Canadian CJD cases are “classical" form

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Several suspected cases of Creutzfeldt-Jakob disease (CJD) in British Columbia have sparked the propagation of rumors and misinformation regarding the cause. Variant CJD, of course, is the extremely rare human disease linked to consumption of beef from cattle with bovine spongiform encephalopathy (BSE). So when health authorities in British Columbia reported potential cases of CJD, conventional media and individuals on social media naturally, and inaccurately, linked the cases to BSE and referred to them as “human mad cow disease.”

News reports cited up to six cases of CJD over the past year in British Columbia, leading to rumors of an “outbreak” of the disease. However, Dr. Paul Van Buynder, Chief Medical Health Officer for Fraser Health in British Columbia set the record straight.

In a public statement, Buynder explained that health officials had investigated six possible cases. One case has been confirmed as CJD after the patient died. Two other cases are likely to be CJD, and the other three are “very unlikely to have CJD.”

Buynder adds that tests conducted on these patients have ruled out variant CJD associated with disease in cattle. “I want to be clear there is absolutely no evidence that these three confirmed or probable cases are linked to food consumption,” he says.

He also points out there has never been a home-grown case of variant CJD in Canada. Two people have been confirmed with the disease in Canada, but both cases were traced to exposure in the United Kingdom.

Buynder also notes that based on statistical probability, about two cases of classical CJD per year would be typical for the Fraser region. Three cases would be within the expected range.

Classical CJD occurs in the global population at a rate of one to two cases per million people per year. In Canada, between 30 and 50 cases of classical CJD are diagnosed each year.
According to the Fraser Health website, classical CJD is recognized to exist in three forms, sporadic, genetic and iatrogenic. Sporadic has an unknown cause, occurs unpredictably and accounts for over 90 per cent of Canadian CJD cases. Genetic CJD is associated with a gene mutation and iatrogenic CJD is acquired from the accidental transmission of the disease through medical procedures.

Read more from Fraser Health.

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Arizona  |  June, 19, 2013 at 09:33 AM

I thought CJD (aka cadaver disease) only occurred in people who were recipients of donated cadavar organs? I've wondered for some time if that information might be available.

Terry S. Singeltary Sr.    
Texas  |  June, 19, 2013 at 04:27 PM

all iatrogenic CJD is, is sporadic CJD, until route and source of the iatrogenic mode of transmission is traced down and documented. 85%+ of all human TSE prion disease, i.e. sporadic CJD, is not a single strain, and does not happen spontaneous. spontaneous TSE prion disease has never been documented in the wild, and proven to be so. it's a myth. there are many routes and sources of the TSE prion here in North America, from many different species, and consumption there from, then those folks have medical, surgical, dental, and or tissue and blood. Monday, October 10, 2011 EFSA Journal 2011 The European Response to BSE: A Success Story *** This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential. snip... Saturday, June 15, 2013 Canada Fraser Health Statement on Creutzfeldt-Jakob Disease outbreak kind regards, terry

Arizona  |  June, 19, 2013 at 04:57 PM

Thank you so much! A great infusion of good information!

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