Thursday, March 19, 2015

Detection and Discrimination of Classical and Atypical L-Type Bovine Spongiform Encephalopathy by Real-Time Quaking-Induced Conversion

Detection and Discrimination of Classical and Atypical L-Type Bovine Spongiform Encephalopathy by Real-Time Quaking-Induced Conversion

 

Christina D. Orrúa, Alessandra Favoleb, Cristiano Coronab, Maria Mazzab, Matteo Mancaa, Bradley R. Grovemana, Andrew G. Hughsona, Pier Luigi Acutisb, Maria Caramellib, Gianluigi Zanussoc, Cristina Casaloneb and Byron Caugheya aRocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana, USA bNational Reference Center for TSE, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy cDepartment of Neurological and Movement Sciences, University of Verona, Verona, Italy

 

B. W. Fenwick, Editor

 

+ Author Affiliations

 

ABSTRACT

 

Statutory surveillance of bovine spongiform encephalopathy (BSE) indicates that cattle are susceptible to both classical BSE (C-BSE) and atypical forms of BSE. Atypical forms of BSE appear to be sporadic and thus may never be eradicated. A major challenge for prion surveillance is the lack of sufficiently practical and sensitive tests for routine BSE detection and strain discrimination. The real-time quaking-induced conversion (RT-QuIC) test, which is based on prion-seeded fibrillization of recombinant prion protein (rPrPSen), is known to be highly specific and sensitive for the detection of multiple human and animal prion diseases but not BSE. Here, we tested brain tissue from cattle affected by C-BSE and atypical L-type bovine spongiform encephalopathy (L-type BSE or L-BSE) with the RT-QuIC assay and found that both BSE forms can be detected and distinguished using particular rPrPSen substrates. Specifically, L-BSE was detected using multiple rPrPSen substrates, while C-BSE was much more selective. This substrate-based approach suggests a diagnostic strategy for specific, sensitive, and rapid detection and discrimination of at least some BSE forms.

 

FOOTNOTES Received 29 October 2014. Returned for modification 30 November 2014. Accepted 12 January 2015. Accepted manuscript posted online 21 January 2015. Address correspondence to Cristina Casalone, cristina.casalone@izsto.it, or Byron Caughey, bcaughey@nih.gov.

 

C.D.O. and A.F. contributed equally to this article.

 

Citation Orrú CD, Favole A, Corona C, Mazza M, Manca M, Groveman BR, Hughson AG, Acutis PL, Caramelli M, Zanusso G, Casalone C, Caughey B. 2015. Detection and discrimination of classical and atypical L-type bovine spongiform encephalopathy by real-time quaking-induced conversion. J Clin Microbiol 53:1115–1120. doi:10.1128/JCM.02906-14.

 

Copyright © 2015, American Society for Microbiology. All Rights Reserved.

 


 

IF, spontaneous BSE was ever to be proven, it would be the industry, and consumer’s worst nightmare. you could never ever eradicate mad cow disease, no matter how hard you try...terry

 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 


 

LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.

 


 


 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)

 


 

her healthy calf also carried the mutation

 

(J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).

 

This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks.

 

Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009

 


 

BANNED MAD COW FEED IN COMMERCE IN ALABAMA

 

______________________________

 

PRODUCT

 

a) EVSRC Custom dairy feed, Recall # V-130-6;

 

b) Performance Chick Starter, Recall # V-131-6;

 

c) Performance Quail Grower, Recall # V-132-6;

 

d) Performance Pheasant Finisher, Recall # V-133-6.

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.

 

REASON

 

Dairy and poultry feeds were possibly contaminated with ruminant based protein.

 

VOLUME OF PRODUCT IN COMMERCE

 

477.72 tons

 

DISTRIBUTION

 

AL

 

______________________________

 

PRODUCT

 

a) Dairy feed, custom, Recall # V-134-6;

 

b) Custom Dairy Feed with Monensin, Recall # V-135-6.

 

CODE

 

None. Bulk product

 

RECALLING FIRM/MANUFACTURER

 

Recalling Firm: Burkmann Feed, Greeneville, TN, by Telephone beginning on June 28, 2006.

 

Manufacturer: H. J. Baker & Bro., Inc., Albertville, AL. Firm initiated recall is complete.

 

REASON

 

Possible contamination of dairy feeds with ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

1,484 tons

 

DISTRIBUTION

 

TN and WV

 

END OF ENFORCEMENT REPORT FOR SEPTEMBER 6, 2006

 

###

 


 

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE - CLASS II

 

______________________________

 

PRODUCT

 

Bulk custom made dairy feed, Recall # V-115-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Hiseville Feed & Seed Co., Hiseville, KY, by telephone and letter on or about July 14, 2006. FDA initiated recall is ongoing.

 

REASON

 

Custom made feeds contain ingredient called Pro-Lak which may contain ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

Approximately 2,223 tons

 

DISTRIBUTION

 

KY

 

______________________________

 

PRODUCT

 

Bulk custom made dairy feed, Recall # V-116-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Rips Farm Center, Tollesboro, KY, by telephone and letter on July 14, 2006. FDA initiated recall is ongoing.

 

REASON

 

Custom made feeds contain ingredient called Pro-Lak which may contain ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

1,220 tons

 

DISTRIBUTION

 

KY

 

______________________________

 

PRODUCT

 

Bulk custom made dairy feed, Recall # V-117-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Kentwood Co-op, Kentwood, LA, by telephone on June 27, 2006. FDA initiated recall is completed.

 

REASON

 

Possible contamination of animal feed ingredients, including ingredients that are used in feed for dairy animals, with ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

40 tons

 

DISTRIBUTION

 

LA and MS

 

______________________________

 

PRODUCT

 

Bulk Dairy Feed, Recall V-118-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Cal Maine Foods, Inc., Edwards, MS, by telephone on June 26, 2006. FDA initiated recall is complete.

 

REASON

 

Possible contamination of animal feed ingredients, including ingredients that are used in feed for dairy animals, with ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

7,150 tons

 

DISTRIBUTION

 

MS

 

______________________________

 

PRODUCT

 

Bulk custom dairy pre-mixes, Recall # V-119-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Walthall County Co-op, Tylertown, MS, by telephone on June 26, 2006. Firm initiated recall is complete.

 

REASON

 

Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

87 tons

 

DISTRIBUTION

 

MS

 

______________________________

 

PRODUCT

 

Bulk custom dairy pre-mixes, Recall # V-120-6

 

CODE

 

None

 

RECALLING FIRM/MANUFACTURER

 

Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete.

 

REASON

 

Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE

 

350 tons

 

DISTRIBUTION

 

AL and MS

 

______________________________

 

PRODUCT

 

a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet,

 

50 lb. bags, Recall # V-121-6;

 

b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet,

 

50 lb. bags, Recall # V-122-6;

 

c) Tucker Milling, LLC #31232 Game Bird Grower,

 

50 lb. bags, Recall # V-123-6;

 

d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6;

 

e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6;

 

f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6;

 

g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6

 

CODE

 

All products manufactured from 02/01/2005 until 06/20/2006

 

RECALLING FIRM/MANUFACTURER

 

Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006.

 

Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.

 

REASON

 

Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".

 

VOLUME OF PRODUCT IN COMMERCE

 

7,541-50 lb bags

 

DISTRIBUTION

 

AL, GA, MS, and TN

 

END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006

 

###

 


 

Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006

 

Date: August 6, 2006 at 6:16 pm PST PRODUCT

 

a) CO-OP 32% Sinking Catfish, Recall # V-100-6;

 

b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6;

 

c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;

 

d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;

 

*** e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;

 

f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;

 

g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6;

 

h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6;

 

i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;

 

j) CO-OP LAYING CRUMBLES, Recall # V-109-6;

 

k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6;

 

l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;

 

m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE

 

Product manufactured from 02/01/2005 until 06/06/2006

 

RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.

 

REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".

 

VOLUME OF PRODUCT IN COMMERCE 125 tons

 

DISTRIBUTION AL and FL

 

END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

 

###

 


 

MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67

 

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II

 

______________________________

 

PRODUCT

 

a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;

 

b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;

 

c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;

 

d) Feather Meal, Recall # V-082-6 CODE

 

a) Bulk

 

b) None

 

c) Bulk

 

d) Bulk

 

RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.

 

REASON

 

Possible contamination of animal feeds with ruminent derived meat and bone meal.

 

VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons

 

DISTRIBUTION Nationwide

 

END OF ENFORCEMENT REPORT FOR July 12, 2006

 

###

 


 

what about that ALABAMA MAD COW, AND MAD COW FEED THERE FROM IN THAT STATE ???

 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 

*** (see mad cow feed in COMMERCE IN ALABAMA...TSS)

 

BANNED MAD COW FEED IN COMMERCE IN ALABAMA

 

Date: September 6, 2006 at 7:58 am PST PRODUCT

 

a) EVSRC Custom dairy feed, Recall # V-130-6;

 

b) Performance Chick Starter, Recall # V-131-6;

 

c) Performance Quail Grower, Recall # V-132-6;

 

d) Performance Pheasant Finisher, Recall # V-133-6.

 

CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.

 

REASON

 

Dairy and poultry feeds were possibly contaminated with ruminant based protein.

 

VOLUME OF PRODUCT IN COMMERCE 477.72 tons

 

DISTRIBUTION AL

 

______________________________

 


 

PLOS Singeltary Comment ;

 

*** ruminant feed ban for cervids in the United States ? ***

 

31 Jan 2015 at 20:14 GMT

 


 

Saturday, January 24, 2015

 

Bovine Spongiform Encephalopathy: Atypical Pros and Cons

 


 

Saturday, January 31, 2015

 

RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE

 


 

Conclusion/Significance: Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

 


 


 


 


 

SPONTANEOUS TSE

 

Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro*

 

Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either "prion diseases" because of the association of a misfolded cellular prion protein in pathogenesis or "transmissible spongiform encephalopathies" (TSEs) because of the spongelike nature of the damaged brain tissue (1).

 

The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

 

Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by "spontaneous" misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or "prion."

 

Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). BSE caused by spontaneous misfolding of the prion protein has not been proven. CREDIT: KATHARINE SUTLIFF/SCIENCE

 

snip...

 

Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent--unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

 

Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD--which is considered by some researchers to begin by spontaneous misfolding of the prion protein--usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

 

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady. ...

 

snip...full text ;

 


 

DR. DEHAVEN: “All right. I think we've got three different questions in there, and I'll try to touch on each one of them.

 

“First of all, let me correct just a technical issue, and that is you mentioned 1 in 10,000. And actually our surveillance system currently is designed, the one that we have in place now is designed to detect 1 positive in 1 million cattle, and I gave some numbers between 200,000 and 268,000 that would allow us to detect 1 in 10 million as opposed to 1 in 10,000.

 

“So we would, if we were able to collect in the ballpark of those numbers of samples then we with increasing numbers of samples have an increasingly statistically valid sample from which to determine, one, whether or not the disease exists and, if so, at what prevalence level.

 

“So our real emphasis is to test as many of those animals as we can, ensure that we get an appropriate geographical distribution, but not setting a specific number as far as a target. Again, consistent with the recommendation from the International Review Team, their recommendation was to test all of them.

 

“So that's consistent with where we're going is to test as many as we possibly can.

 

*** “As far as spontaneous cases, that is a very difficult issue. There is no evidence to prove that spontaneous BSE occurs in cattle; but here again it's an issue of proving a negative. We do know that CJD, the human version of the disease, does occur spontaneously in humans at the rate of about 1 in 1 million. We don't have enough data to definitively say that spontaneous cases of BSE in cattle occur or do not occur.

 

“Again, it's a very difficult situation to prove a negative.

 

“So a lot of research is ongoing. Certainly if we do come up with any positive samples in the course of this surveillance we will be looking at that question in evaluating those samples but no scientifically hard evidence to confirm or refute whether or not spontaneous cases of BSE occur.

 

snip...

 


 


 

1. The BSE epidemic

 

1.1. The origin of the BSE epidemic will probably never be determined with certainty.

 

1.2. We do not know whether or not some of the BARB cases represent truly sporadic classical BSE. If there are spontaneous cases then BSE will never be eradicated although reducing surveillance could make it appear that BSE has been eradicated.

 

snip...

 

5.3. It was stated that the number of sporadic CJD cases was rising. Participants were invited to discuss the reason for this. It was suggested that this was likely to be due to improved surveillance with more cases of sporadic CJD being detected (i.e. through MRI scans). There had been a similar increase in sporadic CJD in countries which did not have a BSE epidemic but improved their surveillance. This supported this theory and suggested that the increase in sporadic CJD was not related to the BSE outbreak.

 


 

Atypical BSE: Transmissibility

 

Linda Detwiller, 5/10/2011

 

 BASE (L) transmitted to:  cattle (IC) - inc < 20 mos and oral?)

 

 Cynomolgus macaques (IC)

 

 Mouse lemurs (IC and oral)

 

 wild-type mice (IC)

 

 bovinized transgenic mice (IC and IP)

 

 humanized transgenic mice (IC)

 

 H cases transmitted to:

 

 cattle – IC incubations < 20 months

 

 bovinized transgenic mice (IC)

 

 ovinized transgenic mice (IC)

 

 C57BL mice (IC)

 

 One study did not transmit to humanized PrP Met 129 mice

 

Evaluation of Possibility of Atypical

 

BSE Transmitting to Humans

 

 Possble interpretation:

 

 L type seems to transmit to nonhuman primates with greater ease than classical BSE

 

 L type also transmitted to humanized transgenic mice with higher attack rate and shorter incubation period than classical?

 

 H type did not transmit to Tg Hu transgenic mice

 

Linda Detwiller, 5/10/2011

 


 

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

 

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.

 


 


 

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 argely 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.

 


 


 


 

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 pathoge esis 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.

 


 

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.

 


 

snip... see more breaches in the BSE aka mad cow Triple Firewall, that never was here ;

 

Friday, January 23, 2015

 

*** Replacement of soybean meal in compound feed by European protein sources and relaxing the mad cow ban $

 


 

Comment from Terry Singeltary Sr.

 

This is a Comment on the Animal and Plant Health Inspection Service (APHIS) Notice: Agency Information Collection Activities; Proposals, Submissions, and Approvals: Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products

 

For related information, Open Docket Folder Docket folder icon

 

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Comment View document:Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ;

 

I believe that there is more risk to the world from Transmissible Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from the United States and all of North America, than there is risk coming to the USA and North America, from other Countries. I am NOT saying I dont think there is any risk for the BSE type TSE prion coming from other Countries, I am just saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present mad cow risk factors in North America like they are not here?

 

North America has more strains of TSE prion disease, in more species (excluding zoo animals in the early BSE days, and excluding the Feline TSE and or Canine TSE, because they dont look, and yes, there has been documented evidence and scientific studies, and DEFRA Hound study, that shows the canine spongiform encephalopathy is very possible, if it has not already happened, just not documented), then any other Country in the world. Mink TME, Deer Elk cervid CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type BSE cattle, atyical HG type BSE cow (the only cow documented in the world to date with this strain), typical sheep goat Scrapie (multiple strains), and the atypical Nor-98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical Scrapie has spread from coast to coast. sporadic CJD on the rise, with different strains mounting, victims becoming younger, with the latest nvCJD human mad cow case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL CDC.

 

typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk assessments for each country, and then made BSE confirmed countries legal to trade mad cow disease, which was all brought forth AFTER that fateful day December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats the day it all started. once the BSE MRR policy was shoved down every countries throat by USDA inc and the OIE, then the legal trading of Scrapie was validated to be a legal trading commodity, also shoved through by the USDA inc and the OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion disease typical and atypical strains, and the BSE TSE Prion aka mad cow type disease was thus made a legal trading commodity, like it or not. its all about money now folks, trade, to hell with human health with a slow incubating disease, that is 100% fatal once clinical, and forget the fact of exposure, sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the infamous VPSPr. ...problem solved $$$

 

the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing but ink on paper.

 

for this very reason I believe the BSE MRR policy is a total failure, and that this policy should be immediately withdrawn, and set back in place the BSE GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all TSE PRION disease in all species of animals, and that the BSE GBR risk assessments be made stronger than before.

 

lets start with the recent notice that beef from Ireland will be coming to America.

 

Ireland confirmed around 1655 cases of mad cow disease. with the highest year confirming about 333 cases in 2002, with numbers of BSE confirmed cases dropping from that point on, to a documentation of 1 confirmed case in 2013, to date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad cow feed ban, and the enforcement of that ban, has drastically reduced the number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow disease in the USA, we still have no clue as to the true number of cases of BSE mad cow disease in the USA or North America as a whole. ...just saying.

 

Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom)

 

Country/Year

 

snip...please see attached pdf file, with references of breaches in the USA triple BSE mad cow firewalls, and recent science on the TSE prion disease. ...TSS No documents available. AttachmentsView All (1) Empty Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission View Attachment:

 


 

Sunday, January 11, 2015

 

Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission

 


 


 

Friday, January 23, 2015

 

*** Replacement of soybean meal in compound feed by European protein sources and relaxing the mad cow ban $

 


 

Saturday, January 24, 2015

 

*** Bovine Spongiform Encephalopathy: Atypical Pros and Cons

 


 

Monday, December 1, 2014

 

Germany Bovine Spongiform Encephalopathy BSE CJD TSE Prion disease A Review December 1, 2014

 


 

Thursday, January 29, 2015

 

Identification of H-type BSE in Portugal

 


 

Thursday, January 29, 2015

 

OIE REPORT Bovine spongiform encephalopathy Prion (atypical BSE type H), Norway Information received on 29/01/2015

 


 

Thursday, July 24, 2014

 

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA

 


 

Saturday, June 12, 2010

 

PUBLICATION REQUEST AND FOIA REQUEST Project Number: 3625-32000-086-05 Study of Atypical Bse

 


 

Wednesday, March 18, 2015

 

Changes in Retinal Function and Morphology Are Early Clinical Signs of Disease in Cattle with Bovine Spongiform Encephalopathy

 


 

Sunday, December 28, 2014

 

Reverse Freedom of Information Act request rFOIA FSIS USDA APHIS TSE PRION aka BSE MAD COW TYPE DISEASE December 2014

 


 

Friday, January 30, 2015

 

*** Scrapie: a particularly persistent pathogen ***

 


 

Tuesday, December 23, 2014

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION

 


 

 

Saturday, August 4, 2012

 

Final Feed Investigation Summary – California Atypical L-type BSE Case - July 2012

 


 

 

Saturday, August 30, 2014

 

Maine Firm Recalls Ribeye and Carcass Products That May Contain Specified Risk Materials SRM TSE PRION aka mad cow type disease

 


 

Friday, December 19, 2014

 

Rancho Alleged Cancerous Eyeball Case Going To Trial

 


 

Thursday, November 28, 2013

 

Department of Justice Former Suppliers of Beef to National School Lunch Program Settle Allegations of Improper Practices and Mistreating Cows

 


 

seems USDA NSLP et al thought that it would be alright, to feed our children all across the USA, via the NSLP, DEAD STOCK DOWNER COWS, the most high risk cattle for mad cow type disease, and other dangerous pathogens, and they did this for 4 years, that was documented, then hid what they did by having a recall, one of the largest recalls ever, and they made this recall and masked the reason for the recall due to animal abuse (I do not condone animal abuse), not for the reason of the potential for these animals to have mad cow BSE type disease (or other dangerous and deadly pathogens). these TSE prion disease can lay dormant for 5, 10, 20 years, or longer, WHO WILL WATCH OUR CHILDREN FOR THE NEXT 5 DECADES FOR CJD ???

 

Saturday, September 21, 2013

 

Westland/Hallmark: 2008 Beef Recall A Case Study by The Food Industry Center January 2010 THE FLIM-FLAM REPORT

 


 

DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ??? this recall was not for the welfare of the animals. ...tss

 

you can check and see here ; (link now dead, does not work...tss)

 


 

try this link ;

 


 

Sunday, November 13, 2011

 

*** California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock

 


 

Monday, October 10, 2011

 

EFSA Journal 2011 The European Response to BSE: A Success Story

 

snip...

 

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far

 

*** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded.

 

*** Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

 

snip...

 


 


 

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...

 


 

2014

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.

 

*** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

snip...

 


 

Monday, March 02, 2015

 

Rapid and Sensitive RT-QuIC Detection of Human Creutzfeldt-Jakob Disease Using Cerebrospinal Fluid

 


 

 TSS

Saturday, January 31, 2015

RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE

Subject: RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE
 
SCIENTIFIC COMMITTEE OF THE BELGIAN FEDERAL AGENCY FOR THE SAFETY OF THE FOOD CHAIN
 
RAPID ADVICE 17-2014
 
Subject: Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE (Dossier SciCom 2014/22) Rapid advice approved by the Scientific Committee on 22nd October 2014.
 
Summary
 
The Scientific Committee was asked to answer two questions in regard to a proposal from the European Commission to no longer obligate Member States with a negligible BSE risk status to remove and dispose the specified risk materials as specified in Annex V to Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies. The aim of this modification of the Regulation is to ensure that conditions for imports of commodities from third countries are not more favorable than the conditions applying to Member States with the same OIE BSE negligible risk status. More specifically it was asked to the Scientific Committee:
 
- If there is a difference in public health risk between the casings imported from third countries with a “negligible risk status for BSE” and casings that come from the 18 EU Member States with a “negligible risk status for BSE”?
 
- If there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM and if the other risk materials for BSE (the skull including the brains and eyes, the spinal cord, the tonsils and the spine) are indeed considered as SRM?
 
Due to lack of availability of data on true prevalence and tissue infectivity of BSE (classical as well as atypical BSE) the Scientific Committee was not able to thoroughly investigate the questions.
 
Removal of specified risk materials from cattle at slaughter prevents BSE infected materials from entering the human food chain.
 
The Scientific Committee is of the opinion that, taking into consideration the uncertainties in regard to the true prevalence of BSE (classical as well as atypical BSE) in countries with a “negligible risk status for BSE” and given the problems related with the early detection of asymptomatic BSE and given the zoonotic significance of atypical BSE, that stopping with the routine removal of specified risk materials during bovine slaughter will increase the risk for public health.
 
2/14
 
The Scientific Committee is not able to compare the public health risk of casings from third countries and from the 18 EU Member States, both with a negligible risk status for BSE, because of lack of data on true BSE prevalence and BSE tissue infectivity (classical BSE and atypical BSE) in the considered countries.
 
The Scientific Committee is also not able to properly answer the second question if there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM due to lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in these countries. There is also no information on tissue infectivity of atypical BSE cases. It is known however that intestines are the portal of entry of prions and that they are already infective before the prions reach the central nervous system.
 
The final decision pertaining the need of removal of all or part of the specified risk materials is a risk management decision and goes beyond the competencies of the Scientific Committee.
 
Samenvatting Sneladvies over de risico’s voor de volksgezondheid van worstenvellen in landen met een “verwaarloosbaar risicostatuut voor BSE” en over de risico’s van wijziging van een lijst van gespecifieerde risicomaterialen (GRM) voor BSE
 
snip...
 
In conclusion the Scientific Committee is not able to answer this question with an acceptable degree of uncertainty because of lack of data on true prevalence of BSE (classical as well as atypical forms of BSE) in the considered countries. It reiterates its concern regarding the import of certain animal products from third countries with a ‘negligible BSE risk status’ as stated in rapid advice SciCom 16-2013.
 
snip...
 
2. Is there a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM while the other risk materials for BSE (the skull including the brains and eyes, the spinal cord, the tonsils and the spine) are indeed considered as SRM?
 
Once again the Scientific Committee is not able to properly answer this question because of lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in EU Member States with a “negligible risk status for BSE”.
 
BSE infected animals may enter undetected the food chain due to the low sensitivity of the diagnostic tests. Further on the classical BSE agent accumulates from the first months post exposure in particular segments of the bovine intestines and persists till clinical onset. In addition no information is available about the infectivity of tissues by the atypical BSE agent, especially in the intestines.
 
If intestines from cattle in EU Member States with a “negligible risk status for BSE” are no longer removed as SRM and are allowed to enter the food chain the public health risk will be increased. The degree of rise in risk level cannot be determined. According to EFSA Journal 2014;12(2):3554, the TSEi model indicated that the removal of the last four meters of the small intestine and of the caecum from the food and feed chain would result in a major reduction of the Classical BSE exposure risk associated with intestine and mesentery in cattle.
 
Referring to its previous advice 16-2013 the Scientific Committee repeats that stopping with the routine removal of all specified risk materials during bovine slaughter will increase the risks of exposure of the population to BSE because of the uncertainty related to the detection of BSE. This uncertainty is the consequence of the long incubation period (especially in cases of atypical BSE), the low sensitivity of the available diagnostic methods, the apparent spontaneous nature of atypical BSE, the lack of a clear clinical picture of atypical BSE cases and the reduction in number of tests in healthy slaughtered animals.
 
The final decision pertaining the need of removal of all or part of the specified risk materials is a decision to be taken by the risk manager and goes beyond the competencies of the Scientific Committee.
 
5. Conclusion
 
Removal of specified risk materials from cattle at slaughter prevents BSE infected materials from entering the human food chain.
 
The Scientific Committee is of the opinion that, taking into consideration the uncertainties in regard to the true prevalence of BSE (including classical as well asaAtypical BSE) in countries with a “negligible risk status for BSE” and given the problems related with the early detection of asymptomatic BSE and given the zoonotic character of atypical BSE, stopping with the routine removal of all specified risk materials during bovine slaughter will increase the risk for public health.
 
12/14
 
The Scientific Committee is not able to compare the public health risk of casings from third countries and from the 18 EU Member States both with a negligible risk status for BSE because of lack of data on true BSE prevalence (classical BSE and atypical BSE) in the considered countries.
 
The Scientific Committee is not able to properly answer the question if there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM due to lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in these countries. There is also no information on tissue infectivity by the agent of atypical BSE.
 
On behalf of the Scientific Committee, The President Prof. Dr. E. Thiry (Sgd.) Brussels, 06/11/2014
 
References
 
snip...end
 
 
Thursday, July 24, 2014
 
Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA
 
 
P7.09
 
Biochemical screening for identification of atypical bse in belgium, 1999-present
 
Authors
 
Alexandre DobIy: Caroline Rodeghiero, Riet Geeroms; Stephanie Durand, Jessica De Sloovere, Emanuel Yanopdenbosch, Stefan Roels,
 
Content
 
Background: Recently atypical forms of BSE have been described. Western blot analyses showed that, in comparison to the classic BSE (C-type), they are demonstrable by a higher or lower molecular weight of the unglycosylated PrPres. They Viere thus named H-type and L-type BSE (L-type is also called BASE). In addition they show a lower proportion of diglycosylated PrPres than C-type. These emerging types represent different strains of BSE. They show unique incubation periods and histological lesions. Such types have been described on different continents. Indeed they might correspond to "sporadic" forms of BSE. In 2004 we already described one L-type in Belgium.
 
Objective: We retrospectively analysed the bovines at least 7-year-old in the Belgian archive of BSE ­diagnosed cattle in order to determine the prevalence of the two types of atypical BSE in Belgium.
 
Methods: We analysed homogenates from 39 bovines of 93 months old in median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We used Western blot with a panel of anti-PrP antibodies (Ab). They detect different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2, Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and analysed PrPres, signals with an image-analysis software (Quantity One, Bio-Rad).
 
Results: The results are still under analysis. We will detail the most crucial characteristics for typing PrPres. These include 1) the apparent molecular mass of the an-, mono- and diglycosylated bands, 2) the binding affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth (unglycosylated) band and 4) the glycoprofile based on the relative proportions of the visible bands.
 
Discussion: The emergence of atypical types of BSE is partially due to a better knowledge of prion strains and more efficient diagnostic techniques. As the area in the brain where the PrPres is deposited can differ drastically between the types, it is essential to ascertain that the sampling techniques and analyses are adapted to these new types. As these new strains seem more virulent than classic types, they represent one of the next challenges in the field of prions.
 
 
 
 
Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium
 
H. De Bosschere, DVM, PhD
 
S. Roels, DVM, PhD
 
E. Vanopdenbosch, DVM, Lic
 
Veterinary and Agrochemical Research Centre (CODA/CERVA)
 
National Reference Laboratorium for Veterinary TSEs
 
Groeselenberg 99, B-1180
 
Ukkel (Brussels), Belgium
 
KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.
 
ABSTRACT
 
For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.
 
 INTRODUCTION
 
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed post-translationally from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely characterized, although PrPsc is believed to be its major if not unique constituent.3
 
Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However, no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages into different species, such as mice, humans, primates, and sheep.7-10 However, very recently, variant forms of BSE have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic, immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in Belgium.
 
snip...
 
In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected worldwide and may contribute to further research studies about epidemiologic significance. Current continued research on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.
 
 
 -------- Original Message --------
 
Subject: Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium
 
Date: Fri, 04 Feb 2005 10:59:33 –0600
 
From: "Terry S. Singeltary Sr."
 
To: Bovine Spongiform Encephalopathy
 
CC: cjdvoice@yahoogroups.com
 
 Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium
 
H. De Bosschere, DVM, PhD
 
S. Roels, DVM, PhD
 
E. Vanopdenbosch, DVM, Lic
 
Veterinary and Agrochemical Research Centre (CODA/CERVA)
 
National Reference Laboratorium for Veterinary TSEs
 
Groeselenberg 99, B-1180
 
Ukkel (Brussels), Belgium
 
KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.
 
ABSTRACT
 
For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.
 
INTRODUCTION
 
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed post-translationally from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely characterized, although PrPsc is believed to be its major if not unique constituent.3
 
Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However, no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages into different species, such as mice, humans, primates, and sheep.7 10 However, very recently, variant forms of BSE have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic, immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in Belgium.
 
MATERIALS AND METHODS
 
Since January 2001, all cattle older than 30 months are tested for TSE via a rapid test (TeSeE-kit, Bio-Rad, Nazareth, Belgium) after EC regulation 999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent assay (ELISA) screening are further subjected to scrapie-associated fibrils (SAF), histopathology, immunohistochemistry, and Western blot (WB) testing16,17 at the National Reference Laboratory (NRL).
 
RESULTS
 
A positive ELISA sample from a 64-month-old East-Flemish cow or Belgian white and red (Figure 1) was presented at the NRL for confirmation. The animal was reported healthy before slaughter. The optical density (OD) titers at the local laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and 0.708 (sample taken at the contralateral side of the first sampling side of the obex region). The histopathology of the obex, pons, and midbrain showed no spongiform changes; immunohistochemistry of the brainstem revealed no signal of PrPsc accumulation typical for BSE; and SAF was negative. However, WB analysis (Bovine WB, Bio-Rad, France; antibodies 12F10 and SAF60) of the same homogenate that was prepared from the obex region for ELISA revealed a small amount of PrPsc with an electrophoretic profile different from that of typical BSE-associated PrPsc.18,19 The band on the gel of the non-glycosylated form of PrPsc of the present case clearly showed a lower migration pattern compared with that of a typical BSE case (Figure 2).
 
DISCUSSION
 
For many years, researchers assumed that only one BSE strain existed.7 10 Only in the past months, reports of atypical BSE cases were announced.11 13 The Japanese case11 describes a very young bull (23 months) characterized by the absence of spongiform changes and PrPsc deposits immunohistochemically. The WB analysis revealed an electrophoretic profile different from that of typical BSE, characterized by low content of the di-glycosylated molecular form of PrPsc and a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE affected cattle with a previously unrecognized neuropathologic profile and PrPsc type were seen. These cases were determined using a different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on immunoblot and a difference in regional distribution of lesions. The two cases in France13 showed variant molecular features with a different PrPsc electrophoretic profile from other BSE cases, mainly characterized by a higher molecular mass of the nonglycosylated PrPsc. The present case shows the most similarities (ie, identical electrophoretic profile, only ELISA and WB positive and histopathology and immunohistochemistry negative) with the Japanese case,11 although the cow in the Japanese case was only 23 months old, and the cow in this case was 64 months old.
 
The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breed-dependent feature involved. It could be that the WB techniques have become more specific within the past year in the detection of minor differences in di-, mono-, and nonglycosylated molecular forms of PrPsc. Infection of cattle by scrapie could also be considered since scrapie can be transmitted by direct contact between animals or through environmental contamination.13
 
In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected worldwide and may contribute to further research studies about epidemiologic significance. Current continued research on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.
 
ACKNOWLEDGMENTS
 
The authors wish to thank Rita Geeroms, Patrick Van Muylem, Stephanie Durand, Raphaël Foubert and Amina Chama for their technical assistance. Mario Vanpoucke is acknowledged for providing references.
 
REFERENCES
 
1. Oesch B, Westaway D, Walchii M, et al: A cellular gene encodes PrP 27 30 protein. Cell 40:735 746, 1985.
 
2. Prusiner SB, De Armond SJ: Prion diseases and neurodegeneration. Annu Rev Neurosci 17:311 339, 1994.
 
3. Prusiner SB: Scrapie prions. Annu Rev Microbiol 43:345 374, 1989.
 
4. Bruce M, Dickinson AG: Biological evidence that scrapie agent has an independent genome. J Gen Virol 68:79 89, 1987.
 
5. Fraser H, Dickinson AG: Scrapie in mice: Agent strain differences in the distribution and intensity of grey matter vacuolation. J Comp Pathol 83:29 40, 1973.
 
6. Bruce M, McConnell I, Fraser H, Dickinson AG: The disease characteristics of different strains of scrapie in Sinc Congenic mice lines: Impications for the nature of the agent and host control of pathogenesis. J Virol 72:595 603, 1991.
 
7. Bruce M, Chree A, McDonnell I, et al: Transmission of bovine spongiform encephalopathy and scrapie to mice: Strain variation and the species barrier. Philos Trans R Soc Lon Ser B 343:405 411, 1994.
 
8. Bruce M, Will RG, Ironside JW, et al: Transmissions to mice indicate that new variant CJD is caused by the BSE agent. Nature 389:498 501, 1997.
 
9. Foster JD, Bruce M, McDonnell I, et al: Detection of BSE infectivity in brain and spleen of experimentally infected sheep. Vet Rec 138:546 548, 1996.
 
10. Lasmezas CI, Fournier J-G, Nouvel V, et al: Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: Implications for human health. Proc Natl Acd Sci U S A 98:4142 4147, 2001.
 
11. Yamakawa Y, Hagiwara K, Nohtomi K, et al, for the Expert Commitee for BSE Diagnosis, Ministry of Health, Labour and Welfare of Japan: Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy 23-month-old Holstein steer. Jpn J Infect Dis 56:221 222, 2003.
 
12. Casalone C, Zanusso G, Acutis PL, et al: Identification of a novel molecular and neuropathological BSE phenotype in Italy: International Conference on Prion Disease: from basic research to intervention concepts. Gasreig, München, 8 10 October, 2003.
 
13. Biacabe AG, Laplanche JL, Ryder S, Baron T: A molecular variant of bovine spongiform encephalopathy. International Conference on Prion Disease: From basic research to intervention concepts. Gasreig, München, 8 10 October, 2003.
 
14. De Becker D, Roels S, Vanopdenbosch E: BSE onderzoek: opsporen van PrPres door middel van de BIO-RAD Platelia BSE-kit. Vlaams Diergeneeskundig Tijdschrift 69:382 384, 2000.
 
15. Roels S, Demeyer G, Tedik K, et al: Variance of mass (volume) taken with the calibrated syringe and of the results provided by the Bio-Rad Platelia BSE test upon storage of brainstem samples at 20°C. Anim Res 51:493 499, 2002.
 
16. Roels S, De Bosschere H, Saegerman C, et al: BSE and scrapie testing in Belgium: general overview. New Food: accepted, 2004.
 
17. Vanopdenbosch E, Dechamps P, Dufey J, et al: Le premier cas d encephalopathie spongioforme bovine diagnostique en Belgique. Annales de Médicine Vétérinaire 142:111 118, 1998.
 
18. Collinge J, Sidle KCL, Meads J, et al: Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 383:685 690, 1996.
 
19. Hill AF, Desbruslais M, Joiner S, et al: The same prion strain causes vCJD and BSE. Nature 389:448 450, 1997.
 
Figure 1. Photograph of the East-Flemish cattle breed or the Belgian white and red.
 
Figure 2. Bovine Western blot (Bio-Rad, France) using antibodies 12F10 and SAF60. MM, Magic mark; Atyp. BSE, Atypical BSE case (present case); Ref1, Reference 1 of a classical BSE case; Ref2, Reference 2 of a classical BSE case. The third band of the non-glycosylated PrPsc of the Atyp. BSE case (left rectangle) shows a markedly faster migration compared to the Ref1 and Ref2 cases (right rectangle).
 
 
P.6.- Atypical case of bovine spongiform encephalopathy in an East-Flemish cow in Belgium.
 
H. De Bosschere1, S. Roels2, E. Vanopdenbosch3
 
1 Veterinary and Agrochemical Research Centre (CODA / CERVA), National Reference Laboratory for Veterinary TSE (Belgium & Luxemburg) , Unit Pathology, Department of Biocontrol, Groeselenberg 99, B-1180 Brussels (Ukkel), Belgium, (hedeb@var.fgov.be)
 
2 idem, (stroe@var.fgov.be)
 
3 idem, (emvan@var.fgov.be)
 
Bovine spongiform encephalopathy (BSE) is a prion disease with a fatal neurodegenerative pathogenesis. It is characterized by the accumulation of an abnormal protein (PrPres), formed posttranslationally from the normal isoform (PrPc). Research in mice showed the existence of different sheep scrapie strains. Scrapie strain discrimination is currently based upon biological typing in a panel of inbred mice. However, no large scale studies of the molecular features of PrPres have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biological and molecular properties even after experimental or accidental passages into different species. Very recently, variant forms of BSE have been reported in Japan, Italy and France. These forms were characterized by atypical histopathological, immunohistochemical and/or biochemical phenotype compared to the classical BSE strain. The present case describes the first Belgian atypical BSE case. Since January 1st 2001, all cattle older than 30 months is tested for TSE via a rapid test following EC regulation 999/2001. Samples positive according to the ELISA screening are further subjected to scrapie associated fibrils (SAF), histopathology, immunohistochemistry and Western blot (WB) at the NRL. A positive ELISA sample from a 64 month-old East-Flemish or Belgian white and red cow was presented at the NRL for confirmation. The histopathology of the obex, pons and midbrain was negative, immunohistochemistry and SAF were also negative. However, WB analysis was positive with an electrophoretic profile different from that of a typical BSE case. The band on the gel of the non-glycosylated form of PrPres of the present case clearly showed a lower migratrion pattern compared to that of a typical BSE case. For many years it was assumed that there was only one BSE strain. Only very recently, reports of atypical BSE cases were announced in Japan, Italy and France. The Japanese case describes a very young bull (23 months) negative on histopathology and immunohistochemistry and a WB electrophoretic profile different from that of classical BSE. The Italians observed two BSE affected cattle with a a different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPres on WB and a difference in regional distribution of lesions. The French two cases showed variant molecular features with a different electrophoretic profile from other BSE cases. The present case shows the most similarities with the Japanese case (except for the age). The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breed dependent feature involved. It could be that the WB techniques have become more specifique within the last year or infection of cattle by scrapie could also be considered. In conclusion, continued research on BSE reveals nowadays different BSE strains in analogy with the different sheep scrapie strains. Atypical BSE cases may question the significance and efficiency of the BSE epidemio-surveillance protocol and the validation of the confirmatory tests.
 
Keywords
 
Bovine spongiform encephalopathy, BSE, Western Blot, atypical BSE
 
 
 
Report on the assessment of the Geographical BSE-risk of BELGIUM July 2000
 
- 42 -
 
5. CONCLUSION ON THE GEOGRAPHICAL BSE RISK
 
5.1 The current GBR
 
The current geographical BSE-risk (GBR) level is III, i.e. BSE is confirmed in domestic cattle (last and only case in 1997) at a lower level. However, the observed incidence of clinical cases over the last 12 months (1 March 1999 to 29 February 2000) was 2.7 per 1 Million adult cattle. This figure is generated by a passive surveillance system that is not able to identify all clinical cases.
 
5.2 The expected development of the GBR
 
Assuming that measures in place continue to be appropriately implemented and no new external challenge occurs, the GBR is expected to decrease over time. However, this does not exclude that cattle infected by the BSE-agent in the past (before 1998/99) may be identified as clinical cases in the foreseeable future.
 
5.3 Recommendations for influencing the future GBR
 
Good implementation of the bans should be ensured.
 
In addition, expanding the surveillance system to target asymptomatic cattle in risk sub-populations such as adult fallen stock and adult cattle presented for emergency slaughter will allow verification of the current GBR-assessment and monitoring its future trend.
 
 
 
 
American Association of Zoo Veterinarians Infectious Disease Committee Manual 2013
 
BOVINE SPONGIFORM ENCEPHALOPATHY (BSE)
 
Little is known about atypical BSE. The origin and natural routes of transmission, if any, have yet to be determined. Almost all cases have been in older cattle (usually > 8 years of age) that have shown little resemblance to the clinic-pathological picture seen in classical disease. It has been suggested that the disease may be sporadic or be caused by a genetic mutation, but no convincing evidence has been found to support either of these ideas. The correct answer will probably only come by study of the future annual incidence curves of both types of disease. Regardless of the origin of atypical BSE, the possibility of recycling the disease in cattle and other ruminants, as well as the potential for transmission to humans, mandate a continuation of feed and specified-risk materials (SRM) bans, together with diagnostic testing programs for some time to come.
 
snip...
 
Naturally occurring cases of BSE in species other than cattle have been very limited and have been linked to exposure to contaminated feed or infected carcasses. The majority of cases originated in the UK and like BSE in cattle, have declined with the implementation of feed controls. None of the exotic animals were infected in the wild.
 
Experts who may be consulted: Linda A. Detwiler, DVM Clinical Professor Department of Pathobiology and Population Medicine
 
College of Veterinary Medicine Mississippi State University 732-580-9391 Fax: 732-741-7751 ldetwiler@belle-terre.com
 
 
 
Published in : European Journal of Epidemiology (2006), vol. 21, pp. 443-447
 
Status : Postprint (Author’s version)
 
Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium
 
B. Van Everbroeck1, A. Michotte2, R. Sciot3, C. Godfraind4, M. Deprez5, S. Quoilin6, J.-J. Martin1 & P. Cras1 1Born-Bunge Institute (BBI), University of Antwerp (UA), Campus Drie Eiken (CDE), Antwerp, Belgium; 2Department of Neuropathology, Academic hospital, Free University of Brussels, Brussels, Belgium; 3Department of Pathology, Catholic University of Leuven, Leuven, Belgium; 4Pathology Laboratory, Catholic University of Louvain, Brussels, Belgium; 5Laboratory of Neuropathology, University of Liège, Sart Tilman, Liège, Belgium; 6Institute of Public Health-Louis Pasteur, Brussels, Belgium
 
Abstract
 
From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In 85 patients a 'definite' diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained 'probable'. We further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72 patients different final diagnoses were made, Alzheimer's disease being the most frequent (N = 20). The demographic parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly increased age-specific incidence (>6/106/ year) of sCJD patients between 70 and 90 years old in the period 2002-2004 compared to 1998-2001 and retrospectively obtained data (1990-1997, p < 0.01). We undertook a detailed clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased vigilance for the diagnosis. In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although these patients are both clinically and neurobiochemically similar to the general sCJD phenotype.
 
Keywords : Diagnosis ; Epidemiology ; Prion disease ; Transmissible spongiform encephalopathy
 
 
To date, 27 cases of L-BSE and 24 cases of H-BSE have been report­ed worldwide (16), thus meaning that the prevalence of atypical BSE is considerably lower than that of C-BSE. However, recent studies showed that L-BSE is easily transmissible to transgenic mice expressing human (17,18) or bovine (19,20) prion protein, as well as to non-human primates (21), with shorter incubation periods than for the transmission of C-BSE to these animals.
 
***The virulent nature of L-BSE has stimulated new concern for human public health since the transmis­sion of C-BSE to humans resulted in variant Creutz­feldt-Jakob disease (vCJD) (4-7), a new emergent prion disease.
 
 
***Infectivity in skeletal muscle of BASE-infected cattle
 
***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.
 
 
 
 
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.
 
 
2014
 
***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.
 
***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.
 
*** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].
 
snip...
 
 
Monday, October 10, 2011
 
EFSA Journal 2011 The European Response to BSE: A Success Story
 
snip...
 
EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far
 
*** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded.
 
*** Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
 
snip...
 
 
 
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...
 
 
 
Atypical BSE: Transmissibility
 
Linda Detwiller, 5/10/2011
 
 
 BASE (L) transmitted to:  cattle (IC) - inc < 20 mos and oral?)
 
 Cynomolgus macaques (IC)
 
 Mouse lemurs (IC and oral)
 
 wild-type mice (IC)
 
 bovinized transgenic mice (IC and IP)
 
 humanized transgenic mice (IC)
 
 H cases transmitted to:
 
 cattle – IC incubations < 20 months
 
 bovinized transgenic mice (IC)
 
 ovinized transgenic mice (IC)
 
 C57BL mice (IC)
 
 One study did not transmit to humanized PrP Met 129 mice
 
Evaluation of Possibility of Atypical
 
BSE Transmitting to Humans
 
 Possble interpretation:
 
 L type seems to transmit to nonhuman primates with greater ease than classical BSE
 
 L type also transmitted to humanized transgenic mice with higher attack rate and shorter incubation period than classical?
 
 H type did not transmit to Tg Hu transgenic mice
 
Linda Detwiller, 5/10/2011
 
 
 
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
 
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.
 
 
 
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 argely 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.
 
 
 
 
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 pathoge esis 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.
 
 
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.
 
 
snip... see more breaches in the BSE aka mad cow Triple Firewall, that never was here ;
 
Friday, January 23, 2015
 
*** Replacement of soybean meal in compound feed by European protein sources and relaxing the mad cow ban $
 
 
 
Comment from Terry Singeltary Sr. This is a Comment on the Animal and Plant Health Inspection Service (APHIS) Notice: Agency Information Collection Activities; Proposals, Submissions, and Approvals: Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products
 
For related information, Open Docket Folder Docket folder icon
 
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Show agency attachment(s) AttachmentsView All (0) Empty
 
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Comment View document:Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ;
 
I believe that there is more risk to the world from Transmissible Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from the United States and all of North America, than there is risk coming to the USA and North America, from other Countries. I am NOT saying I dont think there is any risk for the BSE type TSE prion coming from other Countries, I am just saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present mad cow risk factors in North America like they are not here?
 
North America has more strains of TSE prion disease, in more species (excluding zoo animals in the early BSE days, and excluding the Feline TSE and or Canine TSE, because they dont look, and yes, there has been documented evidence and scientific studies, and DEFRA Hound study, that shows the canine spongiform encephalopathy is very possible, if it has not already happened, just not documented), then any other Country in the world. Mink TME, Deer Elk cervid CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type BSE cattle, atyical HG type BSE cow (the only cow documented in the world to date with this strain), typical sheep goat Scrapie (multiple strains), and the atypical Nor-98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical Scrapie has spread from coast to coast. sporadic CJD on the rise, with different strains mounting, victims becoming younger, with the latest nvCJD human mad cow case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL CDC.
 
typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk assessments for each country, and then made BSE confirmed countries legal to trade mad cow disease, which was all brought forth AFTER that fateful day December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats the day it all started. once the BSE MRR policy was shoved down every countries throat by USDA inc and the OIE, then the legal trading of Scrapie was validated to be a legal trading commodity, also shoved through by the USDA inc and the OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion disease typical and atypical strains, and the BSE TSE Prion aka mad cow type disease was thus made a legal trading commodity, like it or not. its all about money now folks, trade, to hell with human health with a slow incubating disease, that is 100% fatal once clinical, and forget the fact of exposure, sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the infamous VPSPr. ...problem solved $$$
 
the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing but ink on paper.
 
for this very reason I believe the BSE MRR policy is a total failure, and that this policy should be immediately withdrawn, and set back in place the BSE GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all TSE PRION disease in all species of animals, and that the BSE GBR risk assessments be made stronger than before.
 
lets start with the recent notice that beef from Ireland will be coming to America.
 
Ireland confirmed around 1655 cases of mad cow disease. with the highest year confirming about 333 cases in 2002, with numbers of BSE confirmed cases dropping from that point on, to a documentation of 1 confirmed case in 2013, to date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad cow feed ban, and the enforcement of that ban, has drastically reduced the number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow disease in the USA, we still have no clue as to the true number of cases of BSE mad cow disease in the USA or North America as a whole. ...just saying.
 
Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom)
 
Country/Year
 
snip...please see attached pdf file, with references of breaches in the USA triple BSE mad cow firewalls, and recent science on the TSE prion disease. ...TSS No documents available. AttachmentsView All (1) Empty Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission View Attachment:
 
 
Sunday, January 11, 2015
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission
 
 
 
Friday, January 23, 2015
 
*** Replacement of soybean meal in compound feed by European protein sources and relaxing the mad cow ban $
 
 
Saturday, January 24, 2015
 
*** Bovine Spongiform Encephalopathy: Atypical Pros and Cons
 
 
Monday, December 1, 2014
 
Germany Bovine Spongiform Encephalopathy BSE CJD TSE Prion disease A Review December 1, 2014
 
 
Thursday, January 29, 2015
 
Identification of H-type BSE in Portugal
 
 
Thursday, January 29, 2015
 
OIE REPORT Bovine spongiform encephalopathy Prion (atypical BSE type H), Norway Information received on 29/01/2015
 
 
Thursday, July 24, 2014
 
Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA
 
 
Saturday, June 12, 2010
 
PUBLICATION REQUEST AND FOIA REQUEST Project Number: 3625-32000-086-05 Study of Atypical Bse
 
 
Sunday, December 28, 2014
 
Reverse Freedom of Information Act request rFOIA FSIS USDA APHIS TSE PRION aka BSE MAD COW TYPE DISEASE December 2014
 
 
Friday, January 30, 2015
 
*** Scrapie: a particularly persistent pathogen ***
 
 
Tuesday, December 23, 2014
 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
 
 
Saturday, August 30, 2014
 
Maine Firm Recalls Ribeye and Carcass Products That May Contain Specified Risk Materials SRM TSE PRION aka mad cow type disease
 
 
Friday, December 19, 2014
 
Rancho Alleged Cancerous Eyeball Case Going To Trial
 
 
Thursday, November 28, 2013
 
Department of Justice Former Suppliers of Beef to National School Lunch Program Settle Allegations of Improper Practices and Mistreating Cows
 
 
seems USDA NSLP et al thought that it would be alright, to feed our children all across the USA, via the NSLP, DEAD STOCK DOWNER COWS, the most high risk cattle for mad cow type disease, and other dangerous pathogens, and they did this for 4 years, that was documented, then hid what they did by having a recall, one of the largest recalls ever, and they made this recall and masked the reason for the recall due to animal abuse (I do not condone animal abuse), not for the reason of the potential for these animals to have mad cow BSE type disease (or other dangerous and deadly pathogens). these TSE prion disease can lay dormant for 5, 10, 20 years, or longer, WHO WILL WATCH OUR CHILDREN FOR THE NEXT 5 DECADES FOR CJD ???
 
Saturday, September 21, 2013
 
Westland/Hallmark: 2008 Beef Recall A Case Study by The Food Industry Center January 2010 THE FLIM-FLAM REPORT
 
 
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ??? this recall was not for the welfare of the animals. ...tss you can check and see here ; (link now dead, does not work...tss)
 
 
try this link ;
 
 
Sunday, November 13, 2011
 
*** California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock
 
 
Thursday, February 13, 2014
 
HSUS VS USDA ET AL BAN DOWNER CALVES FOR HUMAN CONSUMPTION (*veal) and potential BSE risk factor there from
 
 
Saturday, November 10, 2012
 
Wisconsin Firm Recalls Beef Tongues That May Contain Specified Risk Materials Nov 9, 2012 WI Firm Recalls Beef Tongues
 
 
Saturday, July 23, 2011
 
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
 
 
Sunday, October 18, 2009
 
Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, October 17, 2009
 
 
Thursday, October 15, 2009
 
Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, Oct 15, 2009
 
 
Thursday, June 26, 2008
 
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials
 
 
Tuesday, July 1, 2008
 
Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs
 
 
Friday, August 8, 2008
 
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs 941,271 pounds with tonsils not completely removed
 
 
Saturday, April 5, 2008
 
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
 
 
Wednesday, April 30, 2008
 
Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings
 
 
Wednesday, April 30, 2008
 
Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings
 
 
Friday, October 15, 2010
 
BSE infectivity in the absence of detectable PrPSc accumulation in the tongue and nasal mucosa of terminally diseased cattle
 
 
SPECIFIED RISK MATERIALS SRMs
 
 
Thursday, November 18, 2010
 
UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS
 
Dustin Douglass was indicted and charged with making a fraudulent application to the VA, in an effort to obtain benefits from injuries Douglas represented he suffered while deployed in Iraq. Based on his application, the VA provided benefits totaling $22,148.53. Douglass claimed he suffered various injuries and illnesses as a result of his service in combat. The investigation revealed Douglass had, in fact, been deployed to Iraq, but had served as a computer specialist, had never been in combat, and did not suffer the service-related injuries and illnesses he claimed to have suffered. Douglass was placed on supervised release for 3 years, and required to pay $22,148.53 in restitution. Galen Niehues, an inspector for the Nebraska Department of Agriculture, (NDA), was convicted of mail fraud for submitting falsified reports to his employer concerning inspections he was supposed to perform at Nebraska cattle operations. Niehues was tasked with performing inspections of Nebraska ranches, cattle and feed for the presence of neurological diseases in cattle including Bovine Spongiform Encephalopathy (BSE), also known as “Mad Cow Disease”. Niehues was to identify cattle producers, perform on-site inspections of the farm sites and cattle operations, ask producers specific questions about feed, and take samples of the feed. Niehues was to then submit feed samples for laboratory analysis, and complete reports of his inspections and submit them to the NDA and to the Federal Food and Drug Administration (FDA). An investigation by the FDA and NDA revealed Niehues had fabricated approximately 100 BSE inspections and inspection reports. When confronted, Niehues admitted his reports were fraudulent, and that had fabricated the reports and feed samples he submitted to the NDA. Niehues received a sentence of 5 years probation, a 3-year term of supervised release, and was required to pay $42,812.10 in restitution.
 
 
 
Date: June 21, 2007 at 2:49 pm PST
 
Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program
 
An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.
 
snip...
 
Topics that will be covered in ongoing or planned reviews under Goal 1 include:
 
soundness of BSE maintenance sampling (APHIS),
 
implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS),
 
snip...
 
The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed.
 
4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half
 
 
-MORE Office of the United States Attorney District of Arizona
 
FOR IMMEDIATE RELEASE For Information Contact Public Affairs
 
February 16, 2007 WYN HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681
 
CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENT’S MAD COW DISEASE SURVEILLANCE PROGRAM
 
PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel Knauss stated, “The integrity of the system that tests for mad cow disease relies upon the honest cooperation of enterprises like Farm Fresh Meats. Without that honest cooperation, consumers both in the U.S. and internationally are at risk. We want to thank the USDA’s Office of Inspector General for their continuing efforts to safeguard the public health and enforce the law.” Farm Fresh Meats and Farabee were charged by Information with theft of government funds, mail fraud and wire fraud. According to the Information, on June 7, 2004, Farabee, on behalf of Farm Fresh Meats, signed a contract with the U.S. Department of Agriculture (the “USDA Agreement”) to collect obex samples from cattle at high risk of mad cow disease (the “Targeted Cattle Population”). The Targeted Cattle Population consisted of the following cattle: cattle over thirty months of age; nonambulatory cattle; cattle exhibiting signs of central nervous system disorders; cattle exhibiting signs of mad cow disease; and dead cattle. Pursuant to the USDA Agreement, the USDA agreed to pay Farm Fresh Meats $150 per obex sample for collecting obex samples from cattle within the Targeted Cattle Population, and submitting the obex samples to a USDA laboratory for mad cow disease testing. Farm Fresh Meats further agreed to maintain in cold storage the sampled cattle carcasses and heads until the test results were received by Farm Fresh Meats.
 
Evidence uncovered during the government’s investigation established that Farm Fresh Meats and Farabee submitted samples from cattle outside the Targeted Cattle Population. Specifically, Farm Fresh Meats and Farabee submitted, or caused to be submitted, obex samples from healthy, USDA inspected cattle, in order to steal government moneys.
 
Evidence collected also demonstrated that Farm Fresh Meats and Farabee failed to maintain cattle carcasses and heads pending test results and falsified corporate books and records to conceal their malfeasance. Such actions, to the extent an obex sample tested positive (fortunately, none did), could have jeopardized the USDA’s ability to identify the diseased animal and pinpoint its place of origin. On Wednesday, February 14, 2007, Farm Fresh Meats and Farabee pleaded guilty to stealing government funds and using the mails and wires to effect the scheme. According to their guilty pleas:
 
(a) Farm Fresh Meats collected, and Farabee directed others to collect, obex samples from cattle outside the Targeted Cattle Population, which were not subject to payment by the USDA;
 
(b) Farm Fresh Meats 2 and Farabee caused to be submitted payment requests to the USDA knowing that the requests were based on obex samples that were not subject to payment under the USDA Agreement;
 
(c) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Data Collection Forms to the USDA’s testing laboratory that were false and misleading;
 
(d) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Submission Forms filed with the USDA that were false and misleading;
 
(e) Farm Fresh Meats falsified, and Farabee directed others to falsify, internal Farm Fresh Meats documents to conceal the fact that Farm Fresh Meats was seeking and obtaining payment from the USDA for obex samples obtained from cattle outside the Targeted Cattle Population; and
 
(f) Farm Fresh Meats failed to comply with, and Farabee directed others to fail to comply with, the USDA Agreement by discarding cattle carcasses and heads prior to receiving BSE test results. A conviction for theft of government funds carries a maximum penalty of 10 years imprisonment. Mail fraud and wire fraud convictions carry a maximum penalty of 20 years imprisonment. Convictions for the above referenced violations also carry a maximum fine of $250,000 for individuals and $500,000 for organizations. In determining an actual sentence, Judge Earl H. Carroll will consult the U.S. Sentencing Guidelines, which provide appropriate sentencing ranges. The judge, however, is not bound by those guidelines in determining a sentence.
 
Sentencing is set before Judge Earl H. Carroll on May 14, 2007. The investigation in this case was conducted by Assistant Special Agent in Charge Alejandro Quintero, United States Department of Agriculture, Office of Inspector General. The prosecution is being handled by Robert Long, Assistant U.S. Attorney, District of Arizona, Phoenix. CASE NUMBER: CR-07-00160-PHX-EHC RELEASE NUMBER: 2007-051(Farabee) # # #
 
 
WE can only hope that this is a single incident. BUT i have my doubts. I remember when the infamous TOKEN Purina Feed Mill in Texas was feeding up to 5.5 grams of potentially and probably tainted BANNED RUMINANT feed to cattle, and the FDA was bragging at the time that the amount of potentially BANNED product was so little and the cattle were so big ;
 
"It is important to note that the prohibited material was domestic in origin (therefore not likely to contain infected material because there is no evidence of BSE in U.S. cattle), fed at a very low level, and fed only once. The potential risk of BSE to such cattle is therefore exceedingly low, even if the feed were contaminated."
 
 
On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed. ... FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.
 
 
WE now know all that was a lie. WE know that literally Thousands of TONS of BANNED and most likely tainted product is still going out to commerce. WE know now and we knew then that .005 to a gram was lethal. WE know that CWD infected deer and elk, scrapie infected sheep, BSE and BASE infected cattle have all been rendered and fed back to livestock (including cattle) for human and animal consumption.
 
Paul Brown, known and respected TSE scientist, former TSE expert for the CDC said he had ''absolutely no confidence in USDA tests before one year ago'', and this was on March 15, 2006 ;
 
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
 
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
 
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
 
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
 
 
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
 
 
PAUL BROWN COMMENT TO ME ON THIS ISSUE
 
Tuesday, September 12, 2006 11:10 AM
 
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
 
OR, what the Honorable Phyllis Fong of the OIG found ;
 
Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain
 
 
Table 1. Animal feed ingredients that are legally used in U.S. animal feeds
 
Animal
 
Rendered animal protein from Meat meal, meat meal tankage, meat and bone meal, poultry meal, animal the slaughter of food by-product meal, dried animal blood, blood meal, feather meal, egg-shell production animals and other meal, hydrolyzed whole poultry, hydrolyzed hair, bone marrow, and animal animals digest from dead, dying, diseased, or disabled animals including deer and elk Animal waste Dried ruminant waste, dried swine waste, dried poultry litter, and undried processed animal waste products
 
snip...
 
Conclusions
 
Food-animal production in the United States has changed markedly in the past century, and these changes have paralleled major changes in animal feed formulations. While this industrialized system of food-animal production may result in increased production efficiencies, some of the changes in animal feeding practices may result in unintended adverse health consequences for consumers of animal-based food products. Currently, the use of animal feed ingredients, including rendered animal products, animal waste, antibiotics, metals, and fats, could result in higher levels of bacteria, antibioticresistant bacteria, prions, arsenic, and dioxinlike compounds in animals and resulting animal-based food products intended for human consumption. Subsequent human health effects among consumers could include increases in bacterial infections (antibioticresistant and nonresistant) and increases in the risk of developing chronic (often fatal) diseases such as vCJD. Nevertheless, in spite of the wide range of potential human health impacts that could result from animal feeding practices, there are little data collected at the federal or state level concerning the amounts of specific ingredients that are intentionally included in U.S. animal feed. In addition, almost no biological or chemical testing is conducted on complete U.S. animal feeds; insufficient testing is performed on retail meat products; and human health effects data are not appropriately linked to this information. These surveillance inadequacies make it difficult to conduct rigorous epidemiologic studies and risk assessments that could identify the extent to which specific human health risks are ultimately associated with animal feeding practices. For example, as noted above, there are insufficient data to determine whether other human foodborne bacterial illnesses besides those caused by S. enterica serotype Agona are associated with animal feeding practices. Likewise, there are insufficient data to determine the percentage of antibiotic-resistant human bacterial infections that are attributed to the nontherapeutic use of antibiotics in animal feed. Moreover, little research has been conducted to determine whether the use of organoarsenicals in animal feed, which can lead to elevated levels of arsenic in meat products (Lasky et al. 2004), contributes to increases in cancer risk. In order to address these research gaps, the following principal actions are necessary within the United States: a) implementation of a nationwide reporting system of the specific amounts and types of feed ingredients of concern to public health that are incorporated into animal feed, including antibiotics, arsenicals, rendered animal products, fats, and animal waste; b) funding and development of robust surveillance systems that monitor biological, chemical, and other etiologic agents throughout the animal-based food-production chain “from farm to fork” to human health outcomes; and c) increased communication and collaboration among feed professionals, food-animal producers, and veterinary and public health officials.
 
REFERENCES...snip...end
 
Sapkota et al. 668 VOLUME 115 | NUMBER 5 | May 2007 • Environmental Health Perspectives
 
 
Wednesday, December 4, 2013
 
Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December 4, 2013
 
TO ALL IMPORTING COUNTRIES THAT IMPORTS FROM THE USA, BE WARNED, NEW MAD COW BSE REGULATIONS USDA, AND OIE, not worth the paper the regulations were wrote on, kind of like the mad cow feed ban of August 1997, nothing but ink on paper $$$
 
full text ;
 
 
Friday, January 23, 2015
 
Replacement of soybean meal in compound feed by European protein sources and relaxing the mad cow ban $
 
 
spontaneous atypical BSE ???
 
if that's the case, then France is having one hell of an epidemic of atypical BSE, probably why they stopped testing for BSE, problem solved $$$
 
As of December 2011, around 60 atypical BSE cases have currently been reported in 13 countries, *** with over one third in France.
 
 
so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSS
 
Sunday, October 5, 2014
 
France stops BSE testing for Mad Cow Disease
 
 
Thursday, July 24, 2014
 
*** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigations
 
 
 

Comment

Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ;

I believe that there is more risk to the world from Transmissible Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from the United States and all of North America, than there is risk coming to the USA and North America, from other Countries. I am NOT saying I dont think there is any risk for the BSE type TSE prion coming from other Countries, I am just saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present mad cow risk factors in North America like they are not here?

North America has more strains of TSE prion disease, in more species (excluding zoo animals in the early BSE days, and excluding the Feline TSE and or Canine TSE, because they dont look, and yes, there has been documented evidence and scientific studies, and DEFRA Hound study, that shows the canine spongiform encephalopathy is very possible, if it has not already happened, just not documented), then any other Country in the world. Mink TME, Deer Elk cervid CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type BSE cattle, atyical HG type BSE cow (the only cow documented in the world to date with this strain), typical sheep goat Scrapie (multiple strains), and the atypical Nor-98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical Scrapie has spread from coast to coast. sporadic CJD on the rise, with different strains mounting, victims becoming younger, with the latest nvCJD human mad cow case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL CDC.

typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk assessments for each country, and then made BSE confirmed countries legal to trade mad cow disease, which was all brought forth AFTER that fateful day December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats the day it all started. once the BSE MRR policy was shoved down every countries throat by USDA inc and the OIE, then the legal trading of Scrapie was validated to be a legal trading commodity, also shoved through by the USDA inc and the OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion disease typical and atypical strains, and the BSE TSE Prion aka mad cow type disease was thus made a legal trading commodity, like it or not. its all about money now folks, trade, to hell with human health with a slow incubating disease, that is 100% fatal once clinical, and forget the fact of exposure, sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the infamous VPSPr. ...problem solved $$$

the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing but ink on paper.

for this very reason I believe the BSE MRR policy is a total failure, and that this policy should be immediately withdrawn, and set back in place the BSE GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all TSE PRION disease in all species of animals, and that the BSE GBR risk assessments be made stronger than before.

lets start with the recent notice that beef from Ireland will be coming to America.

Ireland confirmed around 1655 cases of mad cow disease. with the highest year confirming about 333 cases in 2002, with numbers of BSE confirmed cases dropping from that point on, to a documentation of 1 confirmed case in 2013, to date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad cow feed ban, and the enforcement of that ban, has drastically reduced the number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow disease in the USA, we still have no clue as to the true number of cases of BSE mad cow disease in the USA or North America as a whole. ...just saying.

Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom)

Country/Year

snip...please see attached pdf file, with references of breaches in the USA triple BSE mad cow firewalls, and recent science on the TSE prion disease. ...TSS

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Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission

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Sunday, January 11, 2015
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission
 
 
 
 
Terry S. Singeltary Sr.
Bacliff, Texas 77518