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Published On: Sat, Jun 15th, 2013

Canada: Fraser health officer confirms case of Creutzfeldt-Jakob disease (CJD), says there is no outbreak

Responding to several media accounts of an outbreak of the deadly prion disease, Creutzfeldt-Jakob disease (CJD) in Fraser, British Columbia (BC), Dr. Paul Van Buynder, Chief Medical Health Officer of Fraser Health says, “There is no outbreak and I want to reassure residents of the lower mainland that there is no risk to the public or to residents in our facilities.”

Public domain image/John Fowler

Public domain image/John Fowler

Fraser Health has investigated six possible cases of CJD over the past year.  After a case review today we can say that one person has CJD and two others are very likely to have it.

The other three cases are very unlikely to have CJD, according to health officials in a press release June 14.

“I am concerned to see reports this evening in social and traditional media related to our investigation into a small number of possible cases of Creutzfeldt-Jakob disease (CJD). Media have reported that these cases are connected to Bovine Spongiform Encephalopathy – more commonly known as mad cow disease, Van Buynder said.

“I want to be clear there is absolutely no evidence that these three confirmed or probable cases are linked to food consumption.”

These are classical cases of CJD.  Tests conducted on these patients have ruled out variant CJD associated with disease in cattle.

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals, according to the US Centers for Disease Control and Prevention (CDC).

The causative agents of TSEs are believed to be prions. The term “prions” refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain.

Prion diseases are usually rapidly progressive and always fatal.

Classic CJD is a human prion disease. It is a neurodegenerative disorder with characteristic clinical and diagnostic features. This disease is rapidly progressive and always fatal. Infection with this disease leads to death usually within 1 year of onset of illness.

Classic CJD is not related to “mad cow” disease. Classic CJD also is distinct from “variant CJD”, another prion disease that is related to BSE.

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About the Author

- Writer, Co-Founder and Executive Editor of The Global Dispatch. Robert has been covering news in the areas of health, world news and politics for a variety of online news sources. He is also the Editor-in-Chief of the website, Outbreak News Today and hosts the podcast, Outbreak News Interviews on iTunes, Stitcher and Spotify Robert is politically Independent and a born again Christian Follow @bactiman63

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  1. Terry S. Singeltary Sr. says:

    Sadly, in the year 2013, we are still going by science that is 3 decades old, to manage risk factors from the many different strains of the TSE prion disease.

    Dr. Paul Van Buynder et al state ;

    > I want to be clear there is absolutely no evidence that these three confirmed or probable cases are linked to food consumption.

    when in reality, you have no clue Sir.

    sporadic CJD is not a single strain of the TSE prion sporadic phenotypes (there are many, and they are mounting).

    sporadic CJD has now been linked to atypical BSE, and atypical Scrapie, and many reputable scientist around the globe are especially concerned with the CWD of cervids, and it’s different strains, which we now know there are more strains.

    the UKBSEnvCJD only theory is a false myth, and proven to be so.

    also, all iatrogenic CJD is, is sporadic CJD until route and source of the TSE agent is confirmed.

    what fuels this madness, and the spread of this disease, are Doctors and officials that continue to spread this junk science.

    you are part of the problem, in my opinion, and I mean no disrespect Sir.

    if I still sound angry, I am, 15+ years later.

    I have wasted 15 years daily following the science as it emerges with the TSE prion disease, and documenting it. I hope you take the time to read some of it.

    no need to reply, most never do, but I urge you to educate yourself on this topic of the TSE prion disease, and cease spreading the false science that continues to fuel the spread of this TSE prion disease. …

    thank you, kind regards,

    terry

    layperson …lost my mom to the hvCJD ‘confirmed’, and I just made a promise. …

    Saturday, June 15, 2013

    Canada Fraser Health Statement on Creutzfeldt-Jakob Disease outbreak

    http://creutzfeldt-jakob-disease.blogspot.com/2013/06/canada-fraser-health-statement-on.html

    sporadic cjd has been linked to atypical BSE and atypical scrapie. it’s science. deny it if you must. …

    *** 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…

    http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1

    http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf

    Thursday, August 12, 2010

    Seven main threats for the future linked to prions

    First threat

    The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

    ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

    Second threat

    snip…

    http://www.neuroprion.org/en/np-neuroprion.html

    http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html

    http://prionpathy.blogspot.com/

    Rural and Regional Affairs and Transport References Committee

    The possible impacts and consequences for public health, trade and agriculture of the Government’s decision to relax import restrictions on beef Final report June 2010

    2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

    2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. *** Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

    http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf

    on the 10 year anniversary of mad cow disease in Canada, your still just kidding yourself, your readers, and at the same time, putting everyone at risk by this fallacy. …tss

    CFIA, USDA, AND OIE SHOOT, SHOVEL, AND SHUT THE HELL UP SSS BSE TSE PRION MAD COW TYPE POLICY $$$, and the media is buying it hook, line, and sinker $$$

    EDMONTON – Some of former Alberta premier Ralph Klein’s most colourful quotes — and the reactions they elicited:

    SNIP…

    “This all came about through the discovery of a single, isolated case of mad cow disease in one Alberta cow on May 20th.

    The farmer — I think he was a Louisiana fish farmer who knew nothing about cattle ranching.

    *** I guess any self-respecting rancher would have shot, shovelled and shut up, but he didn’t do that.” — Klein recalls how the mad cow crisis started and rancher Marwyn Peaster’s role.

    The premier was speaking at the Western Governors Association meeting in Big Sky, Mont. September 2004.

    http://www.edmontonjournal.com/news/Kleinisms+former+Alberta+premier+Ralph+Klein+sound+bite/8171110/story.html

    Wednesday, December 22, 2010.

    Manitoba veterinarian has been fined $10,000 for falsifying certification documents for U.S. bound cattle and what about mad cow disease?

    http://usdameatexport.blogspot.com/2010/12/manitoba-veterinarian-has-been-fined.html

    CENSORSHIP IS A TERRIBLE THING $$$.

    Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$.

    THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$.

    Thursday, February 10, 2011.

    TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31.

    http://madcowtesting.blogspot.com/2011/02/transmissible-spongiform-encephalopathy.html

    Thursday, January 17, 2013.

    Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection.

    http://madcowtesting.blogspot.com/2013/01/canada-us-agree-on-animal-disease.html

    Wednesday, August 11, 2010.

    REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA.

    http://bse-atypical.blogspot.com/2010/08/report-on-investigation-of-sixteenth.html

    Thursday, August 19, 2010.

    REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA.

    http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html

    Friday, March 4, 2011.

    Alberta dairy cow found with mad cow disease.

    http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/alberta-dairy-cow-found-with-mad-cow.html

    Increased Atypical Scrapie Detections.

    Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.

    http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf

    Thursday, May 30, 2013

    World Organization for Animal Health (OIE) has upgraded the United States’ risk classification for mad cow disease to “negligible” from “controlled”, and risk further exposing the globe to the TSE prion mad cow type disease

    U.S. gets top mad-cow rating from international group and risk further exposing the globe to the TSE prion mad cow type disease

    http://madcowusda.blogspot.com/2013/05/world-organization-for-animal-health.html

    http://transmissiblespongiformencephalopathy.blogspot.com/2013/05/statement-from-agriculture-secretary.html

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