Cerebral white matter affection in myotonic dystrophy type 1 and 2: a diffusion-tensor-imaging study at 3T

Myotonic dystrophy type 1 (DM1) and 2 (DM2) are autosomal dominantly inherited neuromuscular disorders with multisystemic involvement. Although cognitive impairment and white matter changes (WMC) are more common in DM1, brain volume loss and WMC have been described in DM2 as well. Using voxel-based morphometry we previously found grey and white matter loss along cerebral midline structures in DM2. To further investigate WMC in DM1 and DM2, we used diffusion-tensor-imaging (DTI) and correlated changes with clinical data. We compared 21 DM1 (m/f: 9/12, age 44.0±11.8 years (y), disease duration (DD) 13.3±6.6 y) and…