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From: TSS ()
Subject: CJD AND BSE CLUSTERING IN CANADA ???
Date: December 27, 2006 at 11:47 am PST


CANADA CJD DISTRIBUTION

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html

Figure 3: Locations of 1993 BSE Case and the Four Canadian-born Cases Diagnosed from May 2003 to January 2005


http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/eval2005/image4e.gif


Overview of the Canadian-born BSE Cases (May 2003 to January 2005)


http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/eval2005/image3e.gif

Human version of mad cow hits Manitoba
Three cases suspected from same area in province


The Canadian Press
Published: Friday, December 08, 2006
BRANDON, Man. - Three people in Manitoba, including prominent provincial curler Neil Andrews, are suspected of being infected with a degenerative and fatal brain disease, health officials confirmed Thursday.

Doctors from the Brandon Health Region said they have referred three suspected cases of Creutzfeld-Jakob disease, or CJD, for further testing.

CJD infects about one person in every million.

The facts on CJD
Q&A about mad cow and CJD
Mad cow protein may play role in diabetes
More Body & Health news

Dr. Charles Penner, vice-president of medical services for the Brandon Regional Health Authority, doesn't believe the cases are linked, although a cluster of cases is even rarer than the disease.

"For the province to have three cases in one corner of the province, I suppose that would be unusual," he said.

Andrews, a two-time senior provincial men's curling champion, was actually diagnosed with a suspected case of CJD in September. Andrews, 58, noticed something was wrong when he had trouble keeping his balance during his curling delivery.

After a weeklong stay at the Mayo Clinic in Rochester, Minn., doctors told him he likely had CJD.

"(The curlers) knew I couldn't curl and some of them thought that before," he said, laughing, while at a curling club in Brandon in October. "And almost every guy took the time and stopped and talked to me and talked to me openly about the disease and that was good.

"They tried to understand. I'm one of the unfortunate ones who's going to die but they considered themselves lucky."

Typically, one in three suspected cases turns out to be classical CJD, which can lead to rapid brain deterioration, dementia and mobility trouble.

Unlike variant CJD, which has been linked to beef contaminated by mad cow disease, classical CJD cases most often appear sporadically and affect people between 45 and 75. A total of 14 confirmed cases has been reported in Manitoba in the last decade.

Doctors use MRI scans and spinal taps to test for the presence of an abnormal protein to help diagnose a suspected case. But physicians can't confirm anything until an autopsy on brain tissue is performed.

Most people infected with CJD die within a year of the onset of symptoms.

Dr. Michael Coulthard, director of host genetics and prion disease at the National Microbiology Lab in Winnipeg, said the results of the suspected case are still pending. He said there has only been one cluster of cases reported worldwide -- in Switzerland -- in the last five years.

© The Edmonton Journal 2006
http://www.canada.com/vancouversun/news/story.html?id=5ef5f015-0f75-45f1-aac7-c9dd939eb85a&k=66922

Canada Bans SRMs in Feed, Discovers More BSE

By Tina Caparella

Canadian officials have announced that effective July 12, 2007, specified risk materials (SRMs) from cattle will be banned from all animal feeds, pet foods, and fertilizers as an accelerated enhancement to Canada’s eradication of bovine spongiform encephalopathy (BSE). This measure is in addition to feed controls put in place in 1997 prohibiting the use of ruminant proteins in ruminant feed, which has been attributed to the low level of BSE in Canada. Since May 2003, seven cases of BSE have been detected in Canada’s herd of roughly 17 million cattle. In the new measure, SRMs are defined as the skull, brain, trigeminal ganglia (nerves attached to the brain), eyes, tonsils, spinal cord, and dorsal root ganglia (nerves attached to the spinal cord) of cattle 30 months of age or older, and the distal ileum (portion of the small intestine) of cattle of all ages. These tissues have been shown in infected cattle to contain concentrated levels of the BSE agent.


According to the Canadian Food Inspection Agency (CFIA), excluding SRMs in all animal feeds will address potential contamination that could occur during feed production, transportation, storage, and use on the farm. Removing SRMs from pet food and fertilizers is intended to mitigate the risk associated with the potential exposure of cattle and other susceptible animals to BSE through the misuse of these products. CFIA Chief Veterinary Officer Dr. Brian Evans said that the measure does not guarantee that BSE won’t still be found in Canadian cattle, but the new feed ban will help eradicate the disease in 10 years or less based on computer models and “put it [BSE] fully behind us.”


A driving force behind CFIA’s decision to implement the new regulation, under review since December 2004, was Canada’s beef export markets. Many are still closed after the country found its first indigenous BSE case in May 2003. CFIA officials consulted with meat packers, processors, an international scientific panel, and international trading partners prior to going forth with the regulation. The government believes many markets will reopen almost immediately upon announcement of the measure, which is internationally recognized as the most effective way to protect the safety of food from BSE. The measure also applies to imported feeds and has since 2003.


While the regulation does not state how to dispose of the prohibited material, Evans specified that until emerging technologies find new uses for the product, it could be incinerated or rendered and landfilled. As is the current case for dead stock, disposal of SRMs will largely be a provincial responsibility. Provinces are generally supportive of the rationale for full SRM removal. CFIA has set aside $80 million (Canadian) to work with the provinces to assist industry’s implementation of the new feed controls. Handling and processing of the prohibited material, whether at a slaughterhouse or rendering plant, will be done under a permit system.


The U.S. Food and Drug Administration (FDA) stated that both countries have been in close contact as each developed their respective regulations. Even though the risk of BSE is extremely low in the United States, FDA issued a proposed rule in October 2005 that would prohibit the use of certain high-risk cattle materials in animal feed to further reduce the already “extraordinarily” low probability of BSE. FDA is analyzing and evaluating the approximately 800 public comments it received to the proposal and stated in late June that it “plans to develop and issue a final rule as expeditiously as possible.”


According to CFIA, there are 29 rendering facilities in Canada, with only six currently permitted to handle both prohibited and non-prohibited material. Two-thirds of Canada’s rendering facilities are owned and operated by large corporations that include international vertically integrated food companies. Seven rendering facilities are attached to federally registered slaughter plants, which helps to ensure that these companies have very tight control over the rendering and disposition of raw material from their operations.


In 2003, Canadian renderers processed approximately 2.2 million metric tons of inedible animal by-products. Products manufactured included approximately 478,000 metric tons of meat and bone meal, which included cattle, pork, poultry, and fish protein meals; approximately 535,000 metric tons of animal fats, fatty acids, and oils; and 77,600 metric tons of blood and feather protein meals.


All of Canada’s rendering plants are members of the U.S.-based Animal Protein Producers Industry, which sponsors the adoption of Hazard Analysis and Critical Control Point quality assurance programs. Large rendering facilities representing 74 percent of the annual production have implemented these process controls, which includes a third-party audit to ensure compliance.


In a Regulatory Impact Analysis Statement released with the rule, CFIA estimates the aggregate annual volume of raw bovine waste (both non-SRM and SRM) generated by the industry and collected commercially to be 642,509 metric tons, of which 92 percent (589,075 metric tons) is from abattoirs and the remaining eight percent (53,434 metric tons) from dead stock. It is estimated that full SRM removal will result in some 264,140 metric tons (up to 41 percent) of the total raw bovine waste being collected and treated as though it were SRM. This amount can be divided into three SRM sources:


• dead stock – 53,434 metric tons (eight percent of total cattle waste volume);


• provincial abattoirs – 123,484 metric tons (19 percent of total cattle waste volume); and,


• federal abattoirs – 87,222 metric tons (14 percent of total cattle waste volume).


According to the analysis, if rendered to reduce volume and extract fat value, the remaining meat and bone meal volume would be roughly 70,525 metric tons. The full analysis statement is available on the Internet at www.inspection.gc.ca/english/reg/appro/2006/20098ria_e.shtml. Information on BSE activities in Canada can be found at www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/bseesbindexe.shtml.

Two More BSE Cases Found


Following on the heels of the SRM ban announcement, test results within 10 days of each other confirmed two more cases of BSE in Canada. The first, announced July 4, 2006, was in a 15-year-old cross-bred beef cow from Manitoba, which means the animal was born well before the 1997 introduction of Canada’s feed ban. The second case was reported July 13 in a 50-month-old dairy cow from Alberta, meaning the animal was exposed to BSE after the 1997 feed ban. Neither animal entered the human or animal feed systems.


U.S. agricultural officials said the latest case, Canada’s seventh, raises “questions that must be answered,” due to the animal being born roughly four-and-a-half years after the implementation of Canada’s feed ban.


“We need a thorough understanding of all the circumstances involved in this case to assure our consumers that Canada’s regulatory system is effectively providing the utmost protections to consumers and livestock,” stated U.S. Department of Agriculture (USDA) Secretary Mike Johanns. A USDA expert will participate in CFIA’s investigation of this case, particularly as it relates to how the animal may have been exposed to BSE-infected material. CFIA welcomed USDA’s participation.


As of press time, CFIA investigators had already located the birth farm of the 50-month-old cow and were tracing other cattle born on the premises within 12 months before or after the birth of the affected animal. The agency’s investigative efforts into the 15-year-old animal may be constrained by few surviving animals and limited sources of information, such as detailed records, due to the animal’s advanced age. The animal was purchased by the owner as part of an assembled group of cattle in 1992. Nonetheless, the agency is attempting to locate the birth farm, which will provide the basis needed to identify the animal’s herd mates and feed to which it may have been exposed at a young age. A calf born to the affected animal in 2004 is also being traced.


Both infected animals were detected through Canada’s national surveillance program, which targets the highest risk animal populations.

Investigation into Fifth Case Completed


CFIA has completed its investigation into the country’s fifth case of BSE, discovered April 16, 2006, in a six-year-old dairy cow from British Columbia. The investigation identified 148 animals, including the affected animal’s herd mates and recent offspring. From this group, 22 live animals were located and all tested negative for BSE. On additional animal, which is pregnant, was placed under quarantine and will be tested once it has calved.


Of the remaining animals investigated, 77 had died or been slaughtered, 15 were exported to the United States, and 33 were untraceable. According to a U.S. Animal and Plant Health Inspection Service official, all but one of the 15 exported animals had been slaughtered. The one live animal found tested negative.


CFIA investigators examined feed that the affected animal would have been exposed to early in its life. While a specific source of infection was not found, investigators determined that vehicles and equipment used to ship and receive a variety of ingredients likely contaminated cattle feed with the BSE agent.


The investigation noted high compliance with Canada’s feed ban and identified a feed ingredient supplier common to this case and Canada’s fourth BSE animal, confirmed on January 22, 2006. This potential link suggests that all of Canada’s BSE cases fall within the same geographic cluster. The clustering theory is explained in the epidemiological report, Canada’s Assessment of the North American BSE Cases Diagnosed from 2003 to 2005, available on the CFIA Web site at www.inspection.gc.ca.


International Report - August 2006 Render

http://www.rendermagazine.com/August2006/InternationalReport.html

Published online before print February 17, 2004, 10.1073/pnas.0305777101

Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease


Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco ||, and Maria Caramelli *

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.


http://www.pnas.org/cgi/content/abstract/0305777101v1

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse


Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years. ***These results indicate that BASE is
transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.

6:30 Close of Day One


http://www.healthtech.com/2007/tse/day1.asp


-------- Original Message --------

Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD

Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A"

To: "'flounder@wt.net'"


Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

e.asante@ic.ac.uk (until 9/12/02)

New e-mail: e.asante@prion.ucl.ac.uk (active from now)

____________________________________END

Optimal Tracking and Testing of U.S. and Canadian Herds for BSE: A Value-of-Information (VOI) Approach
Louis Anthony Cox, Jr.1,2,*, Douglas A. Popken1, John J. VanSickle3, and Ranajit Sahu4
The U.S. Department of Agriculture (USDA) tests a subset of cattle slaughtered in the United States for bovine spongiform encephalitis (BSE). Knowing the origin of cattle (U.S. vs. Canadian) at testing could enable new testing or surveillance policies based on the origin of cattle testing positive. For example, if a Canadian cow tests positive for BSE, while no U.S. origin cattle do, the United States could subject Canadian cattle to more stringent testing. This article illustrates the application of a value-of-information (VOI) framework to quantify and compare potential economic costs to the United States of implementing tracking cattle origins to the costs of not doing so. The potential economic value of information from a tracking program is estimated to exceed its costs by more than five-fold if such information can reduce future losses in export and domestic markets and reduce future testing costs required to reassure or win back customers. Sensitivity analyses indicate that this conclusion is somewhat robust to many technical, scientific, and market uncertainties, including the current prevalence of BSE in the United States and/or Canada and the likely reactions of consumers to possible future discoveries of BSE in the United States and/or Canada. Indeed, the potential value of tracking information is great enough to justify locating and tracking Canadian cattle already in the United States when this can be done for a reasonable cost. If aggressive tracking and testing can win back lost exports, then the VOI of a tracking program may increase to over half a billion dollars per year.


http://www.blackwell-synergy.com/doi/abs/10.1111/j.1539-6924.2005.00648.x?journalCode=risk

and maybe not $$$

http://www.blackwell-synergy.com/doi/abs/10.1111/j.1539-6924.2005.00648.x?journalCode=risk

Agency can’t trace BSE cow (updated 4:12 p.m.)
Specific records of cow, believed eight to 10 years old, not kept
Hanneke Brooymans, edmontonjournal.com
Published: Tuesday, December 19, 2006
The Canadian Food Inspection Agency has not been able to trace Canada’s eighth case of BSE back to its birth farm.

The Charolais crossbred cow died of bovine spongiform encephalopathy on an Edmonton-area farm in early August. It was estimated to be between eight and 10 years old.

The herd at this farm was assembled since 2001, which meant the agency was looking for the farm where it was born. This is done in order to find other cattle that may have been exposed to the same potentially contaminated feed that infected the positive cow. But the farmer didn’t have specific records about the purchase of this cow, so investigators had to trace all purchases made by the producer since 2001.

Of 56 possible sources of the farm of origin of the positive cow that were investigated, 43 were definitively ruled out, the agency said. But because it was impossible to distinguish among the 13 remaining possible sources of the infected cow, the animal was determined to be untraceable. This is the second time the agency has been unable to find the birth farm of an infected animal.

All 13 remaining farms are in Alberta, said George Luterbach, senior veterinarian with the agency.

All but three of the BSE-infected cattle have been born in Alberta. The remainder were born in Saskatchewan, Manitoba and British Columbia, he said.

Experience suggests that over 90 per cent of the cattle born within 12 months in the same herd as the infected cow would have died by now, says the agency’s report. Same goes for all the cattle that ate the same feed as the infected cow in its first year.

Although it’s possible that some of these animals entered the food system, it’s extremely rare that any of the birth cohort would have got BSE, Luterbach said. Even in the United Kingdom, where thousands of animals died of the disease, it was very rare to find a second case among the birth cohort.

Eight positive animals have been detected among the over 128,000 cattle tested since 2004. These animals were chosen from the 13 million cattle in Canada because they displayed BSE-type symptoms or were found to be diseased after slaughter.

The agency also bans the tissues most likely to contain concentrated levels of the BSE agent, such as the brain and spinal cord, from the human food supply system. This so-called specified risk material has been banned from cattle feed since 1997. Beginning next July, the ban will also be enforced for all pets and livestock.

This latest infected cow would have been born and exposed before the feed ban came into effect or during the early stages of implementation, the agency says.

hbrooymans@thejournal.canwest.com

http://www.canada.com/edmontonjournal/news/local/story.html?id=64007a2c-68f6-49c7-a254-6903f067681c&k=0


http://www.canada.com/saskatoonstarphoenix/story.html?id=56c05da0-40b1-4d71-9a70-a7b29a0aa8a5&k=29140

TSS






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