Figure 2

Case 1. XP-A A: Two consecutive Computed Tomography (CT) images of the brain obtained at age 20y. There are no focal brain abnormalities and no appreciable brain atrophy. B: Two axial T1-weighted Magnetic Resonance Images (MRI) of the brain in the same patient obtained at age 41y. There is evidence of prominent brain atrophy manifested by enlargement of the ventricles and widening of the intracranial subarachnoid spaces. C: Gross atrophy of the right brain hemisphere (right, comparable image to 2B) in comparison to the normal brain at the same age and gender (left). Loss of brain substance appears to be greater in the white matter. D: Thinning of cortex of the right temporal lobe (H&E, x 40). E: Luxol fast blue stain of the right temporal lobe showing patchy loss of myelination.