Variant Creutzfeldt-Jakob Disease
About Variant Creutzfeldt-Jakob Disease (vCJD)1
Rare, fatal brain disorder
Variant Creutzfeldt-Jakob Disease (vCJD) is a rare, degenerative, fatal brain disorder in humans. There is no known treatment of vCJD and it is invariably fatal.
VCJD was first described in 1996 in the United Kingdom. It has different clinical and pathologic characteristics from classic CJD. vCJD is thought to be linked to bovine spongiform encephalopathy (BSE), or mad-cow disease. BSE is a progressive neurologic.
As of June 2005, 177 cases of vCJD had been reported in the world:
- 156 from the United Kingdom
- 12 from France
- 2 from Ireland
- 1 each from Canada, Italy, Japan, The Netherlands, Portugal, Saudi Arabia and the United States
Note: the Canadian, one of the Irish, Japanese and U.S. cases were reported in people who visited or resided in the United Kingdom during a key exposure period of the U.K. population to the BSE agent.
Transmission linked to 'mad cow disease'
It is believed that the people who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE.
In at least one case, blood from a donor who was later identified as having vCJD was transfused to a patient who had subsequently developed vCJD. The case suggest the possibility that vCJD may be transmitted through transfusion.
Early detection designed to improve blood safety
Chiron is working on the development of a nucleic acid testing-enhanced immunoassay to detect prions (protein particles) in blood that cause vCJD.
The PROCLEIX® vCJD Assay* is designed to meet FDA performance guidelines for detecting Variant Creutzfeldt-Jakob disease (vCJD).
No available treatments
No specific therapy has been shown to stop the progression of vCJD, and the disease is invariably fatal.
Prevention is best defense
Governments are also working to prevent vCJD by placing restrictions on animals and animal products from countries where BSE was known to exist.
To reduce the risk of acquiring vCJD from food, travelers to Europe or other areas with indigenous cases of BSE may consider:
- Avoiding beef and beef products
- Selecting beef or beef products that might have a reduced opportunity for contamination with tissues that may harbor the BSE agent (such as solid pieces of muscle meat rather than brains or beef products like burgers and sausages)
Milk and milk products from cows are not believed to pose any risk for transmitting the BSE agent.
References
- Centers for Disease Control and Prevention Website - vCJD Page. Accessed Aug. 12, 2005.