FIG 3. Patient 5: early-onset GLD.

A and B, Axial T2-weighted images (3000/80/1) show subtle increased signal intensity within the cerebellar white matter (straight arrows in A), dentate nuclei (curved arrows in A), and posterior limbs of the internal capsule (arrows in B). There is relatively normal signal intensity within the basal ganglia and thalami. These are the characteristic MR findings in early-onset GLD. Because of the coexistent involvement of the corona radiata pyramidal tract, the MR severity score was 4.

C, Axial noncontrast CT scan at 7 months of age (2 weeks after MR imaging in B) shows abnormal increased density within the basal ganglia and thalamic nuclei (arrowheads).