Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with Duchenne muscular dystrophy: a multicenter cross sectional study

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder that results in functional deficits. However, these functional declines are often not able to be quantified in clinical trials for DMD until after age 7. In this study, we hypothesized that (1)H2O T2 derived using (1)H-MRS and MRI-T...

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Published in:PloS one 2014-09, Vol.9 (9), p.e106435-e106435
Main Authors: Forbes, Sean C, Willcocks, Rebecca J, Triplett, William T, Rooney, William D, Lott, Donovan J, Wang, Dah-Jyuu, Pollaro, Jim, Senesac, Claudia R, Daniels, Michael J, Finkel, Richard S, Russman, Barry S, Byrne, Barry J, Finanger, Erika L, Tennekoon, Gihan I, Walter, Glenn A, Sweeney, H Lee, Vandenborne, Krista
Format: Article
Language:English
Subjects:
Adolescent
Age
Age Distribution
Case-Control Studies
Child
Child, Preschool
Children
Children & youth
Clinical trials
Cohort Studies
Control equipment
Control methods
Cross-Sectional Studies
Data acquisition
Disease Progression
Duchenne's muscular dystrophy
Dystrophy
Edema
Hereditary diseases
Hospitals
Humans
Image acquisition
Leg
Lipid Metabolism
Lipids
Magnetic resonance
Magnetic Resonance Imaging
Male
Medical research
Medicine and Health Sciences
Muscle, Skeletal - metabolism
Muscles
Muscular dystrophy
Muscular Dystrophy, Duchenne - metabolism
NMR
Nuclear magnetic resonance
Physical therapy
Physiology
Principal components analysis
Rodents
Science
Skeletal muscle
Spectroscopy
Spectrum analysis
Therapeutic applications
Thigh
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Summary:Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder that results in functional deficits. However, these functional declines are often not able to be quantified in clinical trials for DMD until after age 7. In this study, we hypothesized that (1)H2O T2 derived using (1)H-MRS and MRI-T2 will be sensitive to muscle involvement at a young age (5-7 years) consistent with increased inflammation and muscle damage in a large cohort of DMD subjects compared to controls. MR data were acquired from 123 boys with DMD (ages 5-14 years; mean 8.6 SD 2.2 years) and 31 healthy controls (age 9.7 SD 2.3 years) using 3-Tesla MRI instruments at three institutions (University of Florida, Oregon Health & Science University, and Children's Hospital of Philadelphia). T2-weighted multi-slice spin echo (SE) axial images and single voxel 1H-MRS were acquired from the lower leg and thigh to measure lipid fraction and (1)H2O T2. MRI-T2, (1)H2O T2, and lipid fraction were greater (p
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0106435