{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T04:46:08Z","timestamp":1777869968008,"version":"3.51.4"},"reference-count":46,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:00:00Z","timestamp":1760054400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:00:00Z","timestamp":1760054400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","award":["HEALTH\u2010F2\u2010 2010\u2010242193"],"award-info":[{"award-number":["HEALTH\u2010F2\u2010 2010\u2010242193"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100018556","name":"Voyager Therapeutics","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100018556","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":[[2026,1]]},"abstract":"Abstract<\/jats:title>\n \n Background<\/jats:title>\n Friedreich's ataxia is a rare, neurodegenerative, multisystem disorder. While ataxia is a hallmark, non\u2010ataxia signs, including muscle weakness, spasticity, and dysphagia are equally disabling. The Inventory of Non\u2010Ataxia Signs (INAS) is a symptom list transformable to a 16\u2010item count.<\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To evaluate the responsiveness of a modified INAS in this population.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Participants were drawn from the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS). The modified\n INAS<\/jats:styled-content>\n count (presence\/absence, 0\u201316 scale) and modified\n INAS<\/jats:styled-content>\n sum (severity\u2010weighted, 0\u201384 scale) were evaluated using linear mixed\u2010models and standardized response means (\n SRMs<\/jats:styled-content>\n ). Items rare (<5%) and uncharacteristic in Friedreich's ataxia were excluded (chorea, myoclonus, fasciculations, resting tremor, rigidity)\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n A total of 1129 participants (mean age, 32.3\u2009years) were assessed for up to 12\u2009years. The mean modified INAS count was 4.6 (\u00b12.2) and modified INAS sum 15.1 (\u00b1\u20099.9). Both correlated strongly with existing outcome measures. Longitudinally, the modified INAS count increased by 0.13 points\/year (95% CI 0.12, 0.14;\n P<\/jats:italic>\n \u2009<\u20090.001) and modified INAS sum by 0.68 points\/year (95% CI 0.64, 0.72;\n P<\/jats:italic>\n \u2009<\u20090.001). The modified INAS sum demonstrated greater responsiveness, with SRMs of 0.26, 0.38, 0.53, and 0.80 at 1, 2, 3, and 5\u2009years, respectively, compared with 0.16, 0.27, 0.31, and 0.46 for the modified INAS count. In non\u2010ambulatory patients and children, responsiveness of the modified INAS sum was higher (SRM 0.82 and 1.7 at 5\u2009years, respectively).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n The modified INAS sum showed good responsiveness over 5\u2009years but not over 1\u20133\u2009years. It may supplement existing outcome measures, contributing to holistic assessment of this multisystem disease, especially in non\u2010ambulatory patients, in whom ataxia\u2010focused measures may show ceiling effects, and children, who typically progress faster. \u00a9 2025 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.70084","type":"journal-article","created":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T09:29:51Z","timestamp":1760088591000},"page":"200-211","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Analysis of a Modified Version of the Inventory of Non\u2010Ataxia Signs Over 12\u2009Years in Patients with Friedreich's Ataxia in the\n EFACTS<\/scp>\n Study"],"prefix":"10.1002","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1165-9153","authenticated-orcid":false,"given":"Stella Andrea","family":"Lischewski","sequence":"first","affiliation":[{"name":"Department of Neurology RWTH Aachen University Aachen Germany"},{"name":"JARA\u2010BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum J\u00fclich GmbH J\u00fclich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Imis","family":"Dogan","sequence":"additional","affiliation":[{"name":"Department of Neurology RWTH Aachen University Aachen Germany"},{"name":"JARA\u2010BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum J\u00fclich GmbH J\u00fclich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3508-4788","authenticated-orcid":false,"given":"Paola","family":"Giunti","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Department of Clinical and Movement Neurosciences UCL\u2010Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael H.","family":"Parkinson","sequence":"additional","affiliation":[{"name":"Ataxia Centre, Department of Clinical and Movement Neurosciences UCL\u2010Queen Square Institute of Neurology London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2405-3564","authenticated-orcid":false,"given":"Caterina","family":"Mariotti","sequence":"additional","affiliation":[{"name":"Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandra","family":"Durr","sequence":"additional","affiliation":[{"name":"Sorbonne Universite, Paris Brain Institute, ICM Institut du Cerveau, AP\u2010HP, INSERM, CNRS, University Hospital Piti\u00e9\u2010Salp\u00eatri\u00e8re Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4864-6287","authenticated-orcid":false,"given":"Claire","family":"Ewenczyk","sequence":"additional","affiliation":[{"name":"Sorbonne Universite, Paris Brain Institute, ICM Institut du Cerveau, AP\u2010HP, INSERM, CNRS, University Hospital Piti\u00e9\u2010Salp\u00eatri\u00e8re Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvia","family":"Boesch","sequence":"additional","affiliation":[{"name":"Department of Neurology Medical University Innsbruck Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Nachbauer","sequence":"additional","affiliation":[{"name":"Department of Neurology Medical University Innsbruck Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Klopstock","sequence":"additional","affiliation":[{"name":"Friedrich\u2010Baur\u2010Institute, Department of Neurology, LMU University Hospital Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Munich Germany"},{"name":"Munic Cluster for Systems Neurology (SynNergy) Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Stendel","sequence":"additional","affiliation":[{"name":"Friedrich\u2010Baur\u2010Institute, Department of Neurology, LMU University Hospital Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisco Javier Rodr\u00edguez","family":"de Rivera Garrido","sequence":"additional","affiliation":[{"name":"Reference Unit of Hereditary Ataxias and Paraplegias, Department of Neurology, IdiPAZ, Hospital Universitario La Paz Madrid 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