What is variant Creutzfeldt-Jakob Disease?
Variant Creutzfeldt-Jakob Disease (vCJD) is a rare and fatal human neurodegenerative disease distinct from Creutzfeldt-Jakob Disease. Although the two diseases similarly destroy the brain, the disorders are different.
Variant CJD is linked to the consumption of beef products from cattle with bovine spongiform encephalopathy (BSE), a disease caused by abnormal prion proteins and sometimes referred to as “mad cow disease.” Since the disease was first described in 1996, at least 200 patients with this disease have been identified. Most cases have occurred in the United Kingdom.
How does variant Creutzfeldt-Jakob Disease differ from Creutzfeldt-Jakob Disease?
Creutzfeldt-Jakob Disease (CJD) is also a rare, fatal neurodegenerative disease but is distinct from vCJD. This disease typically occurs among older people, is rapidly progressive with infection leading to death usually within a year of the onset of illness, while vCJD is less rapid and often strikes younger adults. CJD is not related to beef consumption.
About 85 percent of CJD cases occur for unknown reasons while about 5-15 percent of patients develop CJD because of inherited genetic mutations. CJD strikes about one in one million people, or about 200 people a year nationwide.
Both CJD and vCJD are prion diseases, a group of rare, invariably fatal brain disorders which occur both in humans and certain animals. Diagnosis of either disease is very difficult and often happens through a process of elimination of other disorders.
Are there other things that can cause similar symptoms?
Degenerative diseases of the brain are characterized by changes in brain function and structure and can vary in severity. There is a wide variety of causes of these symptoms including infections from bacteria or viruses; lack of oxygen to the brain; liver or kidney failure; toxic exposures to substances such as solvents, paints or industrial chemicals; and poor nutrition.
Is there any treatment for vCJD or CJD?
There is no known treatment for either disease.
How are the diseases diagnosed?
Confirmation of a diagnosis of vCJD or CJD generally is by brain biopsy or autopsy and the analysis takes weeks to complete. Tests that can help develop a diagnosis include an electroencephalogram (EEG) to see if the brain’s electrical pattern shows specific abnormalities. Magnetic resonance imaging (MRI) scans also can reveal characteristic patterns of brain degeneration. Cerebrospinal fluid can be examined for proteins related to neurodegenerative diseases. A biopsy of the tonsils is also sometimes helpful in establishing a diagnosis.
Have there been cases of vCJD in the United States?
As of April 9, 2008, three cases of vCJD have been reported from the United States. According to the Centers for Disease Control and Prevention, there is strong evidence suggesting that two of the three cases were exposed to the BSE agent in the United Kingdom and that the third was exposed while living in Saudi Arabia.
Is our food safe?
Since 1989, the Food and Drug Administration and the U.S. Department of Agriculture have worked to reduce the risk of consumer exposure to any BSE-contaminated material. Both agencies have issued rules to prevent the use of mammalian protein in the manufacture of ruminant feed. There is no current test available to identify BSE in beef. There is no evidence to suggest that milk and dairy products can transmit the infection. Cooking and irradiation have not been shown to kill the BSE agent.
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