{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,13]],"date-time":"2025-10-13T15:39:32Z","timestamp":1760369972195,"version":"3.40.5"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T00:00:00Z","timestamp":1705536000000},"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":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Current upper limb assessments in pediatric spinal muscular atrophy (SMA) may not adequately capture change with disease progression. Our aim was to examine the relationship between motor function, strength, and hand\/finger mobility of the upper limb in treatment\u2010na\u00efve children with SMA Types 2 and 3 to assess new methods to supplement current outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The Revised Upper Limb Module (RULM), grip and pinch strength, and hand\/finger mobility data were collected from 19 children with SMA Types 2 and 3 aged 5.2\u201316.9\u2009years over a year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A median loss between 0.5 and 2.5 points in the RULM was seen across all SMA subgroups with the biggest median loss recorded between 10 and 14\u2009years of age. The grip strength loss was \u22120.06\u2009kg (\u22124.69 to 3.49; IQR, 1.21); pinch improvement of 0.05 (\u22120.65 to 1.27; IQR, 0.48); hand\/finger mobility test improvement of 4 points (\u221224 to 14; IQR, 6.75) for the whole cohort. Significant correlations were found between the RULM and grip strength (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001), RULM and pinch strength (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001), RULM and revised Brooke (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001), grip strength and pinch strength (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The combined use of the RULM, dynamometry, and hand mobility provide insight about correlations between function and strength in children with SMA. The RULM and grip strength assessments captured a significant decline in upper limb function, whereas the pinch and finger\/hand mobility showed an improvement over the course of 1\u2009year and these results should be considered for future studies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28041","type":"journal-article","created":{"date-parts":[[2024,1,19]],"date-time":"2024-01-19T05:29:13Z","timestamp":1705642153000},"page":"340-348","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Assessment of the upper limb function, strength, and mobility in treatment\u2010naive children with spinal muscular atrophy <scp>Types<\/scp> 2 and 3"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7129-1824","authenticated-orcid":false,"given":"Evelin","family":"Milev","sequence":"first","affiliation":[{"name":"Department of Developmental Neuroscience Dubowitz Neuromuscular Centre, UCL Great 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