Creutzfeldt-Jakob (KROITS-felt YAH-kobe) disease is a degenerative brain disorder that leads to dementia and, ultimately, death. Symptoms of Creutzfeldt-Jakob disease (CJD) can resemble those of other dementia-like brain disorders, such as Alzheimer's. But Creutzfeldt-Jakob disease usually progresses much more rapidly.
CJD captured public attention in the 1990s when some people in the United Kingdom developed a form of the disease â variant CJD (vCJD) â after eating meat from diseased cattle. However, "classic" Creutzfeldt-Jakob disease hasn't been linked to contaminated beef.
Although serious, CJD is rare, and vCJD is the least common form. Worldwide, there is an estimated one case of CJD diagnosed per million people each year, most often in older adults.
Source: http://www.mayoclinic.com
Creutzfeldt-Jakob disease is marked by rapid mental deterioration, usually within a few months. Initial signs and symptoms typically include:
As the disease progresses, mental symptoms worsen. Most people eventually lapse into a coma. Heart failure, respiratory failure, pneumonia or other infections are generally the cause of death. Death usually occurs within a year.
In people with the rarer vCJD, psychiatric symptoms may be more prominent in the beginning, with dementia â the loss of the ability to think, reason and remember â developing later in the illness. In addition, this variant affects people at a younger age than classic CJD does and appears to have a slightly longer duration â 12 to 14 months.
Source: http://www.mayoclinic.com
Creutzfeldt-Jakob disease and its variants belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue.
The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal versions of a kind of protein called a prion. Normally these proteins are harmless. But when they're misshapen, they become infectious and can harm normal biological processes.
The risk of CJD is low. The disease can't be transmitted through coughing or sneezing, touching or sexual contact. The three ways it develops are:
Cases of CJD related to medical procedures are referred to as iatrogenic CJD. Variant CJD is linked primarily to eating beef infected with mad cow disease (bovine spongiform encephalopathy or BSE).
Source: http://www.mayoclinic.com
Only a brain biopsy or an examination of brain tissue after death (autopsy) can confirm the presence of Creutzfeldt-Jakob disease. But doctors often can make an accurate diagnosis based on your medical and personal history, a neurological exam, and certain diagnostic tests.
The exam is likely to reveal characteristic symptoms such as muscle twitching and spasms, abnormal reflexes, and coordination problems. People with CJD also may have areas of blindness and changes in visual-spatial perception.
In addition, doctors commonly use these tests to help detect CJD:
Source: http://www.mayoclinic.com
As with other causes of dementia, Creutzfeldt-Jakob disease profoundly affects the brain as well as the body, although CJD and its variants usually progress much more rapidly. People with CJD usually withdraw from friends and family and eventually lose the ability to recognize or relate to them. They also lose the ability to care for themselves and many eventually slip into a coma. The disease ultimately is fatal.
Source: http://www.mayoclinic.com
There is no known way to prevent sporadic CJD. If you have a family history of neurological disease, you may benefit from talking with a genetics counselor, who can help you sort through the risks associated with your situation.
Hospitals and other medical institutions follow explicit policies to prevent iatrogenic CJD. These measures have included:
To help ensure the safety of the blood supply, people with a risk of exposure to CJD or vCJD aren't eligible to donate blood. This includes people who:
The risk of contracting vCJD in the United States remains extremely low. Only three cases have been reported in the U.S. According to the Centers for Disease Control and Prevention, strong evidence suggests that these cases were acquired abroad â two in the United Kingdom and one in Saudi Arabia.
In the United Kingdom, where the majority of vCJD cases have occurred, fewer than 200 cases have been reported. CJD incidence peaked between 1999 and 2000 and has been declining since.
Most countries have taken steps to prevent BSE-infected tissue from entering the food supply, including:
Source: http://www.mayoclinic.com
Most cases of Creutzfeldt-Jakob disease occur for unknown reasons, and no risk factors can be identified. However, a few factors seem to be associated with different kinds of CJD.
Genetics. People with familial CJD have a genetic mutation that causes the disease. The disease is inherited in an autosomal dominant fashion, which means you need to inherit only one copy of the mutated gene, from either parent, to develop the disease. If you have the mutation, the chance of passing it on to your children is 50 percent.
Genetic analysis in people with iatrogenic and vCJD suggest that inheriting identical copies of certain variants of the prion gene may increase your risk of developing CJD if you're exposed to contaminated tissue.
Exposure to contaminated tissue. People who've received human growth hormone derived from human pituitary glands or who've had grafts of tissue that covers the brain (dura mater) may be at risk of iatrogenic CJD.
The risk of contracting vCJD from eating contaminated beef is difficult to determine. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent.
Source: http://www.mayoclinic.com
We respect your privacy and aim for the best website experience in compliance with local laws. Allowing cookies enables a tailored experience, while disabling them may reduce personalization. For more information, please read our Privacy Policy and Cookie Policy.