Identification of atypical scrapie in Canadian sheep
Gordon B. Mitchell, Katherine I. O'Rourke, Noel P. Harrington, Andrei Soutyrine, Marion M. Simmons, Sandor Dudas, Dongyue Zhuang, Hubert Laude and Aru Balachandran1, Correspondence: 1Corresponding Author: Aru Balachandran, Canadian Food Inspection Agency, Ottawa Laboratory–Fallowfield, 3851 Fallowfield Road, Ottawa, Ontario, Canada, K2H 8P9. Aru.Balachandran@inspection.gc.ca
Scrapie, a transmissible spongiform encephalopathy of sheep and goats, exists in most small ruminant-producing countries of the world. A novel form of this disease was recently recognized and is known by various names, including Nor98, Nor98-like, and atypical scrapie. Differing from classic scrapie in epidemiology, histopathology, and biochemical characteristics, atypical scrapie cases have been identified throughout Europe and in the United States. Enhanced scrapie surveillance efforts recently identified 3 cases of atypical scrapie in Canada.
Key Words: Atypical • Nor98 • prion • scrapie • sheep • transmissible spongiform encephalopathy
http://jvdi.org/cgi/content/abstract/22/3/408?maxtoshow=&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1&FIRSTINDEX=0&volume=22&issue=3&resourcetype=HWCIT
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
Thursday, March 11, 2010
CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010
http://www.inspection.gc.ca/english/anima/disemala/rep/2010scrtree.shtml
Wednesday, March 3, 2010
NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010
http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html
Archive Number 20100312.0803 Published Date 12-MAR-2010 Subject PRO/AH/EDR> Scrapie, atypical, ovine - Australia: (WA) susp
SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED
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A ProMED-mail post
[1] Date: Fri 12 Mar 2010 Source: The Australian [edited]
Archive Number 20100312.0803 Published Date 12-MAR-2010 Subject PRO/AH/EDR> Scrapie, atypical, ovine - Australia: (WA) susp SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED
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A ProMED-mail post
[1] Date: Fri 12 Mar 2010 Source: The Australian [edited]
A West Australian sheep has been found to have signs characteristic of the fatal brain disease atypical scrapie. It comes as Australia faces growing anger from its trade partners over the Rudd government's surprise decision to extend a ban on the importation of beef from countries exposed to mad cow disease for a further 2 years.
Australia's chief veterinarian, Andy Carroll, told the ABC an indicative case of the atypical scrapie had been confirmed but said it posed no risk to human or animal health or the safety of eating meat and animal products.
Nor does atypical scrapie carry the dire trade consequences associated with classical scrapie.
Classical scrapie is in the same transmissible spongiform encephalopathies (TSE) family as BSE, better known as mad cow disease, from which humans can be fatally infected.
Dr Carroll said samples from the sheep's brain were being sent to the World Reference Laboratory in Britain.
Neither atypical scrapie nor classical scrapie has been seen in Australia before, but a sheep in New Zealand tested positive to the atypical form last year [2009].
Atypical scrapie is a relatively recently discovered disease and the common scientific view is that it occurs spontaneously or naturally in very small numbers of older sheep in countries all over the world.
[Byline: Jodie Minus]
-- Communicated by: Sabine Zentis Castleview Pedigree English Longhorns Gut Laach 52385 Nideggen Germany
****** [2] Date: Wed 10 Mar 2010 Source: ABC News (Australian Broadcasting Corporation) [edited]
Animal health authorities are testing a sheep's brain for what could be Australia's 1st case of the disease atypical scrapie.
Although not confirmed, the sheep is thought to be from Western Australia.
This type of scrapie is described as a sporadic degenerative brain condition affecting older sheep, and is not contagious.
Ed Klim, from national advisory group SafeMeat, says a 2nd round of testing is now taking place. "We've been made aware that the Australian Animal Health Laboratory is conducting further routine testing on a sheep sample," he says.
"The disease isn't considered a health risk nor should have any impact on food safety or export markets for sheep meat of live sheep."
Australia's chief veterinarian and WA's Department of Agriculture of Food are both aware of the testing but will not comment.
-- Communicated by: Terry S Singeltary Sr
[Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie.
However, the atypical phenotypic appearance has been shown to be preserved on experimental passage.
Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation.
[Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at
"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]
"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.
"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.
"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."
Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]
The HealthMap/ProMED-mail interactive map of Australia is available at
http://www.promedmail.org/pls/otn/f?p=2400:1001:57555::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,81729
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
Monday, December 14, 2009
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
hmmm, this is getting interesting now...
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,
see also ;
All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html
see full text ;
Monday, December 14, 2009
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html
Saturday, April 10, 2010
TOYOTA VS MAD COW DISEASE USA OIE BSE MRR IMPORT AND EXPORT TRADE WARS
http://usdameatexport.blogspot.com/2010/04/toyota-vs-mad-cow-disease-usa-oie-bse.html
Wednesday, April 28, 2010
BSE, Scrapie, CWD, REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION Paris, 8-12 February 2010
REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION
Paris, 8-12 February 2010
http://usdameatexport.blogspot.com/2010/04/bse-scrapie-cwd-report-of-meeting-of.html
DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN
''they don't wanna know, the dont' care''
watch and listen to video here, turn it up ;
http://maddeer.org/video/embedded/prusinerclip.html
URGENT DATA ON ATYPICAL BSE RISK FACTORS TO HUMANS AND ANIMALS OIE REFUSE TO ACKNOWLEDGE $
position: Post Doctoral Fellow Atypical BSE in Cattle
Closing date: December 24, 2009
Anticipated start date: January/February 2010
Employer: Canadian and OIE Reference Laboratories for BSE CFIA Lethbridge Laboratory, Lethbridge/Alberta
snip...
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.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)
18.173 page 189
Experimental Challenge of Cattle with H-type and L-type Atypical BSE
A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada
Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.
Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.
Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.
http://www.isid.org/14th_icid/
http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf
http://www.isid.org/publications/ICID_Archive.shtml
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
Wednesday, February 24, 2010
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th
ICID International Scientific Exchange Brochure -
http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html
Transmissible Spongiform Encephalopathy
http://transmissiblespongiformencephalopathy.blogspot.com/
Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009
snip...
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
I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS
http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html
I find it appauling that in 2010, the O.I.E. is still going by science on Transmissible Spongiform Encephalopathy that is decades old. New emerging strains of Transmissible Spongiform Encephalopathy have emerged, in different species, along with new science that shows these new strains of T.S.E. are more virulent than the c-B.S.E. MOST every Country that went by the O.I.E. B.S.E. guidelines, most all came down with B.S.E. THE O.I.E. has now shown they are nothing more than a National Trading Brokerage for all strains of animal T.S.E. AS i said before, O.I.E. should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...
still disgusted in Bacliff, Texas USA 77518
Terry S. Singeltary Sr.
