Cerebral white matter affection in myotonic dystrophy type 1 and 2– A Diffusion-Tensor-Imaging Study at3T

Abstract

Myotonic dystrophy type 1 (DM1) and 2 (DM2) are autosomal dominantly inherited neuromuscular disorders with multisystemic involvement. Although cognitive impairment and white matter changes are more common in DM1 patients, brain volume loss and white matter changes have been described in DM2 patients as well. Using voxel-based morphometry (VBM) we previously found grey and white matter loss along cerebral midline structures in DM2 patients [13 DM2 patients (m/f: 8/5, age 53.4±12.0 years, disease duration/DD 12.0±8.8 years); 13 controls (m/f: 8/5, age 53.5±10.2 years)]. To further investigate white matter changes in DM1 and DM2 patients, we used diffusion-tensor-imaging (DTI) and correlated changes with clinical data.