T2 relaxometry of brain in myotonic dystrophy

Neuroradiology. 2001 Mar;43(3):198-204. doi: 10.1007/s002340000459.


We investigated the nature and extent of brain involvement in myotonic dystrophy (DM), examining possible T2 relaxation abnormalities in the brain of 20 patients with adult-onset DM and 20 sex- and age-matched normal controls. Brain MRI was performed at 0.5 T, and T2 values were calculated from signal intensity in two echoes. Regions of interest included: frontal, parietal, temporal, occipital and callosal (rostral and splenial) normal-appearing white matter; frontal, occipital, insular and hippocampal cortex; caudate nucleus, putamen, globus pallidus and thalamus. All white-matter and occipital and right frontal cortex regions showed a significantly longer T2 in the patients. Multiple regression analysis, including grey- and white-matter T2 as dependent variables, plus age at onset and at imaging, disease duration, muscular disability, brain atrophy and CTG trinucleotide repeats as independent variables, revealed that only white-matter T2 elongation and disease duration correlated positively. White-matter involvement in DM is more extensive than previously reported by MRI and neuropathological studies and seems to be progressive in the course of disease.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Brain / pathology*
  • Disease Progression
  • Female
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Middle Aged
  • Myotonic Dystrophy / complications
  • Myotonic Dystrophy / genetics
  • Myotonic Dystrophy / pathology*
  • Regression Analysis
  • Trinucleotide Repeats