What is variant Creutzfeldt-Jakob disease?

The variant form of Creutzfeldt-Jakob disease (vCJD) is caused by proteins that occur naturally in the body (called prions) that have an abnormal form. Prions are found in the brain, among other parts of the body. When normal prions come into contact with abnormal prions, they also change shape. This leads to more and more abnormal prions in the brain, which damage the brain tissue. The patient becomes very ill and eventually dies.

Besides vCJD, there are also other forms of Creutzfeldt-Jakob disease. These other forms are not caused by eating contaminated beef. See the page on Creutzfeldt-Jakob disease for more information.

What are the symptoms of variant Creutzfeldt-Jakob disease?

Variant Creutzfeldt-Jakob disease (vCJD) starts with psychiatric symptoms, such as:

• psychosis (seeing, hearing and thinking things that are not real)
• changes in behaviour
• severe depression

The patient may also suffer from emotional disorders. Later on, there may also be other symptoms affecting the nervous system. The patient will increasingly lose control over movements. For example, the ability to walk may be affected. After that, the patient will become completely paralysed and eventually die. On average, death occurs 12 to 14 months after the first symptoms.

Who can become very ill from variant Creutzfeldt-Jakob disease?

The variant form of Creutzfeldt-Jakob disease (vCJD) mainly affects younger people. The average age of patients with vCJD is 29 years.

How does variant Creutzfeldt-Jakob disease spread?

People can get vCJD by eating beef from cows that have BSE (mad cow disease). The risk is higher if the beef includes brain or nerve tissue. Thanks to many different measures, including strict food safety rules for beef, the risk of contamination is much lower now.

In addition, there is a very low risk of infection during blood transfusions. In theory, it may be possible for a person to become infected during brain or spinal surgery, but there is no evidence of that having happened.

After exposure, it can take years for a person to become ill. It may sometimes even be decades later. It is not clear exactly how long it takes. Researchers suspect that this may be partly determined by hereditary factors. It also depends on how a person was exposed: through contaminated meat, blood or contact with the nervous system.

How can I prevent variant Creutzfeldt-Jakob disease?

As an individual, there is not much you can do to prevent exposure to vCJD. But various measures are in place to guarantee that beef is safe to eat, thus lowering the risk of vCJD. For example, animals that are suspected of having BSE must always be reported to the government. Strict rules are also in place for animal slaughter and for ingredients used in animal feed.

Is there any treatment for variant Creutzfeldt-Jakob disease?

There is no treatment for vCJD. The patient always dies from the disease.

How common is variant Creutzfeldt-Jakob disease?

The variant form of Creutzfeldt-Jakob disease is very rare. Variant CJD has not been seen in the Netherlands in more than 10 years. This is due to strict regulations implemented in 1997, which almost completely eliminated BSE-contaminated meat in the Netherlands.

There are more than 200 known cases of vCJD worldwide. Most of these patients come from the UK. In the Netherlands, there are three patients known to have vCJD.