Wilson disease is a rare genetic condition where excessive copper accumulates in the body, especially in the liver and the brain. Copper plays an important role in development of skin, bones and nerves. Under normal conditions, required amount of copper from food is absorbed and the excess is drained by the liver. In Wilson disease, copper isn’t eliminated properly and accumulates in the body, which can be fatal.
Wilson disease is usually present since birth but signs and symptoms show up only when there is a considerable buildup of copper in the body. Liver disease is the initial presentation with signs and symptoms like jaundice, fatigue, loss of appetite and abdominal swelling. Wilson disease signs and symptoms indicating nervous system involvement include tremors, difficulty in walking, slurred speech, depression and anxiety. When copper deposits in the cornea of the eye, it forms a green-to-brownish ring, called the Kayser-Fleischer ring.
Wilson disease diagnosis is difficult and requires pathology tests and liver biopsy.
The aim of treatment in is to prevent excessive copper build up in the body. This is done by administering chelating agents that bind the copper, which is then excreted. This is followed with administration of zinc acetate that prevents absorption of copper from the food. The Centre for Neurosciences at Kokilaben Dhirubhai Ambani Hospital, Indore is home to the best neurologists to treat wilson’s disease. Wilson’s disease treatment should be continued lifelong irrespective of presence or absence of symptoms.
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