What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease (CJD) 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.

There are various causes of CJD. Based on these causes, CJD can be grouped into four different forms:

• sporadic: the cause is unknown
• genetic: familial (inherited) form
• iatrogenic: infection is transferred during a medical procedure
• variant: caused by eating beef from a cow that had BSE (‘mad cow’ disease). See the page on variant CJD for more information on this form. The information below is about the other three forms.

What are the symptoms of Creutzfeldt-Jakob disease?

The symptoms of CJD are:

• rapidly progressing dementia (forgetfulness and confusion)
• loss of coordination (problems with fine motor control and movements)
• problems with speaking
• impaired vision
• stiffness

People with CJD always die of the disease. Death usually occurs about five months after the symptoms start.

Who can become ill from Creutzfeldt-Jakob disease?

CJD mainly affects older people. The average age of patients with CJD is between 60 and 70 years.

How does Creutzfeldt-Jakob disease spread?

The original cause of the disease is unknown in most patients with CJD. This form is called sporadic CJD. A small percentage of patients have the genetic form: familial CJD. This is caused by a gene that the patient inherited from one of their parents. The iatrogenic form can be transferred to the patient by contaminated surgical instruments. This is very rare. For iatrogenic CJD, the time between exposure and illness (the incubation period) can be as long as 40 years. The incubation period for other forms of CJD is unknown.

How can I prevent Creutzfeldt-Jakob disease?

Since the cause of CJD is often unknown, it is not possible to prevent the disease. You cannot become immune to the disease. Because the disease is caused by a protein that occurs naturally in the body, you cannot produce antibodies against it.

Hospitals use hygiene measures that prevent contamination of surgical instruments.

Is there any treatment for Creutzfeldt-Jakob disease?

There is no treatment for Creutzfeldt-Jakob disease.

How common is Creutzfeldt-Jakob disease in the Netherlands?

On average, one or two people per million inhabitants in the Netherlands contract Creutzfeldt-Jakob disease every year.