The results otained from our  and other groups demonstrate,that there are several abnormalities  in brain MRI of myotonic dystrophy cases,these changes  correlate with personality traits,mood disorders and cognitive performances,specific patterns might explain fatigue, lack of self-awereness,executive and procedural functional domains.

The prevalent brain MRI abnormalities are white matter temporal hyperlucency ,reduction of volume of gray matter, but it is unknown if these lesions are progressive and how they might influence patient performance.

Tractography analysis show an increased mean diffusivity in all the four lobes and a reduction of fractional anisotropy in the main white matter tracts.

The 14 early onset DM1 were 11 males,3 females,the majority had  MIRS score  3,they showed abnormal gyrification of brain MRI. Early-onset cases had a mean age of 33 years and an age of onset of 9 years ,with a disease duration of 21 years,in the late onset group there were 10 males and 4 females with a mean age  of 48 years , disease duration of 14 years, MIRS scores were mostly 3 and 4. Gyrification was not altered respect to control , but was significantly (p<0.05) different versus early onset DM1 ,both in frontal and parieto-occipital areas.