{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T10:23:15Z","timestamp":1790677395307,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,7,31]],"date-time":"2020-07-31T00:00:00Z","timestamp":1596153600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004329","name":"Genzyme","doi-asserted-by":"publisher","award":["GZ2015\u201011496"],"award-info":[{"award-number":["GZ2015\u201011496"]}],"id":[{"id":"10.13039\/100004329","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004339","name":"Sanofi","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004339","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>MRI is a helpful tool for monitoring disease progression in late\u2010onset Pompe disease (LOPD). Our study aimed to evaluate if muscle diffusion tensor imaging (mDTI) shows alterations in muscles of LOPD patients with &lt;10% fat\u2010fraction. We evaluated 6 thigh and 7 calf muscles (both legs) of 18 LOPD and 29 healthy controls (HC) with muscle diffusion tensor imaging (mDTI), T1w, and mDixonquant sequences in a 3T MRI scanner. The quantitative mDTI\u2010values axial diffusivity (\u03bb<jats:sub>1<\/jats:sub>), mean diffusivity (MD), radial diffusivity (RD), and fractional anisotropy (FA) as well as fat\u2010fraction were analyzed. 6\u2010Minute Walk Test (6\u2010MWT) data were correlated to diffusion metrics. We found that mDTI showed significant differences between LOPD and HC in diffusion parameters (<jats:italic>P<\/jats:italic> &lt;\u2009.05). Thigh muscles with &lt;10% fat\u2010fraction showed significant differences in MD, RD, and \u03bb<jats:sub>1\u20103<\/jats:sub>. MD positively correlated with 6\u2010MWT (<jats:italic>P<\/jats:italic> =\u2009.06). To conclude, mDTI reveals diffusion restrictions in muscles of LOPD with and without fat\u2010infiltration and reflects structural changes prior to fatty degeneration.<\/jats:p>","DOI":"10.1002\/mus.27021","type":"journal-article","created":{"date-parts":[[2020,7,12]],"date-time":"2020-07-12T11:39:58Z","timestamp":1594553998000},"page":"541-549","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Diffusion tensor imaging reveals changes in non\u2010fat infiltrated muscles in late onset Pompe disease"],"prefix":"10.1002","volume":"62","author":[{"given":"Robert","family":"Rehmann","sequence":"first","affiliation":[{"name":"Department of Neurology Heimer Institute for Muscle Research, BG\u2010University Hospital Bergmannsheil, Ruhr\u2010University Bochum  Bochum Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3841-0497","authenticated-orcid":false,"given":"Martijn","family":"Froeling","sequence":"additional","affiliation":[{"name":"Department of Radiology University Medical Centre Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marlena","family":"Rohm","sequence":"additional","affiliation":[{"name":"Department of Neurology Heimer Institute for Muscle Research, BG\u2010University Hospital Bergmannsheil, Ruhr\u2010University Bochum  Bochum Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Forsting","sequence":"additional","affiliation":[{"name":"Department of Neurology Heimer Institute for Muscle Research, BG\u2010University Hospital Bergmannsheil, Ruhr\u2010University Bochum  Bochum Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf Andr\u00e9","family":"Kley","sequence":"additional","affiliation":[{"name":"Department of Neurology Heimer Institute for Muscle Research, BG\u2010University Hospital Bergmannsheil, Ruhr\u2010University Bochum  Bochum Germany"},{"name":"Department of Neurology St. Marien\u2010Hospital Borken  Borken Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Schmidt\u2010Wilcke","sequence":"additional","affiliation":[{"name":"St. Mauritius Therapieklinik  Meerbusch Germany"},{"name":"Institute of Clinical Neuroscience and Medical Psychology University Hospital, University of D\u00fcsseldorf  D\u00fcsseldorf Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nesrin","family":"Karabul","sequence":"additional","affiliation":[{"name":"Endokrinologikum Frankfurt a. Main, Center of Hormonal and Metabolic Diseases, Rheumatology, Osteology and Neurology  Frankfurt a. M Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8743-2065","authenticated-orcid":false,"given":"Christine H.","family":"Meyer\u2010Frie\u00dfem","sequence":"additional","affiliation":[{"name":"Department of Anaesthesiology Intensive Care Medicine and Pain Management BG\u2010University Hospital Bergmannsheil, Ruhr\u2010University Bochum  Bochum Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0733-5201","authenticated-orcid":false,"given":"Jan","family":"Vollert","sequence":"additional","affiliation":[{"name":"Pain Research, Department of Surgery and Cancer Imperial College  London UK"},{"name":"Neurophysiology Center of Biomedicine and Medical Technology Mannheim CBTM, Medical Faculty Mannheim, Heidelberg University  Heidelberg 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