Wilson Hepatolenticular degeneration

It is indicated in metallic intoxication due to arsenic, mercury, gold, bismuth, lead, nickel, thallium and antimony in conjunction with sodium calcium edetate for lead poisoning. It is also useful in hepatolenticular degeneration (Wilson s disease). It is contraindicated in iron and cadmium poisoning. 

Scheinberg, I. H., and Gitlin, D., Deficiency of ceruloplasmin in patients with hepatolenticular degeneration (Wilson s disease). Science 116, 484-485 (1952). 

Hepatolenticular degeneration (Wilson s disease) is caused by a genetic failure to eliminate copper absorbed from food so that it accumulates in the liver, brain, cornea and kidneys. Chelating copper in the gut with penicillamine (p. 293) or trientine can establish a negative copper balance (with some clinical improvement if treatment is started early). The patients may also develop cirrhosis, and the best treatment for both may be orthotopic liver transplantation. 

M15. Matthews, W. B., The absorption and excretion of radio-copper in hepatolenticular degeneration (Wilson s disease). J. Neurol. Neurosurg. Psychiat. 17, 242-246 (1954). 

While I have focused primarily on the neuropsychiatry of HE, a number of diverse liver diseases that present with metabolic encephalopathies can be linked to mood dysregulation and depression. This would include illnesses such as hepatolenticular degeneration (Wilson s disease) (Eggers et al., 2003 Hesse et al, 2003 Chan et al., 2005), Gaucher disease (Packman et al., 2006), Fabry disease (Sadek et al., 2004), and hepatitis C infection (Forton et al., 2004). Thus, both the presentation... 

Hepatolenticular degeneration, Wilson s disease, is a severe heritable disorder of copper metabolism. The defect is caused by diminished synthesis of the copper-transporting protein ceruloplasmin and impaired excretion of copper into the bile. Copper concentration is extremely low in blood serum and high in urine furthermore, copper accumulates in liver, brain, kidney, and cornea. Chelating therapy with D-penicillamine is the medication of choice [48]. To improve copper metabolism, zinc therapy is also used [50]. 

Uzman, L., Denny-Brown, D. Amino-aciduria in hepatolenticular degeneration (Wilson s disease). Amer. J. med. Sci. 215, 599-611 (1948)... 

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