Bilateral lucency of the globus pallidus complicating methylmalonic acidemia

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Abstract
A child was seen because of encephalopathy and metabolic ketoacidosis at 19 months. She was found to have a cobalamin‐responsive form of methylmalonic acidemia of the cbl A complementation group. However, after treatment with cyanocobalamin and a protein‐restricted diet, with recovery from the encephalopathy, she was found to have a tremor and bilateral dystonic posturing in association with lucencies in the globus pallidi shown by computed tomographic scan.