{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T18:12:05Z","timestamp":1792260725912,"version":"4.4.0"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T00:00:00Z","timestamp":1769040000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T00:00:00Z","timestamp":1769040000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000346","name":"Ataxia UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000346","id-type":"DOI","asserted-by":"publisher"}]},{"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\/100021828","name":"German Academic Exchange Service","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100021828","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Forschung, Technologie und Raumfahrt","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"ABSTRACT<\/jats:title>\n \n Objective<\/jats:title>\n \n Spinocerebellar ataxia type 3 (SCA3) is a genetically defined ataxia. The Scale for Assessment and Rating of Ataxia (SARA) is a clinician\u2010reported outcome that measures ataxia severity at a single time point. In its standard application, SARA fails to capture short\u2010term fluctuations, limiting its sensitivity in trials. To overcome this, we employed SARA\n home<\/jats:sup>\n , a video\u2010based, self\u2010administered tool for high\u2010frequency, remote ataxia assessment.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n We assessed feasibility and validity of SARA\n home<\/jats:sup>\n in 65 SCA3 patients from seven centers. Participants recorded SARA\n home<\/jats:sup>\n twice daily for 14\u2009days using a mobile e\u2010health app. We analyzed adherence, intraindividual fluctuations and their predictors, and evaluated sensitivity to change in a longitudinal substudy of 11 patients.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Adherence to the study protocol was generally high (80.2%) with valid scores in 79.2% of 1459 recordings. Maximum adherence occurred over a 4\u2010day period (84.8%). Fluctuations ranged 3.0 points between lowest and highest scores (IQR: 2.5\u20134.5) and 1.0 point based on score IQRs (IQR: 0.5\u20131.5), corresponding to 10.7% and 3.6% of the maximal SARA\n home<\/jats:sup>\n score. Fluctuations showed rough agreement with patient global impression. Greater disease severity and longer CAG repeats were associated with smaller relative fluctuations. Over a median follow\u2010up of 411\u2009days, SARA\n home<\/jats:sup>\n showed higher sensitivity to change than conventional SARA (SRM: 0.67 vs. 0.37).\n <\/jats:p>\n <\/jats:sec>\n \n Interpretation<\/jats:title>\n \n SARA\n home<\/jats:sup>\n is a feasible, innovative video\u2010based tool for remote, high\u2010frequency monitoring of ataxia severity. A 4\u2010day recording effectively captures relevant fluctuations and enhances sensitivity to change, supporting its use in future SCA3 trials.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/acn3.70316","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T11:36:47Z","timestamp":1769081807000},"page":"1370-1378","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Remote Assessment of Ataxia Severity in\n SCA3<\/scp>\n Across Multiple Centers and Time Points"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1808-2134","authenticated-orcid":false,"given":"Marcus","family":"Grobe\u2010Einsler","sequence":"first","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn Germany"},{"name":"Department of Neurology, Center for Movement Disorders and Neuromodulation, Medical Faculty Heinrich\u2010Heine University D\u00fcsseldorf Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivian","family":"Maas","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arian Taheri","family":"Amin","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn Germany"},{"name":"Department of Radiology, Medical Faculty Heinrich\u2010Heine University D\u00fcsseldorf Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Faber","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamara","family":"Schaprian","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Hill","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2280-7273","authenticated-orcid":false,"given":"Matthis","family":"Synofzik","sequence":"additional","affiliation":[{"name":"Center for Neurology and Hertie Institute for Clinical Brain Research University of T\u00fcbingen T\u00fcbingen Germany"},{"name":"German Center of Neurodegenerative Diseases (DZNE) T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa H.","family":"Graf","sequence":"additional","affiliation":[{"name":"Center for Neurology and Hertie Institute for Clinical Brain Research University of T\u00fcbingen T\u00fcbingen Germany"},{"name":"German Center of Neurodegenerative Diseases (DZNE) T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Lima","sequence":"additional","affiliation":[{"name":"Faculdade de Ci\u00eancias e Tecnologia Universidade dos A\u00e7ores Ponta Delgada Portugal"},{"name":"UMIB\u2014Unit for Multidisciplinary Research in Biomedicine, ICBAS\u2014School of Medicine and Biomedical Sciences, University of Porto Porto Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana F.","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Faculdade de Ci\u00eancias e Tecnologia Universidade dos A\u00e7ores Ponta Delgada Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bart P.","family":"van de Warrenburg","sequence":"additional","affiliation":[{"name":"Radboud University Medical Center Department of Neurology Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilse H. J.","family":"Willemse","sequence":"additional","affiliation":[{"name":"Radboud University Medical Center Department of Neurology Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Thieme","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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Giunti","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hector","family":"Garcia\u2010Moreno","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magda M.","family":"Santana","sequence":"additional","affiliation":[{"name":"Center for Neuroscience and Cell Biology University of Coimbra (CNC\u2010UC) Coimbra Portugal"},{"name":"Center for Innovative in Biomedicine and Biotechnology (CIBB), University of Coimbra Coimbra Portugal"},{"name":"Gene Therapy Center of Excellence (GeneT) Coimbra Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4973-0923","authenticated-orcid":false,"given":"Jeannette","family":"H\u00fcbener\u2010Schmid","sequence":"additional","affiliation":[{"name":"Institute for Medical Genetics and Applied Genomics University of Tuebingen Tuebingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elin H.","family":"Davies","sequence":"additional","affiliation":[{"name":"Aparito \u2010 a Wholly Owned Subsidiary of Eli Lilly and Company Wrexham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Klockgether","sequence":"additional","affiliation":[{"name":"German Center for Neurodgenerative Diseases (DZNE) Bonn Germany"},{"name":"Center for Neurology, Department of Parkinson's Disease, Sleep and Movement Disorders, University Hospital Bonn Bonn 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