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This disease is probably better known as mad cow disease, which gives it a stigma. This can be a life shattering and even death disease. Dr. Robert Ashley gave some further information on this devastating disease.
Creutzfeldt-jakob disease (CJD) is the most common prion disease in humans. They are infectious but not viruses or bacteria, but are actually proteins. The prions appear to be created from normal proteins that are found on the cell membranes. In prion diseases they reconfigure, but the normal proteins may help inhibit cell death.
Initially this disease will interfere with concentration, memory and judgment that leads to episodes of sleep throughout the day. A person may at first also seem neglectful, pathetic and depressed and quickly gives way to unrelenting mental deterioration. Patients experiencing CJD eventually will develop dementia and can lose the ability to control muscle movements. This results in twitching or jerking contractions. Some researchers believe an unusual “slow virus” or another organism causes CJD. However, they have never been able to isolate a virus or other organism in people with the disease. Furthermore, the agent that causes CJD has several characteristics that are unusual for known organisms such as viruses and bacteria. It is difficult to kill, it does not appear to contain any genetic information in the form of nucleic acids (DNA or RNA), and it usually has a long incubation period before symptoms appear. In some cases, the incubation period may be as long as 50 years. The leading scientific theory at this time maintains that CJD and the other TSEs are caused by a type of protein called a prion.
The harmless and infectious forms of the prion protein have the same sequence of amino acids (the “building blocks” of proteins) but the infectious form of the protein takes a different folded shape than the normal protein. Sporadic CJD may develop because some of a person’s normal prions spontaneously change into the infectious form of the protein and then alter the prions in other cells in a chain reaction.
About 5 to 10 percent of all CJD cases are inherited and arise from a change in the gene that controls formation of the normal prion protein. These prions do not contain genetic information and do not require genes to reproduce themselves. If a mutation occurs in the gene for the body’s normal prior protein, infectious prions can arise. If the prion protein gene is altered in a person’s sperm or egg cells, the mutation can be transmitted to the person’s offspring. Family history is helpful in considering the diagnosis.
CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Exposure to brain tissue and spinal cord fluid from infected individuals should be avoided to prevent transmission of the disease through these materials.
Some animal studies suggest that contaminated blood and related products may transmit the disease, but this has never been shown in humans. Even though millions of people receive blood transfusions each year, there are no reported cases of someone contracting CJD from a transfusion, even among those with hemophilia.
Dr Fredda Branyon