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 

YouTube Video

Sporadic CJD in December 2016


An internet review suggests that sporadic CJD is the main type of Creutzfeldt-Jakob disease in the UK.  It accounts for 85 percent of casesThe 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.  The problem with this argument is that 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 all sporadic CJD is not  linked to mad cow disease (BSE).

The consensus argument suggests that the disease occurs in older adults (adults aged between 45 and 75).  However, it has been argued that the UK is seeing cases of sporadic CJD in younger patients (see blog for 28th March 2012).


The implications of sporadic CJD possibly being linked to BSE


It is argued that 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 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.  See also the Edinburgh University CJD Figures




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 Sporadic CJD and Alzheimer's Disease

YouTube Video

The 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 see 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 sporadic CJD or new variant CJD.

PART 3:  The Possible Link Between BSE and Sporadic CJD in the UK

YouTube Video

It is possible that some sporadic CJD in Britain may be caused by BSE (in any of its variants) 

This video is a response to the Director of the Prion Clinic on the topic of  Creutzfeldt-Jakob Disease (CJD).
This response video comments upon an  'NHS Choices'  video which is no longer available.

In the 'NHS Choices' video, (2 minutes 40 seconds),  only one strain of BSE-related CJD was mentioned. In other words, just variant CJD. 
The commentary on CJD, in the NHS Choices video, appears to be different to the findings of his own research from 2002.
The work discussed below suggests that there is more than one strain of BSE-related CJD; albeit in mice.

To summarise, BSE may cause the sporadic type of CJD.  Therefore, 'the true threat of human BSE may be hidden'.  
See the BBC article below for the full story.

See the link below for an article from Nature: prion data suggest BSE link to sporadic CJD.  Another journal article makes a simiar point.

A journal paper suggests that variant CJD is propagated (caused or transmitted) as sporadic or variant-like CJD strain in the brain of mice (i.e. there appear to be two strains in mice).

Commentary on Sporadic Creutzfeldt–Jakob Disease

The problem is that some apparently "sporadic" (cases of the) disease could be linked with BSE too, which is now under investigation".
According to the scientist "the incidence of Sporadic CJD (which affects older people) has risen each year. We could say its 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 dont think that's a coincidence".
Research has linked some cases of sporadic CJD to BSE.  See "New strain of BSE disease discovered".
It may not be accurate to suggest that cases of sporadic CJD are entirely random or without any cause.