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Diffusion tensor imaging reveals changes in non‐fat infiltrated muscles in late onset Pompe disease
Author(s) -
Rehmann Robert,
Froeling Martijn,
Rohm Marlena,
Forsting Johannes,
Kley Rudolf André,
SchmidtWilcke Tobias,
Karabul Nesrin,
MeyerFrießem Christine H.,
Vollert Jan,
Tegenthoff Martin,
Vorgerd Matthias,
Schlaffke Lara
Publication year - 2020
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.27021
Subject(s) - diffusion mri , fractional anisotropy , thigh , medicine , magnetic resonance imaging , nuclear magnetic resonance , anatomy , radiology , physics
Abstract 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 (λ 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 ( P < .05). Thigh muscles with <10% fat‐fraction showed significant differences in MD, RD, and λ 1‐3 . 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.