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

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

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.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adipose Tissue / drug effects
  • Adipose Tissue / pathology
  • Adipose Tissue / physiopathology
  • Adrenal Cortex Hormones / therapeutic use*
  • Anti-Inflammatory Agents / therapeutic use*
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Humans
  • Leg* / growth & development
  • Leg* / pathology
  • Leg* / physiopathology
  • Longitudinal Studies
  • Magnetic Resonance Imaging
  • Magnetic Resonance Spectroscopy
  • Male
  • Muscle Strength / drug effects
  • Muscle, Skeletal / drug effects*
  • Muscle, Skeletal / growth & development
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Muscular Dystrophy, Duchenne / drug therapy*
  • Muscular Dystrophy, Duchenne / pathology
  • Muscular Dystrophy, Duchenne / physiopathology
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents