Variant Creutzfeldt-Jakob Disease (vCJD)

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What is vCJD?

Variant Creutzfeldt-Jakob disease, also known as human Bovine Spongiform Encephalopathy (BSE) or human mad cow disease, is a degenerative and fatal brain disease that can occur in humans. It damages brain cells and the spinal cord.

What causes vCJD?

It is caused by an abnormal prion, which is a protein found on the surface of cells. The abnormal prion (BSE prion) attaches to other brain cell proteins and bends them out of shape. These attack the brain, killing cells and creating gaps or sponge-like patches in tissue. vCJD is caused by the same prion that is found in cows with Bovine Spongiform Encephalopathy (BSE).

What are the symptoms?

The initial symptoms include mood swings and memory loss and as the disease progresses it causes problems with movement. The infected person advances quickly to a vegetative state and finally death.

How is it spread?

The BSE prion can be passed onto humans when they eat BSE infected cow parts. The BSE prion concentrates in the brain, skull, spine, nerve tissue, and gut lining of cows. The BSE prion is not passed from person to person, and human infection is rare.

What is the incubation period?

It has an incubation period of 5-15 years.

What is the treatment?

There is currently no cure for vCJD. Treatment involves supporting the patient with physical and occupational therapies. A person with vCJD eventually becomes dependent on others for care.

Is vCJD related to CJD?

vCJD and CJD are not the same disease. They are part of a family of diseases caused by abnormal prions. The symptoms are similar, although vCJD usually occurs before the age of 30.