{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T09:16:32Z","timestamp":1780564592455,"version":"3.54.1"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,5,8]],"date-time":"2021-05-08T00:00:00Z","timestamp":1620432000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100013428","name":"Stichting Spieren voor Spieren","doi-asserted-by":"publisher","award":["SvS15"],"award-info":[{"award-number":["SvS15"]}],"id":[{"id":"10.13039\/501100013428","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2021,6]]},"abstract":"Abstract<\/jats:title>\n \n Background<\/jats:title>\n Clinical trials in Duchenne muscular dystrophy (DMD) focus primarily on ambulant patients. Results cannot be extrapolated to later disease stages due to a decline in targeted muscle tissue. In non\u2010ambulant DMD patients, hand function is relatively preserved and crucial for daily\u2010life activities. We used quantitative MRI (qMRI) to establish whether the thenar muscles could be valuable to monitor treatment effects in non\u2010ambulant DMD patients.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Seventeen non\u2010ambulant DMD patients (range 10.2\u201324.1\u00a0years) and 13 healthy controls (range 9.5\u201325.4 years) underwent qMRI of the right hand at 3\u00a0T at baseline. Thenar fat fraction (FF), total volume (TV), and contractile volume (CV) were determined using 4\u2010point Dixon, and T2\n water<\/jats:sub>\n was determined using multiecho spin\u2010echo. Clinical assessments at baseline (\n n<\/jats:italic>\n \u00a0=\u00a017) and 12\u00a0months (\n n<\/jats:italic>\n \u00a0=\u00a013) included pinch strength (kg), performance of the upper limb (PUL) 2.0, DMD upper limb patient reported outcome measure (PROM), and playing a video game for 10\u00a0min using a game controller. Group differences and correlations were assessed with non\u2010parametric tests.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Total volume was lower in patients compared with healthy controls (6.9\u00a0cm\n 3<\/jats:sup>\n , 5.3\u20139.0\u00a0cm\n 3<\/jats:sup>\n vs. 13.0\u00a0cm\n 3<\/jats:sup>\n , 7.6\u201315.8\u00a0cm\n 3<\/jats:sup>\n ,\n P<\/jats:italic>\n \u00a0=\u00a00.010). CV was also lower in patients (6.3\u00a0cm\n 3<\/jats:sup>\n , 4.6\u20138.3\u00a0cm\n 3<\/jats:sup>\n vs. 11.9\u00a0cm\n 3<\/jats:sup>\n , 6.9\u201314.6\u00a0cm\n 3<\/jats:sup>\n ,\n P<\/jats:italic>\n \u00a0=\u00a00.010). FF was slightly elevated (9.7%, 7.3\u201311.4% vs. 7.7%, 6.6\u20138.4%,\n P<\/jats:italic>\n \u00a0=\u00a00.043), while T2\n water<\/jats:sub>\n was higher (31.5\u00a0ms, 30.0\u201332.6\u00a0ms vs. 28.1\u00a0ms, 27.8\u201329.4\u00a0ms,\n P<\/jats:italic>\n \u00a0<\u00a00.001). Pinch strength and PUL decreased over 12\u00a0months (2.857\u00a0kg, 2.137\u20134.010 to 2.243\u00a0kg, 1.930\u20133.339\u00a0kg, and 29 points, 20\u201336 to 23 points, 17\u201330, both\n P<\/jats:italic>\n \u00a0<\u00a00.001), while PROM did not (49 points, 36\u201357 to 44 points, 30\u201354,\n P<\/jats:italic>\n \u00a0=\u00a00.041). All patients were able to play for 10\u00a0min at baseline or follow\u2010up, but some did not comply with the study procedures regarding this endpoint. Pinch strength correlated with TV and CV in patients (rho\u00a0=\u00a00.72 and rho\u00a0=\u00a00.68) and controls (both rho\u00a0=\u00a00.89). PUL correlated with TV, CV, and T2\n water<\/jats:sub>\n (rho\u00a0=\u00a00.57, rho\u00a0=\u00a00.51, and rho\u00a0=\u00a0\u22120.59).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Low thenar FF, increased T2\n water<\/jats:sub>\n , correlation of muscle size with strength and function, and the decrease in strength and function over 1\u00a0year indicate that the thenar muscles are a valuable and quantifiable target for therapy in later stages of DMD. Further studies are needed to relate these data to the loss of a clinically meaningful milestone.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/jcsm.12711","type":"journal-article","created":{"date-parts":[[2021,5,9]],"date-time":"2021-05-09T02:48:47Z","timestamp":1620528527000},"page":"694-703","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Preserved thenar muscles in non\u2010ambulant Duchenne muscular dystrophy patients"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5022-3745","authenticated-orcid":false,"given":"Karin J.","family":"Naarding","sequence":"first","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"Duchenne Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9300-9888","authenticated-orcid":false,"given":"Kevin R.","family":"Keene","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology LUMC Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8813-4876","authenticated-orcid":false,"given":"Aashley S.D.","family":"Sardjoe Mishre","sequence":"additional","affiliation":[{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology LUMC Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8153-8862","authenticated-orcid":false,"given":"Thom T.J.","family":"Veeger","sequence":"additional","affiliation":[{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology LUMC Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1541-130X","authenticated-orcid":false,"given":"Nienke M.","family":"van de Velde","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"Duchenne Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arina J.","family":"Prins","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"Duchenne Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2727-0057","authenticated-orcid":false,"given":"Jedrzej","family":"Burakiewicz","sequence":"additional","affiliation":[{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology LUMC Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4572-1501","authenticated-orcid":false,"given":"Jan J.G.M.","family":"Verschuuren","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"Duchenne Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0797-5711","authenticated-orcid":false,"given":"Menno","family":"van der Holst","sequence":"additional","affiliation":[{"name":"Duchenne Center Leiden Netherlands"},{"name":"Department of Orthopedics, Rehabilitation and Physiotherapy Leiden University Medical Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5892-5143","authenticated-orcid":false,"given":"Erik H.","family":"Niks","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center (LUMC) Leiden Netherlands"},{"name":"Duchenne Center Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5772-7177","authenticated-orcid":false,"given":"Hermien E.","family":"Kan","sequence":"additional","affiliation":[{"name":"Duchenne Center Leiden Netherlands"},{"name":"C.J. Gorter Center for High Field MRI, Department of Radiology LUMC Leiden Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,5,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00314217"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(17)32160-8"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-019-0203-3"},{"key":"e_1_2_8_5_1","unstructured":"CHMP.Guideline on the clinical investigation of medicinal products for the treatment of Duchenne and Becker muscular dystrophy. In European Medicines Agency (EMA). Available at:http:\/\/www.ema.europa.eu\/docs\/en_GB\/document_library\/Scientific_guideline\/2015\/12\/WC500199239.pdf. Accessed December 18 2020."},{"key":"e_1_2_8_6_1","unstructured":"CDER CBER.Duchenne Muscular Dystrophy and Related Dystrophinopathies: Developing Drugs for Treatment. 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