"The basal ganglia, especially the putamina, are affected with swelling in early stages followed by atrophy. Sparing of the mid dorsal putamen (showing normal signal surrounded by T2 hyperintense signal) is referred to as the putaminal eye, a distinct imaging sign seen only in this disease 2,3. This sign is not readily recognised in end stage disease where there is marked volume loss of putamen. This disease has a distinctive imaging evolution on MRI with end stage atrophy of the basal ganglia, cerebrum and cerebellum."