Beeinflussung sensomotorischer und kognitiver Defizite bei Patienten mit Morbus Wilson durch medikamentoese Therapie und Lebertransplantation Schlussbericht

In more than 50 patients with Wilson's disease (WD) sensorimotor and cognitive deficits were quantified using evoked potentials, a motor and a cognitive test battery. Patients with an initial dysarthria and severe dystonia turned out to have a poorer outcome in comparison to the rest of the patients. The analysis of the regional cerebral glucose metabolism revealed a reduction of the glucose utilization throughout the entire brain, especially in the basal ganglia. The improvement of the cerebral glucose metabolic rate was dependent on the duration of therapy and the amount of urinary copper excretion, suggesting not to reduce the DPA-maintenance dose not below 1200 mg DPA/day. Those patients being treated with an orthotopic livertransplantation revealed better test results than 85% of the conventionally treated patients, so that livertransplanted WD-patients are clinically healed. (orig.)SIGLEAvailable from TIB Hannover: DtF QN1(49,14) / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Bildung, Wissenschaft, Forschung und Technologie, Bonn (Germany)DEGerman