NEUROLOGICAL WILSONS DISEASE - THERAPEUTIC STRATEGY

Wilson's disease (WD) is an inherited disease of copper metabolism caused by a failure ofbiliary excretion of excess copper. Accumulated copper causes liver disease in these patients, and inperhaps two thirds of patients, it causes brain damage leading to neurologic or psychiatric manifestations. Treatment involves reversing the positive copper balance. There are many therapeutic approaches to manage these patients. Chelators like penicillamine or trientine induce negative copper balance by cupriuresis. There is high level of endogenous secretion of copper in alimentary canal. Zinc acetate aims at treatment of copper toxicosis by blocking the absorption of copper and increase excretion in the stool. The pivotal role of penicillamine in the management of WD has been a matter of debate .While it induces a negative copper balance, when given in the initial phase of the treatment, it causes worsening of neurological symptoms in about 2030 of patients . Further, in countries with limited resources, the cost of penicillamine for life-long use is rather prohibitive. It has taken many years before it became recognized worldwide that zinc acetate therapy, aiming at the treatment of copper toxicosis, is effective, safe and economic. This is a case report which illustrates the worsening of the neurological symptoms in wilsons disease after d-penicillamine therapy and the use of zinc acetate in ameliorating the symptoms.