Quantitative Magnetic Resonance Imaging of the Forearm in Myotonic Dystrophy Type 1.

INTRODUCTION

Myotonic dystrophy type 1 is the most prevalent muscular dystrophy in adults, characterized by weakness, impaired functional abilities, and myotonia. However, little is known about the relationship between quantitative MRI measures (fat fraction and T2 relaxation time) and clinical findings of the upper extremity. This study assessed forearm muscle structure in patients with myotonic dystrophy using quantitative MRI and correlated these measures with strength, function, and handgrip myotonia.

MATERIALS AND

METHODS

Eighteen adults with myotonic dystrophy type 1 underwent MRI using three-point Dixon and T2 spin echo imaging of the forearm.

RESULTS

The average fat fraction and T2 relaxation time were greatest in the flexor digitorum profundus (26.7% and 55.6 ms, respectively). Correlations were found between quantitative MRI values and clinical tests of strength (r = -0.61 to -0.92, p < 0.01), function (r = -0.64 to -0.83, p < 0.01), and handgrip myotonia (r = 0.48, p < 0.05). Overall, the anterior forearm fat fraction values showed higher correlations with strength and function compared to those of the posterior forearm.

DISCUSSION: Our results support the use of quantitative MRI measures to assess forearm disease pathology and show potential to monitor the effectiveness of therapeutic treatments in patients with myotonic dystrophy type 1.