Definition

Variant Creutzfeldt-Jakob disease (vCJD) is a fatal type of prion disease. Bovine spongiform encephalopathy (BSE) is a prion disease that affects cows. There is evidence that this illness can be transmitted to humans, producing vCJD. This illness is often called mad cow disease.

Causes

It is generally believed that vCJD is caused by infectious proteins called prions. Prions are normal proteins in the body. If these prions fold up in a different way than normal, they may transform into the protein that causes the illness. The build-up of abnormal prions may be linked to the brain damage associated with vCJD.

Risk Factors

Variant CJD is more common in younger people. Factors that may increase your chance of getting vCJD include exposure to prion-containing tissue. This may occur from:

• Eating beef from infected cows
• Receiving a blood transfusion from someone who had the disease

Symptoms

After you are exposed, it can take up to 20 years until symptoms develop. When symptoms develop, they usually follow these 3 phases:

• Early phase (0 to 6 months)— psychiatric symptoms, such as depression, anxiety, withdrawal, memory problems, and difficulty pronouncing words
• Middle phase—neurologic symptoms predominate, such as abnormal gait, problems with coordination, muscle jerks and stiffness, and impaired speech
• Late phase—mute, immobility

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Tests may include:

• Blood tests
• Electroencephalogram (EEG) to record the electical activity of the brain
• Cerebrospinal fluid analysis
• Brain biopsy
• Tonsillar biopsy

Imaging tests take pictures inside your body structures. Imaging tests may include:

• MRI scan
• CT scan
• SPECT imaging

In many cases, a final diagnosis is not determined until an autopsy is completed after death.

Treatment

Currently, there is no cure for vCJD. Treatment is primarily supportive, maximizing function and minimizing discomfort.

Prevention

To reduce your chance of getting vCJD, avoid beef products, particularly processed meat like sausages and hotdogs, or beef items containing brain, spinal cord, or bone marrow.

About 200 worldwide cases of vCJD have occurred to date. Most of these were associated with beef consumption in the United Kingdom. There is a great deal of controversy regarding the safety of US beef. BSE has been detected in the US. However, no cases of vCJD have been attributed to eating US beef. US patients with vCJD were deemed to have obtained it outside of the US.

National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov

Public Health Agency of Canada http://www.phac-aspc.gc.ca

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