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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
Multi-parametric MR in Becker muscular dystrophy patients.
Melissa T Hooijmans1,2, Martijn Froeling3, Zaida Koeks4
1C.J. Gorter Center, Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.
Quantitative MRI and MRS reveal increased T2 heterogeneity and altered metabolic indices in Becker muscular dystrophy (BMD) muscles, suggesting these are early disease markers. Diffusion tensor imaging (DTI) showed no significant group differences.
Area of Science:
- Biomedical Imaging
- Musculoskeletal Research
- Neuromuscular Disorders
Background:
- Quantitative MRI and MRS are vital for assessing pathophysiological changes in Becker muscular dystrophy (BMD).
- While muscle fat fraction correlates with function in BMD, reduced muscle strength per unit area suggests impaired non-fat-replaced (NFR) muscle quality.
- There is a need for markers reflecting intrinsic muscle tissue changes in BMD.
Purpose of the Study:
- To explore water T2 relaxation times, diffusion parameters, and phosphorus metabolic indices as potential early disease markers in BMD.
- To compare these quantitative MRI and MRS measures in fat-replaced (FR) and NFR muscles between BMD patients and healthy controls.
Main Methods:
- Quantitative proton MRI (three-point Dixon, multi-spin-echo, diffusion-weighted spin-echo echo planar imaging) and 2D chemical shift imaging 31P MRS were performed.
- Data were acquired from 24 BMD patients and 13 healthy controls, assessing six lower leg muscles.
- Muscle fat fractions, phosphorus metabolic indices, water T2 relaxation times (average and SD), and diffusion tensor imaging (DTI) parameters were analyzed.
Main Results:
- Increased phosphodiester levels were observed in specific NFR and FR muscles (tibialis anterior, peroneus, gastrocnemius lateralis).
- Increased T2 standard deviation (SD) was prevalent in FR muscles compared to NFR and control muscles, indicating T2 heterogeneity.
- No significant differences were found in average water T2 relaxation times or DTI indices between BMD patients and controls.
Conclusions:
- Increased T2 heterogeneity and altered metabolic indices, particularly in more diseased muscles, may serve as early indicators of BMD progression.
- Diffusion tensor imaging (DTI) parameters did not show significant differences between groups in this study.
- Quantitative MRI and MRS, specifically T2 heterogeneity and metabolic changes, hold promise for monitoring muscle tissue quality in BMD.
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