Creutzfeldt-Jakob disease (CJD) is a rare and fatal brain disorder. CJD is caused by a type of protein called a prion. Prion proteins occur in both a normal form, which is a harmless protein found in the body's cells, and in an infectious form, which causes disease. Prion diseases can be found in human and in animals.
CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one case per million population.
There are different kinds of CJD including Sporadic, Familial, Acquired, and Variant. About 85% of cases are Sporadic CJD and it is unknown how it is spread. Variant CJD is likely to be caused by eating meat from a cow with a certain prion disease. A smaller number of cases are born with mutations of the gene, called familial CJD. It is not known to spread through casual contact.
Signs and Symptoms
- Noticeable social and behavioral changes
- Loss of balance
- Vision problems and blindness
- Slowing of brain function
Symptoms vary based on the type of CJD. In sporadic and familial CJD, symptoms mainly affect the brain. In variant CJD, symptoms affect a person's behavior and emotions followed by neurological symptoms a few months later. Symptoms are unpredictable in iatrogenic CJD.
CJD is diagnosed based on signs and symptoms and progression of the disease. Testing of brain tissue confirms the disease. There is no known treatment for CJD.
Prevention
- Wash hands before eating, drinking, or smoking.
- Cover cuts and abrasions with waterproof dressings.
- Wear surgical gloves when handling a patient's tissues, fluids, or when dressing wounds.
- Use a mask if there is a risk of splashing body fluids.
- To prevent variant CJD, travelers to Europe and other areas with cases of Mad Cow Disease may consider avoiding beef and beef products.
Medical tools that have come in contact with the patient will need special handling after use. People with suspected or confirmed CJD and those with family history should never donate blood, tissues, or organs.
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Additional Resources:
Creutzfeldt-Jakob Disease Fact Sheet (PDF)
You can also call Maine CDC at 1-800-821-5821