Variant Creutzfeldt–Jakob disease

[13] Jonathan Quick, instructor of medicine at the Department of Global Health and Social Medicine at Harvard Medical School, stated that bovine spongiform encephalopathy (BSE) is the first man-made epidemic, or "Frankenstein" disease, because a human decision to feed meat and bone meal to previously herbivorous cattle (as a source of protein) caused what was previously an animal pathogen to enter into the human food chain, and from there to begin causing humans to contract vCJD.

Given the large number of U.S. military personnel and their dependents residing in Europe, it was expected that over 7% of donors would be deferred due to the policy.

Later changes to this policy first relaxed the restriction to a cumulative total of five years or more of civilian travel in European countries (six months or more if military) then, in 2022, removed it entirely.

[23] Similar regulations are in place where anyone having spent more than six months for Germany or one year for France living in the UK between January 1980 and December 1996 is permanently banned from donating blood.

[26] In Poland, anyone having spent cumulatively six months or longer between 1 January 1980 and 31 December 1996 in the UK, Ireland, or France is permanently barred from donating.

[27] In France, anyone having lived or stayed in the United Kingdom a total of over one year between 1 January 1980 and 31 December 1996 is permanently barred from donating.

[29] In Finland, anyone having lived or stayed in the mainland United Kingdom or its Crown Dependencies for a total of over six months between 1 January 1980 and 31 December 1996 is permanently barred from donating.

Emilie Jaumain, who died in 2019, at the age of 33, had been the victim of a work accident in 2010, during which she had pricked herself with a tool contaminated with infected brain.

Indeed, inspections have noted serious failures in the protection of agents in the face of this deadly risk,[42][43] and the long incubation period of this disease leads to fears of new cases in the future, hence great concern.

Creutzfeldt-Jakob Disease and eating squirrel brains, the 1997 article by the Lancet has been almost entirely discredited due its lack of scientific evidence and false statistical assumptions.

This is especially important with a slow moving, and hard to trace disease like vCJD, a point emphasized in their statement that "the Lancet study blundered blithely into such statistical pitfalls".

Since that brief report, there has been no convincing evidence found suggesting that the consumption of squirrel meat, brain or otherwise, is a risk factor for any prion disease.

Without additional experimental or epidemiological evidence, a link between consumption of squirrel brain and human prion disease is unjustifiably speculative.”[48] An article by Democrat and Chronicle provided another primary source debunking this myth.

The human PRNP protein which is subverted in prion disease can occur with either methionine or valine at amino acid 129, without any apparent physiological difference.

[2] The following confirmatory features should be present:[2] vCJD is a separate condition from classic Creutzfeldt–Jakob disease (though both are caused by PrP prions).

[56] The Lancet in 2006 suggested that it may take more than 50 years for vCJD to develop, from their studies of kuru, a similar disease in Papua New Guinea.

[57] The reasoning behind the claim is that kuru was possibly transmitted through cannibalism in Papua New Guinea when family members would eat the body of a dead relative as a sign of mourning.

[58] In the late 20th century, however, kuru reached epidemic proportions in certain Papua New Guinean communities, therefore suggesting that vCJD may also have a similar incubation period of 20 to 50 years.

They have also proposed that individuals having contracted CJD in the early 1990s represent a distinct genetic subpopulation, with unusually short incubation periods for bovine spongiform encephalopathy (BSE).

Large scale studies in the UK have yielded an estimated prevalence of 493 per million, higher than the actual number of reported cases.

[60] The first human death from vCJD occurred in the United Kingdom; Wiltshire teenager Stephen Churchill died on 23 May 1995, aged 19.

[63] The survey provides the most robust prevalence measure to date—and identifies abnormal prion protein across a wider age group than found previously and in all genotypes, indicating "infection" may be relatively common.

Genetic testing of the 16 positive samples revealed a higher proportion of valine homozygous (VV) genotype on the codon 129 of the gene encoding the prion protein (PRNP) compared with the general UK population.

The government assured the public that British beef was safe to eat, with agriculture minister John Gummer famously feeding his daughter a burger.

[72] It is located on the boundary wall of St Thomas' Hospital in Lambeth facing the Riverside Walk of Albert Embankment.

Electroencephalogram of a person with suspected CJD showing typical periodic bursts of triphasic sharp waves
Dark green areas are countries that have confirmed human cases of variant Creutzfeldt–Jakob disease and light green are countries that have bovine spongiform encephalopathy cases
Deaths in the UK from Creutzfeldt–Jakob disease 1990–2014: while cases of vCJD have declined (green), reported cases of sporadic CJD continue to increase (blue)
Commemorative plaque in London paying tribute to people who died from vCJD