{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T06:49:54Z","timestamp":1784616594208,"version":"3.55.0"},"reference-count":29,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,8,9]],"date-time":"2021-08-09T00:00:00Z","timestamp":1628467200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The Hammersmith Functional Motor Scale Expanded (HFMSE) and the Revised Upper Limb Module (RULM) have been widely used in natural history studies and clinical trials. Our aim was to establish how the scales relate to each other at different age points in spinal muscular atrophy (SMA) type 2 and 3, and to describe their coherence over 12\u2009mo.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The study was performed by cross\u2010sectional and longitudinal reanalysis of previously published natural history data. The longitudinal analysis of the 12\u2010mo changes also included the analysis of concordance between scales with changes grouped as stable (\u00b12 points), improved (&gt;+2) or declined (&gt;\u22122).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Three hundred sixty\u2010four patients were included in the cross\u2010sectional analysis, showing different trends in score and point of slope change for the two scales. For type 2, the point of slope change was 4.1\u00a0y for the HFMSE and 5.8 for the RULM, while for type 3, it was 6\u00a0y for the HFMSE and 7.3 for the RULM. One\u2010hundred\u2010twenty\u2010one patients had at least two assessments at 12 mo. Full concordance was found in 57.3% of the assessments, and in 40.4% one scale remained stable and the other changed. Each scale appeared to be more sensitive to specific age or functional subgroups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The two scales, when used in combination, may increase the sensitivity to detect clinically meaningful changes in motor function in patients with SMA types 2 and 3.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27384","type":"journal-article","created":{"date-parts":[[2021,7,30]],"date-time":"2021-07-30T07:43:29Z","timestamp":1627631009000},"page":"552-559","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Different trajectories in upper limb and gross motor function in spinal muscular atrophy"],"prefix":"10.1002","volume":"64","author":[{"given":"Giorgia","family":"Coratti","sequence":"first","affiliation":[{"name":"Pediatric Neurology Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Carmela","family":"Pera","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"},{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Montes","sequence":"additional","affiliation":[{"name":"Departments of Rehabilitation and Regenerative Medicine and Neurology Columbia University Irving Medical Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Pasternak","sequence":"additional","affiliation":[{"name":"Department of Neurology, Boston Children\u02bcs Hospital Harvard Medical School  Boston Massachusetts United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariacristina","family":"Scoto","sequence":"additional","affiliation":[{"name":"Dubowitz Neuromuscular Centre UCL Institute of Child Health &amp; Great Ormond Street Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Baranello","sequence":"additional","affiliation":[{"name":"Dubowitz Neuromuscular Centre UCL Institute of Child Health &amp; Great Ormond Street Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7994-3391","authenticated-orcid":false,"given":"Sonia","family":"Messina","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine and Centro Clinico Nemo Sud University of Messina  Messina Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sally","family":"Dunaway Young","sequence":"additional","affiliation":[{"name":"Department of Neurology Stanford University  Stanford California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allan M.","family":"Glanzman","sequence":"additional","affiliation":[{"name":"Department of Physical Therapy Children\u02bcs Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tina","family":"Duong","sequence":"additional","affiliation":[{"name":"Department of Neurology Stanford University  Stanford California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"De Sanctis","sequence":"additional","affiliation":[{"name":"Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena Stacy","family":"Mazzone","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelin","family":"Milev","sequence":"additional","affiliation":[{"name":"Dubowitz Neuromuscular Centre UCL Institute of Child Health &amp; 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