Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and simi...

Hepatolenticular degeneration (Wilson's disease) is a rare condition characterised by a defect in biliary excretion of copper resulting in excessive copper accumulation and toxicity. To the most frequent symptoms of this disorder belong liver, neurological or psychiatric disturbances, although other less common clinical features may sometimes be present. Since the clinical presentation of the d...

Starch gel electrophoresis has demonstrated that graded concentrations of sodium sulphate do not precipitate the serum globulins in any reasonable degree of purity. The use of this form of electrophoresis has shown that caeruloplasmin is associated with a globulin in the Fa(2) position.This technique can be employed as a screening test for patients suspected of suffering from hepatolenticular d...

Reports have appeared of abnormal copper metabolism in retinitis pigmentosa, and of a family with vitelliform retinal degeneration in which other members suffered from hepatolenticular degeneration. In the present study 15 patients with retinitis pigmentosa, 4 with various other retinal degenerations, and 1 with a family disposition to retinitis pigmentosa were examined. The copper concentratio...

Holmberg and Laurell (1948) gave the name " caeruloplasmin " to a blue copper globulin complex which they had isolated from both pig and human blood (1947). It was obtained by ammonium sulphate precipitation and was found to be active as an oxidase against p-phenylenediamine. Tlhey considered it to be an a globulin with a molecular weight of about 150,000. Other workers (Scheinberg and Gitlin, ...