Figure 2

MRIs of patients V-2, V-3, V-4 and V-6 at various ages. Areas of abnormal intensity in the thalami and basal ganglia (arrowheads) were distinct at 4 months in V-2 (A, B). The sagittal and axial views of T1- and T2-weighted images of V-2 at 1 year showed severe brain atrophy (D, E, F). The sagittal and axial views of T1- and T2-weighted images of V-3 at 7 years, 5 months (G, H, I), V-4 at 6 years, 3 months (J, K, L), and V-6 at 3 years, 5 months (M, N, O) showed severe brain atrophy, including cerebellar atrophy (I, L, O) and abnormal intensity areas in the thalami and basal ganglia.