Magnetic Resonance Imaging and Spectroscopy Assessment of Lower Extremity Skeletal Muscles in Boys with Duchenne Muscular Dystrophy: A Multicenter Cross Sectional Study
Open Access
- 9 September 2014
- journal article
- research article
- Published by Public Library of Science (PLoS) in PLOS ONE
- Vol. 9 (9), e106435
- https://doi.org/10.1371/journal.pone.0106435
Abstract
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 1H2O T2 derived using 1H-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 1H2O T2. MRI-T2, 1H2O T2, and lipid fraction were greater (p2, 1H2O T2 and lipid fraction and vastus lateralis MRI-T2 and 1H2O T2. In the boys with DMD, MRI-T2 and lipid fraction were greater (p1H2O T2 was lower in the oldest age group compared to the young age group. Overall, MR measures of T2 and lipid fraction revealed differences between DMD and Controls. Furthermore, MRI-T2 was greater in the older age group compared to the young age group, which was associated with higher lipid fractions. Overall, MR measures of T2 and lipid fraction show excellent sensitivity to DMD disease pathologies and potential therapeutic interventions in DMD, even in the younger boys.Keywords
This publication has 42 references indexed in Scilit:
- Global T2 versus water T2 in NMR imaging of fatty infiltrated muscles: Different methodology, different information and different implicationsNeuromuscular Disorders, 2014
- Assessment of intramuscular lipid and metabolites of the lower leg using magnetic resonance spectroscopy in boys with Duchenne muscular dystrophyNeuromuscular Disorders, 2014
- Orphan drug development in muscular dystrophy: update on two large clinical trials of dystrophin rescue therapies.2013
- Quantitative Muscle MRI as an Assessment Tool for Monitoring Disease Progression in LGMD2I: A Multicentre Longitudinal StudyPLOS ONE, 2013
- Permanent muscular sodium overload and persistent muscle edema in Duchenne muscular dystrophy: a possible contributor of progressive muscle degenerationZeitschrift für Neurologie, 2012
- Lower leg muscle involvement in Duchenne muscular dystrophy: an MR imaging and spectroscopy studySkeletal Radiology, 2011
- Changes in muscle T2 and tissue damage after downhill running in mdx MiceMuscle & Nerve, 2011
- Quantification of fat infiltration in oculopharyngeal muscular dystrophy: Comparison of three MR imaging methodsJournal of Magnetic Resonance Imaging, 2010
- MR measurements of muscle damage and adaptation after eccentric exerciseJournal of Applied Physiology, 1999
- Localized Proton NMR spectroscopy using stimulated echoes: Applications to human skeletal muscle in vivoMagnetic Resonance in Medicine, 1991