{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T04:08:21Z","timestamp":1791432501533,"version":"4.3.3"},"reference-count":32,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,8,9]],"date-time":"2025-08-09T00:00:00Z","timestamp":1754697600000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004243","name":"Prinses Beatrix Spierfonds","doi-asserted-by":"publisher","award":["W.OR12\u201022"],"award-info":[{"award-number":["W.OR12\u201022"]}],"id":[{"id":"10.13039\/501100004243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004243","name":"Prinses Beatrix Spierfonds","doi-asserted-by":"publisher","award":["W.OR18\u201007"],"award-info":[{"award-number":["W.OR18\u201007"]}],"id":[{"id":"10.13039\/501100004243","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess changes in the facioscapulohumeral muscular dystrophy Rasch\u2010Built Overall Disability Scale (FSHD\u2010RODS) over 6.5\u2009years in FSHD patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>FSHD patients of 18\u2009years or older were assessed at baseline (T1) and followed up at 5\u2009years (T2) and 6.5\u2009years (T3). The patient\u2010reported FSHD\u2010RODS and Sickness Impact Profile 68 (SIP68) questionnaires were evaluated, alongside the FSHD clinical severity score, FSHD clinical score and Motor Function Measure (MFM). Individual minimal clinically important differences (MCID\u2010SE) of the FSHD\u2010RODS were calculated to assess the clinical importance of the change over time. A subgroup analysis included patients eligible for current clinical trials.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010two patients were included, representing the full disease spectrum. The FSHD\u2010RODS and SIP68 questionnaire outcomes remained stable, whereas overall disease severity slightly increased (FSHD clinical severity score 6 to 6.5, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) and MFM slightly decreased (94% to 91%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). FSHD\u2010RODS correlated strongly with SIP68, MFM and disease severity (<jats:italic>\u03c1<\/jats:italic>\u2009\u2265\u20090.69). The clinical trial subgroup (<jats:italic>n<\/jats:italic>\u2009=\u200933) showed similar results. Ten patients (16%), all with moderate\u2013to\u2010severe baseline disease (FSHD clinical severity score \u2265\u20096), showed clinically important deterioration over 6.5\u2009years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>FSHD progression is generally slow, with FSHD\u2010RODS only detecting meaningful decline in moderately to severely affected patients. The ability to compensate for muscle weakness may limit the sensitivity of patient\u2010reported outcomes like FSHD\u2010RODS in clinical trials. These findings highlight the need for alternative measures to capture disease progression and treatment effects in FSHD research.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70238","type":"journal-article","created":{"date-parts":[[2025,8,9]],"date-time":"2025-08-09T08:41:56Z","timestamp":1754728916000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["The Facioscapulohumeral Muscular Dystrophy Rasch\u2010Built Overall Disability Scale (<scp>FSHD<\/scp>\u2010<scp>RODS<\/scp>): Longitudinal Assessment of a Disease\u2010Specific Patient Reported Outcome"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1023-2182","authenticated-orcid":false,"given":"Sjan","family":"Teeselink","sequence":"first","affiliation":[{"name":"Department of Neurology Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0412-0670","authenticated-orcid":false,"given":"Sanne C. 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