Dystonic signs include slowness of speech, unsteady gait, dystonic facies and posturing. Psiqiatric disturbances are present in majority of pacients with symptomatic disease, and include several forms of psychosis and neuroses. The Kayser-Fleischer ring denotes neurologic impairment and consists of copper deposition in the cornea. Total symptomatic recovery can be achived if intervention is made early. Otherwise death may not be prevented or recovery will be only partial.
Penicillamine is the drug of choice.
Response is quite slow and takes one year to maximum effect. Neurologic symptons may worse in the first months of treatment. After 14 days of penicillamine intake, patients often experiment some sensitivity effects, like fever, rash, lymphadenopathy, neutropenia and thrombocytopenia. Late side effects include proteinuria, nefrotic syndrome, systemic lupus erythematosus, goodpasture's syndrome and chronic skin diseases. In these cases, penicillamine should be discontinued for a few months.