MRI was performed in 13 patients with the adult form of myotonic dystr
ophy (MD) and compared with that of sex- and age-matched normal contro
ls. There was some cerebral atrophy in the patients and marked thicken
ing of the skull in three of them, associated with ossification of the
falx cerebri in two. We found high-signal areas on T2-weighted images
in the white matter in 9 (70 %) of the patients; five showed high-sig
nal areas in the subcortical white matter of the temporal lobes. These
findings were associated with intellectual impairment in only one pat
ient. who had a history of a difficult birth and temporal lobe epileps
y.