Symmetric basal ganglia calcification in a 9-year-old child with MELAS.

A9-year-old boy presented with headache, vomiting, and leftward eye gaze deviation. On examination, left homonymous hemianopia, horizontal nystagmus, and anisocoric pupils were noted. Brain CT disclosed symmetric calcification in basal ganglia (figure A). Blood examination showed lactic academia. Further MRI revealed right occipito-temporo-parietal cortical hyperintensities (figure, B and C). Muscle biopsy revealed raggedred fibers, and genetic study showed an A3243G point mutation, confirming the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and strokelike symptoms (MELAS). Symmetric basal ganglia calcification, focal cerebral lesions not confined to the vascular territories in a young patient warrant further workup for mitochondrial cytopathy.1,2