Tuesday, November 15, 2011

Alternative BSE Risk Assessment Methodology of Imported Beef and Beef Offal to Japan

Journal of Veterinary Medical Science

Advance Publication

[PDF (443K)]

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Alternative BSE Risk Assessment Methodology of Imported Beef and Beef Offal to Japan

Yasuhiro YOSHIKAWA1), Motohiro HORIUCHI2), Naotaka ISHIGURO3), Mutsuyo KADOHIRA4), Satoshi KAI5), Hidehiro MIZUSAWA6), Chisato NAGATA7), Takashi ONODERA8), Tetsutaro SATA9), Toshiyuki TSUTSUI10), Masahito YAMADA11) and Shigeki YAMAMOTO12)

1) School of Veterinary Medicine, Kitasato University 2) Laboratory of Veterinary Hygiene, Department of Applied Veterinary Sciences, Graduate School of Veterinary Medicine, Hokkaido University 3) Laboratory of Food and Environmental Hygiene, Department of Veterinary Medicine, Faculty of Applied Biological Sciences, Gifu University 4) Department of Life Science and Agriculture, Obihiro University of Agriculture and Veterinary Medicine 5) Faculty of Business, Marketing and Distribution, Nakamura Gakuen University 6) Department of Neurology and Neurological Science, Tokyo Medical and Dental University 7) Department of Epidemiology and Preventive Medicine, Graduate School of Medicine, Gifu University 8) Graduate School of Agricultural and Life Sciences, The University of Tokyo 9) Department of Pathology, National Institute of Infectious Diseases 10) Epidemiological Research Team, National Institute of Animal Health 11) Department of Neurology and Neurobiology of Aging, Graduate School of Medical Science, Kanazawa University 12) Division of Biomedical Food Research, National Institute of Health

(Received 11-Sep-2010) (Accepted 31-Oct-2011)

ABSTRACT. Food Safety Commission (FSC) of Japan, established in July 2003, has its own initiative to conduct risk assessments on food stuffs known as 'self-tasking assessment'. Within this framework, FSC decided to conduct the risk assessment of beef and beef offal imported to Japan from countries with no previous BSE reports, thus a methodology was formed to suit to this purpose. This methodology was partly based on the previous assessments on Japanese domestic beef and beef imported from US/Canada, but some modification was added. Other organizations' assessment methods, such as those used for BSE status assessment in live cattle by OIE and EFSA's GBR, were also consulted. In this review, the authors introduce this alternative methodology that reflects (1) the risk of live cattle in the assessed country including temporal risks of BSE invasion and domestic propagation, with the assessment results verified by surveillance data, and (2) the risk of beef and beef offal consisting of cumulative BSE risk by types of slaughtering and meat production processes implemented, and the status of mechanically recovered meat production. Other possibly influencing factors such as atypical BSE cases were also reviewed. The key characteristic of the current assessment is a combination of time sequential risk level of live cattle and qualitative risk level of meat productions at present in an assessed country.

snip...

However, for transgenic mice expressing human prion protein, only L-type but not H-type could be transmitted according to the previously published reports.

snip...

The origin of atypical BSE is not yet determined. According to EFSA's scientific opinion published in 2008, all the cases of atypical BSE were reported with birth dates before the real feed ban in January 2001 in Europe. Therefore, the possibility of those atypical cases attributing to the contaminated feeds, just as in classical BSE, cannot be completely denied.

snip...

In contrast to classical BSE, the systemic distribution of abnormal prion protein in atypical BSE cases is barely known, therefore it is unclear whether the brainstem is truly the optimal part of the sampling and testing in H/L type detection. Likewise, information regarding infectivity distribution of atypical BSE is scarce in bovine peripheral tissues and body fluid. All together, lack in those essential data raises a certain hindrance to evaluating relative risk-reducing effects of various SRM removal measures from the cattle.

snip...

In the previous risk assessments done by PEC of FSC in Japan on US/Canadian imports to Japan, the surveillance-based BSE prevalence of US and Canadian cattle were estimated to be 1 case and 5-6 cases per one million cattle in US and Canada respectively (6).

snip...

It is in this context that the feed ban has been implemented in countries as a preventive measure against BSE.

snip...

KEY WORDS: beef, BSE, importation, prion diseases, risk assessment


http://www.jstage.jst.go.jp/article/jvms/advpub/0/advpub_1111070682/_article


http://www.jstage.jst.go.jp/article/jvms/advpub/0/1111070682/_pdf


====================================



Greetings,

Sadly, I think Japan put their country, and it's consumers, at greater risk, by this assessment of BSE TSE Prion risk factors from Imported Beef and Beef Offal to Japan from BSE Counrties such as the USA, Canada, and Mexico, all three of which were graded as BSE GBR III, all three have typical and atypical CJD cases, and these cases are rising.

IT seems trade has won over human and animal safety in Japan, as it did in most every other country $

Seems a disease with long incubation period, but yet is 100% fatal, is just not worthy to be feared from Government and Industry.

BIG mistake. ...

TSS



=======================

"However, for transgenic mice expressing human prion protein, only L-type but not H-type could be transmitted according to the previously published reports."

=======================


this is wrong. please see ;



P.4.23

Transmission of atypical BSE in humanized mouse models

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

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

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

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

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

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

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


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

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

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

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

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


I ask Professor Kong ;

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

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

Professor Kong reply ;

.....snip

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

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

END...TSS


Thursday, December 04, 2008 2:37 PM

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

personal communication with Professor Kong. ...TSS



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


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


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

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


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


=====================

"The origin of atypical BSE is not yet determined. According to EFSA's scientific opinion published in 2008, all the cases of atypical BSE were reported with birth dates before the real feed ban in January 2001 in Europe. Therefore, the possibility of those atypical cases attributing to the contaminated feeds, just as in classical BSE, cannot be completely denied."

=====================



atypical BSE TSE cases have been around for a long long time, so yes indeed MBM or SRM could very well and most likely did contain atypical TSE of all strains in the USA, and the typical strains as well, of all species. the North America and the USA has typical and atypical BSE, typical and atypical Scrapie, and two strains now of Chronic Wasting Disease, call them typical and atypical, or call them what you want, all this has been rendered and fed to food producing livestock for humans and animals for years in the USA. these are the facts, like them or not. please see ;


1992

IN CONFIDENCE

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367)

http://tna.europarchive.org/20080609145105/http://www.bseinquiry.gov.uk/files/yb/1993/03/14001001.pdf



1992

NEW BRAIN DISORDER

3. WHAT ABOUT REPORTS OF NEW FORM OF BSE ?

THE VETERINARY RECORD HAS PUBLISHED AN ARTICLE ON A NEW BRAIN DISORDER OF CATTLE DISCOVERED THROUGH OUR CONTROL MEASURES FOR BSE. ALTHOUGH IT PRESENTS SIMILAR CLINICAL SIGNS TO BSE THERE ARE MAJOR DIFFERENCES IN HISTOPATHOLOGY AND INCUBATION PERIODS BETWEEN THE TWO. MUST EMPHASISE THAT THIS IS _NOT_ BSE.

4. IS THIS NEW BRAIN DISORDER A THREAT ?

WE DO NOT EVEN KNOW WHETHER THE AGENT OF THIS DISEASE IS TRANSMISSIBLE. IN ANY CASE, CASES SO FAR IDENTIFIED HAD SHOWN SIMILAR SYMPTOMS TO THOSE OF BSE, AND THEREFORE HAVE BEEN SLAUGHTERED AND INCINERATED, SO THAT IF A TRANSMISSIBLE AGENT WERE INVOLVED IT WOULD HAVE BEEN ELIMINATED. ...

http://collections.europarchive.org/tna/20090114131343/http://www.bseinquiry.gov.uk/files/yb/1992/10/26001001.pdf



Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1

http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html


NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS

"All of the 15 cattle tested showed that the brains had abnormally accumulated PrP"

2009

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html



''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$



1995

page 9 of 14 ;

30. The Committee noted that the results were unusual. the questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr. Tyrell noted that the feeling of the committee was that this did not represent a new agent but it was important to be prepared to say something publicly about these findings. A suggested line to take was that these were scientifically unpublishable results but in line with the policy of openness they would be made publicly available and further work done to test their validity. Since the BSE precautions were applied to IBNC cases, human health was protected. Further investigations should be carried out on isolations from brains of IBNC cases with removal of the brain and subsequent handling under strict conditions to avoid the risk of any contamination.

31. Mr. Bradley informed the Committee that the CVO had informed the CMO about the IBNC results and the transmission from retina and he, like the Committee was satisfied that the controls already in place or proposed were adequate. ...

snip... see full text



http://collections.europarchive.org/tna/20080102204938/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



NOW, what about the 'obex only' mode of testing used by the USDA et al for TSE, prions $$$ works for them too, a sure fire way NOT TO FIND MAD COW DISEASE $$$


NOW, read the following please, and then ask yourself, WHY the USDA et al were ONLY TESTING THE OBEX PART OF THE BRAIN in USA cattle for BSE $$$


BECAUSE they knew that would be the least likely way to find BSE/TSE in USA cattle $$$...TSS



Tuesday, November 02, 2010

IN CONFIDENCE

The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992

http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html


1979

In May, 1990 (J/AVMA/196/1679) a paper was published in the Journal of the American Veterinary Medical Association detailing work on the transmission of scrapie to cattle initiated by the USDA at Mission, Texas, in January, 1979. In this study, ten calves were each challenged intracerebrally, orally, intramuscularly and subcutaneously with scrapie which had been subpassaged in either a sheep or a goat.Three of these animals went on to develop clinical symptoms of a spongiform encephalopathy which differed from those seen with BSE infection. PrP was detected in tissues from these cattle, but neurohistologically the changes seen in the brains from these cattle were consistent with scrapie and differed from BSE. Material from the affected cattle was bioassayed in mice which were observed for 2 years. The mice remained clinically and neurohistologically negative *** apart from one group which,although they showed CNS signs, were histologically negative.

http://www.bseinquiry.gov.uk/files/ws/s100bxii.pdf

http://74.125.47.132/search?q=cache:Geyf-zmNTSkJ:www.bseinquiry.gov.uk/files/ws/s100bxii.pdf+mission+texas+bse+inquiry+yb&cd=1&hl=en&ct=clnk&gl=us

3.56 A further difference in the transmission properties of the two diseases was the pattern of disease caused in the brains of experimental animals. Mice inoculated with scrapie material from geographically and temporally distinct sources were found to have variable brain lesions, whereas mice inoculated with BSE material similarly derived from different sources all had very similar patterns of disease. 30 These results showed that, unlike scrapie, only one strain of BSE was present in the inocula derived from different sources. As the current hypothesis suggested that scrapie had transmitted to cattle at a number of geographically separate sites, it might have been expected that several strains of BSE would have been evident, given that over 20 strains of scrapie were known. Since 1996, strain-typing studies in mice have shown that the lesion profile produced by BSE is different to all known scrapie strains. 31

3.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988, 35 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988-89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA. 36 It was also felt that the results of such an experiment would be hard to interpret. While a negative result would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE. 37 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest. 38

3.59 Nevertheless, from the first demonstration of transmissibility of BSE in 1988, the possibility of differences in the transmission properties of BSE and scrapie was clear. Scrapie was transmissible to hamsters, but by 1988 attempts to transmit BSE to hamsters had failed. Subsequent findings increased that possibility.

http://web.archive.org/web/20010224062436/http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820550



The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820546

The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988, 35 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988-89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA. 36 It was also felt that the results of such an experiment would be hard to interpret. While a negative result would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE. 37 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest. 38

http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820550


http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm




Saturday, June 25, 2011

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



"BSE-L in North America may have existed for decades"


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html



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

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

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...



http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1


http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf


see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;



http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html



Wednesday, March 31, 2010

Atypical BSE in Cattle

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

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

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

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



Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat

snip...

http://www.neuroprion.org/en/np-neuroprion.html



Rural and Regional Affairs and Transport References Committee

The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf



And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf



please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html


[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

http://whqlibdoc.who.int/publications/2003/9241545887.pdf



Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

http://www.promedmail.org/direct.php?id=20100405.1091



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

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

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


Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html



SEE FULL TEXT OF ALL THIS HERE ;

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html


Thursday, June 23, 2011

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

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


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.

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156


http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF


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)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html



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

http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html



P.9.21

Molecular characterization of BSE in Canada

Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.


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




October 2009

O.11.3

Infectivity in skeletal muscle of BASE-infected cattle

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

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

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

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

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



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



 

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.


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




STRICTLY PRIVATE AND CONFIDENTIAL 25, AUGUST 1995

snip...

To minimise the risk of farmers' claims for compensation from feed compounders.

To minimise the potential damage to compound feed markets through adverse publicity.

To maximise freedom of action for feed compounders, notably by maintaining the availability of meat and bone meal as a raw material in animal feeds, and ensuring time is available to make any changes which may be required.

snip...

THE FUTURE

4..........

MAFF remains under pressure in Brussels and is not skilled at handling potentially explosive issues.

5. Tests _may_ show that ruminant feeds have been sold which contain illegal traces of ruminant protein. More likely, a few positive test results will turn up but proof that a particular feed mill knowingly supplied it to a particular farm will be difficult if not impossible.

6. The threat remains real and it will be some years before feed compounders are free of it. The longer we can avoid any direct linkage between feed milling _practices_ and actual BSE cases, the more likely it is that serious damage can be avoided. ...

SEE full text ;

http://web.archive.org/web/20060517074958/http://www.bseinquiry.gov.uk/files/yb/1995/08/24002001.pdf



2011

Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD

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


Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html


Sunday, November 13, 2011

Atypical Scrapie Isolates Involve a Uniform Prion Species with a Complex Molecular Signature

PrPres peptides of low molecular mass have also been described in other types of prion disease, such as Gerstmann-Sträussler-Scheinker disease [32], [33] and Creutzfeldt-Jakob disease [34], [35] in humans as well as in H-BSE in cattle [36], [37]. Forthcoming directions of research are likely to focus on more precise comparative analyses of truncated PrPres peptides and their role in the biology of human and animal prion diseases...

http://nor-98.blogspot.com/2011/11/atypical-scrapie-isolates-involve.html


Thursday, July 14, 2011

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

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



Monday, May 23, 2011

Atypical Prion Diseases in Humans and Animals 2011 Top Curr Chem (2011)

DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011

Atypical Prion Diseases in Humans and Animals

Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar

Abstract

Although prion diseases, such as Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the “scrapie form” (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

M.A. Tranulis (*)

Norwegian School of Veterinary Science, Oslo, Norway

e-mail: Michael.Tranulis@nvh.no

S.L. Benestad

Norwegian Veterinary Institute, Oslo, Norway

T. Baron

Agence Nationale de Se´curite´ Sanitaire, ANSES, Lyon, France

H. Kretzschmar

Ludwig–Maximilians University of Munich, Munich, Germany

Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type

http://resources.metapress.com/pdf-preview.axd?code=f433r34h34ugg617&size=largest


see full text and more here ;

http://bse-atypical.blogspot.com/2011/05/atypical-prion-diseases-in-humans-and.html


Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

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


Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

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


Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

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


Thursday, June 2, 2011

USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES Pennsylvania AND California

http://nor-98.blogspot.com/2011/06/usda-scrapie-report-for-april-2011-new.html


Monday, June 20, 2011 2011

Annual Conference of the National Institute for Animal Agriculture ATYPICAL NOR-98 LIKE SCRAPIE UPDATE USA

http://nor-98.blogspot.com/2011/06/2011-annual-conference-of-national.html


Thursday, July 14, 2011

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

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


Wednesday, October 12, 2011

White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation

http://chronic-wasting-disease.blogspot.com/2011/10/white-tailed-deer-are-susceptible-to.html


Monday, November 14, 2011

WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wyoming-creutzfeldt-jakob-disease-cwd.html




UPDATED DATA ON 2ND CWD STRAIN



Wednesday, September 08, 2010

CWD PRION CONGRESS SEPTEMBER 8-11 2010

http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html



Wednesday, January 5, 2011

ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011

Prions

David W. Colby1,* and Stanley B. Prusiner1,2

http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html



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

Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

Date: Fri, 16 May 2003 11:47:37 -0500

From: "Terry S. Singeltary Sr."

To: fdadockets@oc.fda.gov

Greetings FDA,

i would kindly like to comment on;

Docket 03D-0186

FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

http://madcowfeed.blogspot.com/2008/07/docket-03d-0186-fda-issues-draft.html



10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm



USDA FDA FAILED BSE TRIPLE FIRE WALLS, a feed ban and surveillance system that was nothing more than ink on paper ;

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)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html




Monday, September 12, 2011

BSE PRION Agriculture Animal Feed Question House of Lords Thursday, 8 September 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/09/bse-prion-agriculture-animal-feed.html



Saturday, July 23, 2011

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

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


Saturday, November 6, 2010

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS

INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation

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


Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)

PRION DISEASE UPDATE 2010 (11)

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



CANADA CJD UPDATE 2011

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

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

snip...

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


USA 2011

USA

National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

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

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

3 Disease acquired in the United Kingdom;

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

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

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

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


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

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

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

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



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


PLEASE NOTE, CWRU, CDC, GAMBETTI, CJD FOUNDATION ET AL, HAVE FAILED TO PUT OUT AN UPDATE ON THE CJD CASE FILES SINCE AUGUST 2011 ???



Creutzfeldt-Jakob disease in Mexico

Leora Velásquez-Pérez1,*, Daniel Rembao-Bojorquez2, Jorge Guevara3, Rosa María Guadarrama-Torres1, Araceli Trejo-Contreras1Article first published online: 18 SEP 2007

DOI: 10.1111/j.1440-1789.2007.00807.x

2007 Japanese Society of Neuropathology

Keywords:Creutzfeldt-Jakob disease;encephalopathy;epidemiology;prion

Creutzfeldt-Jakob disease (CJD) is classified within the group of transmissible spongiform encephalopathies (TSE). It is a rapidly progressive illness that affects mental functions. The average age of onset is 50 years. Various tests can help orient the clinical diagnosis, but the confirmatory test is still the post mortem analysis. The aim of this study was to describe the epidemiological, clinical and histopathological characteristics of patients diagnosed as suffering from CJD, at the National Institute of Neurology and Neurosurgery of Mexico (NINN). An observational, descriptive and transversal study was conducted. We collected information concerning these cases from the Departments of Epidemiology and Pathology, as well as the clinical charts of the patients with a diagnosis of CJD. Fifteen cases were registered of which three CJD cases were definite, five probable cases were identified, and seven were possible. The average age of the patients was 49 years. Two definite cases were female and one was male. It is important to improve the systems for surveillance of this type of disease and, furthermore, to permit greater accessibility to laboratories where the procedures necessary for supporting diagnosis can be followed.

http://onlinelibrary.wiley.com/doi/10.1111/j.1440-1789.2007.00807.x/abstract;jsessionid=90711B9F726B5F6523A087E60E1A136F.d02t01


Subject: Creutzfeldt-Jakob disease in Mexico

Date: September 20, 2007 at 8:38 am PST

Creutzfeldt-Jakob disease in Mexico

Leora Velásquez-Pérez,1 Daniel Rembao-Bojorquez,2 Jorge Guevara,3 Rosa María Guadarrama-Torres1 and Araceli Trejo-Contreras1 Departments of 1Epidemiology and 2Pathology, and 3Neurodegenerative Diseases Laboratory, National Institute of Neurology and Neurosurgery, Mexico DF, Mexico

Creutzfeldt-Jakob disease (CJD) is classified within the group of transmissible spongiform encephalopathies (TSE). It is a rapidly progressive illness that affects mental functions. The average age of onset is 50 years. Various tests can help orient the clinical diagnosis, but the confirmatory test is still the post mortem analysis. The aim of this study was to describe the epidemiological, clinical and histopathological characteristics of patients diagnosed as suffering from CJD, at the National Institute of Neurology and Neurosurgery of Mexico (NINN). An observational, descriptive and transversal study was conducted. We collected information concerning these cases from the Departments of Epidemiology and Pathology, as well as the clinical charts of the patients with a diagnosis of CJD. Fifteen cases were registered of which three CJD cases were definite, five probable cases were identified, and seven were possible.The average age of the patients was 49 years. Two definite cases were female and one was male. It is important to improve the systems for surveillance of this type of disease and, furthermore, to permit greater accessibility to laboratories where the procedures necessary for supporting diagnosis can be followed.

Key words: Creutzfeldt-Jakob disease, encephalopathy, epidemiology, prion.

snip...

In Mexico there are some deficiencies in the surveillance system, among other reasons due to limited knowledge concerning this disease on the part of administrative and medical staff, which causes a lack of notification of cases and an under-registration of these diseases. On the other hand, the National System of Epidemiological Surveillance has not rigorously integrated and made obligatory the notification and control of TSE, and there are no centers or laboratories of microbiology and genetics where tests to support the diagnosis of the disease can be conducted. Another problem in Mexico is the fact that many of the suspected or probable cases are never confirmed because of the refusal of relatives to allow deceased patients to undergo autopsies. The aim of this study was to describe and present the epidemiological, clinical, and histopathological characteristics of CJD cases, detected between January 1, 2000 and May 31, 2005 in our institution; this being one of the world’s main neurological institutions.

snip...

RESULTS Between January 1, 2000 and May 31, 2005, three definite CJD cases were identified (20%), five probable cases were identified (33%), and seven were classified as possible (47%). The laboratory investigations used for the patients are presented in Table 1. The year of diagnosis of these patients is shown in Table 2. By the end of May 2005, eight patients were still alive (53%), and seven had died (47%). Of the seven deceased patients, the neurohistophatological study was performed in three patients. Of these, autopsy was carried out in two and stereotactic biopsy was performed in one. Histopathological images of definite cases of CJD are presented in Figure 1. Of these 15 cases,47% were male and 53% were female. The youngest patient was 23 years old, while the oldest was 75 (average: 49 years). The clinical manifestations observed most frequently at the beginning of the disease included cognitive symptoms, behavioral changes, cephalalgia and depression.The rest of the symptoms are shown in Table 2. The elapsed time between the beginning of symptoms and the patient’s arrival at the NINN for medical attention consisted of a mean of 71 days (range: 10–210).The elapsed time from the beginning of clinical manifestations until the date of death and that between their arrival at the NINN and date of death are shown in Table 2. When analyzing their family history with respect to the presence of CJD and any type of dementia, all patients denied having this kind of antecedent. The past medical history of each patient is shown in Table 3. Ten patients (66.6%) were married or lived as couples, four (26.7%) were single, and only one (6.7%) was a widower. Regarding their educational level, nine (60%) had completed elementary education or had taken the first 3 years of courses and knew how to read and write; two (13%) had completed secondary education; three (20%) had completed high school or had at least a technical degree; and one (7%) had a bachelor degree. Concerning their residential locations, it was found that seven patients (47%) lived in Mexico City, five (33%) in the state of Mexico or suburban zones of the City, and three (20%) in other states of the Mexican Republic. The distribution of the cases in Mexico City, according to political divisions of the city, is shown in Figure 2. Regarding patients living in the state of Mexico, two (40%) were from Chalco, one (20%) was from Cuautitlan, one (20%) was from Ecatepec, and one (20%) from San JuanTeotihuacan. The three states of the Mexican Republic from where the other patients came were Guerrero, Hidalgo, and Sonora, each with one case. Regarding the occupational activities of the seven female patients, six (86%) were housewives and one (14%) was a secretary.With respect to the occupational activities of the male patients, two (25%) were retailers, two (25%) were office employees, and the remaining 50% was made up of farmers, drivers, bricklayers, and students, each with one case.

DISCUSSION The average age in this study was 49 years, with an age range of 23–75 years. The age range reported by Bateman et al.13 is 45–75 years, and they mentioned that sCJD is extremely rare under age 30. However, in our study we had patients younger than this latter age. Nevertheless, gender frequency was similar, with 53% of female patients and 47% of male patients. Our results are more consistent with the work of Olov et al.32 who reported cases of CJD in younger patients, ranging from 34 to 84 years of age. Of the three definite cases, two were female, an interesting fact, as in 1995, in Mexico, Martínez et al.33 reported three cases, all of them female patients, but only one of them had a brain biopsy result. We are aware that PrP genotyping is important to classify prion diseases. However, in Mexico this technique is not available, a limitation of this study. However, we consider that this study reveals important information about CJD in Mexico. In the future, it would be interesting to perform a retrospective study with genetic analysis. Although seven cases died over a 5-year period, only 43% of them had a confirmed diagnosis. Despite the clinical profile, the laboratory, and the imaging studies that

could be made due to the refusal by the patients’ relatives. Unfortunately, Mexican culture is not oriented towards organ donation and post mortem studies. Besides this fact, specialized centers or laboratories where 14.3.3 protein determinations can be carried out are scarce. In Mexico, only one National Institute of Health conducts this type of analysis, which means blood samples have to be analyzed in foreign countries, making studies more expensive for patients, most of whom have low economic resources.This makes it impossible for them to obtain studies that support the CJD diagnosis. A tendency therefore exists to underestimate the real frequency of the disease, thus it may be more common in Mexico than it appears. In effect, the lack of knowledge among the population and among the medical staff of some institutions, as well as different levels of medical attention provided countrywide, may cause this disease not to be consistently diagnosed. This contrasts greatly with what happens in many European countries, where prompt post mortem studies for BSE have been carried out since January 1, 2001.

Fig. 2 Geographical areas of the Mexican Republic where the studied cases were located.

DELEGATIONS OF FEDERAL DISTRICT

GUSTAVO A MADERO 2 CASES

VENUSTIANO CARRANZA 1 CASE

IZTACALCO 1 CASE

COYOACAN 2 CASES

XOCHIMILCO 1 CASE

Table 2 indicates that most of the reported cases applied for medical attention 1–2 months after the appearance of symptoms, due to the limited importance patients give to behavioral and psycho-affective disorders. Early identification of the first symptoms in sporadic CJD, like depression, agitation, irritability, and memory loss, is important for public health reasons and potential timely interventions when treatments become available.34 No similarities were observed among the occupational activities of the studied male patients. Besides this, the fact that most of the female patients were housewives is most probably a reflection of the usual occupation of lowincome Mexican women. Forty-seven percent of the cases died, and the available information indicates that the elapsed time from the initiation of symptoms and the patient’s death is short – less than a year – indicating the damaging and aggressive impact of this disease. In spite of the fact that CJD is sporadic and its frequency of appearance is relatively low, it is necessary to make patients and their relatives aware of the importance of brain donation, to be able to reach more precise diagnoses and avoid many of these cases being classified either as probable or possible. Information about TSE must be widespread, particularly that concerning CJD; epidemiological surveillance and diagnostic systems must be established, so that more precise data concerning the incidence of these diseases are available, allowing for stricter control and prevention to be imposed. The training process should be enriched by increasing the number of autopsies performed in the NINN. From 1998 onwards, an institutional autopsy program has been

established; however, proven cases of dementia including prion diseases are still low.35 It is necessary to increase the study of prion diseases by including autopsies as an integral part of medical education programs, along with a participating academic committee that should promote, assess, and evaluate the results obtained.

REFERENCES

snip...end...TSS

2007 Japanese Society of Neuropathology

http://www.blackwell-synergy.com/doi/abs/10.1111/j.1440-1789.2007.00807.x


Rocio on July 21, 2006 12:55 PM MEXICO I hope some one of the foundation speak spanish....I am from Mexico, My father has CJD, his mom, 2 brothers and his sister are dead because of the same sickness...I cant understand...why my family...and why my dad??? he is 64, he still alive...he doesnt know he is sick...because he is losting his memory...so he feells good...I dont have enough vocabulary but I feell terrible, In Mexico anydody knows about this sickness so is difficult for us because we have to be in contact from San Francisco.....the only think I need....is talk with someone who had the same problem.....thank you and sorry for my written...(I dont speak neither write a good english)


http://www.cjdfoundation.org/guestbook.php?query=&page=50&limit=10



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Reyes M, Aguilar S, Corona R, Vega I Creutzfeldt-Jakob disease: Case report and literature review Original title: Enfermedad de Creutzfeldt-Jakob: Reporte de un caso y revisión de la literatura Med Sur 2002; 9 (2): 79-87

ABSTRACT

Creutzfeldt-Jakob disease is a neurodegenerative and neuroselective entity, unusually reported in Mexico. We report a case of a 66 year-old women patient with rapidly progressive dementia, associated with cerebellum syndrome, myoclonus, abnormal movements and motor dysfunction, with diagnostic studies positively suggesting Creutzfeldt-Jakob disease. The clinical characteristics and diagnostic studies are presented. A review of the literature discussing pathogenic mechanisms, variants of the disease and diagnostic clues, are also presented.

http://www.medigraphic.com/ingles/i-htms/i-medsur/i-ms2002/i-ms02-2/im-ms022d.htm



PLEASE NOTE ;

Creutzfeldt-Jakob disease is a neurodegenerative and neuroselective entity,

unusually reported in Mexico

UNUSUALLY REPORTED is key word here. ...TSS


======================================

Thursday, August 4, 2011

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

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



see source science here ;



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




Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease (SEE VIDEO)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/british-disease-or-disease-gone-global.html


Saturday, March 5, 2011

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

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


Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html


#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Author: T. Singeltary; Bacliff, TX/US

Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange

This abstract has been ACCEPTED.

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Authors: T. Singeltary; Bacliff, TX/US

Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Body: 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.

Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion

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


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

From: Terry S. Singeltary Sr.

To: Debra.Beasley@aphis.usda.gov

Sent: Tuesday, November 24, 2009 11:01 AM

Subject: OIE has recently published its proposed animal welfare guidelines for public comment

Greetings USDA/APHIS et al,

I would kindly like to comment on OIE proposed guidelines.

AS I said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. THE reason most every country around the globe came down with BSE/TSE in their cattle, were due to the failed and flawed BSE/TSE testing and surveillance policy of the O.I.E. NOW, they don't even acknowledge atypical scrapie it seems, as one for concern $

Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html


Tuesday, May 24, 2011 2:24 PM

O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/oie-terrestrial-animal-health-standards.html


IN SHORT, AND IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

http://www.oie.int/eng/Session2007/RF2006.pdf


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

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



EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1211902594180.htm


Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of the USA Question number: EFSA-Q-2003-083

Adopted: 1 July 2004

Summary

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.

A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1211902594180.htm


Note1: It is also worth noting that the current GBR conclusions are not dependent on the large exchange of imports between USA and Canada. External challenge due to exports to the USA from European countries varied from moderate to high. These challenges indicate that it was likely that BSE infectivity was introduced into the North American continent.

snip...please see full text ;

http://www.efsa.europa.eu/en/scdocs/doc/3rax1.pdf


Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Mexico Question number: EFSA-Q-2003-083 Adopted date: 01/07/2004 Summary

Document

Summary

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in Mexico, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Mexico. This scientific report addresses the GBR of Mexico as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into Mexico and could have reached domestic cattle. These cattle imported could have been rendered and therefore led to an internal challenge in the mid to late 1990s. It is possible that imported meat and bone meal (MBM) into Mexico reached domestic cattle and leads to an internal challenge around 1993.

It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’ MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late 1990s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable. Thus it is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.

EFSA concludes that the current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.

Publication date: 20/08/2004 Last updated: 08/09/2004


http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1178620779452.htm



SUMMARY

http://www.efsa.europa.eu/EFSA/Scientific_Document/sr04_biohaz02_mexico_report_summary_en1,0.pdf



DOCUMENT

http://www.efsa.europa.eu/EFSA/Scientific_Document/sr04_biohaz02_mexico_report_v2_en1,0.pdf



====================================================================



Subject: Mexico SAGARPA Assessment of BSE VS EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of Mexico

Date: February 5, 2007 at 1:11 pm PST

Empresa solicitante: SAGARPA

Tipo del análisis efectuado: Cuantitativo

Temática: “Análisis de riesgo sobre la ocurrencia de la encefalopatía espongiforme bovina en México”

INTRODUCTION:

The bovine spongiform encephalopathy (BSE), it is a neurological disease, invariably fatal and with long period of incubation, that affects cattle. Its etiologic agent is the prion. General consensus exists with respect to that the feeding of contaminated meat and bone flours, it is the most significant source in the dissemination and transmission of this etiologic agent. At this time there is no exist evidence that BSE is transmitted by means of embryos, their semen and in case of existing maternal transmission, if this could happened it would be in a so extremely low rate that it could not be considered like a trigger or leading factor of an epidemic. Controversy in respect to other probable ways of transmission remains. The BSE was diagnosed for the first time in 1986 in the United Kingdom. At this time it exists in 26 countries, including a Canada and the United States of North America (USA).

This document summarizes the analyzed elements and the results of the study of the evaluation of the risk factors, of the epidemiology surveillance and related activities, as well as the quantitative estimation of the risk with respect to the probability of introduction of the disease to the Mexican herd.

EVALUATED ELEMENTS:

Demography and characteristics of the Mexican cattle industry: Cattle is one of the main activities in the Mexican farming sector, due to its contribution in the supply of meat (beef) products, dairy, among others; as well as its participation in the international trade on cattle exports, mainly to the United States of North America.

According to data of the 2001, cattle population is of 30.620.933 of heads, of which 28.480.803 are beef cattle and 2.140.130 dairy cattle. The main cattle production states are located in the center-north, where its operation is intensive and its feeding is based on grains; as well as in the coast of the Gulf of Mexico and the south-southeastern, with intensive programs and feeding is based mainly on the pasturing (grass). The national dairy herd, is calculated as specialized or technified that represent 17,44% of the herd, semi-specialized 14,90% of the herd, double-purpose herd (beef and dairy) 59,68% and the small family-run herd or the referred as “backyard” (traspatio) 7,98%.Previous numbers are to be considered as an estimation of the dairy livestock inventory by production units. Nevertheless, it is necessary to consider that all races of pure breed can be found in anyone of those groups.

Legal grounds: Mexico counts on a normative frame that covers (deals with) the relevant aspects of the Epidemiology Surveillance of the BSE, like the Federal Law of Animal Health, the Federal Law of Metrology and Regulation, the General Law of Health and several Mexican Official Norms (NOM-009-Z00-1994, Sanitary process of the meat, NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products destined for animal feed and NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding). Wider and extended covertures of these regulations were evaluated.

Veterinary infrastructure: The veterinary services in the country are structural and normative organized by the Mexican State through the Secretariat of Agriculture, Livestock, Rural Affairs, Fishery and Alimentary (SAGARPA) Federally Empower, that is to say, that has the capacity and authority to negotiate an to come to agreement with the States Governments that integrate the Republic; to coordinate itself with the other Secretariats of State; to deal with organizations of the Private and Social sector as well as with the rest of the Civil Society as a whole.

The National Service of Health, Food Safety and Ag-alimentary Quality (SENASICA), it is an organism of this Secretariat, which has attributions in the matter of vegetable health, animal health and ag-alimentary safety and is conformed by the following main directorates: Sanidad Vegetal, Salud Animal, Inocuidad Agroalimentaria, Acuícola y Pesquera, Inspección Fitozoosanitaria, (equivalent to U.S. APHIS, FSIS and VS –Veterinary Services) Jurídica, Administración e Informática. In accordance with the assigned attributions, it corresponds to central offices the substantive part and the operative part, to the personnel assigned to the State Delegations of the SAGARPA and other instances of the SENASICA.

Consequently, the four main areas are assigned to the Main directorate of Salud Animal-Animal Health (DGSA) and to the Main directorate of Inspección Fotozoosanitaria-Plant Inspection (DGIF) and Veterinary Services (SV) in Mexico are in charge of: surveillance, epidemiology, animal movement, zoo-sanitary campaigns and emergencies.

Imports This is perhaps one of the medullar points, in the sense that it represents the information of the imports made during the risk periods and therefore, it provides the fundamental information for the risk assessment. In 1991, Mexico implemented measures to avoid the appearance of BSE, as the disease had become a serious worldwide problem, reason why, live bovines imports were prohibited, beef, beef products and by-products and in 1994 flour of meat and bone from countries affected by this disease was also prohibited and in the 2000 MBM feeding ban was imposed. In order to mitigate the risk of transmission of the EEB, a revision of the established requirements for import for ruminants’ products began.

Cattle imports and its products and by-products, as well as specific risk materials played a very important role in this study, where considerable amounts of cattle imports from countries now affected by BSE were identified, countries that at the time of the import they remained clean and therefore just some preventive risk measures were in place.

Slaughter, Cattle disposition and Offal.- Different cattle slaughter schemes were analyzed as well as the processes in use, finding some significant differences among them, being the most important the sanitary jurisdiction of the organizations that regulate us.

In Mexico, the slaughter is divided in three different systems, Federal Inspection Type Plants (TIF), which has been increased in the past years; in 1992 they participated with the 13,5%, in 1997 with the 19,40% and in 2002 with the 26,60% of the national total. In the case of the municipal slaughterhouses from 1992 to 1997, their slaughtered animals corresponded to the 49,5% and for 2002 it was increased to 73,4%, whereas the slaughter in private plants decreased of 37,10% in 1992 to 31,10%, in 1997 and from 1998 to date, we have no information.

The procedures to be followed by the establishments in the animal slaughter and those that industrialize, process, packing, chilled/froze beef products or by-products for human consumption, in order to obtain products of optimal hygienic quality, are written in the NOM-009-ZOO-1994 “sanitary Process of Beef”.

The direct consumption of beef can be stratified in three great destinies, differentiated by the market that are destined to, the rural one, the one of small centers of population, (and) the one of the big cities, characterized each one of them by its consumption and the partial or integral industrialization by direct consumer and by means of commercialization or points of sale, as well as for the origin of the own supplier.(?)

Rendering of Cattle Products.

The processes applied by the rendering plants for obtaining the protein from inedible offal, were evaluated.

Food elaboration and its use for animal feeding.- This analysis was focused in the processes of food elaboration for animal consumption.

In Mexico, the control in the production of food from animal origin, as much as the elaboration of the meat flour as that of the balanced food manufacture it is regulated by the Mexican Official Norm NOM-061-ZOO-1999, “Zoo-sanitary Specifications of nutritional products for animal consumption”, which bans the use of MBM flours of ruminant origin or any mixture that contains it for the elaboration of balanced meals for ruminants, and the Mexican Official Norm NOM-060-ZOO-1999, “Zoo-sanitary Specifications for the transformation of animal offal and its use in the animal feeding”.

In accordance with the Section of Manufacturers of Balanced Food for Animals of the National Camera of the Industry of Transformation (CANACINTRA), there are 396 balanced food plants registered, same that have the capacity to produce more than 20 million tons a year, according to the numbers registered during 1999-2002. 63% of such plants are integrated and produce 64% of the animal feed produced nationwide, the rest corresponds to commercial plants.

The animal feed produced by the integrated plants, that is the most significant part, during the 2002 it produced the 58,7% of the products destined for raising of poultry, the 16,5% for swine, the 14,3% for dairy and 9,2% for feedlots (cattle) and 1,3% for other species.

As far as the composition of the main raw materials to produce balanced foods, these mainly correspond in 45% to domestic sorghum and 55% sorghum concentrated; 16% to domestic yellow maize and 84% imported; 91% domestic protein pastes and 9% imported; 80% of other domestic forage grains (broken maize, wheat, barley, oats, etc.) and 20% imported and other ingredients (wheat by-products, maize, vitamins, minerals, oils, etc.).

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Neuropathies in Mexico, Epidemiology Surveillance Program.- For this analysis, the legal elements related to the notification of the BSE were taken into account, in Mexico, as well as the activities made by the Commission Mexico - United States for the Prevention of the Aftosa Fever and Other Exotic Diseases of Animals (CPA), official entity in charge of carrying out this activity and other connected activities as training, taking of samples and the diagnosis of laboratory.

BSE Diagnosis.

Veterinary Services diagnoses capacity was evaluated as well as its adherence to the international standards, according to what is indicated by the International Organization of Animal Health (OIE), as well as the processes of taking and shipping of samples.

For the diagnosis of the BSE, the OIE recommends five laboratory tests: Histopathology (HP), Immunohistochemistry (IHQ), Western blot (immunotransferency), ELISA (enzimoinmunoassay) and Bio-assay in mouse. At this time Mexico counts with two laboratories of diagnosis for this disease: the National Center of Services of Diagnosis in Animal Health (CENASA) and the Laboratory of high security of the CPA. The CENASA performs the histopathology test and at the CPA the Immunohistochemistry test is carried out.

The reception of samples at CPA, it depends to a great extent on the economic resources which are accounted for this activity, expense that is approximately of $400,00 ($ 36.50 USD.) per sample received (includes the material for conservation, packing and shipping), reason for what, have to wait for the collection of several to be sent at the same time and in order to reduce costs, but delaying the result. As the CPA does not have a certified pathologist to carry-out the HP test technique, these samples are sent to the CENASA for their diagnosis; this implies that such samples are stored by approximately one week, since it doesn’t have the human resources for its transfer.

The main problem at CENASA, for the right operation of the diagnosis of the BSE, it is the lack of coordination on shipping and receiving of samples, which is not done accordingly to the calendar of the laboratory and the operative area, because in a short period of time the expected/projected number of samples is exceeded, resulting in delays in accomplishment of the tests and the disposition in excess of material and human resources.

At this moment, the techniques are being standardized, Immunohistochemistry at the CENASA and the western blot (immunmotransferency) at the CPA, which will allow us to have more tools for the diagnose in Mexico; in addition, the WB allow us to count on another technique of the higher sensitivity and specificity, that guarantees optimal result in less time (approximately 8 hours).

It must be mentioned that, we have had contemplated the formation of a network of laboratories of diagnosis of TSE´s to specialized on the HP technique, where we will have 6 regional laboratories and 4 universities involved, this will in the future allow the processing and diagnosis of the sample from its place of origin and only its confirmation by other techniques at central level. For this, we already count with the procedure for the authorization and verification of a laboratory of histopathology for the diagnosis of the BSE.

Monetary Compensation to cattle dealers: Because the BSE is considered as an exotic disease, a contingency fund that could be put to work in case the disease appears, does not exist at this time.

In the case of the contingency funds, the national campaign for diseases relies on a section on this subject. Nevertheless, for the exotic diseases official norms do not exist and article 36 of the Federal Law of Animal Health only establishes that will be due to create, but it does not explain the mechanism to be considered for its creation.

The pre-established form to compensate the possible producers that are themselves affected by the presence of BSE in their cattle, it will be from the federal budget that is agreed upon the program Alliance for the Country for the corresponding fiscal year.

In this sense, it is necessary to pinpoint that the minimum amount to consider for this budget will be a 4% of the total assigned to the Fito-zoo-sanitary Contingencies Plan on behalf of the Federal Government.

This will have a distribution by federal entity, which a specific amount will be able to be assigned to joint, if necessary, to the DINESA against the BSE. Also, the State Governments will proportionally contribute an equal amount to the federal to be incorporated to the compensation funds of the Device of Emergency. As for the cattlemen, they will have to come-up with resources equivalent to the third part of the total amount assigned by the Federal and State governments.

Animal identification and traceability of cattle products.- Different elements were considered with which Mexico counts on to carry out the traceability of animals and its products upon a sanitary problem, including the animal identification and the organizations related to this activity.

Actually, the identification system of the cattle in Mexico is organized in two forms, one State-ID with aims of demonstration of property and control of cattle rustling and another Federal-ID, with aims of identification for the development of the zoo-sanitary campaigns against the bovine tuberculosis and brucellosis, first it is based on the registry and recognition of the Hot-Brands of each producer, and the second in addition to the previous system, one is based on a metallic earring of blue color with a number of identification, which is described in the NOM-031-ZOO-1995, Campaña Nacional Contra la Tuberculosis Bovina (Mycobacterium bovis), National Campaign Against Bovine Tuberculosis.

This procedure assigns a number to an animal, which is used during zoo-sanitary surveillance campaigns, these activities are registered along with an identification number, in a document called test-opinion, in which it is written down, in addition to the test results applied to the animals, the identification and data of the cattle herd and ranch of origin of the animal for its later traceability. This test-opinion is along with the certificate of “Herd free of bovine tuberculosis” as described in the same Mexican Official Norm.

According to the procedure previously described, in Mexico, there were around 3.291 registered herds with 282.932 heads of bovines identified in 2003, that represents 0,94% of the total population in this country.

Nevertheless, in the same NOM, it’s expressed in point 11 referring to mobilization that the animals coming from disease-free herds, they will be able to be mobilized to any destination within the national territory with no need to be tested for tuberculosis before its mobilization, if the following requirements are met: obtain a zoo-sanitary certificate, and for the zoo-sanitary certificate to be issued, certification that they come from a disease-free herd and that the animals must have a disease-free herd identification

.

Considering that in order to mobilize the animals it is necessary to have a valid disease-free zoo-sanitary certificate, we can estimate that there are more than 3,431,022 identified animals, according to the information obtained from the Statistical Report of the Cattle Mobilization of FY2000, with information captured up to the 24 of August of 2001 by the National Organism of Herd Certification, A.C., that represents the 11,4% of the bovine total population on which we can observe that more than 50% of these mobilizations are directed to slaughterhouses, 17% to feedlots, 15% for export and 11% for pasturing.

Based on the above, experience of a suitable animal traceability is shown specially in the case of the animals destined to be exported, where the USDA when finding a positive animal reactor to the tests of tuberculosis in the United States, it has been possible to trace it back to its the original herd; on the other hand, the identification system used on dairy cattle, which counts on a homogenous system of identification for production and genetic improvement control, nevertheless, this mechanism although is available for the federal government, it would make use of, only in the presence of a serious epidemiology event.

Educational Programs, Awareness and Training.- The CPA, one of its activities, is to maintain a permanent program of training courses on exotic diseases of the animals, on a national context. In 1994, BSE awareness programs were incorporated , with the diffusion of information, talks and courses on the following areas: disease history, economic consequences, etiology, transmission mechanisms, clinical signage, histopathology injuries, differential diagnosis, measures of prevention and activities of epidemiologist surveillance, supported by audio-visual means, these programs are taken to a diverse audience, including the students of the last semesters of Veterinary Medicine, to the personnel that conforms the Quarantine National System, as well as Veterinary Doctors, government, private and to other specialists.

ESTIMATION OF RISK (Risk Assessment)

According to the qualitative estimation in this assessment, it was determined that the risk of occurrence of the disease in the bovine population, is low.

The quantitative estimation index was located at 5.268908E 08 of the risk of disease exposure of the national herd, number that represents numerically like a low probability of occurrence of the problem in Mexico.

CONCLUSIONS AND RECOMMENDATIONS

Conclusions

The BSE is a disease that was described for the first time in 1986, nevertheless, today, epidemiologists have many unanswered questions on how is transmitted.

The introduction of the BSE in Mexico would cause a serious socioeconomic impact, commercial, political and probably of public health concern, because the presence of the disease would restrict sanitarily and commercially, disrupting the actual distribution of meat products at national level and to other countries, independently of the impact in the consumption of the inhabitants with respect to the beef consumption and products of bovine origin.

Considering the way of transmission, in case of a breakout, the native animals that are at greater risk of being infected in Mexico, those are the dairy cattle in specialized systems and the bovines at feedlots in the arid and tropical regions.

In Mexico, we got Laws, Mexican Official Norms and Agreements, that cover relevant aspects of the epidemiology surveillance of the BSE, same that must be fortified in its operative phase, mainly in its application and enforcement.

The Mexican Official Norm NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, prohibits the import of cattle products, however, fresh beef has been imported, chilled, frozen and beef preparations, as long as it comes from animals smaller of thirty months of age, which diminishes the risk but does not exclude it.

The evaluation showed that the four great areas of concern are assigned to the Main Directorate of Animal Health (DGSA) and to the Main Directorate of Fito-zoo-sanitary Inspection (DGIF); responsibility of the Veterinary Services in Mexico, in relation to the BSE are: epidemiology surveillance, animal movement control, zoo-sanitary campaigns and emergencies; functionality and capability of communicating among them was evaluated as we as the capacity of response before a sanitary emergency caused by the BSE.

It is necessary to increase and to better coordination of the surveillance activities, particularity between the areas of diagnoses and operational, for the correct execution of the surveillance activities in the field.

The imported bovines (1996-2003) have been slaughtered and those destined to improve genetics, once they conclude their productive life and are discarded, will also be slaughtered.

The actions of detection of downer-cows need to be reinforced for its processing at TIF plants, till now a deficient activity, where the majority of the animals with such clinical characteristics, regularly are not taken this plants but rather are slaughtered at same ranch/location and consumed regionally or they are taken to slaughterhouses without supervision and sanitary inspection.

Ante mortem inspections need to be reinforced at Federal Inspection Type Plants, municipal and private slaughterhouses, mainly in these two last ones, with the purpose of detecting bovines clinically affected by BSE.

There is commercial interest to incorporate flours of meat and bone of ruminants in the rations destined to the feeding of the bovines, like an alternative source of protein matter, reason why official mechanisms must be reinforced in preventing this type of illegal practices.

One of the tools in preventing the BSE is to avoid the exposure of the native bovines to the consumption of presumably contaminated feed with the pathological agent or unless is processed by means of a thermal process that guarantees its inactivation. However, the heat treatment that the flours of meat and bone are put under in XXXXXX XXXXX XXXXX XXXXXX XXXXXX XXXXXX XXXXXX XXXXX XXXXX XXXX XXXXX XXXXXX XXXXX XXXXX XXXXXX XXXXX XXXXX XXXX during 20 minutes), even though this one, it does not guarantee the destruction of the prion either, but it reduces its infectivity significantly.

The Country counts on regulations, in respect to the transformation of offal (NOM-061-ZOO-1999), there still are deficiencies as to the number and qualification of the personnel responsible in supervising their fulfillment through inspection and verification.

Deficiencies in the availability of technical information at official and private levels were detected, crucial information necessary for the elaboration of the present assessment, such as the case of the information on product imports and on rendering plants, were not available for the study.

Blood was not considered as a potential source of transmission of the BSE, by-product in form of flour (dry blood), that is also produced by the rendering plants and is used in animals feeds.

The BSE epidemiology surveillance program in Mexico must be reinforced by focusing on a target animal study (bovine suspected of BSE and with suggestive clinical signs of the disease). On the other hand, as a result from this study, we found that a percentage of the obtained samples for BSE testing have been inadequately collected and among other causes were: absence of cerebral stem, incomplete cerebral stem, over-manipulated samples, advanced changes (decompose) postmortem, not enough tissue to work on, low concentration of conservative (solution) or samples taken from inadequate age of animal (too young); showing all of these, a necessity to review these procedures.

It was also detected the fact that, as a routine practice the samples sent for the diagnosis of bovine rabies, whenever they come out positive to this disease, they are no longer processed for the BSE testing, discarding with this, the possibility of finding both diseases in a same animal, rabies virus and the BSE prion. It is also concluded that with the loss of diagnosis material, it prevented us from obtaining valuable epidemiology information useful in restructuring our surveillance program.

The identification of the cattle, as well as the traceability of its products and by-products, presents serious deficiencies at national level, which is important in case the BSE is detected in the Country, given its importance like a primordial component to trace, to prevent and to eradicate this and other animal diseases, turning out to be an additional vital tool to determine the dissemination degree in case of break-out in the country, that would immediately allow us to be able to establish its origin (native or imported) and to take the appropriate counter-epidemic measures.

From 1994, the Commission Mexico - United States for the Prevention of Foot-and-Mouth Disease (FMD) and other Exotic Diseases (CPA), it has carried out activities of awareness and training on BSE, however, this has been centered to certain zones of the country, leaving some other zones, particularly the rural zones without cover, same that can provide with valuable epidemiology information and some cases for diagnosis of neuropathy in ruminants.

According to the analysis made on the risk assessment in its qualitative modality, it is considered like low-risk, the risk of introduction of the BSE to the national herd, whereas the quantitative study locates it in values of 5.268908E-08.

Recommendations:

We ought:

to reinforce the inspection and supervision activities by the sanitary authority of the SAGARPA over all of those involved in the cattle production chain, in respect to the fulfillment and application of the established technical regulations expressed on the official norms on the monitoring of BSE, specially the NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding and the NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products for animal consumption;

to increase the number of inspectors (Vet Doctors) as much as governmental as private, with a vision of having a better supervision of the rendering plants and feed factories. It is recommendable that such inspectors have a veterinary doctor’s degree.

to reinforce the active epidemiology surveillance subsystem, having special attention to aim at target animals and the size of the statistical test, as well as its stratification at national level;

to review, to update and to homologate the criteria and definitions of the Mexican official norms related to the monitoring of the BSE and the requirements of import, according to norms NOM-008, NOM-030 and NOM-060;

to provide technical and legal elements in the official norms, that may allow to optimize the use of financial and human resources (federal, state and private), with the purpose of that the material and human infrastructure, the installed diagnoses and the potential, can be used with greater efficiency, in the prevention activities, diagnosis and surveillance of the BSE in Mexico;

to homologate the mechanism of training in the obtaining of the samples for the BSE, using the technique of the teaspoon, by means of a national program;

to have a certified pathologist for the high security laboratory of the CPA, because this situation of not having one, causes the delay in the processing of samples, as well as the loss of economic resources by requiring the support of the CENASA;

to plan the taking of samples at a national level and to coordinate its shipment to the CPA for its processing in the laboratory of high security or its re-expedition to the CENASA, with the purpose of optimizing the diagnosis;

to obtain funds and allocate them at each state, in order to compensate cattle dealers affected by the animal culling at risk by BSE, in case of BSE showing up in Mexico, the same or similar mechanism are to established for the handling of monetary compensation, like the one used on the Alliance for the Country or to extend the already existing state government faculties, by means of an exclusive and specific account for the implementation of BSE comp payment.

to implement a national animal identification and traceability system, its products and by-products, that it may allow us to apply prevention measures and control of diseases, as it would be the case of the BSE.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 6, paragraph 1):

Gallinaza and pollinaza- feather meal (hay bed or substrate on which birds grow up, constituted by rice husk, straw or another type of hay, agriculturist, that at the end of the raising cycle of young hens or chicken, contains the feces of the animals that were bred on it, as well as rest of non-consumed food by the birds), it has been considered in multiple occasions, like an element of potential risk in the transmission of the bovine spongiform encephalopathy (BSE), when it is used to feed ruminants. The risk is generated, as it is common, the bird feed, contains flours of meat and bone of ruminant like source of protein. In this way, in theory, if some of the bovines with which the meat and bone flour was prepared as bird feed were infected with the BSE prion and given the high resistance of the agent (prion) to high temperatures, in the industrial process as the making of the flour, like the making of the nutritional concentrated feed for birds, and even the passage by digestive-tract of the bird, it would not guarantee the destruction of the BSE prion, reason why the possibility would exist, when gallinaza or pollinaza is used in the feeding of ruminants, this could infect susceptible ruminants.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 6, paragraph 2):

As much gallinaza as pollinaza, they can contain up to a 3% of wasted food, independently of bird feces that could also contain the prion, all implying that the flours of meat and bone of bovine origin, can be consumed by other bovines and by doing this, constituting a possible situation of BSE risk.

Norma NOM-060-ZOO-1999 Zoo-sanitary specifications for the transformation of animal’s offal and its use in animal feeding and the NOM-061-ZOO-1999 Zoo-sanitary Specifications of nutritional products for animal consumption, they clearly indicate the prohibition to feed ruminants with flours of meat and bone of ruminant origin, however, the prohibition to feed ruminants with gallinaza or pollinaza, is not contemplated in these norms.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 6, paragraph 3):

Other elements to consider are the production cycles of the farms of birds in Mexico, a common practice is that when a cycle is reached (ended) “all inside, all outside”, and the pollinaza and gallinaza are destined to feed the cattle. Depending on the type and the characteristics of the bed, it is possible to calculate an approximated weight of 13,9 kg. by square meter of bird farm surface.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 6, paragraph 4):

In the Mexican market, two types of products are accepted: pollinaza and gallinaza, which has been consolidated as a production system, considering that near 90% of the feces are used as ruminant’s feed, with prices reaching near those of cereal grains, the rest is used in agriculture.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 6, paragraph 5):

The use of the animal feces like source of high nutrients supply, it obeys mainly to its high content of mineral matter and non-protein nitrogen. In general, nitrogen is concentrated in greater amount in bird feces. What is doubtless, it is that the feces are raw material available all the year long for animal feeding, especially bovines.

The FAO (1980) made a description of the physical composition of pollinaza as it is detailed next:

Feces 62%

Bed 31%

Wasted Feed 3%

Feathers 2%

Unknown ingredients related to fresh matter 2%

Source: The FAO. Feed from Wastes Animal: State of knowledge, Production animal and Health, to paper 18. Rome, Italy 1980.

Conclusion:

Making public the information that has been eliminated of the report, it would open the door for those in the grain business to use it for their benefit and by pressing the government/the authority to establish a NOM banning such products as ruminant feed. This would bring/cause an important alteration in the commercialization of these products nationwide, which in turn would remarkably increase the production costs of the cattle in feed lots. Today, we foresee escalating grains prices at medium term, originated by its use in the ethanol production; this would aggravate the situation and force a NOM as described before, which in addition, if our sanitary status with respect to the BSE is considered low, it would be obviously excessive cost and highly harmful for the producer of birds and cattle. It is why, that it was decided to block the reference information.

With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:

Justification of the blockade (p. 12, paragraph 3):

During the period between 1996 to 2003 years in which, considering the long period of incubation of the BSE, the disease was already present in the United States of America and Canada, Mexico as usual, imported considerable amounts of calves destined for dairy production. In the same term “bullfight” bulls from Spain were imported once Europe reached a free status from FMD, same that allowed the import of some cattle for reproduction from other European countries, with exception of the United Kingdom and Ireland, countries in which BSE already existed.

In all the cases these imports were immediately stopped even before the confirmation of BSE in those countries, nevertheless, as already indicated, the ample period of incubation of the disease, those imports are looked as of certain risk, even though in that moment they were not.

The nonexistence of a national animal identification and traceability system at that time made it impossible to establish the destiny of most of those animals and to even know if they have been eliminated at the end of its productive life. It is possible to indicate that the recent imports of heifers coming from the United States of America and in the near future from Canada, new requirements and actions that guarantee their traceability and other measures to mitigate the BSE risk, are in place.

Even though during the administration of the Lic. Vicente Fox, the SAGARPA made a concerted effort to establish the National System of Individual Cattle Identification (SINIIGA), the magnitude, cost and coverage of the project, its conclusion in the short and medium term are way far distant, what implies that it will be long time before Mexico can count on a suitable (working) national system of identification and traceability of animals and products of origin animal.

The blockade of the above paragraph obeys to the convenience of not exposing to the Federal Government to unnecessarily critics that even though funded, it would not contribute to the solution of a problem that, although is of urgent attention, by its magnitude and cost, it exceeds in much, the present capacities as much of the Government, like of the National Cattlemen Sector. The critic would sustain in that what it is said in such paragraph is purely speculative, without possibility of corroborating it documentarily.

END...



Saturday, March 05, 2011

MAD COW DISEASE BSE PRION MEXICO ???

http://bse-atypical.blogspot.com/2011/03/mad-cow-disease-bse-prion-mexico.html


HOWEVER, my files show 44 tons of greaves for USA. ...TSS

Subject: Re: exports from the U.K. of it's MBM to U.S.???

From: S.J.Pearsall@esg.maff.gsi.gov.uk

Date: Tue, 8 Feb 2000 14:03:16 +0000

To: flounder@wt.net (Receipt Notification Requested) (Non Receipt Notification Requested)

Terry Meat and bonemeal is not specifically classified for overseas trade purposes. The nearest equivalent is listed as flours and meals of meat or offals (including tankage), unfit for human consumption; greaves.

UK exports of this to the US are listed below:

Country Tonnes

1980

1981 12

1982

1983

1984 10

1985 2

1986

1987

1988

1989 20

1990

Data for exports between 1975 and 1979 are not readily available. These can be obtained (at a charge) from data retailers appointed by HM Customs and Excise: BTSL (Tel: 01372 463121) or Abacus (01245 252222).

Best wishes Simon Pearsall

Overseas trade statistics Stats (C&F)C

====================================== END...TSS



Intraspecies transmission of L-type-like bovine spongiform encephalopathy detected in Japan

Shigeo Fukuda 1*, Yoshifumi Iwamaru 2*, Morikazu Imamura 2 , Kentarou Masujin 2 , Yoshihisa Shimizu 2 , Yuichi Matsuura 2 , Yujing Shu 2 , Megumi Kurachi 2 , Kazuo Kasai 2 , Yuichi Murayama 2 , Sadao Onoe 1 , Ken'ichi Hagiwara 3 , Tetsutaro Sata 4 , Shirou Mohri 2 , Takashi Yokoyama 2 and Hiroyuki Okada 2 1 Molecular Biotechnology Laboratory, Hokkaido Animal Research Center, Shintoku, Hokkaido 081-0038, Japan 2 Prion Disease Research Center, National Institute of Animal Health, 3-1-5 Kan-nondai, Tsukuba, Ibaraki 305-0856, Japan 3 Departments of Biochemistry and Cell Biology , and 4 Pathology, National Institute of Infectious Diseases, Toyama 1-23-1 Shinjuku-ku, Tokyo, 162-8640, Japan Correspondence Hiroyuki Okada, Prion Disease Research Center, National Institute of Animal Health, Kannonndai 3-1-5, Tsukuba, Ibaraki 305-0856, Japan. Tel & fax: +81-29-838-7757; email: okadahi@affrc.go.jp

*These authors contributed equally to this work.

Copyright © 2009 Japanese Society for Bacteriology, Japanese Society for Virology, Japanese Society for Host Defense Research, and Blackwell Publishing Asia Pty Ltd KEYWORDS atypical bovine spongiform encephalopathy • cattle • L-type-like • transmission

ABSTRACT

It has been assumed that the agent causing BSE in cattle is a uniform strain (classical BSE); however, different neuropathological and molecular phenotypes of BSE (atypical BSE) have been recently reported. We demonstrated the successful transmission of L-type-like atypical BSE detected in Japan (BSE/JP24 isolate) to cattle. Based on the incubation period, neuropathological hallmarks, and molecular properties of the abnormal host prion protein, the characteristics of BSE/JP24 prion were apparently distinguishable from the classical BSE prion and closely resemble those of bovine amyloidotic spongiform encephalopathy prion detected in Italy.

--------------------------------------------------------------------------------

Received 7 May 2009; revised 2 August 2009; accepted 4 August 2009.

DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1348-0421.2009.00169.x About DOI

snip...

In Japan, two atypical BSE cases have been identified to date. The first case showed an L-type-like electrophoretic mobility of the unglycosylated PrPSc on western blot analysis (9). The second casewas identified in an aged beef cattle, Japanese Black (BSE/JP24), and showed PrP-positive amyloid plaques in histopathological examination of the brain and a distinct glycoformprofile (10). Although such properties seem to be similar to those reported in a BASE case (7), unlike with the BASE prion, shortening of the incubation periods was observed in bovinized mice serially passaged with the BSE/JP24 prion (11). Thus, it remains controversial whether the BSE/JP24 prion is identical to the BASE prion. These observations prompted us to characterize the phenotypes of the BSE/JP24 prion propagated in its natural host by comparison with those of the classical BSE prion. Hence, we have inoculated with brain homogenates from classical BSE and BSE/JP24 isolates into Holstein cattle and assessed their risk against cattle species.

snip...

In summary, we demonstrated the successful transmission of the BSE/JP24 prion to cattle. The BSE/JP24 prion-affected cattle sustained the molecular properties of PK-treated PrPSc as those of the original BSE/JP24 isolate. Although most brain regions except for the medulla oblongata of the original BSE/JP24 isolate were unable to be investigated due to inadequate specimen collection, in comparison to experimentally BSE/JP24 prion-affected cattle, both neuropathological features, such as severe vacuolation in the medulla oblongata at the obex level and the presence of PrPSc plaques, closely resembled each other. Based on molecular properties of PK-treated PrPSc and a detailed comparison of the immunohistochemical and neuropathological properties, the BSE/JP24 prion was distinguishable from those in the classical BSE prion, and appear to be rather similar to the BASE prion (8). Of interest, experimental transmission of the BSE/JP24 prion to cattle induced a shorter incubation period and more severe neuropathological changes compared to the classical BSE prion, suggesting that the BASE and BSE/JP24 prion might be more virulent in cattle species. However, such speculation conflicts with reports that atypical BSE field cases have been mainly found in adult and aged cattle (5). The reason for this discrepancy in incubation periods between experimentally and naturally affected cattle is unknown. These observations may imply that atypical BSE are sporadic forms of BSE. Alternatively, the route of infection and/or prion titer may be attributed to the relatively long incubation period in natural atypical cases. Further studies using orally BSE/JP24 prion-affected cattle will be needed to address this issue.

http://www3.interscience.wiley.com/journal/122570969/abstract?CRETRY=1&SRETRY=0



FINAL REPORT JAPAN-UNITED STATES BSE WORKING GROUP JULY 22, 2004 $$$

http://www.mhlw.go.jp/kinkyu/bse/yunyu/dl/040723-1b.pdf


http://www.mhlw.go.jp/english/topics/foodsafety/bse/dl/3-2-2.pdf



Subject: Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Date: August 24, 2005 at 2:47 pm PST

August 24, 2005

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Greetings APHIS ET AL,

My name is Terry S. Singeltary Sr.

http://www.regulations.gov/search/Regs/contentStreamer?objectId=0900006480086ebc&disposition=attachment&contentType=msw6


"These observations may imply that atypical BSE are sporadic forms of BSE."



PLEASE SEE BELOW ;



"So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE."




Prions: Protein Aggregation and Infectious Diseases

ADRIANO AGUZZI AND ANNA MARIA CALELLA

Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland

snip...

3. Sporadic Creutzfeldt-Jakob disease Approximately 85% of all human prion diseases are sporadic forms of CJD. For sCJD, there is no association with a mutant PRNP allele, nor is there any epidemiological evidence for exposure to a TSE agent through contact with people or animals infected with TSEs. sCJD cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the size and glycoform ratio of proteaseresistant prion protein identified on western blot (type 1 or type 2) (174). Heterozygosity (Met/Val) at PrP codon 129 appears to be associated with a lower risk (378) and/or prolonged incubation time (119, 387). The lack of routine laboratory testing for preclinical diagnosis makes the search for agent sources and other risk factors extremely difficult. At present, the means of acquisition of a TSE agent in these patients remains a mystery. So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE. In humans, the peak age incidence of sporadic CJD is 55–60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

snip...

http://physrev.physiology.org/cgi/content/abstract/89/4/1105



CREUTZFELDT JAKOB DISEASE JAPAN

Asia-Oceania Symposium on Prion Disease AOSPD 2010

Poster 10

Human prion diseases in Japan: a prospective surveillance from 1999

Sakai, K, Nozaki, I., Hamaguchi, T., Noguchi-Shinohara, M., Nakamura Y., Sato, T,

Kitamot, T., Mizusawa, H., Sanjo, N., Moriwaka, F., Shiga, Y., Kuroiwa, Y.,

Nishizawa, M., Inuzuka, T., Takeda, M., Abe, K., Murai, H., Murayama, S., Tateishi, J., Shirabe, S., Takumi, I., Harada, M., Yamada, M.

Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School Science, Japan; 2) Creutzfeldt-Jakob disease Surveillance Committee, Japan

ksakai@med.kanazawa-u.ac.jp

[Introduction]

The current surveillance system of prion diseases by the Creutzfeldt-Jakob (CJD) Surveillance Committee, Japan, started in 1999. The purpose of this study is to describe the epidemiology and clinical features of human prion diseases in Japan.

[Materials and Methods]

The clinical, neuropathological, and molecular genetic data of patients suspected as having prion disease were prospectively collected and analyzed by the CJD Surveillance Committee, Japan, from 1999 to 2009.

[Results and Discussion]

We have obtained the information of 1,823 patients. A total of 1,421 cases of prion diseases was identified, including 1,088 cases of sporadic CJD (sCJD) (76.6%), 245 cases of genetic prion diseases (17.2%), 80 cases of dura mater graft-associated CJD (dCJD) (5.6%), 1 case of variant CJD (vCJD) (0.1 %), and 7 cases of unclassified CJD (0.5%). The overall annual incidence rate was 0.92 cases per million person­-year. In sCJD, codon 129 polymorphism of the prion protein (PrP) gene was Met/Met in 94.9%, Met/Val in 4.3%, and Val/Val 0.7%. MM2 type occupied the majority of atypical sCJD cases. In genetic prion diseases, the most frequent mutation of the PrP gene was V1801 (42.9%), followed by P102L (18.0%), E200K (16.3%), and M232R (13.9 %), and so on. Eighty cases of dCJD were found in this surveillance system; when combined with cases identified by previous surveillance systems, the total number of dCJD cases in Japan was 138. One case of vCJD with a history of a short stay in the UK was identified in 2005.

[Conclusions]

Human prion diseases in Japan were characterized by frequent occurrence of dCJD relative commonness of MM2 among atypical sCJD cases, and unique PrP gene mutations in genetic prion diseases, which were different from those in countries.



http://www.gakkai.co.jp/apps2011/greet.html


http://www.gakkai.co.jp/apps2011/index.html



" and 7 cases of unclassified CJD (0.5%). "   ???



Saturday, March 5, 2011




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



Greetings,




WITH more and more atypical Transmissible Spongiform Encephalopathy cases showing up in more and more species here in North America, and the enormous monumental amount of banned mad cow protein in commerce since the infamous partial and voluntary mad cow feed ban inked on paper, with tons and tons crossing back and forth between the USA, Canada, and Mexico, it just does not surprise me of all these "PENDING CLASSIFICATIONS" of human TSE in Canada, and the USA. UK c-BSE transmitted to humans became nvCJD. WE now have atypical strains of BSE in cattle. Mission Texas experiments long ago showed that transmitted USA sheep scrapie to USA bovine, produced a TSE much different than the UK typical c-BSE. SO why would human TSE in the USA look like UK human TSE ? The corruption is mind boggling. The UK saw a suspicious TSE in humans, and science linked it to cattle. North America is awash with human and animal TSE, CJD is rising in young and old, with the same pathology and same symptoms, and none of it is related to the other. isn't that nice. who, what, bestowed such miracles upon North America $



snip...see full text of the ''PENDING CLASSIFICATION OF CJD CASES'' here ;


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




Prospective 10-year surveillance of human prion diseases in Japan

Ichiro Nozaki1,2, Tsuyoshi Hamaguchi1, Nobuo Sanjo3,4, Moeko Noguchi-Shinohara1, Kenji Sakai1, Yosikazu Nakamura4,5, Takeshi Sato4,6, Tetsuyuki Kitamoto4,7, Hidehiro Mizusawa3,4, Fumio Moriwaka4,8, Yusei Shiga4,9, Yoshiyuki Kuroiwa4,10, Masatoyo Nishizawa4,11, Shigeki Kuzuhara4,12, Takashi Inuzuka4,13, Masatoshi Takeda4,14, Shigetoshi Kuroda4,15, Koji Abe4,16, Hiroyuki Murai4,17, Shigeo Murayama4,18, Jun Tateishi4,19, Ichiro Takumi4,20, Susumu Shirabe4,21, Masafumi Harada4,22, Atsuko Sadakane5 and Masahito Yamada1,4

+ Author Affiliations

1 Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan

2 Department of Neurology, Noto General Hospital, Nanao, Japan

3 Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan

4 Creutzfeldt–Jakob Disease Surveillance Committee, Japan

5 Department of Public Health, Jichi Medical University, Shimotsuke, Japan

6 Department of Neurology, Higashi Yamato Hospital, Higashiyamato, Japan

7 Department of Prion Protein Research, Division of CJD Science and Technology, Tohoku University Graduate School of Medicine, Sendai, Japan

8 Department of Communication Disorders, Health Sciences University of Hokkaido Graduate School of Psychological Science, Ishikari, Japan

9 Department of Neurology, Aoba Neurosurgical Clinic, Sendai, Japan

10 Department of Neurology, Yokohama City University School of Medicine, Yokohama, Japan

11 Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan

12 Department of Neurology, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan

13 Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu, Japan

14 Department of Psychiatry, Osaka University Graduate School of Medicine, Suita, Japan

15 Department of Neuropsychiatry, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan

16 Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Okayama, Japan

17 Department of Neurology, Iizuka Hospital, Fukuoka, Japan

18 Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan

19 Harukaze Healthcare Service Institution, Fukuoka, Japan

20 Department of Neurosurgery, Nippon Medical School Musashi Kosugi Hospital, Kawasaki, Japan

21 Centre of Health and Community Medicine, Nagasaki University, Nagasaki, Japan

22 Department of Medical Imaging, Institute of Health Biosciences, the University of Tokushima Graduate School, Tokushima, Japan

Correspondence to: Masahito Yamada, Department of Neurology and Neurobiology of Ageing, Kanazawa University Graduate School of Medical Science, 13-1 Takara-machi, Kanazawa 920-8640, Japan E-mail: m-yamada@med.kanazawa-u.ac.jp Received May 1, 2010. Revision received June 14, 2010. Accepted June 15, 2010.

Next Section Summary

We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999. We obtained information on 1685 Japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n?=?180, 14.7%) and probable (n?=?1029, 84.2%) cases, except for dura mater graft-associated Creutzfeldt–Jakob disease which also included possible cases (n?=?13, 1.1%). They were classified into 922 (75.5%) with sporadic Creutzfeldt–Jakob disease, 216 (17.7%) with genetic prion diseases, 81 (6.6%) with acquired prion diseases, including 80 cases of dura mater graft-associated Creutzfeldt–Jakob disease and one case of variant Creutzfeldt–Jakob disease, and three cases of unclassified Creutzfeldt–Jakob disease (0.2%). The annual incidence rate of prion disease ranged from 0.65 in 1999 to 1.10 in 2006, with an average of 0.85, similar to European countries. Although methionine homozygosity at codon 129 polymorphism of the prion protein gene was reported to be very common (93%) in the general Japanese population, sporadic Creutzfeldt–Jakob disease in Japan was significantly associated with codon 129 homozygosity (97.5%), as reported in western countries. In sporadic Creutzfeldt–Jakob disease, MM1 type (Parchi’s classification) is the most common, as in western countries. Among atypical sporadic Creutzfeldt–Jakob disease cases, the MM2 type appeared most common, probably related to the very high proportion of methionine allele in the Japanese population. As for iatrogenic Creutzfeldt–Jakob disease, only dura mater graft-associated Creutzfeldt–Jakob disease cases were reported in Japan and, combined with the data from previous surveillance systems, the total number of dura mater graft-associated Creutzfeldt–Jakob disease was 138, comprising the majority of worldwide dura mater graft-associated Creutzfeldt–Jakob disease patients. Regarding genetic prion diseases, the most common mutation of prion protein gene was V180I (41.2%), followed by P102L (18.1%), E200K (17.1%) and M232R (15.3%), and this distribution was quite different from that in Europe. In particular, V180I and M232R were quite rare mutations worldwide. Patients with V180I or M232R mutations rarely had a family history of prion diseases, indicating that a genetic test for sporadic cases is necessary to distinguish these from sporadic Creutzfeldt–Jakob disease. In conclusion, our prospective 10-year surveillance revealed a frequent occurrence of dura mater graft-associated Creutzfeldt–Jakob disease, and unique phenotypes of sporadic Creutzfeldt–Jakob disease and genetic prion diseases related to the characteristic distribution of prion protein gene mutations and polymorphisms in Japan, compared with those in western countries.

Key words prion disease dura mater graft-associated Creutzfeldt–Jakob disease 14-3-3 protein periodic synchronous wave complexes codon 129 or 219 polymorphism

snip...

Sporadic Creutzfeldt–Jakob disease

The very high frequency of PSWCs in sporadic Creutzfeldt–Jakob disease (97%), compared with the data of western countries (Collins et al., 2006), is related to the application of the diagnostic criteria by Masters et al. (1979) and the low autopsy rate in Japan. Regarding the subtypes according to Parchi’s classification (Parchi et al., 1999), the MM1 type was the most common (25/44, 56.8%), characterized by typical Creutzfeldt–Jakob disease features: rapid clinical course, positive PSWCs and CSF 14-3-3 protein and typical MRI findings (Table 4). Among atypical cases other than the MM1 type, the proportion of the MM2 type was relatively high (10/44, 22.7%) compared with Europe, the USA (12/300, 4.0%) (Parchi et al., 1999) and Germany (12/243, 4.9%) (Heinemann et al., 2007). MM2 type cases included cortical (50%), thalamic (40%) and combined (cortical and thalamic) forms (10%). Our results were influenced by the bias that atypical cases might have been more selectively autopsied to confirm the diagnosis; however, the relatively high proportion of the MM2 type in Japanese patients with sporadic Creutzfeldt–Jakob disease reflected the high proportion of the methionine homozygote genotype in the Japanese population.

Clinical characteristics of each sporadic Creutzfeldt–Jakob disease subtype (MM1, MM2-cortical, MM2-thalamic, MV2 and VV2) were almost the same as in previous reports (Parchi et al., 1999; Collins et al., 2006), except for the higher frequency of extrapyramidal signs (72%) in the MM1 type [7% in a previous report (Parchi et al., 1999)] and the lower frequency of pyramidal signs (0%) in MM2-cortical subtype [83% in previous reports (Parchi et al., 1999; Krasnianski et al., 2006)]. The deficiency of pyramidal or other neurological signs in the MM2-cortical subtype would lead to difficulties in the clinical diagnosis of MM2-type sporadic Creutzfeldt–Jakob disease on the basis of the current sporadic Creutzfeldt–Jakob disease criteria, although cortical hyperintensities on MRI suggest the diagnosis (Hamaguchi et al., 2005). In this study, the age at onset of the MM2-thalamic subtype was younger with a longer duration than the MM1 type, and neither PSWCs on EEG nor hyperintensities on MRI were identified in the MM2-thalamic subtype.

http://brain.oxfordjournals.org/content/133/10/3043.full


Prospective 10-year surveillance of human prion diseases in Japan

Table 2

Classification of each type of prion disease according to accuracy of diagnosis

Sporadic Creutzfeldt–Jakob disease Variant Creutzfeldt–Jakob disease Dura mater graft-associated Creutzfeldt–Jakob disease Genetic prion disease Total (%)

Definite

111 1 33 35 180 (13.6)

Probable

811 0 34 181 1026 (77.7)

Possible

97 0 13 4 114 (8.6)

Total (%)

1019 (77.2) 1 (0.1) 80 (6.1)

http://brain.oxfordjournals.org/content/133/10/3043/T2.expansion.html



Monday, June 06, 2011

Duration of Prion Disease is Longer in Japan Than in Other Countries

http://creutzfeldt-jakob-disease.blogspot.com/2011/06/duration-of-prion-disease-is-longer-in.html


Thursday, October 23, 2008

Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts – Japan, 1979-2008 : UPDATE

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/creutzfeldt-jakob-disease-associated.html


Wednesday, August 12, 2009

Unique clinicopathological features and PrP profiles in the first autopsied case of dura mater graft-associated Creutzfeldt–Jakob disease

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/unique-clinicopathological-features-and.html


http://www.mhlw.go.jp/shingi/2009/12/dl/s1210-8n.pdf



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