Creutzfeldt-Jakob disease (CJD) is a rare fatal disease of humans caused by an unusual infectious agent, a prion protein. It is one of a group of diseases which cause the brain to turn spongy, hence the generic name spongiform encephalopathy, and the inevitable outcome, mental and physical collapse, and death. There are about 50 confirmed cases a year in the UK, mostly in the elderly, although since the diagnosis can only be made after death some cases used to get misdiagnosed as things like Alzheimer’s. Cows suffer from a similar disease, bovine spongiform encephalopathy (BSE) or mad cow disease.
In 1995/6 a new variant of CJD (vCJD) was reported in ten younger people aged 19 to 41, with an average age of 29. The clinical features seemed to differ from the usual pattern, with apathy a big feature of the early stages. Nine of the ten were first referred for psychiatric treatment. After death, signs of spongiform change and the prion proteins were found in the brain.
In March 1996 scientists convinced the government that the most likely cause of the new variant was eating BSE-infected cattle, and a marvellous health scare was launched. Nearly everyone eats meat, and the new disease had a long incubation period so hey presto, everyone except the vegetarians might die. Wild predictions were made. Up to 500,000 deaths over the following ten years were widely predicted.
How did the numbers pan out? The figures are easily available from the UK Dept of Health. Prior to 1995 there were no cases.
*partial figures because of the delay between onset and firm classification (mean 11 months).
We'll keep these numbers updated from time to time. The present table is taken from Andrews et al. Lancet 2003; 361: 751-2.
vCJD confirmed deaths updated from the CJD Surveillance Unit April 2004 click here
Nottingham 22 Dec 2002
We keep hearing rumours of a similar scare in the United States, presumably drummed up by vegetarians, and public health officials with a vested interest in such things. To date (Dec 2002) the number of vCJD cases in the US is zero.
Postscript July 2004
The above may not be strictly true. A Florida woman, Charlene Singh, who was born in London and had moved to the US in 1992, was diagnosed with variant Creutzfeldt-Jakob disease in 2002 and died in June 2004. All the evidence suggests that she contracted the disease in England.
Table updated 7 November 2005 from CJD surveillance unit.
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