[Case reports of patients with hepatolenticular degeneration-Wilson's disease with emphasis on work capacity]

We presented four patients with hepatolenticular degeneration--Wilson or hepatocerebral dystrophy-Wilson-Konovalov by soviet authors. Our cases were nervous variable of this disease or pseudosclerose (Westphal-Strumphell). The first three patients showed rigid--trembling and one trembling form of disease. All, patients were male. We think that it is very important early identification of this disease, to reduce time from acute manifestation to make a diagnosis and to apply appropriate therapy. This time period in our cases lasted from 0 to 4 years. Except use d- penicillamine (to 900 mg/daily) we think that is also very important use the acidum ascorbicum (to 4.5 gr/daily). This acidum shows less consumption of d- penicillamine and has low complication. Recurrent working ability begins with disappearance of neurological phenomenology and with rehabilitation of psychic status. All our patients we examined by ergometric and psychological tests and we obtained in results working ability population. In our experience and from therapeutic results to make this diagnosis does not mean a priori definite working ability.