Fig. 1

Brain MRI of case 1. a and b Brain magnetic resonance (MR) images of the patient 1 at age 36. Atrophy is present in the frontal and temporal cortices and the caudate nucleus. a and b Brain MR images of the patient taken 1 year after those shown in c and d. The atrophy is more severe and the anterior horn of the lateral ventricles is markedly enlarged. Permission was obtained from the right holder [25]