Robert Rehmann, Martijn Froeling, Marlena Rohm, Johannes Forsting, Rudolf André Kley, Tobias Schmidt-Wilcke, Nesrin Karabul, Christine Meyer-Frießem, Jan Vollert, Martin Tegenthoff, Matthias Vorgerd, Lara Schlaffke
- MRI is a helpful tool for monitoring disease progression in late-onset Pompe disease (LOPD). Our study aimed to evaluate if muscle diffusion tensor imaging (mDTI) shows alterations in muscles of LOPD patients with <10% fat-fraction. 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-values axial diffusivity (\(\lambda_{1}\)), mean diffusivity (MD), radial diffusivity (RD), and fractional anisotropy (FA) as well as fat-fraction were analyzed. 6-Minute Walk Test (6-MWT) data were correlated to diffusion metrics. We found that mDTI showed significant differences between LOPD and HC in diffusion parameters (\(\it P\) < .05). Thigh muscles with <10% fat-fraction showed significant differences in MD, RD, and \(\lambda_{1-3}\). MD positively correlated with 6-MWT (\(\it P\) = .06). To conclude, mDTI reveals diffusion restrictions in muscles of LOPD with and without fat-infiltration and reflects structural changes prior to fatty degeneration.
MetadatenAuthor: | Robert RehmannORCiDGND, Martijn FroelingORCiDGND, Marlena RohmORCiDGND, Johannes ForstingORCiDGND, Rudolf André KleyGND, Tobias Schmidt-WilckeGND, Nesrin KarabulGND, Christine Meyer-FrießemORCiDGND, Jan VollertORCiDGND, Martin TegenthoffGND, Matthias VorgerdORCiDGND, Lara SchlaffkeORCiDGND |
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URN: | urn:nbn:de:hbz:294-99139 |
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DOI: | https://doi.org/10.1002/mus.27021 |
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Parent Title (English): | Muscle & nerve |
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Publisher: | Wiley |
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Place of publication: | Hoboken, New Jersey |
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Document Type: | Article |
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Language: | English |
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Date of Publication (online): | 2023/05/24 |
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Date of first Publication: | 2020/07/12 |
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Publishing Institution: | Ruhr-Universität Bochum, Universitätsbibliothek |
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Tag: | 6-Minute Walk Test; diffusion tensor imaging; glycogen storage disease type II; magnetic resonance imaging; muscle; skeletal; tractography |
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Volume: | 62 |
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Issue: | 4 |
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First Page: | 541 |
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Last Page: | 549 |
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Note: | Dieser Beitrag ist auf Grund des DEAL-Wiley-Vertrages frei zugänglich. |
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Institutes/Facilities: | Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil, Neurologische Universitätsklinik und Poliklinik |
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Dewey Decimal Classification: | Technik, Medizin, angewandte Wissenschaften / Medizin, Gesundheit |
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open_access (DINI-Set): | open_access |
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Licence (English): | Creative Commons - CC BY-NC-ND 4.0 - Attribution-NonCommercial-NoDerivatives 4.0 International |
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