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The human form of BSE is broadly similar to Creutzfeldt-Jakob disease, but differs in a number of clinical and anatomical respects. For example, it affects younger patients (average age 29, versus 65 for the classic disease) and has a relatively longer course (median 14 months, versus 4.5 months). [ 7 ]
Time from onset of symptoms to death is generally weeks to months. [2] Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). [3] As of 2018, a total of 231 cases of vCJD had been reported globally. [5] BSE is thought to be due to an infection by a misfolded protein, known as a prion.
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 ...
TSEs in non-human mammals include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle – popularly known as "mad cow disease" – and chronic wasting disease (CWD) in deer and elk. The variant form of Creutzfeldt–Jakob disease in humans is caused by exposure to bovine spongiform encephalopathy prions. [4] [5] [6]
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]
The disease is found most frequently in people 55–65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms.
Lacey warned of the dangers of BSE before the crisis was revealed by the government. [8] Lacey believed there was a "systematic cover-up" from the government and scientists about the dangers of food that British people eat. [8] [9] He made headlines after a Sunday Times interview in which he called for the slaughter of all BSE-infected herds. [3]
Scrapie and other transmissible spongiform encephalopathies are caused by prions. [19] Prions were determined to be the infectious agent because transmission is difficult to prevent with heat, radiation and disinfectants, the agent does not evoke any detectable immune response, and it has a long incubation period of between 18 months and 5 years. [20]