Three-point technique of fat quantification of muscle tissue as a marker of disease progression in Duchenne muscular dystrophy: preliminary study.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Wren
Tishya A L
TA
Department of Radiology, Children's Hospital Los Angeles, 4650 Sunset Blvd., MS#81, Los Angeles, CA 90027, USA.
Bluml
Stefan
S
Tseng-Ong
Linda
L
Gilsanz
Vicente
V
D000273:Adipose Tissue / Q000737:chemistry* / Q000473:pathology*; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000737:chemistry* / Q000473:pathology* / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology* / Q000503:physiopathology*; D012720:Severity of Illness Index