‘Face of the giant panda’ sign in Wilson disease

Excerpt

A 16-year-old boy presented with truncal dystonia and arm tremor of 3 years’ duration. He had suffered from fulminant hepatitis at 12 years of age. Icterus with hepatomegaly was noted. Neurological examination revealed dystonia, scanning dysarthria, emotional lability, ‘wing-beating tremor’ of the arms and bilateral Kayser-Fleischer rings. Laboratory investigation confirmed Wilson disease. MR imaging showed hyperintensity of the mid-brain with relative sparing of the red nucleus, superior colliculus and part of the pars reticulate of the substantia nigra with hypointensity of the aqueduct (face of the giant panda appearance; Fig. 1). He was treated with penicillamine and zinc. At 6 months, there was moderate improvement.

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