Quantitative assessment of skeletal muscle degeneration in patients with myotonic dystrophy type 1 using MRI

J Magn Reson Imaging. 2012 Mar;35(3):678-85. doi: 10.1002/jmri.22849. Epub 2011 Nov 8.

Abstract

Purpose: To identify MRI biomarkers that could be used to follow disease progression and therapeutic efficacy in one individual muscle in patients with myotonic dystrophy type 1 (DM1).

Materials and methods: Lower limb MRI and maximal ankle dorsiflexor strength assessment, using a hand-held dynamometer, were performed in 19 DM1 patients and 6 control subjects. The volume of residual muscle tissue of Tibialis Anterior (TA) muscle was chosen as an index for muscle atrophy, and the T2-relaxation-time of the residual muscle tissue was measured to evaluate edema-like lesions. The fat-to-water ratio was assessed using three-point Dixon images to quantify fat infiltration in the entire muscle.

Results: The intra-observer variability of MRI indices (∼5.2% for the residual muscle tissue volume and 2.5% for the fat-to-water ratio) was lower than that of the dorsiflexor torque measurement (∼11.5%). A high correlation (r = 0.91) was found between maximal ankle dorsiflexor strength and residual TA muscle tissue volume in DM1 patients. Increases in the fat-to-water ratio and T2-relaxation-time were associated with a decrease in maximal ankle dorsiflexor strength.

Conclusion: MRI appears as a noninvasive method which can be used to follow disease progression and therapeutic efficacy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Ankle Joint / pathology*
  • Biomarkers / analysis
  • Female
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Muscle Strength / physiology
  • Muscle, Skeletal / pathology*
  • Muscular Atrophy / pathology*
  • Myotonic Dystrophy / pathology*
  • Reproducibility of Results
  • Statistics, Nonparametric

Substances

  • Biomarkers