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Atypical BSE Has Never Led To Human vCJD – But Could It?

There is good news and bad news about the “L-type” atypical mad cow phenotype, found in the nearly 11-year-old, dead dairy cow discovered two weeks ago in California.

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The good news is no human has ever contracted variant Creutzfeldt-Jakob (vCJD) disease from cattle infected with atypical bovine spongiform encephalopathy (BSE).   vCJD is the human version of mad cow disease, a fatal neurodegenerative disorder.

The bad news is peer-reviewed findings by French neuroscientist Thierry Baron published in the January edition of Emerging Infectious Diseases indicate it is too soon to say incidents like the California cow are just a spontaneous cases. Baron found mouse lemurs may contract L-type BSE more easily than the classic version.

But with prion researchers around the world working on the topic, that likely will not be the last word. Spontaneous vCJD occurs in humans in about one in one million cases.

Dr. Michael Hansen, a Consumers Union scientist who has long tracked mad cow disease, thinks there is a possibility L-type BSE is “not necessarily a spontaneous case.”

Hansen, speaking for CU last week, called upon USDA to adopt the following steps in the wake of the atypical BSE finding in a non-ambulatory cow:

- Increase the BSE surveillance program beyond the current 40,000 tests annually that Hansen calls “very small” but USDA says is 10 times more than required by world standards.

- Allow private companies to test for BSE at their own expense. Federal courts have said USDA has power over BSE tests and so far it has blocked private testing.

- Prohibit the use of poultry litter for feed, because birds may have consumed agents harboring the infectious mad cow agent, and prohibit cow blood as a milk replacer for weaning calves.  USDA has not seen either practice as high risk.

USDA officials have said, over and over, during the past week that a system of interlocking defenses they’ve erected to combat BSE is working.   Among these are:

- Removal of specified risk materials, meaning those parts of the animal that would contain BSE if the animal had the disease. These include brain, spinal cord and digestive tracts.

- The ruminant-to-ruminant feed ban that has been in effect since 1997.

- The BSE surveillance program, which includes those 40,000 tests a year, many conducted at targeted places like the rendering transfer station in Hanford, CA where the cow infected with atypical mad cow was found.

Until recently, many prion researchers believed the L-type BSE strain is probably not posing a danger to human because it is so rare people are typically not exposed to it.  

But the University of Edinburgh’s Rona Barron, a molecular biologist, has recently found that the L-type prion has a harder time infecting normal human brain protein. 

There are actually two atypical BSE phenotypes, L and K. They are classified according to high and low molecular mass. Classic BSE (C-type) is thought to stem from food chain contamination by a single prion strain.   

Feed is a common transmission source for Classic BSE.

The world campaign against mad cow disease has driven the number of BSE cases down to 29 in 2011, a 99 percent reduction since the peak of 37,311 cases in 1992.  The U.S. announced the nation’s fourth mad cow discovery on April 24.  

Three others turned up in 2003 in Washington state, in 2005 in Texas, and in 2006 in Alabama.  The first U.S. mad cow was a classical case, imported from Canada and infected from eating feed contaminated with diseased brain and spinal bits.

Two California dairies were under quarantine,  awaiting the USDA investigation into whether the two herds include any birth cohorts of the BSE-infected cow.  The diseased cow had two calves in the last two years, one stillborn.

Dr. John Clifford, USDA chief veterinarian, said the surviving calf was euthanized.  Tests on it for BSE were negative.  

The exact location of the two dairies has not been disclosed.  Once any cohorts are identified, the quarantine will be scaled back to those animals or eliminated if none exist on one dairy or the other.

Clifford says about 60 cases of atypical BSE have been identified worldwide.

USDA recently agreed to comply with all World Health Organization protocols for mad cow.   As a result, it will provide brain tissues from the latest diseased cow to laboratories in both Canada and Europe.

That participation may have helped the U.S. beef industry avoid any export restrictions  in any of its four largest markets –Canada, Mexico, Japan and South Korea — following the latest mad cow discovery.  Only tiny Indonesia has suspended U.S. beef.

© Food Safety News
  • http://www.bse-expert.cz hlasny

    Acording to my opinion; what is the common denominator of the neurodegenerative diseases (mad cow disease…) , including Alzheimer’s disease? This was 11 years ago ( March 2001), when I published (in Czech) an alternative theory ( BSE ammonia- magnesium theory), where the main role of NMDA receptors was described. In addition, in scientific literature there was described the effect of drugs, in Alzheimer’s disease in humans, on the principle of control hyperfunction of NMDA receptors, which is consistent with my BSE alternative theory of BSE. See more about the Nameda; A medication known as Namenda® (memantine), an N-methyl D-aspartate (NMDA) antagonist, is prescribed to treat moderate to severe Alzheimer’s disease (www.nia.nih.gov/alzheimers/publication/alzheimers-disease-medications-fact-sheet
    I worked in the USA almost one year- West Virginia University (1991, Morgantown). There I obtained a lot of information from scientific journals (in former Czechoslovakia it was not possible), what I used to create an alternative theory of the origin and the spread of mad cow disease (BSE). Relevant findings I have published 11 years ago in Czech (March 2001) and in English (May 2002; Netherlands, International Journal “Feed Mix”; http://www.warmwell.com/lone_voices_in_the_bse_debate1.pdf ). Later, in August 2006, I published own website, see; http://www.bse-expert.cz
    Since then I pointed my website in the order of hundreds of magazines around the world, especially in the U.S.. See some of them, for example on Google; http://www.google.com/search?q=hlasny+bse+expert&hl=cs&lr=&start=0&sa=N&filter=0 . In 2008, I repeatedly visited the USA, it was about the occasion of my presentation in Vancouver, Canada (July 2008; 29th World Veterinary Congress; Neurodegenerative Diseases and Schizophrenia as a Hyper or Hypofunction of the NMDA Receptors (www.bse-expert.cz/pdf/Veter_kongres.pdf).
    According to my theory, the origins of the neurodegenerative diseases may lie in chronic magnesium deficiency coupled with a high protein intake. So defective prions are markers of the diseases rather than the cause and BSE can be a naturally occurring disease, not an infectious disease. WHY?
    Because, about the BSE/ vCJD diseases; this was never justified scientifically! It was pure, math-model-driven science fiction. But it was pushed very vigorously by the British science establishment, which has never confessed to its errors… See more about the; BSE/ vCJD mathematical- models, see recent large three comments (February 2010) in Telegraph.co.uk (www.telegraph.co.uk/health/healthnews/7168326/Does-vCJD-still-pose-a-major-public-health-threat.html ).
    However, well-known circumstances can be show (as a detective story); in documenting the first case of disease transmission, by blood transfusion. For more informations see my other large comments (January 12, 2011) in Western Star (www.thewesternstar.com/News/Canada%20-%20World/Business/1969-12-31/article-2095060/Womans-death-in-northern-Italy-is-nations-2nd-fatal-case-of-mad-cow-disease/1 ). At the present time I will complete my website to the final version, so it would be clear among other things, that mad cow disease has never been and is not an infectious disease. Among other things, there much contributed recent research about the Alzhemeir´s disease.

  • Avery

    Existing USDA restrictions have successfully controlled “classic BSE” in the U.S. for a decade and a half. Those same controls should effectively control spontaneous encephalopathies. This article obviously is a sop to Consumers Union scaremongering, jumbling pathogenesis of prion diseases and emphasizing emotion over science the way it does. Very poor journalism here, Dan. Not up to your recent high standards at all.

  • doc raymond

    I wonder if Dr. Hansen eats beef or does he have an agenda against beef that is not clearly stated along with his unreason able demands. Why ask for a ban chicken on litter from feed for cattle when all SRMs are removed before rendered cattle are turned into chicken feed? Because it would drive up the cost of poultry meat AND beef, that’s why. Not one single case of vCJD from eating American beef, only 29 BSE positive tests in the world last year, and only 4 positives ever in the US after more than one million samples, and he asks for more testing?

  • http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/ars-flip-flops-on-srm-removal-for.html Terry S. Singeltary Sr.

    existing USDA restrictions have been a joke since the inception of the partial and voluntary feed ban, that failed terribly. the only poor journalizm is coming from the USDA et al where they have been covering up mad cow disease for decades, AND the same BSe there from after a mad cow is accidently detected. just read the gao and the oig reports. for Pete’s sake, 10 years post partial and voluntary feed ban, 10,000,000 MILLION POUNDS OF BANNED MAD COW PROTEIN went into commerce, that was 2007. 2006 it was measured in TONS, not pounds, much worse. after that, the FDA ceased publishing those reports, that is how bad it got.
    the industry can fool some of us some of the time, but they will not fool all of us all of the time.
    because of the continued lies and cover-ups of mad cow disease here in the USA by the USDA et al, i again call on the OIE to reclassify the USA BSE GBR, to BSE GBR IV.
    also, atypical BSE has been linked to sporadic CJD as well.
    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
    and there is about as much likelyhood that any of these cases are a spontaneous BSE, that there is man will walk on the moon today. please see ;
    However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons.
    “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.
    BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded. It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.
    http://www.washingtonpost.com/national/health-science/case-of-mad-cow-disease-found-in-california-animal-but-food-supply-said-safe/2012/04/24/gIQAtelqfT_story.html
    What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”
    The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. “(The agency) has no foundation on which to base that statement.”
    http://vitals.msnbc.msn.com/_news/2012/05/02/11501754-are-usda-assurances-on-mad-cow-case-gross-oversimplification?lite
    MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story…
    ***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model
    http://wwwnc.cdc.gov/eid/article/18/1/pdfs/11-1092.pdf
    ***Infectivity in skeletal muscle of BASE-infected cattle
    http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf
    ***feedstuffs- It also suggests a similar cause or source for atypical BSE in these countries.
    http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf
    ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.
    http://www.neuroprion.org/en/np-neuroprion.html
    full text ;
    atypical L-type BASE BSE
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/update-from-usda-regarding-detection-of.html
    Tuesday, May 1, 2012
    BSE MAD COW LETTERS TO USDA (Tom Vilsack, Secretary of Agriculture) and FDA (Magaret Hamburg, Commissioner of FDA) May 1, 2012
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/bse-mad-cow-letters-to-usda-tom-vilsack.html
    Wednesday, May 2, 2012
    ARS FLIP FLOPS ON SRM REMOVAL FOR ATYPICAL L-TYPE BASE BSE RISK HUMAN AND ANIMAL HEALTH
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/ars-flip-flops-on-srm-removal-for.html
    Friday, May 4, 2012
    May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/may-2-2012-update-from-aphis-regarding.html
    http://www.regulations.gov/#!documentDetail;D=APHIS-2008-0010-0008
    http://www.regulations.gov/#!docketDetail;D=APHIS-2008-0010
    Sunday, March 11, 2012
    APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html
    Wednesday, April 4, 2012
    Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68
    http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/bovine-spongiform-encephalopathy.html
    TSS