NEUROLOGICAL WILSONS DISEASE - THERAPEUTIC STRATEGY

Abstract

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.
Keyword :Wilson's disease ,dpenicillamine,
zinc acetate.