Abstract
A 3.5-year-old girl with tyrosinemia is reported. A computed tomography scan of the abdomen revealed multiple hepatic nodules. Brain magnetic resonance imaging revealed bilateral high-signal changes confined to the globus pallidus on T2-weighted images. Globus pallidus lesions likely represented neuropathologic changes such as astocytosis, delayed myelination, and status spongiosus (myelin splitting and vacuolation).
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