Liver Health Wilson's Disease

Wondering About Wilson's Disease?

There are small amounts of copper in many things we eat, and, normally, the body knows how to get rid of the excess. But when a genetic defect renders the body incapable of processing this mineral, there are many negative effects.

As the copper starts to build up, it may affect either the liver or nervous system. George Brewer, MD, emeritus professor of human genetics and internal medicine at the University of Michigan explains this rare problem called Wilson's disease.

What is Wilson's disease?
Wilson's disease is an inherited disease that causes the accumulation of copper in the body. The disease generally presents in teenagers or young adults and it develops as either a liver disease or a neurologic abnormality—about half are seen to have neurological effects and about half have problems with the liver.

The neurological effects occur when the copper accumulation affects the parts of the brain that coordinate movement; they're not affecting the strength of muscles, but rather the coordination of muscles. Speech may be affected, as well as swallowing. Small motor movements may also be very clumsy. There is often tremor or shakiness as part of a movement disorder. Another aspect of the neurological disease is dystonia, where some muscle groups tighten up, causing a limb or the head, for example, to get pulled into abnormal positions.

The disease can also affect the liver, causing hepatitis-like symptoms, in which the patient may be jaundiced, or yellow-colored. If Wilson's disease is caught later on, it may cause cirrhosis, or fibrosis, of the liver. Occasionally, it will present as acute liver failure. Usually this happens in younger people, age 15 or 20 or so. Suddenly, there's an acute liver failure and the liver is so damaged that the only thing that will save the patient's life is transplantation.

Who is at risk for inheriting the disease?
It's recessively inherited, meaning both parents either have Wilson's disease or are carriers of the abnormal gene that causes the disease. And both parents must have at least one copy of the gene for the child to inherit the disease.

When does someone start to show symptoms of the disease?
In Western countries, the most common age of onset is 21 years of age, but the age of onset is quite broad, ranging all the way from 15 to 45 for those with neurologic problems. The average liver presentation tends to be a little bit younger.

In countries like India and the Far East, the age that Wilson's disease begins to show tends to be much earlier, so it is often that young children have this disease.

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