{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T21:17:54Z","timestamp":1783977474712,"version":"3.55.0"},"reference-count":29,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,8,12]],"date-time":"2022-08-12T00:00:00Z","timestamp":1660262400000},"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":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Oculopharyngeal muscular dystrophy (OPMD) is a late\u2010onset, progressive muscle disease. Quantitative muscle ultrasound (QMUS) assesses structural changes in muscles and is a sensitive biomarker in neuromuscular disorders. Our aim of this study was to determine whether QMUS can detect muscle pathology and can be used as longitudinal imaging biomarker in OPMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Genetically confirmed OPMD patients, recruited by their treating physicians or from the national neuromuscular database, were examined twice, 20\u2009months apart, using QMUS of orofacial and limb muscles, and measurements of functional capacity and muscle strength. Absolute echo intensity (AEI) and muscle thickness of all muscles were analyzed and correlated with clinical data.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The tongue, deltoid, iliopsoas, rectus femoris, and soleus muscles showed increased AEI at baseline compared with normal values in 43 OPMD patients, with the rectus femoris being most often affected (51%).The AEI and muscle thickness of 9 of 11 muscles correlated significantly with the motor function measure, 10\u2010step stair test, swallowing capacity, dynamometry, Medical Research Council grade, tongue strength, and bite force (r\u00a0=\u00a00.302 to \u22120.711). Between baseline and follow\u2010up, deterioration in AEI was found for the temporalis, tongue, and deltoid muscles, and decreased muscle thickness was detected for the temporalis, masseter, digastric, tongue, deltoid, iliopsoas, and soleus muscles (<jats:italic>P<\/jats:italic>\u00a0&lt;\u2009.05). No relation was found between the change in AEI and repeat length or disease duration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>QMUS detected muscle pathology and disease progression in OPMD over 20\u2009months. We conclude that QMUS should be considered as a biomarker in treatment trials.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27679","type":"journal-article","created":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T05:05:35Z","timestamp":1658379935000},"page":"453-461","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6609-0649","authenticated-orcid":false,"given":"Rosemarie H. M. J. M.","family":"Kroon","sequence":"first","affiliation":[{"name":"Radboud university medical center, Donders Institute for Brain Cognition and Behaviour, Department of Rehabilitation  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5790-9109","authenticated-orcid":false,"given":"Johanna G.","family":"Kalf","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute for Brain Cognition and Behaviour, Department of Rehabilitation  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rutger L.","family":"Meijers","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute of Brain Cognition and Behaviour, Department of Neurology  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bert J. M.","family":"de Swart","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute for Brain Cognition and Behaviour, Department of Rehabilitation  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian G. M.","family":"Cameron","sequence":"additional","affiliation":[{"name":"Radboud University, Donders Institute for Brain Cognition and Behaviour, Centre for Neuroscience  Nijmegen The Netherlands"},{"name":"University of Twente Biomedical Signals and Systems, Faculty of Electrical Engineering, Mathematics and Computer, Science  Enschede The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonne","family":"Doorduin","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute of Brain Cognition and Behaviour, Department of Neurology  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7839-8125","authenticated-orcid":false,"given":"Nens","family":"van Alfen","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute of Brain Cognition and Behaviour, Department of Neurology  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baziel G. M.","family":"van Engelen","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute of Brain Cognition and Behaviour, Department of Neurology  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne G. C.","family":"Horlings","sequence":"additional","affiliation":[{"name":"Radboud university medical center, Donders Institute of Brain Cognition and Behaviour, Department of Neurology  Nijmegen the Netherlands"},{"name":"Medical University of Innsbruck Department of Neurology  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,8,12]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003554"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000072861"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-5937-9"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.22431"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.09.010"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-319578"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.cnd.0000152060.57673.25"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-9037-y"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-010-1799-2"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24247"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0720-048X(01)00399-0"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.27081"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24904"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000009211"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2006.09.017"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000012640"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.55.9.822"},{"key":"e_1_2_10_19_1","first-page":"155","article-title":"Maximum swallowing volume: comparison between Flemish and Dutch normal values","volume":"35","author":"Paepens M","year":"2020","journal-title":"Dysphagia."},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/1460-6984.12332"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1080\/13682820701234444"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1044\/jshr.3506.1239"},{"key":"e_1_2_10_23_1","volume-title":"Aids to the Investigation of Peripheral Nerve Injuries","author":"Medical Research Council","year":"1976"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2005.03.004"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1682\/JRRD.2013.06.0148"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1017\/cjn.2018.269"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1213\/ANE.0000000000002864"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(99)00206-1"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-140060"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-6393-2"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27679","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.27679","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27679","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T14:04:43Z","timestamp":1692626683000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.27679"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,12]]},"references-count":29,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,10]]}},"alternative-id":["10.1002\/mus.27679"],"URL":"https:\/\/doi.org\/10.1002\/mus.27679","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,12]]},"assertion":[{"value":"2021-04-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-08-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}