COMMISSION DECISION of 11 November 2009
amending the Annex to Decision 2007/453/EC as regards the BSE status of Chile, Colombia and Japan
(notified under document C(2009) 8590)
(Text with EEA relevance)
THE COMMISSION OF THE EUROPEAN COMMUNITIES,
Having regard to the Treaty establishing the European Community,
Having regard to Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies ( 1 ), and in particular the third subparagraph of Article 5(2) thereof,
(1) Regulation (EC) No 999/2001 lays down rules for the prevention, control and eradication of transmissible spongiform encephalopathies (TSEs) in animals. For that purpose, the bovine spongiform encephalopathy (BSE) status of Member States or third countries or regions thereof (countries or regions) is to be determined by classification into one of three categories depending on the BSE risk involved, namely a negligible BSE risk, a controlled BSE risk and an undetermined BSE risk.
(2) The Annex to Commission Decision 2007/453/EC of 29 June 2007 establishing the BSE status of Member States or third countries or regions thereof according to their BSE risk ( 2 ) lists countries or regions according to their BSE risk status.
(3) The World Organisation for Animal Health (OIE) plays a leading role in the categorisation of countries or regions according to their BSE risk. The list in the Annex to Decision 2007/453/EC takes account of Resolution No XXI — Recognition of the Bovine Spongiform Encephalopathy
Status of Members — adopted by the OIE in May 2008 regarding the BSE status of Member States and third countries.
(4) Decision 2007/453/EC currently lists Finland and Sweden as having a negligible BSE risk and all other Member States as having a controlled BSE risk. It also lists the BSE status of third countries. In May 2009, the OIE adopted Resolution No XXII — Recognition of the Bovine Spongiform Encephalopathy Risk Status of Members. That Resolution recognised Chile as having a negligible BSE risk and Colombia and Japan as having a controlled BSE risk. The list in Decision 2007/453/EC should therefore be amended to be brought into line with that Resolution as regards those three third countries. However, pending a final conclusion of the OIE on the BSE risk status of all Member States and taking into account the harmonised stringent BSE protective measures applied within the Community, no changes should at present be made as regards the recognised BSE status of the Member States.
(5) Decision 2007/453/EC should therefore be amended accordingly.
(5) Decision 2007/453/EC should therefore be amended accordingly.
(6) The measures provided for in this Decision are in accordance with the opinion of the Standing Committee on the Food Chain and Animal Health,
HAS ADOPTED THIS DECISION:
The Annex to Decision 2007/453/EC is replaced by the text in the Annex to this Decision.
This Decision is addressed to the Member States.
Done at Brussels, 11 November 2009.
For the Commission
Member of the Commission
‘LIST OF COUNTRIES OR REGIONS
A. Countries or regions with a negligible BSE risk
— New Zealand,
B. Countries or regions with a controlled BSE risk
— Belgium, Bulgaria, the Czech Republic, Denmark, Germany, Estonia, Ireland, Greece, Spain, France, Italy, Cyprus, Latvia, Lithuania, Luxembourg, Hungary, Malta, Netherlands, Austria, Poland, Portugal, Romania, Slovenia, Slovakia, United Kingdom,
— United States,
C. Countries or regions with an undetermined BSE risk
bought and paid for by your local cattle dealers. ...TSS
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,
Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary
Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028 Public Submission Title Comment from Terry S Singletary
Comment 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY
THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.
MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???
go figure. ...
Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment
January 28, 2007
I would kindly like to submit the following to ;
BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01
Wednesday, November 18, 2009
R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission
Tuesday, November 10, 2009
Surveillance On the Bovine Spongiform Encephalopathy and rabies in Taiwan
Monday, October 26, 2009
MAD COW DISEASE, AND U.S. BEEF TRADE
MAD COW DISEASE, CJD, TSE, SOUND SCIENCE, COMMERCE, AND SELLING YOUR SOUL TO THE DEVIL
Posted: Wed Dec 19, 2007 3:47 pm Post subject: OIE
Maybe familirise yourself with the OIE. The primary concern is animal health of the world they are the animal version of the WHO. It is a long way down from that ivory tower but here we go, until pressured by the USA repesentatives a country could not export animals for 6 years after finding a BSE/BASE positive animal so under the old rules the US would not be able to export anywhere in the world for another 4 1/2 years. Who got the risk levels system put in to allow some trade - your US representatives. You guys want to change rules - OK , but you do not get special rules that only apply to the US.
As i have told you before Sand h I market all my own slaughter animals and you know that, so don't do the whole holier than thow act.
With all due respect, it is obvious that you know little about the OIE and how it actually works. Having been to their offices in Paris and talked personally with the Head of the Animal Test Section, you would choke if you knew how many lobby groups attend that office daily. There is a steady stream of paid lobby groups that have one goal in life and that is to sway the Section Heads of each department within the OIE to suit the needs of different juristictions around the world, which curiously enough, also includes the USA and Canada. Anyone can go there and chat with them - providing they can privide valid cause to be let in. To say that the only goal of the OIE is animal health is actually only part of their function. They are more than that and my discussions with Dr. Diaz there has showed me that. But to blindly make a statement regarding what they do when you have no idea what they actually do is like eating the skin of the orange and not knowing what is actually under.
Interstingly you state that the US Government applied pressure (to the OIE) I assume and that is a great example of the lobby groups doing their job. So, at the end of the day, one can safely assume that it is the pressure applied by certain influential lobby groups that will determine a likely aoutcome to an apparent OIE directive. Man alive, isn't it great to live in a democracy wherein the people get to make the choices and not just some "other" interested party or group - say like........Cargyll or Tyson for example?
So, one last question, question?
Who wags the tail of that dog?? And for what reason other than one that is purely associated with trade and international agreements and greed?
And you think it is so simply explainable.
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of the United States of America (USA) Question number: EFSA-Q-2003-083 Adopted date: 1 July 2004 Summary (0.1Mb)
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.
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of CANADA
Question N° EFSA-Q-2003-083 Adopted 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 Canada, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Canada. This scientific report addresses the GBR of Canada as assessed in 2004 based on data covering the period 1980-2003. The BSE agent was probably imported into the country middle of the eighties and could have reached domestic cattle in the early nineties. 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 90s. It is possible that imported meat and bone meal (MBM) into Canada reached domestic cattle and led to an internal challenge in the early 90s. A certain risk that BSE-infected cattle entered processing in Canada, and were at least partly rendered for feed, occurred in the early 1990s when cattle imported from UK in the mid 80s could have been slaughtered. 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 Canada is III, i.e. it is confirmed at a lower level that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as the system remains unstable, it is expected that the GBR continues to grow, even if no additional external challenges occur.
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of MEXICO
Question N° EFSA-Q-2003-083 Adopted 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 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 1990's. 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 BSEagent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.
MY comments/questions are as follows ; 1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?
*** Suppressed peer review of Harvard study October 31, 2002 ***
Response to Public Comments on the Harvard Risk Assessment of ... RESPONSE TO COMMENTS FROM TERRY S. SINGELTARY SR. Comment #1: SINCE the first Harvard BSE Risk Assessment was so flawed and fraught ...
Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles
Thursday, November 13, 2008
Katharina Stoeck1, 6, Klaus Hess2, Lorenz Amsler3, 7, Tobias Eckert3, Dieter Zimmermann4, Adriano Aguzzi1, 8 and Markus Glatzel5, 8
(1) Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland (2) Dept. of Neurology, University Hospital Zurich, Zurich, Switzerland (3) Swiss Federal Office of Public Health, Bern, Switzerland (4) Institute of Surgical Pathology, University Hospital Zurich, Zurich, Switzerland (5) Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany (6) Dept. of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany (7) CSL Behring, Bern, Switzerland (8) Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany
Received: 23 February 2007 Revised: 8 February 2008 Accepted: 11 February 2008 Published online: 29 October 2008
Abstract Incidences of human transmissible spongiform encephalopathies are monitored by national registries in the majority of countries in Western Europe. During the past 13 years incidences for Creutzfeldt-Jakob disease (CJD) in Switzerland fluctuated between 0.4 and 2.63 cases/106 inhabitants. We have compared clinicpathological patient profiles including geographic and gender distribution, age at disease onset, duration of disease, clinical symptoms, and recognized or hypothetical risk factors for CJD, genetic risk factors, biochemical and histopathological data for two cohorts of Swiss sporadic CJD patients from years of regular sporadic CJD incidence (1996–2000, mean incidence 1.3 cases/106 inhabitants, n = 47) to Swiss sporadic CJD patients from years of elevated sporadic CJD incidence (2001–2004, mean incidence 2.3 cases/106 inhabitants, n = 73). Sporadic CJD patients from the cohort with elevated sporadic CJD incidence presented with a higher frequency of rare sporadic CJD subtypes. Patients of these subtypes were significantly older and showed a skewed male/female ratio when compared to published patients of identical sporadic CJD-types or to patients from the 1996–2000 cohort and indicates that improved detection of rare sporadic CJD subtypes may have contributed to increased incidence.
In summary, this analysis confirms our initial finding of an increased incidence of sCJD in Switzerland. Although, the reason for this phenomenon remains unexplained to date, our analysis demonstrates that patients from the years 2001–2004 with increased sCJD incidence differ in several aspects from published sCJD cohorts. The fact that the MV2 subgroup of patients showed an increase in mean age at disease onset when compared to published cohorts, together with the fact that these patients demonstrate distinct features in sensitive imaging methods, may indicate that improved detection of these patients has contributed to the rise in sCJD incidence. Further studies investigating biochemical and genetic aspects will contribute to our understanding of the mechanisms underlying sCJD.
Electronic supplementary material The online version of this artiecle (DOI10.1007/s00415-008-0900-00) contains supplementary material, which is available to authorized users. Key words Creutzfeldt-Jakob disease - prions - dementia - epidemiology
M. Glatzel and A. Aguzzi coordinated the design and operation of the study. Katharina Stoeck and Klaus Hess were involved in clinical assessment of patients. M. Glatzel and Dieter Zimmermann were involved in assessment of specimen. All authors contributed to the manuscript and approved the final version. M. Glatzel and A. Aguzzi had full access to all data in the study and had final responsibility for the decision to submit for publication. The study was performed according to established ethical guidelines This study was supported by grants of the Swiss Federal Office of Public Health and the Swiss National Science Foundation.
A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001–2004
Jessica Ruegger* 1 , Katharina Stoeck* 2,3 , Lorenz Amsler4,5 , Thomas Blaettler2,6 , Marcel Zwahlen7 , Adriano Aguzzi2 , Markus Glatzel2,8 , Klaus Hess1 and Tobias Eckert4,9
1Department of Neurology, University Hospital Zurich, Zurich, Switzerland
2Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland
3Department of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Hamburg, Germany
4Federal Office of Public Health, Bern, Switzerland
5CSL Behring, Bern, Switzerland
6Bristol-Myers Squibb, Wallingford, CT, USA
7Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
8Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Hamburg, Germany
9Swiss Tropical Institute, Basel, Switzerland
author email corresponding author email* Contributed equally
BMC Public Health 2009, 9:18doi:10.1186/1471-2458-9-18
The electronic version of this article is the complete one and can be found online at:
Received: 11 July 2008 Accepted: 14 January 2009 Published: 14 January 2009
© 2009 Ruegger et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background In 2001, the observed annual mortality from Creutzfeldt-Jakob disease (CJD) in Switzerland increased from less than 1.5 to 2.6 per million inhabitants. An underlying cause could not be identified.
Methods To analyse potential risk factors for sCJD in Switzerland, close relatives of 69 sCJD-patients and 224 frequency age-matched controls were interviewed in a case-control study using a standardised questionnaire. 135 potential risk factors including socio-demographics, medical history, occupation and diet were analysed by logistic regression adjusting for age, sex and education.
Results sCJD patients were more likely to have travelled abroad, worked at an animal laboratory, undergone invasive dental treatment, orthopaedic surgery, ophthalmologic surgery after 1980, regular GP visits, taken medication regularly, and consumed kidney. No differences between patients and controls were found for residency, family history, and exposure to environmental and other dietary factors.
Conclusion Although some factors were significantly more frequent among sCJD-cases, this study did not reveal specific explanations for the increased incidence of deaths due to sporadic CJD observed in Switzerland since 2001. Results have to be interpreted with caution due to multiple testing and possible recall bias in association with a long incubation period. The most plausible reason for the increase in Swiss sCJD cases after 2000 is an improved case ascertainment. Therefore, underreporting of cases might well have occurred before the year 2001, and the "real" yearly incidence of sCJD might not be lower than, but rather above 2 per million inhabitants.
Biochemical typing of pathological prion protein in aging cattle with BSE
Seraina Tester1 , Valerie Juillerat1 , Marcus G Doherr1 , Bianca Haase2 , Miroslaw Polak3 , Felix Ehrensperger4 , Tosso Leeb2 , Andreas Zurbriggen1 and Torsten Seuberlich1
1NeuroCenter, Reference Laboratory for TSE in animals, Department of Clinical Research and Veterinary Public Health, Vetsuisse Faculty, University of Berne, Switzerland
2Institute of Genetics, Vetsuisse Faculty, University of Berne, Switzerland
3National Veterinary Research Institute, Pulawy, Poland
4Institute of Veterinary Pathology, Vetsuisse Faculty, University of Zürich, Switzerland
author email corresponding author email
Virology Journal 2009, 6:64doi:10.1186/1743-422X-6-64
The electronic version of this article is the complete one and can be found online at:
Received: 23 March 2009 Accepted: 26 May 2009 Published: 26 May 2009
© 2009 Tester et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background The broad enforcement of active surveillance for bovine spongiform encephalopathy (BSE) in 2000 led to the discovery of previously unnoticed, atypical BSE phenotypes in aged cattle that differed from classical BSE (C-type) in biochemical properties of the pathological prion protein. Depending on the molecular mass and the degree of glycosylation of its proteinase K resistant core fragment (PrPres), mainly determined in samples derived from the medulla oblongata, these atypical cases are currently classified into low (L)-type or high (H)-type BSE. In the present study we address the question to what extent such atypical BSE cases are part of the BSE epidemic in Switzerland.
Results To this end we analyzed the biochemical PrPres type by Western blot in a total of 33 BSE cases in cattle with a minimum age of eight years, targeting up to ten different brain regions. Our work confirmed H-type BSE in a zebu but classified all other cases as C-type BSE; indicating a very low incidence of H- and L-type BSE in Switzerland. It was documented for the first time that the biochemical PrPres type was consistent across different brain regions of aging animals with C-type and H-type BSE, i.e. independent of the neuroanatomical structure investigated.
Conclusion Taken together this study provides further characteristics of the BSE epidemic in Switzerland and generates new baseline data for the definition of C- and H-type BSE phenotypes, thereby underpinning the notion that they indeed represent distinct prion disease entities.
Conclusion Taken together these results indicate that the prevalence of H- and L-type BSE in Switzerland remains under the detection limit of the Swiss active surveillance program. However one H-type BSE case was identified by passive BSE surveillance and proves in principle the capacity to identify such cases in the population. Hence, the overall prevalence of atypical BSE in Switzerland appears very low and similar to what has been reported from other countries. It has been speculated and strengthened by experimental data [53,54] that atypical BSE once recycled in the cattle population was the origin of the worldwide BSE epidemic in the last 20 years. If this holds true and such cases occur spontaneously in the population, then BSE might never be completely eradicated. Furthermore, in these circumstances, it would be hazardous to relieve certain disease control measures, including the total prohibition of MBM in ruminant feed.
Finally the authors consider the possibility that CJD in Switzerland is related to a prion epizootic, and pay considerable attention to this possibility since between 1995 and 1998, Switzerland reported a larger incidence of BSE than did all other continental European countries (415 cases between 1990 and 2002). Exposure to BSE-infected products might have taken place mainly before high-risk bovine food products were banned from the human food chain in 1990. However, BSE is thought to cause variant CJD [abbreviated as vCJD or CJD (new var.) in ProMED-mail] rather than sporadic CJD, yet all evidence indicates that none of the Swiss cases fulfill the diagnostic criteria of vCJD. Swiss CJD could be related to BSE only if the strain of Swiss BSE prion differs from the strain of BSE prevalent in the UK. Available data, though limited, suggest that this is not the case.
At present there is no evidence that the Swiss CJD cases might result from transmission of BSE to people after one or more serial passages through species other than cattle. Scrapie is exceedingly rare in Switzerland: only 7 cases have been reported in the past 10 years. Chronic wasting disease of deer has not been reported in Europe, although surveillance data on transmissible spongiform encephalopathies in European game are incomplete.
All recognized clinical and molecular markers combine to indicate that none of the Swiss patients developed vCJD. The authors conclude that the elucidation of the underlying chain of events is a national research priority, and may uncover previously unrecognized modes of prion infection and transmission. It remains to be seen whether this increase in the incidence of CJD in Switzerland will be sustained, or whether it represent a statistical anomaly. According to Will RG, et al. (Ann Neurol 1998; 43: 763-767) there was a doubling in the annual death rates for sporadic CJD in the United Kingdom between the 1980s and the 1990s, and similar increases in the apparent death rates for sporadic Creutzfeldt-Jakob disease had occurred in other European countries, attributable to improvement in diagnosis. - Mod.CP].................as/mpp/cp/mpp
Prions: Protein Aggregation and Infectious Diseases
ADRIANO AGUZZI AND ANNA MARIA CALELLA
Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
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.
Physiol Rev • VOL 89 • OCTOBER 2009 • www.prv.org
Tuesday, November 17, 2009
SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1
Tuesday, November 17, 2009
SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2
Thursday, November 05, 2009
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification