Fig. 2.

1.5 T low-quality images of the first MRI examination of the boy (16 months); the best available images were chosen. Axial FLAIR (a) slice shows the typical pattern of LS with basal ganglia involvement (caudate and lenticular nuclei) and mild involvement of thalami bilaterally. Sagittal T1 slice (b) shows normal tropism of the corpus callosum and cerebellum. Last MRI examination at 12 yrs. (c-d) shows marked progression of basal ganglia involvement with enlargement of lateral and third ventricles, indicating global atrophy. Sagittal FLAIR slice (d) highlights marked thinning of the corpus callosum, a consequence of brain atrophy, and severe cerebellum atrophy. The presence of significant movement artifacts is related to MRI without anesthesia, because of critical conditions of the patient