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Neurology. 2014 Sep 9;83(11):974-80. doi: 10.1212/WNL.0000000000000775. Epub 2014 Aug 6.

Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS.

Author information

1
From the Departments of Physical Therapy (I.A., R.J.W., S.C.F., D.J.L., W.T.T., C.R.S., K.V.), Physiology and Functional Genomics (G.A.W.), and Pediatrics (B.J.B.), and Molecular Genetics & Microbiology, Powell Gene Therapy Center (B.J.B.), University of Florida, Gainesville; The Children's Hospital of Philadelphia (R.S.F., D.-J.W., G.I.T.), PA; Oregon Health & Science University (W.D.R., E.L.F., B.S.R.), Portland; the Departments of Integrative Biology and Statistics and Data Sciences (M.J.D.), The University of Texas at Austin; Shriners Hospital for Children (B.S.R.), Philadelphia, PA; and the Department of Physiology (H.L.S.), University of Pennsylvania, Philadelphia.
2
From the Departments of Physical Therapy (I.A., R.J.W., S.C.F., D.J.L., W.T.T., C.R.S., K.V.), Physiology and Functional Genomics (G.A.W.), and Pediatrics (B.J.B.), and Molecular Genetics & Microbiology, Powell Gene Therapy Center (B.J.B.), University of Florida, Gainesville; The Children's Hospital of Philadelphia (R.S.F., D.-J.W., G.I.T.), PA; Oregon Health & Science University (W.D.R., E.L.F., B.S.R.), Portland; the Departments of Integrative Biology and Statistics and Data Sciences (M.J.D.), The University of Texas at Austin; Shriners Hospital for Children (B.S.R.), Philadelphia, PA; and the Department of Physiology (H.L.S.), University of Pennsylvania, Philadelphia. kvandenb@phhp.ufl.edu.

Abstract

OBJECTIVE:

To evaluate the effects of corticosteroids on the lower extremity muscles in boys with Duchenne muscular dystrophy (DMD) using MRI and magnetic resonance spectroscopy (MRS).

METHODS:

Transverse relaxation time (T2) and fat fraction were measured by MRI/MRS in lower extremity muscles of 15 boys with DMD (age 5.0-6.9 years) taking corticosteroids and 15 corticosteroid-naive boys. Subsequently, fat fraction was measured in a subset of these boys at 1 year. Finally, MRI/MRS data were collected from 16 corticosteroid-naive boys with DMD (age 5-8.9 years) at baseline, 3 months, and 6 months. Five boys were treated with corticosteroids after baseline and the remaining 11 served as corticosteroid-naive controls.

RESULTS:

Cross-sectional comparisons demonstrated lower muscle T2 and less intramuscular (IM) fat deposition in boys with DMD on corticosteroids, suggesting reduced inflammation/damage and fat infiltration with treatment. Boys on corticosteroids demonstrated less increase in IM fat infiltration at 1 year. Finally, T2 by MRI/MRS detected effects of corticosteroids on leg muscles as early as 3 months after drug initiation.

CONCLUSIONS:

These results demonstrate the ability of MRI/MRS to detect therapeutic effects of corticosteroids in reducing inflammatory processes in skeletal muscles of boys with DMD. Our work highlights the potential of MRI/MRS as a biomarker in evaluating therapeutic interventions in DMD.

PMID:
25098537
PMCID:
PMC4162304
DOI:
10.1212/WNL.0000000000000775
[Indexed for MEDLINE]
Free PMC Article

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