Creutzfeldt-Jakob disease

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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:

Age. Sporadic CJD tends to develop later in life, usually around age 60. Onset of familial CJD occurs slightly earlier, and vCJD has affected people at a much younger age, usually in their late 20s.
Genetics. People with familial CJD have a genetic mutation that causes the disease. To develop familial CJD, a child must have one copy of the mutated gene, which is inherited from either parent. If you have the mutation, the chance of passing it on to your children is 50%.
Exposure to contaminated tissue. People who’ve received infected manufactured human growth hormone, or who’ve had transplants of the infected tissues that cover the brain (dura mater), may be at risk of iatrogenic CJD.

The risk of getting vCJD from eating contaminated beef is very low. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent. Chronic wasting disease (CWD) is a prion disease that affects deer, elk, reindeer and moose. It has been found in some areas of North America. To date, no documented cases of CWD have caused disease in humans.

Creutzfeldt-Jakob disease greatly affects the brain and body. CJD usually progresses quickly. Over time, people with CJD 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 is always fatal.

There’s no known way to prevent sporadic Creutzfeldt-Jakob disease (CJD). If you have a family history of neurological disease, you may benefit from talking with a genetics counselor. He or she can help you sort through the risks associated with your situation.

Preventing iatrogenic CJD
Hospitals and other medical institutions follow clear policies to prevent iatrogenic CJD. These measures have included:

Exclusive use of man-made human growth hormone, rather than the kind derived from human pituitary glands
Destruction of surgical instruments used on the brain or nervous tissue of someone with known or suspected CJD
Single-use kits for spinal taps (lumbar punctures)
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 in the United States. This includes people who:

Have a biological relative who has been diagnosed with familial CJD
Have received a dura mater brain graft
Have received cadaveric human growth hormone
Spent at least three months in the United Kingdom from 1980 to 1996
Spent five years or more in France or Ireland between1980 and 2001
Received a blood transfusion in the U.K., France or Ireland since 1980
The U.K., as well as some other countries, also has certain restrictions regarding blood donations from people with a risk of exposure to CJD or vCJD.

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