A case is reported of a 20-year-old female presenting with confusion and progressive sensory aphasia. CT and MRI showed bilateral and symmetric acute necrosis of the basal ganglia and of the left temporal and occipital lobe, besides chronic spinocerebellar degeneration. The imaging findings suggested a mitochrondrial encephalopathy. Genetic examination confirmed a MELAS syndrome (mitochondrial myopathy, encephalopathy, lactate acidosis and stroke like episodes).
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