Alteration in copper metabolism is well established as a consistent feature of hepatolenticular degeneration (Wilson's disease). A number of different aspects of this metabolic abnormality have been described. The copper content of brain, liver, and other organs is increased.* Urinary excretion of copper is increased,† but fecal excretion is decreased,‡ the patients showing a positive dietary balance with respect to copper. Total serum copper is usually decreased,§ due to a marked reduction in ceruloplasmin,∥ whereas the amount of plasma copper loosely bound to protein and reacting directly with sodium diethyldithiocarbamate is increased.4 Studies with radioactive copper have indicated increased absorption of copper from the gastrointestinal tract ¶ and demonstrated relative persistence of administered copper in the albumin fraction of serum, as opposed to the transfer into the ceruloplasmin-containing globulin fraction which occurs in normal persons.# Improvement in neurological symptoms in some cases following administration of dimercaprol U. S. P.
PORTER H, FOLCH J. Brain Copper-Protein Fractions in the Normal and in Wilson's Disease. AMA Arch NeurPsych. 1957;77(1):8–16. doi:10.1001/archneurpsyc.1957.02330310018002
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