TY - JOUR
T1 - Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with duchenne muscular dystrophy
T2 - A multicenter cross sectional study
AU - Forbes, Sean C.
AU - Willcocks, Rebecca J.
AU - Triplett, William T.
AU - Rooney, William D.
AU - Lott, Donovan J.
AU - Wang, Dah Jyuu
AU - Pollaro, Jim
AU - Senesac, Claudia R.
AU - Daniels, Michael J.
AU - Finkel, Richard S.
AU - Russman, Barry S.
AU - Byrne, Barry J.
AU - Finanger, Erika L.
AU - Tennekoon, Gihan I.
AU - Walter, Glenn A.
AU - Sweeney, H. Lee
AU - Vandenborne, Krista
N1 - Publisher Copyright:
© 2014 Forbes et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
PY - 2014/9
Y1 - 2014/9
N2 - Introduction: 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. Methods: 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 1HMRS were acquired from the lower leg and thigh to measure lipid fraction and 1H2O T2. Results: MRI-T2, 1H2O T2, and lipid fraction were greater (p,0.05) in DMD compared to controls. In the youngest age group, DMD values were different (p,0.05) than controls for the soleus MRI-T2, 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 (p,0.05) in the oldest age group (11-14 years) than the youngest age group (5-6.9 years), while 1H2O T2 was lower in the oldest age group compared to the young age group. Discussion: 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.
AB - Introduction: 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. Methods: 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 1HMRS were acquired from the lower leg and thigh to measure lipid fraction and 1H2O T2. Results: MRI-T2, 1H2O T2, and lipid fraction were greater (p,0.05) in DMD compared to controls. In the youngest age group, DMD values were different (p,0.05) than controls for the soleus MRI-T2, 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 (p,0.05) in the oldest age group (11-14 years) than the youngest age group (5-6.9 years), while 1H2O T2 was lower in the oldest age group compared to the young age group. Discussion: 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.
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U2 - 10.1371/journal.pone.0106435
DO - 10.1371/journal.pone.0106435
M3 - Article
C2 - 25203313
AN - SCOPUS:84908397254
SN - 1932-6203
VL - 9
JO - PloS one
JF - PloS one
IS - 9
M1 - 0106435
ER -