CT scan appearances in Leigh's disease (subacute necrotizing encephalomyelopathy)

Four cases of Leigh's disease (subacute necrotizing encephalomyelopathy), two confirmed at postmortem examination, have been investigated recently by CT scanning. All four showed the same symmetrical areas of low attenuation in the basal ganglia, especially the putamina. These areas corresponded with the typical areas of necrosis seen in this disease. There was also some evidence of atrophic change in the posterior fossa in some cases. This CT scan appearance may be diagnostic in the appropriate clinical context.