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Bovine Spongiform Encephalopathy, Mad Cow Disease
10-08-2014, 12:52 PM
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Bovine Spongiform Encephalopathy, Mad Cow Disease
Mad Cow Disease is a Prion that effects the brain causing a degenerative neurological disease.

Info

Quote:Can people get Mad Cow Disease?

No. Mad Cow Disease is an illness of cattle. However, a new form of human TSE has appeared in England called the new variant of Creutzfeld-Jakob disease (vCJD). In recent years, over 100 cases of vCJD have been confirmed in England. Mounting evidence suggests that the cluster of vCJD is due to the same agent that caused BSE in cattle.

Animal Transmission,

Bovine, Swine, Ovine, Caprine, Extreme Zoolgy

Human Transmission

Blood, Sperm

Symtoms
Quote:The first symptom of CJD is rapidly progressive dementia, leading to memory loss, personality changes and hallucinations. Other frequently occurring features include anxiety, depression, paranoia, obsessive-compulsive symptoms, and psychosis.[11] This is accompanied by physical problems such as speech impairment, jerky movements (myoclonus), balance and coordination dysfunction (ataxia), changes in gait, rigid posture, and seizures. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks.[citation needed] In some people, the symptoms can continue for years. In most patients, these symptoms are followed by involuntary movements and the appearance of an atypical diagnostic electroencephalogram tracing. Most victims die six months after initial symptoms appear, often of pneumonia due to impaired coughing reflexes. About 15% of patients survive for two or more years.[12] Some patients have been known to live 4–5 years with mostly psychological symptoms until the disease progresses causing more physical symptoms leading to a diagnosis and inevitable death usually within the first year of diagnosis.

The symptoms of CJD are caused by the progressive death of the brain's nerve cells, which is associated with the build-up of abnormal prion proteins forming amyloids. When brain tissue from a CJD patient is examined under a microscope, many tiny holes can be seen where whole areas of nerve cells have died. The word "spongiform" in "transmissible spongiform encephalopathies" refers to the sponge-like appearance of the brain tissue.

Quote:The defective protein can be transmitted by contaminated harvested human brain products,[18] immunoglobulins (IVIG), corneal grafts, dural grafts or electrode implants (acquired or iatrogenic form (iCJD); it can be familial (fCJD); or it may appear for the first time in the patient (sporadic form: sCJD). In the familial form, a mutation occurs in the gene for PrP, PRNP. Ten to fifteen percent of CJD cases are familial. (CDC)

The disease has also been shown to result from use of human growth hormone obtained from the pituitary glands of persons who died from Creutzfeldt–Jakob Disease,[19] though the known incidence of this cause is (as of April 2004) quite small. The risk of infection via cadaveric HGH in the US ceased when the medication was withdrawn in 1985.[citation needed]

It is thought that humans can contract the disease by consuming material from animals infected with the bovine form of the disease.[20] The only suspected cases to arise thus far have been vCJD with cases in the UK and Canada, moreover there are fears--based on animal studies[21]--that consuming beef or beef products containing prion particles can also cause the development of classic CJD. When BSE material infects humans, the resulting disease is known as (new) variant CJD (nvCJD).[14]

Cannibalism has also been implicated as a transmission mechanism for abnormal prions, causing the disease known as kuru, once found primarily among women and children of the Fore people in Papua New Guinea. While the men of the tribe ate the body of the deceased and rarely contracted the disease, the women and children, who ate the less desirable body parts, including the brain, were eight times more likely than men to contract kuru from infected tissue.

Prions, the infectious agent of CJD, may not be inactivated by means of routine surgical instrument sterilization procedures. The World Health Organization and the US Centers for Disease Control and Prevention recommend that instrumentation used in such cases be immediately destroyed after use; short of destruction, it is recommended that heat and chemical decontamination be used in combination to process instruments that come in contact with high-infectivity tissues. No cases of iatrogenic transmission of CJD have been reported subsequent to the adoption of current sterilization procedures, or since 1976.[22][23][24] Copper-hydrogen peroxide has been suggested as an alternative to the current recommendation of sodium hydroxide or sodium hypochlorite.[25] Thermal depolymerization also destroys prions in infected organic and inorganic matter, since the process chemically attacks protein at the molecular level, although more effective and practical methods involve destruction by combinations of detergents and enzymes similar to biological washing powders.[26]
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