Leigh syndrome is a rare autosomal recessive defect in mitochondrial enzymes also known as subacute necrotizing encephalopathy. There are 3 different forms of the disease: infantile, juvenile and adult. Increased serum pyruvate is a typical laboratory finding. It is characterized on MR by symmetrically increased T2 signal within the basal ganglia extending into the brainstem. Increased signal may also be seen in the cerebral white matter and the cortex. Multiple toxic agents may produce symmetric increased basal ganglia signal including carbon monoxide and methanol. Hypoxic events such as near drowning or cardiac arrest may also have this appearance. Wilson's disease is another entity which can produce symmetrically increased signal in the basal ganglia. In this case the patients history and laboratory findings were helpful in narrowing down the differential. Related Cases
