This is an autosomal recessive disorder related to copper metabolism. Hepatic disease appears first, typically in late adolescence.

•  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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