{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T22:26:44Z","timestamp":1767652004863,"version":"3.37.3"},"reference-count":38,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T00:00:00Z","timestamp":1671580800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Trust and University College London","award":["515048"],"award-info":[{"award-number":["515048"]}]},{"DOI":"10.13039\/100011708","name":"SMA Trust","doi-asserted-by":"publisher","award":["13\/LO\/1748; 07DN02; 37787"],"award-info":[{"award-number":["13\/LO\/1748; 07DN02; 37787"]}],"id":[{"id":"10.13039\/100011708","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005614","name":"Biogen","doi-asserted-by":"publisher","award":["13\/LO\/1748"],"award-info":[{"award-number":["13\/LO\/1748"]}],"id":[{"id":"10.13039\/100005614","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008164","name":"Muscular Dystrophy UK","doi-asserted-by":"publisher","award":["07DN02; 37787"],"award-info":[{"award-number":["07DN02; 37787"]}],"id":[{"id":"10.13039\/501100008164","id-type":"DOI","asserted-by":"publisher"}]},{"name":"MRC Translational Research Centre at UCL and Newcastle","award":["MR\/K501074\/1"],"award-info":[{"award-number":["MR\/K501074\/1"]}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>The Revised Hammersmith Scale (RHS) for Spinal Muscular Atrophy (SMA) was designed as a psychometrically robust clinical outcome assessment to assess physical abilities of patients with type 2 and 3 SMA. The reliability properties of the RHS have not yet been reported. A prospective RHS reliability study was undertaken in a UK cohort of experienced neuromuscular paediatric Physiotherapists. Reliability testing was conducted via a virtual survey platform two weeks apart. Through the virtual platform participants scored videos of two RHS assessments, one of a child with SMA 2 and one of a child with SMA 3. Inter and intra-rater reliability was analysed using a type 3 Intraclass Correlation Coefficient (ICC). Intra-rater agreement was further analysed using Bland Altman (BA) Limits of Agreement (LOA) and plots. The acceptable inter and intra-rater variability was set as a change of \u00b1 2 by the international team of expert physiotherapists who developed the RHS. Inter-rater agreement, n = 22 raters, type 3 ICC was 0.989 (95% CI 0.944 to 1.00), 97.7% of scores were within the acceptable limits of \u00b1 2 points. Intra-rater agreement, n = 21 raters, type 3 ICC ranged from 0.922 to 1.0, with 97.6% of scores within the acceptable limits of \u00b1 2 points. The mean SMA 2 intra-rater difference was -0.10 (-0.6 to 0.4), with lower LOA -2.24 and upper LOA +2.04. Intra-rater difference between tests for SMA 3 intra-rater difference was -0.05 (-0.6 to 0.5), with lower LOA -2.48 and upper LOA +2.38. Intra-rater scoring precision fell within BA agreement limits of \u00b12 points. The results demonstrate that the RHS is highly reliable when used by experienced UK physiotherapists, and variability of test scores regarding inter and intra-rater reliability was confirmed to lie within \u00b12 points.<\/jats:p>","DOI":"10.1371\/journal.pone.0278996","type":"journal-article","created":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T18:29:45Z","timestamp":1671647385000},"page":"e0278996","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":6,"title":["Revised Hammersmith Scale for spinal muscular atrophy: Inter and intra-rater reliability and agreement"],"prefix":"10.1371","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1275-2640","authenticated-orcid":true,"given":"Danielle","family":"Ramsey","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gita","family":"Ramdharry","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariacristina","family":"Scoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9102-5232","authenticated-orcid":true,"given":"Francesco","family":"Muntoni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda","family":"Wallace","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"on behalf of the SMA REACH UK network","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2022,12,21]]},"reference":[{"key":"pone.0278996.ref001","first-page":"591","volume-title":"Handbook of Clinical Neurology","author":"ES Arnold","year":"2018"},{"key":"pone.0278996.ref002","first-page":"145","volume-title":"Neuromuscular Disorders of Infancy","author":"TO Crawford","year":"2003"},{"issue":"4","key":"pone.0278996.ref003","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1038\/nrneurol.2018.4","article-title":"Advances in therapy for spinal muscular atrophy: promises and challenges","volume":"14","author":"EJN Groen","year":"2018","journal-title":"Nature Rev Neurol"},{"issue":"5","key":"pone.0278996.ref004","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/S1474-4422(12)70061-3","article-title":"Childhood spinal muscular atrophy: controversies and challenges","volume":"11","author":"E Mercuri","year":"2012","journal-title":"Lancet Neurol"},{"key":"pone.0278996.ref005","first-page":"117","volume-title":"Neuromuscular Disorders of Infancy, Childhood, and Adolescence","author":"BT Darras","year":"2015","edition":"2"},{"issue":"4","key":"pone.0278996.ref006","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1007\/s40265-017-0711-7","article-title":"Nusinersen: First Global Approval","volume":"77","author":"SM Hoy","year":"2017","journal-title":"Drugs"},{"issue":"9","key":"pone.0278996.ref007","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1038\/gt.2017.47","article-title":"The SMA Trust: the role of a disease-focused research charity in developing treatments for SMA","volume":"24","author":"V Christie-Brown","year":"2017","journal-title":"Gene Ther"},{"key":"pone.0278996.ref008","unstructured":"European Medicines Agency. Assessment report: Spinraza. Committee for Medicinal Products for Human Use (CHMP); 2017 April 21. [Cited 2021 October 29]. 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Managed Access Agreement\u2013Nusinersen (Spinraza) for the treatment of 5q Spinal Muscular Atrophy. 2019 July. [Cited 2021 October 29]. Available from: https:\/\/www.nice.org.uk\/guidance\/ta588\/resources\/managed-access-agreement-july-2019-pdf-6842812573."},{"key":"pone.0278996.ref020","unstructured":"National Insitute for Health and Care Excellence (NICE). Nusinersen for treating spinal muscular atrophy. 2019 July 24. [Cited 2021 October 29]. Available from: https:\/\/www.nice.org.uk\/guidance\/ta588."},{"journal-title":"Scholar Rock Announces Initiation of Patient Dosing in Phase 2 Trial of SRK-015 in Spinal Muscular Atrophy GlobeNewswire.","year":"2019","author":"Scholar Rock","key":"pone.0278996.ref021"},{"issue":"1","key":"pone.0278996.ref022","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/j.jclinepi.2010.03.002","article-title":"Guidelines for Reporting Reliability and Agreement Studies (GRRAS) were proposed.","volume":"64","author":"J Kottner","year":"2011","journal-title":"J Clin Epidemiol."},{"key":"pone.0278996.ref023","volume-title":"BMJ Books","author":"MD Altman D","year":"2013","edition":"2"},{"key":"pone.0278996.ref024","unstructured":"UCL. Opinio 2021. Available from: https:\/\/www.ucl.ac.uk\/isd\/services\/learning-teaching\/e-learning-services-for-staff\/e-learning-core-tools\/opinio."},{"key":"pone.0278996.ref025","unstructured":"Department of Health. Information Governance Toolkit. 2015. 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