Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2 and Creutzfeldt Jakob Disease report 2012

Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2 and Creutzfeldt Jakob Disease report 2012


AUSTRALIAN VETERINARY EMERGENCY PLAN

AUSVETPLAN

Disease Strategy Bovine spongiform encephalopathy Version 3.2, 2012

snip...

Two cases of feline TSE have been diagnosed in imported animals in Australian zoos. In 1992, a case was seen in a cheetah imported from the UK to a zoo in Western Australia, and the agent was subsequently typed as the classical BSE strain (Peet and Curran 1992). This animal and two littermates imported at the same time were destroyed and incinerated. The source of infection was traced to a zoo in the UK. In July 2002, a second case was diagnosed in an Asiatic golden cat imported from the Netherlands (Young and Slocombe 2003). The cat, which was born in Germany, died suddenly of a pancreatic condition, and the TSE was detected as an incidental finding on routine histopathology of the brain.

snip...

see full text 61 pages ;

http://www.animalhealthaustralia.com.au/wp-content/uploads/2011/04/BSE3.2-14-FINAL7Mar12.pdf





First occurrence of atypical scrapie

Australia is free of scrapie, also known as ‘classical’ scrapie, and has been assessed as a ‘negligible bovine spongiform encephalopathy (BSE) risk’ (the lowest risk) by the World Organisation for Animal Health (OIE). Both diseases belong to a group of diseases termed transmissible spongiform encephalopathies (TSEs) or ‘prion diseases’.

Active surveillance occurs to validate Australia’s status for both diseases, through the National Transmissible Spongiform Encephalopathies Surveillance Program (NTSESP), consistent with OIE recommendations. Results are routinely reported in Animal Health Surveillance Quarterly.

The first case of atypical scrapie (another TSE) in Australia has been confirmed in a single sheep, through the NTSESP. This is not a surprising finding. Atypical scrapie is a rare, sporadic, degenerative brain condition that spontaneously occurs in a very small proportion of older sheep and, less commonly, in goats. Most countries that test large numbers of sheep for scrapie have found one or more cases of atypical scrapie.

Testing on samples from the affected sheep at the CSIRO Australian Animal Health Laboratory in March 2010 showed preliminary results consistent with atypical scrapie. The results were confirmed by the Veterinary Laboratory Agencies at Weybridge in the United Kingdom, an OIE reference laboratory.

Atypical scrapie is clinically, pathologically, biochemically and epidemiologically unrelated to classical scrapie, and has been recognised as a distinct disease of sheep and goats for about a decade. During this time, the disease has been diagnosed in more than 20 countries worldwide. It does not pose a risk to human health or to the productivity of the Australian sheep flock. There is evidence that it is not naturally spread to other animals. It is not known to have any causal relationship to other TSEs, including BSE in cattle, chronic wasting disease in deer, or any form of Creutzfeldt–Jakob disease in people.

As atypical scrapie is a different disease to classical scrapie, Australia’s internationally recognised status as free from scrapie will not change as a result of this case. Contributed by Reg Butler, Biosecurity Services Group, Australian Government Department of Agriculture, Fisheries and Forestry

http://www.animalhealthaustralia.com.au/fms/Animal%20Health%20Australia/ADSP/AHSQ/AHSQ%20Q1%202010.pdf



Scrapie

Scrapie has been recognised in sheep for more than 250 years, and occurs at a low annual incidence in many countries, but is not present in Australia or New Zealand.

Atypical scrapie In 2009, atypical/Nor98 scrapie was detected in one sheep brain from a consignment of sheep and goat brains sent from New Zealand to the European Union, for use as negative control materials for evaluating rapid tests for BSE and scrapie.50 In 2010, a case of atypical/Nor98 scrapie was diagnosed in a sheep in Australia.

Australian and New Zealand Standard Diagnostic Procedure, August 2010. Page 7 of 27

http://www.animalhealthaustralia.com.au/wp-content/uploads/2011/03/Australia-and-New-Zealand-Standard-Diagnostic-Protocols-for-TSE.pdf



The first case of atypical scrapie in Australia was recently detected through the active surveillance program for transmissible spongiform encephalopathies (TSEs). Atypical scrapie is a rare, degenerative brain condition that occurs spontaneously in a very small proportion of older sheep and goats. It is a different disease to classical scrapie and other known TSEs. Australia remains free from scrapie.


http://www.animalhealthaustralia.com.au/wp-content/uploads/2011/05/AHSQ-Q1-2010.pdf



http://www.animalhealthaustralia.com.au/


Thursday, October 7, 2010

Australia first documented case of atypical scrapie confirmed First occurrence of atypical scrapie

http://nor-98.blogspot.com/2010/10/australia-first-documented-case-of.html





Tuesday, March 16, 2010

COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard

RRA&T 2 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

[9.03 am]

BELLINGER, Mr Brad, Chairman, Australian Beef Association CARTER, Mr John Edward, Director, Australian Beef Association CHAIR—Welcome. Would you like to make an opening statement?

Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so:

You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heidenhain Variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:

snip...see full text 110 pages ;

http://www.aph.gov.au/hansard/senate/commttee/S12742.pdf


for those interested, please see much more here ;

http://docket-aphis-2006-0041.blogspot.com/2010/03/commonwealth-of-australia-hansard.html





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




Tuesday, July 13, 2010

(SEE BEEF PRODUCTS EXPORTED TO AUSTRALIA FROM USA...TSS)

AUSTRALIAN QUESTIONNAIRE TO ASSESS BSE RISK (OIE) Terrestrial Animal Health Code, 2009 and USA export risk factor for BSE to Australia

http://usdameatexport.blogspot.com/2010/07/australian-questionnaire-to-assess-bse.html



Saturday, August 14, 2010

USA NON-SPECIES CODING SYSTEM (BEEF IMPORT EXPORT BSE RISK THERE FROM)

US denies it's illegally sending beef to Australia ?

Friday, 13/08/2010

http://usdameatexport.blogspot.com/2010/08/usa-non-species-coding-system-beef.html


Saturday, June 19, 2010

U.S. DENIED UPGRADED BSE STATUS FROM OIE

http://usdameatexport.blogspot.com/2010/06/us-denied-upgraded-bse-status-from-oie.html





Wednesday, June 15, 2011

Galveston, Texas - Isle port moves through thousands of heifers headed to Russia, none from Texas, Alabama, or Washington, due to BSE risk factor

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/galveston-texas-isle-port-moves-through.html




Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.

In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2





Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68

Comment from Terry Singeltary Document ID: APHIS-2008-0010-0008 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Docket ID: APHIS-2008-0010 RIN:0579-AC68

Topics: No Topics associated with this document View Document: More

Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS

Comment: comment submission Document ID APHIS-2008-0010-0001 Greetings USDA, OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor. SEE REFERENCE SOURCES IN ATTACHMENTS

SEE Terry S. Singeltary Sr. Attachment WORD FILE ;

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



L-BSE, TME, AND SPORADIC CJD aka mad cow disease in North America

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


Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Sophie Freire,1 Jürgen Richt,2 Justin Greenlee,3 Juan-Maria Torres,4 Paul Brown,1 Bob Hills5 and Jean-Philippe Deslys1

1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Kansas State University; Manhattan, KS USA; 3USDA; Ames, IA USA; 4INIA; Madrid, Spain; 5Health Canada; Ottawa, ON Canada†Presenting author; Email: emmanuel.comoy@cea.fr

The epidemiology of Transmissible mink encephalopathy (TME) indicates an alimentary origin. Several inter-species transmission experiments have not succeeded in establishing with certainty any natural reservoir of this prion strain, although both ovine and bovine sources have been suspected. Cattle exposed to TME develop a spongiform encephalopathy that is distinct from classical Bovine Spongiform Encephalopathy (c-BSE).

Inoculation of c-BSE to cynomolgus macaque provided early evidence of a possible risk to humans, and remains an important model to define the risk of both primary (oral transmission from cattle to primate) and secondary (intravenous intra-species transmission) exposures. We have also evaluated the transmissibility of other cattle prion strains to macaques, including L- and H- atypical forms of BSE, namely BSE-L and BSE-H, and cattle-adapted TME.

BSE-L induced a neurological disease distinct from c-BSE. Peripheral exposures demonstrate the transmissibility of BSE-L by oral, intravenous, and intra-cerebral routes, with incubation periods similar to c-BSE. Cattle-adapted TME also induced a rapid disease in cynomolgus macaque. The clinical features, lesion profile, and biochemical signature of the induced disease was similar to the features observed in animals exposed to BSE-L, suggesting a link between the two prion strains. Secondary transmissions to a common host (transgenic mouse overexpressing bovine PrP) of cattle-TME and BSE-L before or after passage in primates induced diseases with similar incubation periods: like the c-BSE strain, these cattle strains maintained their distinctive features regardless of the donor species and passages.

If the link between TME and BSE-L is confirmed, our results would suggest that BSE-L in North America may have existed for decades, and highlight a possible preferential transmission of animal prion strains to primates after passage in cattle.



=====================end...tss====================


link url not available, please see PRION 2011 ;


http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf


Volume 13, Number 12–December 2007 Research

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* andRichard A. Bessen†*Agence Française de Sécurité Sanitaire des Aliments–Lyon, Lyon, France; and†Montana State University, Bozeman, Montana, USA

Abstract

Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine-passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, H-type BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profiles, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME.The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE.

snip...

Conclusion

These studies provide experimental evidence that the Stetsonville TME agent is distinct from typical BSE but has phenotypic similarities to L-type BSE in TgOvPrP4 mice. Our conclusion is that L-type BSE is a more likely candidate for a bovine source of TME infection than typical BSE. In the scenario that a ruminant TSE is the source for TME infection in mink, this would be a second example of transmission of a TSE from ruminants to non-ruminants under natural conditions or farming practices in addition to transmission of typical BSE to humans, domestic cats, and exotic zoo animals(37). The potential importance of this finding is relevant to L-type BSE, which based on experimental transmission into humanized PrP transgenic mice and macaques, suggests that L-type BSE is more pathogenic for humans than typical BSE (24,38).

http://www.cdc.gov/eid/content/13/12/1887.htm?s_cid=eid1887_e


PLEASE NOTE *

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf



When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



P.4.23

Transmission of atypical BSE in humanized mouse models

Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA

Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.

Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.

Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.

Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf


P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS

Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.

III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf



I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''

Best regards,

Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

BSE-H is also transmissible in our humanized Tg mice.

The possibility of more than two atypical BSE strains will be discussed.

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/experimental-h-type-bovine-spongiform.html




PLoS One. 2012; 7(2): e31449.

Published online 2012 February 21. doi: 10.1371/journal.pone.0031449

PMCID: PMC3283643

Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy

The present data offer novel information on the tropism of the BASE agent and highlight relevant public health issues. While the transmission barrier for classical BSE is high in most species, BASE prions are readily transmissible to a variety of mammals including non-human primates [11]–[13], [35]. Accordingly, the possibility of spreading of BASE prions through skeletal muscle to other species should be taken into account and evaluated in risk analysis studies.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3283643/?tool=pubmed


Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html


Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html





Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html



Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html



Sunday, March 28, 2010

Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ?

http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-atypical-bse.html



Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html



I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS



Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html



Thursday, July 14, 2011

Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/histopathological-studies-of-ch1641.html


Monday, June 27, 2011

Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease

http://prionopathy.blogspot.com/2011/06/comparison-of-sheep-nor98-with-human.html



BSE: TIME TO TAKE H.B. PARRY SERIOUSLY

If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf



Thursday, November 18, 2010

Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep

http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html



Sunday, December 12, 2010

EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010

http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsa-reviews-bsetse-infectivity-in.html



Monday, November 22, 2010

Atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control

REVIEW ARTICLES

http://transmissiblespongiformencephalopathy.blogspot.com/2010/11/atypical-transmissible-spongiform.html




Wednesday, January 19, 2011

EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html





Thursday, March 29, 2012

atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012

NIAA Annual Conference April 11-14, 2011San Antonio, Texas

http://nor-98.blogspot.com/2012/03/atypical-nor-98-scrapie-has-spread-from.html



WHICH CAME FIRST, THE CART OR THE HORSE ???


Minnesota

CAPTIVE CWD CONFIRMED 2002

FREE RANGING CWD CONFIRMED 2011

http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm



Colorado

Captive CWD discovered 1967

Free ranging CWD discovered 1981



PLEASE STUDY THIS MAP !


SEE CWD MAP, RELATE TO DATES OF GAME FARM INFECTION, TO DATE OF INFECTION RATE IN WILD, SURROUNDING SAID INFECTED GAME FARMS. ...TSS

http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm



*** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 ***

Saturday, February 18, 2012

Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease

CDC Volume 18, Number 3—March 2012

SNIP...

Long-term effects of CWD on cervid populations and ecosystems remain unclear as the disease continues to spread and prevalence increases. In captive herds, CWD might persist at high levels and lead to complete herd destruction in the absence of human culling. Epidemiologic modeling suggests the disease could have severe effects on free-ranging deer populations, depending on hunting policies and environmental persistence (8,9). CWD has been associated with large decreases in free-ranging mule deer populations in an area of high CWD prevalence (Boulder, Colorado, USA) (5).

SNIP...

Reasons for Caution There are several reasons for caution with respect to zoonotic and interspecies CWD transmission. First, there is strong evidence that distinct CWD strains exist (36). Prion strains are distinguished by varied incubation periods, clinical symptoms, PrPSc conformations, and CNS PrPSc depositions (3,32). Strains have been identified in other natural prion diseases, including scrapie, BSE, and CJD (3). Intraspecies and interspecies transmission of prions from CWD-positive deer and elk isolates resulted in identification of >2 strains of CWD in rodent models (36), indicating that CWD strains likely exist in cervids. However, nothing is currently known about natural distribution and prevalence of CWD strains. Currently, host range and pathogenicity vary with prion strain (28,37). Therefore, zoonotic potential of CWD may also vary with CWD strain. In addition, diversity in host (cervid) and target (e.g., human) genotypes further complicates definitive findings of zoonotic and interspecies transmission potentials of CWD. Intraspecies and interspecies passage of the CWD agent may also increase the risk for zoonotic CWD transmission. The CWD prion agent is undergoing serial passage naturally as the disease continues to emerge. In vitro and in vivo intraspecies transmission of the CWD agent yields PrPSc with an increased capacity to convert human PrPc to PrPSc (30). Interspecies prion transmission can alter CWD host range (38) and yield multiple novel prion strains (3,28). The potential for interspecies CWD transmission (by cohabitating mammals) will only increase as the disease spreads and CWD prions continue to be shed into the environment. This environmental passage itself may alter CWD prions or exert selective pressures on CWD strain mixtures by interactions with soil, which are known to vary with prion strain (25), or exposure to environmental or gut degradation. Given that prion disease in humans can be difficult to diagnose and the asymptomatic incubation period can last decades, continued research, epidemiologic surveillance, and caution in handling risky material remain prudent as CWD continues to spread and the opportunity for interspecies transmission increases. Otherwise, similar to what occurred in the United Kingdom after detection of variant CJD and its subsequent link to BSE, years of prevention could be lost if zoonotic transmission of CWD is subsequently identified, SNIP...SEE FULL TEXT ;

*** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 ***

Saturday, February 18, 2012

Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease

CDC Volume 18, Number 3—March 2012

http://wwwnc.cdc.gov/eid/article/18/3/11-0685_article.htm


see much more here ;

http://chronic-wasting-disease.blogspot.com/2012/02/occurrence-transmission-and-zoonotic.html



Sunday, January 22, 2012

Chronic Wasting Disease CWD cervids interspecies transmission

http://chronic-wasting-disease.blogspot.com/2012/01/chronic-wasting-disease-cwd-cervids.html



Thursday, January 26, 2012

The Risk of Prion Zoonoses

Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167

http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html



Thursday, January 26, 2012

Facilitated Cross-Species Transmission of Prions in Extraneural Tissue

Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659

http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html


CJD9/10022

October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf



THIRD CJD REPORT UK 1994

snip...

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats, there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

http://www.cjd.ed.ac.uk/Archive%20reports/report3.pdf



Saturday, March 10, 2012

Enhanced Surveillance Strategies for Detecting and Monitoring Chronic Wasting Disease in Free-Ranging Cervids Open-File Report 2012–1036 National Wildlife Health Center

Open-File Report 2012–1036

http://chronic-wasting-disease.blogspot.com/2012/03/enhanced-surveillance-strategies-for.html

a few things to consider please. one, CWD has already been transmitted to many cattle in the lab (86% in one study). the oral route would have a much longer incubation period, but we already know that CWD will transmit back to cervids via the oral route, very efficiently. the threat of spreading CWD via close contact, like at feeding grounds is great. every bit of science to date shows this. so to congregate deer together by unnatural means is not smart in my opinion. another fear has come to pass as well, another strain of CWD, yes a second strain. and just recently science has shown that a natural case of BSE has been transmitted to a GOAT. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease.

http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194089



Thursday, February 09, 2012

50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE

http://chronic-wasting-disease.blogspot.com/2012/02/50-game-farms-to-date-in-usa-infected.html



and when these game farms claim they are testing, and everything is o.k., think again...



Saturday, February 04, 2012

Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised

http://chronic-wasting-disease.blogspot.com/2012/02/wisconsin-16-age-limit-on-testing-dead.html



Saturday, November 6, 2010

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU

Berne, 2010 TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation

http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html



Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>

Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)

http://www.promedmail.org/direct.php?id=20101206.4364



> > > Ackerman says downed cattle are 50 times more likely to have mad cow disease (also known as Bovine Spongiform Encephalopathy, or BSE) than ambulatory cattle that are suspected of having BSE. Of the 20 confirmed cases of mad cow disease in North America since 1993, at least 16 have involved downer cattle, he said. < < <



don’t forget the children...


PLEASE be aware, for 4 years, the USDA fed our children all across the Nation (including TEXAS) dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???


WAS your child exposed to mad cow disease via the NSLP ???


SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE


http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/



DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???


you can check and see here ; http://www.fns.usda.gov/fns/safety/pdf/Hallmark-Westland_byState.pdf



Saturday, March 5, 2011


MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA


http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html



Sunday, February 12, 2012


National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas


http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html



Thursday, April 12, 2012


Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010 Eurosurveillance, Volume 17, Issue 15, 12 April 2012

Research articles



http://creutzfeldt-jakob-disease.blogspot.com/2012/04/health-professions-and-risk-of-sporadic.html




Saturday, July 23, 2011


CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE


http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/cattle-heads-with-tonsils-beef-tongues.html



Saturday, November 6, 2010



TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation



http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html



Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>

Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)

http://www.promedmail.org/direct.php?id=20101206.4364


October 2009 O.11.3

Infectivity in skeletal muscle of BASE-infected cattle

Silvia Suardi1, Chiara Vimercati1, Fabio Moda1, Ruggerone Margherita1, Ilaria Campagnani1, Guerino Lombardi2, Daniela Gelmetti2, Martin H. Groschup3, Anne Buschmann3, Cristina Casalone4, Maria Caramelli4, Salvatore Monaco5, Gianluigi Zanusso5, Fabrizio Tagliavini1 1Carlo Besta" Neurological Institute,Italy; 2IZS Brescia, Italy; 33FLI Insel Riems, D, Germany; 4CEA-IZS Torino, Italy; 5University of Verona, Italy

Background: BASE is an atypical form of bovine spongiform encephalopathy caused by a prion strain distinct from that of BSE. Upon experimental transmission to cattle, BASE induces a previously unrecognized disease phenotype marked by mental dullness and progressive atrophy of hind limb musculature. Whether affected muscles contain infectivity is unknown. This is a critical issue since the BASE strain is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible.

Objectives: To investigate the distribution of infectivity in peripheral tissues of cattle experimentally infected with BASE. Methods: Groups of Tg mice expressing bovine PrP (Tgbov XV, n= 7-15/group) were inoculated both i.c. and i.p. with 10% homogenates of a variety of tissues including brain, spleen, cervical lymph node, kidney and skeletal muscle (m. longissimus dorsi) from cattle intracerebrally infected with BASE. No PrPres was detectable in the peripheral tissues used for inoculation either by immunohistochemistry or Western blot.

Results: Mice inoculated with BASE-brain homogenates showed clinical signs of disease with incubation and survival times of 175±15 and 207±12 days. Five out of seven mice challenged with skeletal muscle developed a similar neurological disorder, with incubation and survival times of 380±11 and 410±12 days. At present (700 days after inoculation) mice challenged with the other peripheral tissues are still healthy. The neuropathological phenotype and PrPres type of the affected mice inoculated either with brain or muscle were indistinguishable and matched those of Tgbov XV mice infected with natural BASE.

Discussion: Our data indicate that the skeletal muscle of cattle experimentally infected with BASE contains significant amount of infectivity, at variance with BSE-affected cattle, raising the issue of intraspecies transmission and the potential risk for humans. Experiments are in progress to assess the presence of infectivity in skeletal muscles of natural BASE.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf



Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf


International Society for Infectious Diseases Web: http://www.isid.org/




Friday, November 06, 2009

Surveillance of Creutzfeldt-Jakob disease in Australia: 2009 update

http://creutzfeldt-jakob-disease.blogspot.com/2009/11/surveillance-of-creutzfeldt-jakob.html





Thursday, August 05, 2010

Surveillance of Creutzfeldt-Jakob disease in Australia: 2010 update


http://creutzfeldt-jakob-disease.blogspot.com/2010/08/surveillance-of-creutzfeldt-jakob.html





Communicable Diseases Intelligence Volume 35 No 2 - June 2011

Surveillance of Creutzfeldt-Jakob disease in Australia: update to December 2010 This annual report prepared by the Australian National CJD Registry provides updated Australian human transmissible spongiform encephalopathies (TSE) figures, up till December 2010.



Genevieve M Klug, Alison Boyd, Amelia McGlade, Christiane Stehmann, Colin L Masters, Steven J Collins

Abstract

Since the establishment of the Australian National Creutzfeldt-Jakob disease Registry (ANCJDR) it’s activities have expanded from prospectively investigating additional iatrogenic Creutzfeldt-Jakob disease cases to include: retrospective ascertainment to 1970; provision of expert opinions in the area of infection control management; provide diagnostic testing services for all suspect cases; and maintenance of national and international collaborations in conjunction with routine surveillance responsibilities. An update of the ANCJDR’s surveillance activities and outcomes between 1 April and 31 December 2010 is herein presented, including a summation of a recent publication by the ANCJDR. The shorter reporting period is due to a contractual change with the Department of Health and Ageing in 2010, resulting in the reporting timeframe shifting to align with full calendar years. Commun Dis Intell 2011;35(2):149–153.



Introduction In October 1993, the Australian Government Department of Health and Ageing established the Australian National Creutzfeldt-Jakob disease Registry (ANCJDR) and have since charged this unit with the task of the surveillance of human prion diseases in Australia. The formation of this unit was underscored by the recommendations of the Allars Report,1 which investigated the identification of 4 women who were recipients of human-derived pituitary hormones and who died of Creutzfeldt-Jakob disease (CJD) between 1988 and 1991. CJD is one form of the prion group of neurological disorders, which in humans, includes Gerstmann Sträussler-Sheinker syndrome, fatal familial insomnia, Kuru and variant CJD (vCJD) while bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and chronic wasting disease in deer and elk represent principal animal forms of disease. This family of disorders, also known as transmissible spongiform encephalopathies (TSEs), causes a rapidly progressive neurological illness, ultimately leading to death. Globally, the annual incidence of CJD is around 1 case per million, although it is speculated that this may well be higher as has been indicated from several international surveillance centres,2 where the annual incidence of 2 cases per million per year has been observed. The large majority of CJD cases have no known underlying cause and are thus classified as sporadic. The remaining cases are attributed to iatrogenic transmission or genetic predisposition. All suspect TSE cases referred to the ANCJDR are actively investigated and where possible, classified as definite, probable or possible according to the internationally recognised and validated clinical and neuropathological criteria.3,4



ANCJDR surveillance update to 31 December 2010 Notifications Between 1 April and 31 December 2010 46 new suspect cases of CJD were notified to the ANCJDR. While this figure is reduced from previous reports based on 12 month periods, it is a reflection of the shorter reporting period of 9 months due to a change in contractual timeframes. Of these new suspect cases, nine have been confirmed as definite or probable cases, one has been classified as a possible case and three have been removed from the register. The remaining 33 are still under investigation with 16 of these cases still alive and 17 deceased. Neuropathological examination for nine of the deceased cases is pending.

Since establishment, a total of 1,468 cases of suspect CJD have been notified to the ANCJDR, comprising 308 notifications of case deaths prior to 1993 (retrospective cases) and 1,160 suspects notified prospectively. While this equates to around 80 notifications annually, the notification of prospective cases provides a more accurate estimate of annual national notifications. The average number of suspect case notifications for the period 1993 to 2010 is 64 cases per year or 3.2 cases per million population per year. This is almost 3 times greater than the rate of Australian confirmed cases for the same period (1.2 cases per million per year). Almost half of the prospective notifications stem from referrals for cerebrospinal fluid (CSF) 14-3-3 protein analysis (one of the diagnostic tests offered by the ANCJDR since 1997), while around a third are derived from personal communication from clinicians, family or hospitals. The remaining cases are ascertained through death certificate searches, hospital and health department searches and requests for other diagnostic services such as genetic testing.

By state and territory, analysis of the prospective suspect case notifications shows relatively stable levels since 2006 compared with previous years (Figure 1). Prior to this report however, Tasmania was an exception to stable notification levels due to declining notifications since 2006. In 2010, 3 cases have been notified to the ANCJDR, returning the number of notifications to expected levels. A reduced number of notifications in New South Wales for 2008–2009 has been sustained in 2010, with around 10 fewer cases being notified for these 3 years compared with the 2006–2007 period.

Figure 1: Prospective, suspect Creutzfeldt-Jakob disease case notifications to the Australian National Creutzfeldt-Jakob Disease Registry, 1997 to 2010, by state or territory



Case outcomes Of the 1,468 cases notified to the ANCJDR, 653 of these have been classified as probable or definite CJD cases (Table 1). An additional case of definite iatrogenic CJD is included in Table 1, due to pituitary hormone treatment occurring within Australia. However, due to the non-domestic location of onset and death, this case is not included in the Australian statistical analyses. The remaining, notified cases have been excluded after detailed follow-up investigation (573); are currently under evaluation (229); or have been classified as possible cases (13). Possible cases, classified as clinically likely but unable to meet diagnostic criteria, are excluded from all statistical analyses in this report.

Table 1: Classification of cases by the ANCJDR, 1 January 1970 to 31 December 2010



* Includes 1 definite iatrogenic case who received pituitary hormone treatment in Australia but disease onset and death occurred while a resident of the United Kingdom. This case is not included in statistical analysis since morbidity and mortality did not occur within Australia.

† Includes 159 living cases.

Since the last reporting period, 14 suspect cases have been removed from the register, with 11 of these after neuropathological confirmation. A further definite CJD case, who was initially referred to the register during treatment in Australia, died overseas and was therefore removed from the register and not included as an Australian case due to the non-domestic location at death. For the 9-month reporting period, 20 cases were confirmed as definite cases and four confirmed as probable cases, which is consistent with the number of cases classified in previous full 12 month reporting periods. This finding stems from the greater number of definite cases confirmed after post-mortem examinations performed in 2010 and relates to the reduced turnaround time for post-mortem examinations across some states and territories within this 9-month period, translating into more cases being confirmed in a shorter period of time. Of the new cases, 23 were classified as sporadic cases and one as a familial case.

The annual proportion of suspect cases notified to the ANCJDR where death is known to have occurred and have undergone post-mortem examination, has increased over time. This is to be expected given the active approach that the ANCJDR has undertaken to seek and facilitate post-mortem examinations in recent years. For the 1993 to 2010 period, 60% of all suspect case deaths have undergone post-mortem. It should be noted that this proportion is related only to cases where death is known to have occurred and the ANCJDR is aware that not all deaths are notified. In Victoria, further assistance with post-mortems has been provided through the formalisation of a contractual agreement with the Victorian Department of Health and the ANCJDR. The agreement has led to more expeditious and higher rates of autopsy in this state.

Based on the Australian population, the average crude rate of CJD-related post-mortems between 1993 and 2010 is 1.4 post-mortems per million per year, which is considerable given CJD is a particularly rare condition. By state and territory, the rate ranges from 0.8 CJD post-mortems performed annually per million in Tasmania to 1.5 in both Victoria and New South Wales. Despite the smaller populations in the Tasmania, the Northern Territory and the Australian Capital Territory, the post-mortem rates are all relatively consistent with more populous states and provide a level of confidence that suspect case deaths in these states and territories have a similar likelihood of undergoing post-mortem examination.

Top of page

As reported previously, the annual incidence of CJD has steadily increased from 1970 to peak in 2000, 2006 and 2008 (Figure 2) with 1.4–1.6 cases per million per year being recorded, equating to 32 to 37 cases per year. For the overall period of 1993 to 2010, an average of 24.5 CJD cases per year are confirmed in Australia and the average age-standardised mortality rate is 1.2 cases per million per year. Although these long term averages align closely with rates observed in other countries with similar surveillance mechanisms in place,2 it is believed that the incidence in the peak years, more closely reflects the true incidence of CJD in Australia. The ANCJDR therefore aims to achieve this level of case ascertainment.

Figure 2: ANCJDR definite and probable cases 1970 to 2010,* number and age-standardised mortality rate



Age-standardised mortality rates were calculated using the Australian Bureau of Statistics 2000 estimated resident population for Australia.

* To 31 December 2010.

Top of page

Delineation of the total case deaths by state and territory shows absolute numbers reflecting regional population distributions. The annual number of deaths from definite and probable TSE according to state and territory during 2000–2010 is shown (Table 2). The mean age-standardised rates (1993–2010) indicates that there is little variability between the larger regions of Australia with between 1.0–1.5 deaths per million occurring annually. These rates are in alignment with reported figures from other countries with similar surveillance mechanisms as those in Australia.5 Furthermore, analysis of sporadic CJD standardised mortality ratios indicate that the rate of death was not found to be significantly different in any state or territory compared with the rate in the Australian general population, indicating that no state or territory had a greater or lower risk of CJD.

Table 2: Transmissible spongiform encephalopathy deaths and mortality rate, by state and territory



* Includes all deaths occurring between the complete years 1 January 1993 or 1 January 2000 and 31 December 2010.

The highest TSE (all forms) mortality rates (1993–2010) were observed in Victoria and Western Australia (1.4 and 1.5 deaths per million per year, respectively). Previously, the lowest rates of mortality were observed in the Northern Territory and Tasmania and it was postulated that cases were being under-ascertained in these regions. More recently, an increase in confirmed cases in these less populated states and territories has contributed to the re-alignment of mortality rates to that of the larger states and territories. Tasmania continues to have the lowest TSE mortality in Australia; however, as previously discussed5 an under-ascertainment of cases prior to 2000 may be responsible for skewing the overall incidence. Furthermore, a confirmed CJD case who was a permanent Tasmanian resident, but died interstate was not attributed to Tasmania due to the non-Tasmanian location at death. It should be noted that the effect of 2 additional confirmed CJD cases in Tasmania would result in the mortality increasing to 0.9 cases per million per year, re-aligning mortality rates more closely to expected levels.

The age group with the highest mortality from all forms of CJD is amongst those aged 65–69 years where 8.3 deaths per million persons occur annually. From the age of 50, incidence increases to peak at 9.0 deaths per million per year in females in the 65–69 year age group and at 7.6 deaths per million per year in males in the 70–74 year age group (Figure 3). After these gender-specific peak age groups, mortality rates decline for both genders, although it should be noted that an increase in the detection of older age cases in recent years has led to a more rounded decline in the age-specific trend in the older age groups. Females are in slight excess (53%) for all forms of CJD, and this is true for familial (55%) and sporadic (53%) groups. In the small number of Australian iatrogenic cases, an equal number of males and females have been affected overall; 3 female pituitary hormone-related cases, 4 male and 1 female dura mater related cases.

Since the last reporting period, there has been little change in the aetiological proportions of Australian TSE cases with the large majority occurring sporadically (90.7%) and the remainder classified as familial (8.1%) and iatrogenic (1.2%). A slight reduction in the genetic CJD forms has been observed in recent years and while the explanation for this is unclear at present, the incidence of genetic CJD will be closely monitored in future years. There have been no confirmed cases of vCJD, Kuru or further cases of iatrogenic CJD relating to recipients of dura mater grafts or pituitary hormone. The last deaths from iatrogenic CJD occurred in 1991 (pituitary hormone-related CJD) and 2000 (dura mater-related CJD).

As shown in Figure 3, the majority of Australian TSE cases occur after the age of 50 and this is true for all TSE aetiologies. The median age at death in the 653 confirmed cases is 66 years (range, 18–90 years), with the median age younger in familial cases (59 years, range 18–82 years) and iatrogenic cases (39 years, range 26–62 years), but overall closely aligns with the median age death of sporadic cases, given this CJD form represents 90.7% of all cases. Similarly, the median duration of disease from onset to death is 4 months for all cases (range 0.9–192 months) and the sporadic only case group (range 0.9–60 months), yet longer for both the familial case group (6 months, range 1.5–192 months) and the iatrogenic case group (6.5 months, range 2–25 months).

Figure 3: Age- and sex-specific mortality rates in all Creutzfeldt-Jakob disease cases, 1993 to 2010



Recent publication During 2010, the ANCJDR published several articles including an update on pituitary hormone cases in Australia, drawing on the experience in other countries for comparative analysis.6 In brief, this review examined the ongoing risk for individuals who received pituitary hormone extracted from cadavers between 1967 to mid-1985 for the treatment of infertility and short stature under the Australian Human Pituitary Hormone Program. This program ceased in mid-1985 after the recognition of a linkage between treatment and CJD in a recipient in the United States of America. Australia had the lowest rate of pituitary hormone-related CJD cases across the countries compared, with the reasons for this not entirely clear. In addition, given 20 years has passed since the last case of pituitary hormone-related CJD was identified in Australia, the review discusses the current risk for the recipient community and raises the potential for changes to the infection control measures for this recipient cohort in the future.

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Acknowledgements The ANCJDR wishes to thank families, medical practitioners and associated staff for their generous support of Australian CJD surveillance. The ANCJDR also thanks Dr Handan Wand, Dr Matthew Law and Professor John Kaldor (National Centre in HIV Epidemiology and Clinical Research at the University of New South Wales) for their expert epidemiological and statistical support.

Author details Ms Genevieve M Klug, Research Assistant

Ms Alison Boyd, Registry Co-ordinator

Miss Amelia McGlade, Research Assistant

Dr Christiane Stehmann, Administrative Assistant

Professor Colin L Masters, Director

Associate Professor Steven J Collins, Director

Corresponding author: Genevieve Klug, Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, The University of Melbourne, Victoria, 3010, Australia. Telephone: +61 8344 1949. Facsimile: +61 9349 5105. Email: gmjak@unimelb.edu.au

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References

snip...see full text ;

http://www.health.gov.au/internet/main/publishing.nsf/content/cda-cdi3502c.htm





ODD, we see NO ‘classification pending’ type CJD in Australia ???




like we do the USA ?





SEE RISE IN cpsCJD i.e. classification pending sporadic CJD ;

CANADA CJD UPDATE 2011

CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.

snip...

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf





USA 2011

USA

National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

http://www.cjdsurveillance.com/pdf/case-table.pdf



Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

========end=====tss=====2011


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html





Wednesday, March 28, 2012

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $

http://prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html





THE steady rise of sporadic CJD cases in Canada AND USA, with many unusual cases of ''PENDING CLASSIFICATIONS" which have been pending now FOR 3 YEARS. HOW long can this cover-up continue $$$




The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf






http://ideas.health.vic.gov.au/diseases/cjd-facts.asp#cases



http://access.health.qld.gov.au/hid/BrainSpinalCordandNerveHealth/BrainInfectionsandParasites/creutzfeldtJakobDisease_fs.asp



2011 Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD

http://bse-atypical.blogspot.com/2011/09/l-bse-base-prion-and-atypical-sporadic.html





SEE RISE OF SPORADIC CJD YEAR TO YEAR ;

http://www.cjd.ed.ac.uk/figures.htm





Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011

(SEE VIDEO)

http://www.youtube.com/watch?v=zf3lfz9NrT4


http://www.youtube.com/watch?v=c0tWkNvhO4g


http://www.youtube.com/watch?v=zf3lfz9NrT4&feature=results_main&playnext=1&list=PL780BE2AF0B62A944





full text with source references ;

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/terry-singeltary-sr-on-creutzfeldt.html





please see full text ;



http://transmissiblespongiformencephalopathy.blogspot.com/



http://www.health.gov.au/internet/main/publishing.nsf/Content/ohp-vcjd-response-plan/$File/vCJD-response-plan.pdf



http://www.registries.org.au/registries/creutzfeldtjakob_disease.html




pink slime and a ship of fools, with Governor Rick Perry at the helm.


john gummer of England, force fed his daughter mad cow beef. a few years later, a young friend of theirs (23) died from mad cow disease. NOW, Governor Rick Perry, shows he is as big a fool as John Gummer.

http://media.kansascity.com/smedia/2012/03/29/21/01/MiPpi.SlMa.81.jpg


http://i.dailymail.co.uk/i/pix/2009/12/30/article-0-01F258B0000004B0-450_468x286.jpg


see more on this sad sad saga here ;


Wednesday, March 14, 2012

PINK SLIME, MRM’s, BSE AKA MAD COW DISEASE, AND THE USDA NSLP

http://madcowusda.blogspot.com/2012/03/pink-slime-mrms-bse-aka-mad-cow-disease.html


Sunday, August 28, 2011

Rick Perry, Texas, BSE aka mad cow disease, CJD, and 12 years of lies there from

http://sciencebushwhacked.blogspot.com/2011/08/rick-perry-texas-bse-aka-mad-cow.html


BY the way, ammonia treated beef DOES NOT KILL MAD COW DISEASE !!!


MOM DOD 12-14-97 Heidenhain Variant Creutzfeldt Jakob Disease ‘confirmed’ TEXAS, YEARS OF RICK PERRY BEING TEXAS AGRICULTURE COMMISSIONER, THEN ON TO GOVERNOR OF TEXAS, WHERE TWO MAD COWS WERE COVERED UP under Perry’s watch, ONE SUCCESSFULLY, AND ONE THAT TOOK AN ACT OF CONGRESS and 7 MONTHS TO FINALLY CONFIRM via WEYBRIDGE ENGLAND.



layperson


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net




Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68




Comment from Terry Singeltary

Document ID: APHIS-2008-0010-0008 Document Type: Public Submission

This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

Docket ID: APHIS-2008-0010 RIN:0579-AC68



Topics: No Topics associated with this document

View Document:

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Document Subtype: Public Comment

Status: Posted

Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time

Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time

Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time

Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time

Tracking Number: 80fdd617

First Name: Terry

Middle Name: S.

Last Name: Singeltary

City: Bacliff

Country: United States

State or Province: TX

Organization Name: CJD TSE PRION

Submitter's Representative: CONSUMERS







Comment:

comment submission Document ID APHIS-2008-0010-0001 Greetings USDA, OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor. SEE REFERENCE SOURCES IN ATTACHMENTS



SEE Terry S. Singeltary Sr. Attachment WORD FILE ;









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








layperson




Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net