400621 Lecture Notes - Lecture 20: Bovine Spongiform Encephalopathy, Transmissible Spongiform Encephalopathy, Amyloid

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Variant creutzfeldt jakob disease (vcjd) is a type of brain disease within the transmissible spongiform encephalopathy family. Symptoms include psychiatric problems, behavioural changes, and painful sensations. The length of time between exposure and the development of symptoms is unclear, but is believed to be years. Average life expectancy following the onset of symptoms is 13 months. It is caused by prions, which are mis-folded proteins. Spread is believed to be primarily due to eating bovine spongiform encephalopathy (bse)-infected beef. Infection is also believed to require a specific genetic susceptibility. Spread may potentially also occur via blood products or contaminated surgical equipment. Diagnosis is by brain biopsy but can be suspected based on certain other criteria. It is different from classic creutzfeldt-jakob disease though both are due to prions. 170 cases of vcjd have been recorded in the united kingdom, and 50 cases in the rest of the world. The disease has become less common since 2000.

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