If the liver injury is acute the copper may be acutely released in the blood and cause hemolytic anemia. If liver injury is more cronic, the copper may acumulate in the brain and cause neuropsiquiatric symptoms. Also, by an not clear mechanism, the protein that carries copper in the blood, ceruloplasmin, is characteristically low. The disease affects mostly young pacients, the median age being between 8 and 20 years. Any person with recurrent hepatic disease and unexplained neurologic symptoms should be investigated to have Wilson's disease.
Liver disease occurs in half of the patients.
Ultimately, all pacients develop cirrhosis. The fulminant liver injury may lead to sudden release of copper in the bloodstream and hemolytic anemia with negative coomb's test. This may be a life threatening condition if not promptly corrected. Neurologic manifestations may occur without liver disease and present in a large variety of ways, including resting and intention tremors, spasticity, rigidity and chorea. Dystonic signs include slowness of speech, unsteady gait, dystonic facies and posturing.