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Creutzfeldt Jakob Disease

Get the facts on Creutzfeldt Jakob Disease treatment, diagnosis, staging, causes, types, symptoms. Information and current news about clinical trials and trial-related data, Creutzfeldt Jakob Disease prevention, screening, research, statistics and other Creutzfeldt Jakob Disease related topics. We answer all your qestions about Creutzfeldt Jakob Disease.

Question: how do humans acquire creutzfeldt-jakob disease or any prion disorders? people from papua new guinea obtained a strain of TSE, and some have obtained it from cows that are infected with mad cow disease. it can also be transmitted by blood transfusion. any other examples in which how humans obtain them? or better yet, how do they appear?

Answer: By far the most common (but still rare): Creutzfeld Jakob Disease (CJD) can be hereditary--the usual example is an hereditary disease most common in Sepharidic (particularly Libyan) Jews. This is known as familial CJD. Close to 15% of all cases are hereditary. Or sporadic CJD--the result of a spontaneous gene mutation or perhaps just sponeous conversion of the prion protein to the misfolded, aggregated state. 85% of all cases. The above two sources kill about 5000 people per year in the United States. Last I heard only one American had developed the disease originating with mad cow disease (vCJD), a woman who used to live in the UK. Rare: Cannibalism I believe is where it was first observed--the disease is called Kuru, the tribe is the Fore of Papua New Guinea. People caught it from eating the brains of cannibalism victims. And of course mad cow disease (vCJD in humans)--by eating cows (or potentially other animals). Not all the cow--just the brain or spinal tissue. Obviously this is more common in the UK at the moment, although I understand the number of cases is dropping. Other potential sources of transmission include such things as neurosurgery, corneal grafts and hormone injections. Also by reuse of surgical equipment. All of these situations lead to proteins that are misfolded and aggregated, can propagate themselves and are infectious. The aggregated proteins are insoluble and accumulate, killing brain cells. Many of the mutations in the prion protein leading to CJD are known. See the second reference. .


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