CJD: The Increasing Number of Sporadic CJD Cases

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PART 1: A Video on the Increase in Sporadic CJD in the United Kingdom

An internet review suggests that sporadic CJD is the main type of Creutzfeldt-Jakob disease in the UK. It accounts for 85 percent of cases. The NHS website suggests that the cause of the disease is unclear but that it occurs when "a normal brain protein changes abnormally ... and turns into a prion". The Alzheimer's Association argues that the disease "develops spontaneously for no known reason". The UCL prion unit states that the disease "occurs 'out of the blue', at random, in the population". There appears to be a consensus that sporadic CJD has an unknown cause. However, there has been a clear increase in the incidence of sporadic CJD as shown in the video. This increase is unlikely to have occurred by chance. It would be unwise to argue that every case of sporadic CJD is not linked to mad cow disease (BSE).

Arguably, kuru and other prion diseases that affect humans are "just variants of the same disease (as) all of these diseases consist of a prion infection in the brain" (Waldman and Lamb 2004:204). If sporadic and variant CJD are similar diseases, with similar causes, then they could be categorised together. If the variant disease is combined, with the sporadic disease, then the total number of official CJD deaths in the UK rises from nearly 200, to nearly 2,000, at the end of 2016. This is by combining the sporadic CJD with the variant CJD. The figures for CJD would also be higher if 'possible' cases of CJD were included as well as definite and probable cases.

The number of cases of sporadic CJD has doubled since the middle of the 1990s which justifies the enforcement of food safety regulations. The Edinburgh University CJD Figures provide the latest information.

Reference: Waldman, M. and Lamb, M. (2004), Dying for a Hamburger: Modern Meat Processing and the Epidemic of Alzheimer's Disease, New York: Thomas Dunne Books

PART 2: The Possible Link Between BSE and Sporadic CJD in the UK

The consensus argument suggests that sCJD occurs in older adults (adults aged between 45 and 75). However, research now needs to find out whether sporadic CJD is increasing in younger people. According to the scientist in the video "the incidence of Sporadic CJD (which affects older people) has risen each year. We could say it is because we are better at diagnosis, but I believe it could be due to BSE in the food chain; which can make people more susceptible to developing sporadic CJD. I have also seen much younger cases of sporadic CJD patients. I do not think that's a coincidence". The source for this quote was on the Justice for Andy website; but the quote is no longer available. Many ‘sporadic’ cases of the disease could be linked with BSE. Research has linked some cases of sporadic CJD to BSE according to the CJD Support Network ("New strain of BSE disease discovered", March 2004). Again, it may not be accurate to state that cases of sporadic CJD are entirely random or without any cause.

PART 3: Sporadic CJD and Alzheimer's Disease

There is concern over an increase in sporadic CJD; and its potential misdiagnosis as Alzheimer's Disease.

The 1 in 33,000 figure, used in the presentation, is a 'headline figure'. It would be very difficult to generalise to 30 cases per million or 1,800 cases in the total U.K. population. These figures are only intended as a starting point for further investigation. This 1 in 33,000 figure, is taken from the 'Justice for Andy' website; blog for Friday 8th January 2010.

CJD is no longer a rare disease according to University College London's Prion Unit. In a UCL lecture, on December 8th 2009, he stated that: "In a person's lifetime the risk of developing sporadic CJD is now 1 in 33,000". The figure could be as high as 1 in 9,000 in the United States. Although this appears to be for over age 50's only. One of the problems, with estimating the number of people with CJD, is that there may not be enough testing being undertaken. More testing is needed for CJD; whether this is for sporadic CJD or new variant CJD.

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