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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Clinically feasible diffusion MRI in muscle: Time dependence and initial findings in Duchenne muscular dystrophy
Amy R McDowell1, Thorsten Feiweier2, Francesco Muntoni3,4,5
1Developmental Imaging and Biophysics, University College London, London, United Kingdom.
Purpose:
To characterize the diffusion time-dependence in muscle in healthy adult volunteers, boys with Duchenne's muscular dystrophy (DMD), and age-matched controls in a clinically feasible acquisition time for pediatric applications.
Methods:
Diffusion data were acquired using a pulsed gradient stimulated echo diffusion preparation at 5 different diffusion times (70, 130, 190, 250, and 330 ms), at 4 different b-values (0, 200, 400, 600, and 800 s/mm2 ) and 6 directions (orthogonal x, y, and z and diagonal xy, xz, and yz) and processed to obtain standard diffusion indices (mean diffusivity [MD] and fractional anisotropy [FA]) at each diffusion time.
Results:
Time-dependent diffusion was seen in muscle in healthy adult volunteers, boys with DMD, and age-matched controls. Boys with DMD showed reduced MD and increased FA values in comparison to age matched controls across a range of diffusion times. A diffusion time of Δ = 190 ms had the largest effect size.
Conclusions:
These results could be used to optimize diffusion imaging in this disease further and imply that these diffusion indices may become an important biomarker in monitoring progression in DMD in the future.
Insights
Diffusion MRI reveals time-dependent changes in muscle for healthy individuals and Duchenne muscular dystrophy (DMD) patients. Boys with DMD exhibit altered diffusion indices, suggesting potential biomarkers for disease monitoring.
Area of Science:
- Biomedical Engineering
- Radiology
- Neuromuscular Disorders
Background:
- Diffusion MRI is a valuable tool for characterizing tissue microstructure.
- Understanding diffusion time-dependence is crucial for accurate interpretation of diffusion MRI data, especially in pediatric applications.
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle tissue.
Purpose of the Study:
- To investigate the time-dependence of diffusion MRI metrics in the muscle of healthy volunteers and boys with DMD.
- To establish clinically feasible acquisition times for pediatric diffusion imaging.
- To identify potential diffusion MRI biomarkers for monitoring DMD progression.
Main Methods:
- Diffusion-weighted imaging was performed using a pulsed gradient stimulated echo sequence at various diffusion times (70-330 ms) and b-values (0-800 s/mm²).
- Standard diffusion indices, including mean diffusivity (MD) and fractional anisotropy (FA), were calculated at each diffusion time.
- Data were analyzed in healthy adults, boys with DMD, and age-matched controls.
Main Results:
- Time-dependent diffusion patterns were observed in all study groups.
- Boys with DMD demonstrated significantly reduced MD and increased FA compared to controls across diffusion times.
- The diffusion time of 190 ms yielded the largest effect size for differentiating DMD from controls.
Conclusions:
- Diffusion MRI metrics exhibit time-dependence in muscle, relevant for both healthy individuals and DMD patients.
- Altered diffusion indices in DMD suggest their potential as sensitive biomarkers for disease progression.
- Optimized diffusion imaging protocols incorporating time-dependence may enhance diagnostic and monitoring capabilities for DMD.
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