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Washington University Experience | NEURODEGENERATION | Wilson Disease | 2A1 Wilson Dz AANP DSS 2005 Case 2
Necropsy Findings: The general autopsy showed hepatosplenomegaly with macronodular cirrhosis, focal acute pneumonia, and ascites. --- Comment: After a clinical diagnosis of Wilson’s disease was entertained, an examination disclosed Kayser-Fleischer rings in the cornea. The 24 hour urine copper excretion was 74 micrograms, which is elevated, but not greatly. The middle third of the putamen on each side had necrosis. Alzheimer type I astrocytes were tentatively identified, in addition to the readily demonstrable Alzheimer type II astrocytes. A rhodamine stain for copper was negative in brain sections. ---- 2A1-4 The progressive magnification of the putamen in the area of most significant basal ganglia damage shows neuron loss, astrocytosis and preservation of many pencil fibers. (H&E)