Vanishing White Matter Disease (6 yrs male)

There is marked cerebral white matter volume loss, with widening of the sulci throughout, and enlargement of the lateral and third ventricles. The white matter returns abnormal high T2 signal/ low FLAIR signal suggesting the white matter has been almost completely replaced by near CSF intensity fluid. Appearances are typical of vanishing white matter disease. Cerebellar volume is relatively well preserved. There appears to be symmetrical restricted diffusion within the subthalamic nuclei and tectum of the midbrain. This may just represent relative sparing of these tissues.