Diseases, Conditions, Syndromes

Cascade of events leading to prion disease described

Prion diseases are deadly neurodegenerative disorders in humans and animals that are characterized by misfolded forms of prion protein (PrP). Development of effective treatments has been hampered by the lack of good experimental ...

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Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of mule deer, whitetailed deer, elk (wapiti), and moose. TSEs are caused by unusual infectious agents known as prions. To date, CWD has been found mainly in cervids (members of the deer family). First recognized as a clinical "wasting" syndrome in 1967 in mule deer in a wildlife research facility in northern Colorado, USA, it was identified as a TSE in 1978 and has spread to a dozen states and two Canadian provinces. CWD is typified by chronic weight loss leading to death. There is no known relationship between CWD and any other TSE of animals or people.

Although there have been reports in the popular press of humans being affected by CWD, a study by the CDC suggests that "[m]ore epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions." The epidemiological study further concludes that, "[a]s a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified."

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