Creutzfeldt- Jacob

Creutzfeldt-Jakob disease (CJD) is a brain disease and belongs to the group of spongy brain abnormalities, also called prion diseases. The classic form of CJD was first described in 1920. There are two forms of the disease, the classic form and the variant CJD (vCJD Creutzfeldt-Jakob variant).

The classic form of CJD is divided into three forms. The majority (85-90%) of the disease has an unknown cause (sporadic form), a small part (5-15%) has a genetic cause (familial form) and a very small part (1-2%) is caused through medical treatment (iatrogenic form).

Symptoms of the classic form of CJD

The classic form of CJD is very rare and occurs on average once per million people per year. The classic form mainly affects older people and has a fatal outcome after an average of 5 months. Symptoms are: rapid dementia (forgetfulness, difficulty with daily activities), coordination disorders, speech disorders, problems with vision and stiffness.

Cause of the classic form of CJD

The disease is caused by the body’s prion proteins with a different shape. Prions occur in the brain, among others. How prions can get a deviant shape is largely unknown for the time being.

Eventually the abnormal prions accumulate in the brain, causing the brain tissue to be broken down with death as a result.

In addition to the classic form, the variant form of Creutzfeldt-Jakob disease (vCJD) was described for the first time in 1996 in the United Kingdom. This form of CJD is associated with mad cow disease (either BSE blood sedimentation, bovine spongiform encephalopathy, a prion disease in cattle).

The Creutzfeldt-Jakob variant

The Creutzfeldt-Jakob variant (vCJD Creutzfeldt-Jakob variant) is a rare brain disease that belongs to the sponge-like brain abnormalities. vCJD leads to psychiatric disorders and increasing uncertainty in walking and making movements. The disease mostly occurs in young people and has a fatal outcome.

The symptoms of Creutzfeldt-Jakob variant

The incubation period of vCJD is uncertain but can probably be decades. In contrast to the characteristic dementia in the classical form of CJD Creutzfeld Jacob, vCJD is characterized by psychiatric complaints, such as psychoses, behavioral changes or very severe depressions.

Subsequently an increasing uncontrollability occurs when walking and making movements, followed by complete paralysis and eventually death. The course of the disease lasts about 12 to 14 months.

Contamination and prevention of Creutzfeldt-Jakob variant

Contamination of people with vCJD is probably caused by consumption of beef (mainly brain and nerve tissue) contaminated with BSE (bovine spongiform encephalopathy). In addition, transfer from person to person is possible via blood.

Numerous measures, including rules that guarantee the food safety of beef, can minimize the risk of contamination.

Life expectancy of the Creutzfeldt-Jakob variant

vCJD is very rare, more than two hundred cases of vCJD are known worldwide, the majority of them in the United Kingdom. The disease mainly occurs in young people, the average age of vCJD patients is around 30 years.

Currently, there is no cure for Creutzfeldt-Jakob disease. Treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Drugs can help relieve pain if it occurs.