{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T16:22:33Z","timestamp":1787674953517,"version":"build-2784847793"},"reference-count":29,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,6,7]],"date-time":"2024-06-07T00:00:00Z","timestamp":1717718400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2024,6,7]],"date-time":"2024-06-07T00:00:00Z","timestamp":1717718400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["441409627"],"award-info":[{"award-number":["441409627"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003042","name":"Else Kr\u00f6ner-Fresenius-Stiftung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003042","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009397","name":"Medizinischen Fakult\u00e4t, Eberhard Karls Universit\u00e4t T\u00fcbingen","doi-asserted-by":"publisher","award":["439\u20100\u20100"],"award-info":[{"award-number":["439\u20100\u20100"]}],"id":[{"id":"10.13039\/501100009397","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RC 5\u00d71000"],"award-info":[{"award-number":["RC 5\u00d71000"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF\u20102019\u2010 12370417"],"award-info":[{"award-number":["RF\u20102019\u2010 12370417"]}],"id":[{"id":"10.13039\/501100003196","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":[[2024,9]]},"abstract":"Abstract<\/jats:title>\n \n Background<\/jats:title>\n With treatment trials on the horizon, this study aimed to identify candidate digital\u2010motor gait outcomes for autosomal recessive spastic ataxia of Charlevoix\u2010Saguenay (ARSACS), capturable by wearable sensors with multicenter validity, and ideally also ecological validity during free walking outside laboratory settings.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Cross\u2010sectional multicenter study (four centers), with gait assessments in 36 subjects (18 ARSACS patients; 18 controls) using three body\u2010worn sensors (Opal, APDM) in laboratory settings and free walking in public spaces. Sensor gait measures were analyzed for discriminative validity from controls, and for convergent (ie, clinical and patient relevance) validity by correlations with SPRS\n mobility<\/jats:sup>\n (primary outcome) and Scale for the Assessment and Rating of Ataxia (SARA), Spastic Paraplegia Rating Scale (SPRS), and activities of daily living subscore of the Friedreich Ataxia Rating Scale (FARS\u2010ADL) (exploratory outcomes).\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Of 30 hypothesis\u2010based digital gait measures, 14 measures discriminated ARSACS patients from controls with large effect sizes (|Cliff's \u03b4|\u2009>\u20090.8) in laboratory settings, with strongest discrimination by measures of spatiotemporal variability Lateral Step Deviation (\u03b4\u2009=\u20090.98), SPcmp (\u03b4\u2009=\u20090.94), and Swing CV (\u03b4\u2009=\u20090.93). Large correlations with the SPRS\n mobility<\/jats:sup>\n were observed for Swing CV (Spearman's \u03c1\u2009=\u20090.84), Speed (\u03c1\u2009=\u2009\u22120.63), and Harmonic Ratio V (\u03c1\u2009=\u2009\u22120.62). During supervised free walking in a public space, 11\/30 gait measures discriminated ARSACS from controls with large effect sizes. Large correlations with SPRS\n mobility<\/jats:sup>\n were here observed for Swing CV (\u03c1\u2009=\u20090.78) and Speed (\u03c1\u2009=\u2009\u22120.69), without reductions in effect sizes compared with laboratory settings.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n We identified a promising set of digital\u2010motor candidate gait outcomes for ARSACS, applicable in multicenter settings, correlating with patient\u2010relevant health aspects, and with high validity also outside laboratory settings, thus simulating real\u2010life walking with higher ecological validity. \u00a9 2024 The Author(s).\n Movement Disorders<\/jats:italic>\n published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/mds.29876","type":"journal-article","created":{"date-parts":[[2024,6,7]],"date-time":"2024-06-07T08:11:11Z","timestamp":1717747871000},"page":"1544-1555","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Digital Gait Outcomes for Autosomal Recessive Spastic Ataxia of Charlevoix\u2010Saguenay (\n ARSACS)<\/scp>\n : Discriminative, Convergent, and Ecological Validity in a Multicenter Study (\n PROSPAX<\/scp>\n )"],"prefix":"10.1002","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-9259-9230","authenticated-orcid":false,"given":"Lukas","family":"Beichert","sequence":"first","affiliation":[{"name":"Division Translational Genomics of Neurodegenerative Diseases Hertie\u2010Institute for Clinical Brain Research and Center for Neurology, University of T\u00fcbingen T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE), University of T\u00fcbingen T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1537-1336","authenticated-orcid":false,"given":"Winfried","family":"Ilg","sequence":"additional","affiliation":[{"name":"Section Computational Sensomotorics, Hertie Institute for Clinical Brain Research T\u00fcbingen Germany"},{"name":"Centre for Integrative Neuroscience (CIN) T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Kessler","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases Hertie\u2010Institute for Clinical Brain Research and Center for Neurology, University of T\u00fcbingen T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8165-5898","authenticated-orcid":false,"given":"Andreas","family":"Trasch\u00fctz","sequence":"additional","affiliation":[{"name":"Division Translational Genomics of Neurodegenerative Diseases Hertie\u2010Institute for Clinical Brain Research and Center for Neurology, University of T\u00fcbingen T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE), University of T\u00fcbingen T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selina","family":"Reich","sequence":"additional","affiliation":[{"name":"Division Translational Genomics of Neurodegenerative Diseases Hertie\u2010Institute for Clinical Brain Research and Center for Neurology, University of T\u00fcbingen T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE), University of T\u00fcbingen T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo M.","family":"Santorelli","sequence":"additional","affiliation":[{"name":"Molecular Medicine, IRCCS Fondazione Stella Maris Pisa 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