Bovine spongiform encephalopathy, Switzerland
Information received on 03/05/2011 from Mr Hans Wyss, Chief Veterinary Officer, Schwarzenburgstrasse 161, Swiss Federal Veterinary Office, LIEBEFELD BERNE, Switzerland
Report type Immediate notification (Final report) Start date 08/04/2011 Date of first confirmation of the event 18/04/2011 Report date 03/05/2011 Date submitted to OIE 03/05/2011 Date event resolved 03/05/2011 Reason for notification Reoccurrence of a listed disease Date of previous occurrence 10/2006 Manifestation of disease Sub-clinical infection Causal agent Prion Nature of diagnosis Laboratory (advanced) This event pertains to the whole country
New outbreaks Summary of outbreaks Total outbreaks: 1 Location(s) SANKT GALLEN (Sankt Gallen)
Total animals affected Species Susceptible Cases Deaths Destroyed Slaughtered Cattle 32 1 0 1 0
Outbreak statistics Species Apparent morbidity rate Apparent mortality rate Apparent case fatality rate Proportion susceptible animals lost* Cattle 3.13% 0.00% 0.00% 3.13%
* Removed from the susceptible population through death, destruction and/or slaughter
Epidemiology Source of the outbreak(s) or origin of infection Unknown or inconclusive
Control measures Measures applied No vaccination No treatment of affected animals
Measures to be applied No other measures
Diagnostic test results Laboratory name and type National Reference Laboratory (National laboratory) Tests and results Species Test Test date Result Cattle western blotting 18/04/2011 Positive
Future Reporting The event is resolved. No more reports will be submitted.
Switzerland sporadic CJD ; Swiss rise in CJD raises concerns over possible BSE link [LONDON]
Plaque attack: Swiss patients have spongiform patterns in the brain typical of sporadic CJD. The number of people dying from Creutzfeldt-Jakob disease (CJD) has risen sharply in Switzerland -- sparking fears of a possible link with bovine spongiform encephalopathy (BSE). BSE is thought to be the cause of a distinctive form of the brain-wasting disease known as variant CJD. The Swiss cases, in contrast, are standard 'sporadic' CJD. Each year between 1997 and 2000, no more than 11 Swiss people developed CJD. But 19 cases were reported in 2001, and seven were recorded in the first quarter of this year. This is some four times higher than the incidence elsewhere, reports a team led by Adriano Aguzzi of the University Hospital Zurich (M. Glatzel et al. Lancet 360, 139-141; 2002). The increase could be a mere statistical blip, or it may be due to increased awareness of the disease leading to more diagnoses. More disturbing is the possibility that the cases are linked to the consumption of BSE-infected meat products -- which would mean that the BSE agent can cause two distinct forms of CJD. Possible links between the Swiss CJD cases and BSE will now be explored by strain-typing experiments in which the disease is transmitted to mice. These tests will take at least a year to complete. "It's the best way to establish or exclude any suspected link," says Moira Bruce of the UK Institute for Animal Health's Neuropathogenesis Unit in Edinburgh.
Experiences in England and Switzerland -- two countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since mad cow was first detected in British herds in 1986. Switzerland discovered last year its CJD rate was twice that of any other country in the world. Switzerland had been seeing about eight to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.
Mouse model sheds new light on human prion disease
Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.
Monday, May 19, 2008
SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS
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.
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
Sunday, May 01, 2011
STUDY OF ATYPICAL BSE 2010 Annual Report May 2011
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5–May 2011
"THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. 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."
NOW, some history on the failed OIE BSE/TSE policy, and why the OIE allowed BSE and other TSE to spread around the globe $$$
while i applaud Switerland for reporting this case of BSE, i must say again that the OIE is a failed organization. to have a reporting system set up for TSE that only reports a case of TSE when a country sees fit to report a case, and only relies on the GOOD WORD of a country, is a failed system. apparently, the USA and Canada and Mexico i.e. North America, is exempt from the OIE reporting base of TSE on the OIE news feed. ...tss
The two Commissions discussed the issue of ‘atypical’ scrapie in terms of notification requirements and the issue of the host genetic resistance. In response to questions of Members, the Code Commission clarified that ‘classical’ scrapie is reportable to the OIE but that ‘atypical’ scrapie is not reportable (in accordance with the recommendations made by the ad hoc Group on Atypical Scrapie and Atypical BSE, which met in November 2007). However, the sharing of scientific information on ‘atypical’ scrapie is encouraged. At this time, the Code Commission considered that more scientific information would be needed to fully address the issues associated with host genotype.
OIE Terrestrial Animal Health Standards Commission / September 2010
The EU takes note of the fact that atypical scrapie is not an OIE listed disease. Nevertheless, it will remain notifiable in the EU. Moreover it must be stressed that any emergence of this disease should be notified to the OIE by Members and that scientific data should continue to be gathered.
Has transmission to humans been proven? (with the exception of artificial circumstances) AND Is human infection associated with severe consequences? (death or prolonged illness)
What is Nor98-Like (Nonclassical) Scrapie?
Nor98-like scrapie is a prion disease. The prion diseases include classical scrapie in sheep and goats, Bovine Spongiform Encephalopathy (BSE) primarily of cattle, chronic wasting disease (CWD) of deer and elk, and in humans Creutzfeldt-Jakob Disease (CJD) and variant Creutzfeldt-Jakob Disease (vCJD). What each of these diseases has in common is that they cause a progressive and ultimately fatal degeneration of the nervous system. While the underlying cause(s) are still debated, the theory most widely accepted in the scientific community is that the agent is a prion – an abnormal form of a normally occurring cellular protein.
Unlike BSE, classical scrapie and Nor98-like scrapie have not been shown to be a threat to human health. Classical scrapie has been known to exist for over 250 years, and cases have occurred in the United States since 1947. It is likely that Nor98-like scrapie has also existed for a long time; however, it was first identified in Norway in 1998, hence the name Nor98. This type of scrapie is referred to as “atypical scrapie”, “Nor98 scrapie”, “Nor98-like scrapie”, or “nonclassical scrapie” in the literature.
Since 1998, almost every country in Europe, as well as the Falkland Islands, New Zealand, Canada, and the United States have found similar cases. The first cases in the United States were identified in 2007.
Nor98-like (nonclassical) scrapie and classical scrapie are separate diseases with distinct features.
Classical scrapie is an infectious disease that is transmitted to other sheep and goats under natural conditions.
Nor98-like scrapie is either not transmitted or is poorly transmitted, under natural conditions. Many scientists believe that Nor98-like scrapie is not an infectious disease under natural conditions and that it is instead caused by a random conversion of the normal prion protein into the abnormal form (often referred to as “sporadic”).
Nor98-like scrapie has been found in all countries where extensive surveillance has been conducted using sensitive test methods; whereas, classical scrapie has not been reported in some of these countries.
Nor98-like scrapie cases are widely distributed and proportionate with sheep/goat populations; whereas, classical scrapie cases often occur in clusters.
Number of affected sheep or goats in a flock or herd
Classical scrapie usually infects more than one animal in an infected flock. In the US, approximately 18 percent of the mature, genetically susceptible sheep in an infected flock are infected.
Nor98-like scrapie is rarely found in more than one animal in a flock or herd. When an additional case is found, it is usually in flocks with more than 500 sheep.
Average age of onset of clinical signs in animals
With classical scrapie, clinical signs typically first appear and result in death in animals that are between 3-5 years of age.
In Nor98-like scrapie, clinical signs are rarely documented and the animals are typically diagnosed when they are sampled at slaughter, usually at greater than 5 years of age.
Variations in protection against disease conferred by genotype
Sheep with genotypes that are resistant to classical scrapie are susceptible to Nor98-like scrapie.
Clinical signs associated with disease
Unlike classical scrapie, clinical signs are rarely reported in Nor98-like scrapie cases.
In the few Nor98-like scrapie cases where clinical signs were reported, the signs observed were indistinguishable from those described for classical scrapie. These include incoordination, gait abnormalities, collapse while running, tremors, loss of condition, leg biting, nibble response and/or behavioral changes.
One potential clinical difference is that intense rubbing is a frequently occurring clinical sign in classical scrapie cases; whereas, it has not been reported in Nor98-like scrapie cases.
Laboratory findings readily distinguish Nor98-like scrapie from classical scrapie.
In 2009, the World Animal Health Organization (OIE) recognized Nor98-like scrapie as a separate disease from classical scrapie because of differences in laboratory findings, transmissibility, and distribution. This determination means that Nor98-like scrapie is not a reportable disease to OIE, and should be of no trade concern.
How has APHIS’ policy changed regarding Nor98-like scrapie?
APHIS will no longer require the depopulation or movement restriction of Nor98-like scrapie exposed sheep and goats. APHIS will propose changes to the Code of Federal Regulations (CFR) in 2010 to allow the APHIS Administrator to eliminate or reduce post exposure requirements for certain scrapie types (or certain/specific types of scrapie)-- such as Nor98-like scrapie -- that are determined to pose minimal risk of lateral transmission under natural conditions.
In the meantime, APHIS is conducting a national scrapie control pilot project for Nor98-like scrapie. The project is for flocks and herds in which animals positive to Nor98-like scrapie were born, lambed, or kidded.
What will the pilot project do for producers?
The pilot project will allow producers that have sheep and goats exposed to Nor98-like (nonclassical) scrapie to retain, sell, exhibit or move them for any purpose.
What will happen if Nor98-like scrapie case is found on or traced back to an owner’s flock or herd?
The owner will be contacted by a Federal or State veterinarian who will schedule a visit to the farm with the owner. The activities listed below will be performed.
Confirm the identification of the positive animal.
Provide the owner with information about scrapie and its control.
Determine if the positive animal was born in or gave birth in the flock/herd. If so, the veterinarian will:
Work with state animal health authority to ensure animals are not moved from the premises until they have been officially identified;
Develop a Nor98-like scrapie flock plan and a 5 year monitoring plan with the producer;
If not already officially identified, apply official eartags to sheep and goats exposed to Nor98-like scrapie; and
Inventory all sheep and goats, and any sheep or goat embryos.
The officially identified sheep and goats will then be classified and handled as low-risk exposed animals, allowing the owner to move the animals from the premises for any purpose including sale.
SCRAPIE The United States is unable to support the proposed new draft Code Chapter on Scrapie. The draft chapter, as written, departs significantly from the existing chapter, is confusing and is difficult to understand. This version of the scrapie chapter uses much of the same wording as the BSE chapter and is written as if the predominance of evidence revealed that scrapie was a food-borne disease similar to BSE in cattle which is inappropriate. Moreover, several of the new changes are not supported by current scientific evidence. As a result, detailed comments on individual articles would not meaningful at this time. The United States is not supportive of the proposed draft chapter for the following reasons: 1. Inclusion of “atypical” scrapie: The scientific evidence indicates that “atypical” scrapie, also referred to as Nor-98, Nor-98-like, or non-classical scrapie, is not the same disease as classical scrapie. Further, “atypical” scrapie does not meet the criteria for listing diseases of trade concern by the OIE, as described in Chapter 2.1.1 of the Code. The United States recommends that the scope of this chapter be limited to classical scrapie in sheep and goats. Further, the United States recommends that OIE clearly adopt the position that “atypical” scrapie represents a distinct disease entity from classical scrapie and that it not be a listed disease. • There is no evidence that “atypical” scrapie is a contagious disease. If it is contagious, available evidence suggests that it has a much lower transmission efficiency. (Hopp, et al, 2006; Green, et al, 2007; Benestad, et al 2008; McIntyre, et al, 2008) • The disease appears to be ubiquitous in that it has been found wherever sufficient surveillance has been conducted. (Buschmann et al, 2004; De Bosschere et al, 2004; Orge, et al, 2004; Everest et al, 2006; Arsac, 2007; Benestad, et al 2008; Fediaevsky, et al, 2008) • The disease does not appear to be economically significant in that the prevalence of clinical disease is low and it typically occurs in older animals. (Luhken, et al., 2007; Benestad, et al 2008). • The disease is as likely as not to be the result of a spontaneous conversion of normal prion protein. (Benestad, et al 2008, De Bosschere et al 2007) • Removal of exposed sheep is unlikely to reduce the prevalence of “atypical” scrapie infection and removing only those exposed sheep that are phenylalanine (F) at codon 141 is scientifically unsound since the disease is known to affect sheep of most other genotypes. Further, sheep with AHQ alleles have a similar risk of infection with “atypical” strains as sheep with F at codon 141. (Luhken, et al., 2007). • If “atypical” scrapie is included as a listed disease, the surveillance and diagnostic requirements which are needed to identify these cases should be described in detail in both this Chapter and the Manual of Diagnostic Tests and Vaccines for Terrestrial 2 Animals. Data from Europe illustrates that using the proper test(s) is essential for the identification of atypical scrapie (Fediaevsky et al., 2008).
6. Overemphasis on importation and use of bovine meat and bone meal as a route of scrapie transmission: Given that the draft Chapter is not intended to address risk mitigation for BSE in small ruminants, we believe there is an over-emphasis on this potential route of transmission in the current draft. The United States recommends that the requirements in this chapter be limited to the inclusion of products from sheep and goats (instead of from all ruminants) in feed or feed ingredients intended for consumption by animals • The use of products from sheep and goats as feed or feed ingredients for ruminant or non-ruminant animals represent one possible route of transmission (Philippe, et al, 2005) and a source of environmental contamination with the classical scrapie agent. However, this is not the primary route of transmission for the scrapie agent. • The need for the exclusion of cattle-derived protein or other animal protein to mitigate BSE risk should be based on a country’s BSE risk status and should be addressed in Chapter 2.3.13 of the Code.
14. Failure to provide scientific justification for the list of permitted commodities in Item 1 of Article 220.127.116.11. . We recommend that the list be re-evaluated and those items that have not been substantiated as presenting no risk be excluded or those with some risk but where the intended use mitigates the risk the use be specified. • There is no known human health risk associated with scrapie. As such, if meat and meat products for human consumption are included in this list, sheep and/or goat milk intended for human consumption should also be added to the list of permitted commodities in Item 1 of Article 18.104.22.168. • In the vast majority of sheep infected with classical scrapie, actual infectivity or PrPres has been identified in most tissues including the lymphoreticular system (tonsils, spleen, lymph nodes), the gastrointestinal tract, brain, and spinal cord (Hadlow et. al. 1979; Hadlow et al., 1980; van Kuelen et al., 1996; van Kuelen et al., 1999, Andreoletti et al., 2000; Heggebø et al., 2002; Caplazi et al., 2004). Infectivity and/or PrPres has also been identified in the placenta (see Hourrigan et al., 1979; Onodera et al., 1993; Pattison et al., 1972; Pattison et al., 1974; Race et al., 1998), blood (Hunter et al., 2002; Houston et al. 2008); peripheral nerves (Groschup et al., 1996), muscle (Pattison and Millson, 1962; Andreoletti et al., 2004; Casalone et al., 2005), salivary gland (Hadlow et al., 1980; Vascellari et al., 2007), kidney (Siso et al., 2006), and skin ( Thomzig et al., 2007). In addition, recent work has shown milk and/or colostrum from scrapie infected ewes transmitted the disease to 17 of 18 lambs (Konold et al., 2008). • The data on the risk of low protein tallow made from scrapie infected tissues particularly for use in milk replacer is limited and some epidemiologic studies suggest an association of milk replacer use with scrapie risk. Taylor et al., 1997 examined the inactivation capacity of different rendering system in regards to scrapie. The presence of infectivity was determined by bioassay into mice. From the onset of this study, it was assumed that tallow was not the vehicle for the transmission of TSE. Hence only 2 tallow samples were examined.
Increased Atypical Scrapie Detections
Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.
Saturday, December 18, 2010
OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5–May 2011
Friday, March 4, 2011
Alberta dairy cow found with mad cow disease
Wednesday, August 11, 2010
REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA
Thursday, August 19, 2010
REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA
Thursday, February 10, 2011
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 a nd how to hide mad cow disease in Canada Current as of: 2011-01-31
i wonder if CFIA Canada uses the same OBEX ONLY diagnostic criteria as the USDA ?
Tuesday, November 02, 2010
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992
Sunday, March 27, 2011
SCRAPIE USA UPDATE FEBRUARY 2011
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5-May 2011
Wednesday, March 9, 2011
27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD
March 8, 2011
President Barack Obama The White House
1600 Pennsylvania Avenue, W Washington, DC 20500
Dear President Obama: