Subacute necrotizing encephalomyelopathy (Leigh's disease): clinical correlation with computerized tomography in the diagnosis of the juvenile and adult form

Abstract A computerized tomography (CT) scan was performed on 6 surviving patients with a suspected diagnosis of subacute necrotizing encephalomyelopathy of the juvenile and adult forms. Bilateral low density areas were observed in the putamina in all cases. This CT pattern conformed with the characteristic pathological changes of the disease. Once the possibility of Wilson's disease or an earlier episode of acute cerebral hypoxia, syndromes which both display a similar appearance in CT, is excluded, a diagnosis of subacute necrotizing encephalomyelopathy may be considered in cases showing bilateral low density areas in the basal ganglia, especially in the putamina, and with a consistent clinical history.