“ This is an autosomal recessive disorder related to copper metabolism . Hepatic disease appears first, typically in late adolescence. MRI ...
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This is an autosomal recessive disorder related to copper metabolism. Hepatic disease appears first, typically in late adolescence.
MRI
• Variable signal abnormality mostly in the basal ganglia (especially the putamen) and midbrain (tegmentum).
• There may be high signal on T1 from the paramagnetic effects of copper, but mostly high T2.
• Characteristically, the red nuclei and substantia nigra are spared, giving the ‘face of the giant panda’ sign on T2.
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