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Variant Creutzfeldt–Jakob disease (vCJD), formerly known as New variant Creutzfeldt–Jakob disease (nvCJD) and referred to colloquially as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. [7]
Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. [ 4 ] [ 1 ] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. [ 4 ]
The United Kingdom was afflicted with an outbreak of bovine spongiform encephalopathy (BSE, also known as "mad cow disease"), and its human equivalent variant Creutzfeldt–Jakob disease (vCJD), in the 1980s and 1990s. Over four million head of cattle were slaughtered in an effort to contain the outbreak, and 178 people died after contracting ...
Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). [3] The agent can be transmitted to humans by eating food contaminated with it. [18] Though any tissue may be involved, the highest risk to humans is believed to be from eating food contaminated with the brain, spinal cord, or digestive tract.
The variant form of Creutzfeldt–Jakob disease in humans is caused by exposure to bovine spongiform encephalopathy prions. [ 4 ] [ 5 ] [ 6 ] Unlike other kinds of infectious disease, which are spread by agents with a DNA or RNA genome (such as virus or bacteria ), the infectious agent in TSEs is believed to be a prion , thus being composed ...
The 2021 French moratorium on prion research was a three-month moratorium on research on prions in France. The moratorium was announced in July 2021 by several public research institutions after a retired lab worker was diagnosed with Variant Creutzfeldt–Jakob disease and came two years after the death of Émilie Jaumain from the same disease after acquiring it in a lab accident.
Jonathan Simms (1 June 1984 – 5 March 2011) was a man from Belfast, Northern Ireland, who contracted variant Creutzfeldt–Jakob disease (vCJD) in his late teenage years. He was given a post-diagnosis life expectancy of one year, similar to that of other young people who were diagnosed in the same age bracket.
In 2013, Coulthart Cashman et al described at an academic conference Creutzfeldt-Jakob disease reporting in Canada. [ 15 ] In 2014, Coulthart et al studied the RT-QuIC assay, which is "able to detect low levels of the disease-inducing isoform of the prion protein (PrP(d)) in brain tissue biopsies and (CSF)," and "has great potential to become a ...