Wilson's disease: Symptomatic treatment of neuropsychiatric disturbances. Some comments

Neuropsychiatric disturbances in Wilson's disease may require symptomatic relief beyond often lengthy anticopper treatment. The concomitance of brain and liver damage make it necessary to choose drugs with care, their dosage and the identification of these symptoms due to copper accumulation in the brain and those to hepatic encephalopathy. Short halflife benzodiazepines, 'non classical' neuroleptics, anticholinergics and phenobarbital can be valuable therapeutical tools in symptomatic treatment of Wilson's disease. Wilson's disease is a rare condition characterized by degenerative changes in the brain, with softening of the basal ganglia, and cirrhosis of the liver. Biliary excretion of copper is defective and the metal accumulates in the liver, brain and other tissues. Patients usually present in the second or third decade of life with hepatic, neurologic and psychiatric involvement. the administration of agents that remove copper from the body, penicillamine, or prevent its absorption, zinc, or possible have both actions such, as trientine, at least improve or reverse, the manifestations of most established Wilson's disease, even after months or years, and prevent its appearance in asymptomatic affected siblings. The anticopper therapy of wilson's disease has been xhaustively reviewed (Walshe, 1986; Brewer et al., 1987; Hogenraad et al., 1987; Marsden, 1987; Walshe, 1988). However, besides specific anticopper therapy, in severe cases, particularly in those patients whose clinical clinical picture worsens on the initiation of penicillamine treatment (Brewer et al., 1987) symptomatic treatment may be used and selection of the proper treatment may be awkward in presence of brain damage concomitant with liver involvement. In this paper we will discuss the advisability of symptomatic treatment and, in some cases, indicate the drug of choice.