Initials><AffiliationInfo><Affiliation>Department of Neurology, University Hospital of Bonn, Bonn, Germany; German Center for Neurodegenerative Diseases, Germany. Electronic address: klockgether@uni-bonn.de.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><DataBankList CompleteYN="Y"><DataBank><DataBankName>ClinicalTrials.gov</DataBankName><AccessionNumberList><AccessionNumber>NCT02440763</AccessionNumber></AccessionNumberList></DataBank></DataBankList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2015</Year><Month>09</Month><Day>13</Day></ArticleDate></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>Lancet Neurol</MedlineTA><NlmUniqueID>101139309</NlmUniqueID><ISSNLinking>1474-4422</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="C000593749">ATXN1 protein, human</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="C000593752">ATXN2 protein, human</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000067696">Ataxin-1</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000067698">Ataxin-2</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="C105656">CACNA1A protein, human</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D015220">Calcium Channels</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D012097">Repressor Proteins</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 3.4.19.12</RegistryNumber><NameOfSubstance UI="C092341">ATXN3 protein, human</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 3.4.19.12</RegistryNumber><NameOfSubstance UI="D000067699">Ataxin-3</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><CommentsCorrectionsList><CommentsCorrections RefType="CommentIn"><RefSource>Lancet Neurol. 2015 Nov;14(11):1067-9</RefSource><PMID Version="1">26377378</PMID></CommentsCorrections></CommentsCorrectionsList><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000067696" MajorTopicYN="N">Ataxin-1</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000067698" MajorTopicYN="N">Ataxin-2</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000067699" MajorTopicYN="N">Ataxin-3</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D015220" MajorTopicYN="N">Calcium Channels</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D015331" MajorTopicYN="N">Cohort Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D018450" MajorTopicYN="Y">Disease Progression</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D039781" MajorTopicYN="N">PubMed</DescriptorName><QualifierName UI="Q000706" MajorTopicYN="N">statistics & numerical data</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D012097" MajorTopicYN="N">Repressor Proteins</DescriptorName><QualifierName 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