Variant Creutzfeldt-Jakob Disease
(Human Mad Cow Disease; vCJD)
Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease that can cause death. Bovine spongiform encephalopathy (BSE) is a prion disease in cows. BSE can spread to humans and results in vCJD. It is also known as mad cow disease.
It is believed that vCJD is caused by proteins called prions. Prions are normal proteins in the body. These prions may fold up in a way that is not normal. This can change them into the protein that causes the illness. The buildup of these prions may be linked to vCJD.
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Variant CJD is more common in younger people. Being around tissue that contains prions may raise the risk. This may happen from:
- Eating beef from infected cows
- Getting a blood transfusion from a person who had the disease
After a person is exposed, it can take up to 20 years until they feel sick. Problems get worse over time:
- Early phase (0 to 6 months)—mental health problems, such as depression, anxiety, withdrawal, memory problems, and trouble speaking
- Middle phase—nervous system problems, such as difficulty with walking and coordination, muscle jerking and stiffness, and a hard time speaking
- Late phase—being unable to talk or move
The doctor will ask about symptoms and health history. A physical exam will be done.
These tests may be done to learn more about the brain:
- Blood tests
- Electroencephalogram (EEG) to record the electrical activity of the brain
- to test the fluid that protects the brain and spinal cord
Images may be taken with:
- A brain biopsy
- An autopsy done after a person has died
The goals of treatment are to offer support and ease discomfort. There is no cure for vCJD.
The risk of vCJD may be lowered by not eating beef products when traveling to places where BSE is a problem.
- Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/creutzfeldt-jakob-disease.
- Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/creutzfeldt-jakob-disease-fact-sheet.
- Geschwind, M.D. Prion Diseases. Continuum (Minneap Minn). 2015; 21 (6 Neuroinfectious Disease): 1612-1638.
- Mackenzie, G. and Will, R. Creutzfeldt-Jakob disease: recent developments. F1000 Research, 2017; 6: 2053.
- Shir, D., Lazar, E.B., et al. Analysis of clinical features, diagnostic tests, and biomarkers in patients with suspected Creutzfeldt-Jakob Disease, 2014-2021. JAMA Netw Open, 2022; 5(8): e2225098.
- Rimas Lukas, MD
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