{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,4]],"date-time":"2026-04-04T14:24:04Z","timestamp":1775312644309,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T00:00:00Z","timestamp":1657238400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100002243","name":"National Ataxia Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007155","name":"Medical Research Council Canada","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007155","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008382","name":"Fundo Regional para a Ci\u00eancia e Tecnologia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008382","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01ED1602A\/B"],"award-info":[{"award-number":["01ED1602A\/B"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013278","name":"EU Joint Programme \u2013 Neurodegenerative Disease Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013278","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To investigate the temporal dynamics of SARA item scores in SCA3 patients and evaluate if clinical and demographic factors are differentially associated with evolution of axial and appendicular ataxia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In a prospective, multinational cohort study involving 11 European and 2 US sites, SARA scores were determined longitudinally in 223 SCA3 patients with a follow\u2010up assessment after 1\u2009year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>An increase in SARA score from 10 to 20 points was mainly driven by axial and speech items, with a markedly smaller contribution of appendicular items. Finger chase and nose\u2010finger test scores not only showed the lowest variability at baseline, but also the least deterioration at follow\u2010up. Compared with the full set of SARA items, omission of both tests would result in lower sample size requirements for therapeutic trials. Sex was associated with change in SARA sum score and appendicular, but not axial, subscore, with a significantly faster progression in men. Despite considerable interindividual variability, the average annual progression rate of SARA score was approximately three times higher in subjects with a disease duration over 10\u2009years than in those within 10\u2009years from onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our findings provide evidence for a difference in temporal dynamics between axial and appendicular ataxia in SCA3 patients, which will help inform the design of clinical trials and development of new (etiology\u2010specific) outcome measures. \u00a9 2022 The Authors. <jats:italic>Movement Disorders<\/jats:italic> published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.29135","type":"journal-article","created":{"date-parts":[[2022,7,9]],"date-time":"2022-07-09T01:15:25Z","timestamp":1657329325000},"page":"1850-1860","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Differential Temporal Dynamics of Axial and Appendicular Ataxia in <scp>SCA3<\/scp>"],"prefix":"10.1002","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0823-1287","authenticated-orcid":false,"given":"Roderick P.P.W.M.","family":"Maas","sequence":"first","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven","family":"Teerenstra","sequence":"additional","affiliation":[{"name":"Department for Health Evidence, Biostatistics Section Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manuela","family":"Lima","sequence":"additional","affiliation":[{"name":"Faculdade de Ci\u00eancias e Tecnologia Universidade dos A\u00e7ores  Azores Portugal"},{"name":"Instituto de Biologia Molecular e Celular (IBMC), Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade (i3S) Universidade do Porto  Porto Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paula","family":"Pires","sequence":"additional","affiliation":[{"name":"Department of Neurology Hospital Santo Esp\u00edrito da ilha Terceira  Azores Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5831-3307","authenticated-orcid":false,"given":"Lu\u00eds","family":"Pereira de Almeida","sequence":"additional","affiliation":[{"name":"Center for Neuroscience and Cell Biology (CNC) University of Coimbra  Coimbra Portugal"},{"name":"Center for Innovation in Biomedicine and Biotechnology (CIBB) University of Coimbra  Coimbra Portugal"},{"name":"Faculty of Pharmacy University of Coimbra  Coimbra Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Judith","family":"van Gaalen","sequence":"additional","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dagmar","family":"Timmann","sequence":"additional","affiliation":[{"name":"Department of Neurology and Center for Translational Neuro\u2010 and Behavioral Sciences (C\u2010TNBS), Essen University Hospital University of Duisburg\u2010Essen  Essen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4025-4606","authenticated-orcid":false,"given":"Jon","family":"Infante","sequence":"additional","affiliation":[{"name":"Neurology Service, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CINERNED), University Hospital Marques de Valdecilla\u2010IDIVAL University of Cantabria\u2010UC  Santander Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiadi","family":"Onyike","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Behavioral Sciences Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khalaf","family":"Bushara","sequence":"additional","affiliation":[{"name":"Ataxia Center, Department of Neurology University of Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6986-3969","authenticated-orcid":false,"given":"Heike","family":"Jacobi","sequence":"additional","affiliation":[{"name":"Department of Neurology University Hospital Heidelberg  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9730-9228","authenticated-orcid":false,"given":"Kathrin","family":"Reetz","sequence":"additional","affiliation":[{"name":"Department of Neurology RWTH Aachen University  Aachen Germany"},{"name":"JARA\u2010BRAIN Institute Molecular Neuroscience and Neuroimaging Forschungszentrum J\u00fclich GmbH and RWTH Aachen University  Aachen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magda M.","family":"Santana","sequence":"additional","affiliation":[{"name":"Center for Neuroscience and Cell Biology (CNC) University of Coimbra  Coimbra Portugal"},{"name":"Center for Innovation in Biomedicine and Biotechnology (CIBB) University of Coimbra  Coimbra Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5712-3106","authenticated-orcid":false,"given":"Joana","family":"Afonso Ribeiro","sequence":"additional","affiliation":[{"name":"Department of Neurology, Child Development Centre Coimbra's Hospital and University Centre  Coimbra Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4973-0923","authenticated-orcid":false,"given":"Jeannette","family":"H\u00fcbener\u2010Schmid","sequence":"additional","affiliation":[{"name":"Institute of Medical Genetics and Applied Genomics University of T\u00fcbingen  T\u00fcbingen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeroen J.","family":"de Vries","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Center Groningen University of Groningen  Groningen The Netherlands"},{"name":"Expertise Center Movement Disorders Groningen University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2280-7273","authenticated-orcid":false,"given":"Matthis","family":"Synofzik","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, Center for Neurology and Hertie Institute for Clinical Brain Research University of T\u00fcbingen  T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE)  T\u00fcbingen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ludger","family":"Sch\u00f6ls","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, Center for Neurology and Hertie Institute for Clinical Brain Research University of T\u00fcbingen  T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE)  T\u00fcbingen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hector","family":"Garcia\u2010Moreno","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology University College London  London United Kingdom"},{"name":"Department of Neurogenetics, National Hospital for Neurology and Neurosurgery University College London Hospital NHS Foundation Trust  London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Giunti","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology University College London  London United Kingdom"},{"name":"Department of Neurogenetics, National Hospital for Neurology and Neurosurgery University College London Hospital NHS Foundation Trust  London United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"Faber","sequence":"additional","affiliation":[{"name":"Department of Neurology University Hospital Bonn  Bonn Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE)  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Klockgether","sequence":"additional","affiliation":[{"name":"Department of Neurology University Hospital Bonn  Bonn Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE)  Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bart P.C.","family":"van de Warrenburg","sequence":"additional","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,7,8]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-71779-1_11"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22132"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-019-0074-3"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000358801"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e3282f7673d"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2007.00910.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-177"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(13)70104-2"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00202-1"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001711"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000219042.60538.92"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2021.08.014"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXG.0000000000000415"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311-022-01231-w"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00201-X"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25803"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-013-0532-5"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnhum.2017.00605"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000324863.76290.19"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-012-0421-3"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181d1a6c9"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0725-y"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.28610"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brs.2021.01.020"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25297"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.28670"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2011.2713"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.4.589"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31822e7ca0"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.28313"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.29135","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mds.29135","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.29135","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T12:28:23Z","timestamp":1760963303000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.29135"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,8]]},"references-count":30,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2022,9]]}},"alternative-id":["10.1002\/mds.29135"],"URL":"https:\/\/doi.org\/10.1002\/mds.29135","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,8]]},"assertion":[{"value":"2022-02-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-06-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-08","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}