Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3,Movement Disorders

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Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3
Movement Disorders ( IF 8.6 ) Pub Date : 2022-07-08 , DOI: 10.1002/mds.29135
Roderick P P W M Maas ₁ , Steven Teerenstra ₂ , Manuela Lima _{3,

4} , Paula Pires ₅ , Luís Pereira de Almeida _{6,

7,

8} , Judith van Gaalen ₁ , Dagmar Timmann ₉ , Jon Infante ₁₀ , Chiadi Onyike ₁₁ , Khalaf Bushara ₁₂ , Heike Jacobi ₁₃ , Kathrin Reetz _{14,

15} , Magda M Santana _{6,

7} , Joana Afonso Ribeiro ₁₆ , Jeannette Hübener-Schmid ₁₇ , Jeroen J de Vries _{18,

19} , Matthis Synofzik _{20,

21} , Ludger Schöls _{20,

21} , Hector Garcia-Moreno _{22,

23} , Paola Giunti _{22,

23} , Jennifer Faber _{24,

25} , Thomas Klockgether _{24,

25} , Bart P C van de Warrenburg ₁

Affiliation

Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
Department for Health Evidence, Biostatistics Section, Radboud University Medical Center, Nijmegen, The Netherlands.
Faculdade de Ciências e Tecnologia, Universidade dos Açores, Azores, Portugal.
Instituto de Biologia Molecular e Celular (IBMC), Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Porto, Portugal.
Department of Neurology, Hospital Santo Espírito da ilha Terceira, Azores, Portugal.
Center for Neuroscience and Cell Biology (CNC), University of Coimbra, Coimbra, Portugal.
Center for Innovation in Biomedicine and Biotechnology (CIBB), University of Coimbra, Coimbra, Portugal.
Faculty of Pharmacy, University of Coimbra, Coimbra, Portugal.
Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), Essen University Hospital, University of Duisburg-Essen, Essen, Germany.
Neurology Service, Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CINERNED), University Hospital Marques de Valdecilla-IDIVAL, University of Cantabria-UC, Santander, Spain.
Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Ataxia Center, Department of Neurology, University of Minnesota, Minneapolis, Minnesota, USA.
Department of Neurology, University Hospital Heidelberg, Heidelberg, Germany.
Department of Neurology, RWTH Aachen University, Aachen, Germany.
JARA-BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH and RWTH Aachen University, Aachen, Germany.
Department of Neurology, Child Development Centre, Coimbra's Hospital and University Centre, Coimbra, Portugal.
Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.
Department of Neurology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Expertise Center Movement Disorders Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Department of Neurodegenerative Diseases, Center for Neurology and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
German Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.
Department of Neurogenetics, National Hospital for Neurology and Neurosurgery, University College London Hospital NHS Foundation Trust, London, United Kingdom.
Department of Neurology, University Hospital Bonn, Bonn, Germany.
German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.

Disease severity in spinocerebellar ataxia type 3 (SCA3) is commonly defined by the Scale for the Assessment and Rating of Ataxia (SARA) sum score, but little is known about the contributions and progression patterns of individual items.

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