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Diffusion tensor imaging reveals changes in non-fat infiltrated muscles in late onset Pompe disease

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 a...

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Autores principales: Rehmann, Robert (Autor) , Froeling, Martijn (Autor) , Rohm, Marlena (Autor) , Forsting, Johannes (Autor) , Kley, Rudolf André (Autor) , Schmidt-Wilcke, Tobias (Autor) , Karabul, Nesrin (Autor) , Meyer-Frießem, Christine H. (Autor) , Vollert, Jan (Autor) , Tegenthoff, Martin (Autor) , Vorgerd, Matthias (Autor) , Schlaffke, Lara (Autor)
Formato: Article (Journal)
Lenguaje:inglés
Publicado: 12 July 2020
In: Muscle & nerve
Year: 2020, Volumen: 62, Número: 4, Pages: 541-549
ISSN:1097-4598
DOI:10.1002/mus.27021
Acceso en línea:Verlag, kostenfrei, Volltext: https://doi.org/10.1002/mus.27021
Verlag, kostenfrei, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1002/mus.27021
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Notas de Autor:Robert Rehmann, MD; Martijn Froeling, PhD; Marlena Rohm, MSc; Johannes Forsting, Rudolf André Kley, MD; Tobias Schmidt-Wilcke, MD; Nesrin Karabul, MD; Christine H. Meyer-Frießem, MD; Jan Vollert, PhD; Martin Tegenthoff, MD; Matthias Vorgerd, MD; Lara Schlaffke, PhD
Descripción
Sumario: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 (λ1), 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 (P < .05). Thigh muscles with <10% fat-fraction showed significant differences in MD, RD, and λ1-3. MD positively correlated with 6-MWT (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.
Notas:Gesehen am 10.06.2026
Descripción Física:Online Resource
ISSN:1097-4598
DOI:10.1002/mus.27021

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