Three-Point Technique of Fat Quantification of Muscle Tissue as a Marker of Disease Progression in Duchenne Muscular Dystrophy: Preliminary Study

TLDR

Clinical trials in Duchenne muscular dystrophy are limited by the lack of suitable objective end points. This study sought to determine whether muscle fat infiltration measured by the three‑point Dixon MRI technique could serve as an objective marker of disease severity. The authors compared functional ability scores with manual muscle testing, isokinetic dynamometer strength, and quadriceps/hamstring fat percentages quantified by the Dixon MRI technique. MRI‑derived muscle fat percentages correlated more strongly with functional grades than manual or dynamometer strength measures, increased with age, and offered a more objective, reproducible assessment of disease severity.

Abstract

Clinical trials involving patients with Duchenne muscular dystrophy are hindered by the lack of suitable objective end points. The purpose of this study was to examine whether muscle lipid infiltration measured with the three-point Dixon MRI technique has value as a marker of disease severity.Disease severity in nine boys (mean age, 8.6 +/- 2.7 years) with Duchenne muscular dystrophy was determined with the functional ability scale of Brooke and associates. Functional scores were compared with strength measurements obtained by manual testing of muscles of the lower extremities, knee extensor strength measured with an isokinetic dynamometer, and muscle fat percentage in the quadriceps and hamstrings determined with the three-point Dixon MRI technique.MRI measurements of fat infiltration had stronger correlation (p < 0.05) with functional grade than did measurements obtained with manual muscle testing (p = 0.07) or quantitative strength measured with the isokinetic dynamometer (p = 0.54). Muscle fat percentage did not correlate with strength measurements from manual or dynamometer muscle testing but increased with age in subjects with Duchenne muscular dystrophy.Muscle adiposity values obtained with three-point Dixon MRI are accurate in assessment of disease severity in patients with Duchenne muscular dystrophy. Because they are not influenced by patient effort or examiner variability, these measurements are more objective and reproducible than measurements of muscle strength.