Monday, May 5, 2014

FSA H R Jasper & Son Limited prosecuted failing to remove specified risk material (SRM) from sheep carcasses destined for the food chain

H R Jasper & Son Limited prosecuted


2 May 2014 04:58 PM

The Food Standards Agency has welcomed the successful prosecution of a Cornwall-based slaughter house for a breach of the regulations introduced to control the risks from BSE. 

H R Jasper & Son Limited, a red meat slaughterhouse based near Launceston, Cornwall, has been ordered to pay almost £9,000 in fines and costs for failing to remove specified risk material (SRM) from sheep carcasses destined for the food chain.
SRM is the parts of cattle, sheep and goats most likely to carry BSE. All SRM must be removed in either the slaughterhouse or, in certain circumstances, cutting plant. It must not enter the food chain.
The company entered not guilty pleas on six charges and, following a two day hearing, was convicted on all counts.
Andrew Rhodes, Chief Operating Officer at the FSA, said: 'The meat industry has worked hard over many years to restore confidence in the safety of UK meat. Unfortunately, some companies occasionally fail to meet the standards which underpin the strict and successful system of controls we have in place to tackle BSE. As the enforcer of those regulations we will take action against any company that potentially puts consumers at risk.'
The case was heard at Truro Magistrates’ Court on 29 April 2014. More information about the BSE controls can be found at the link below.

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Last updated on 1 May 2014
 
H R Jasper & Son Limited prosecuted
 
The Food Standards Agency has welcomed the successful prosecution of a Cornwall-based slaughter house for a breach of the regulations introduced to control the risks from BSE.
 
H R Jasper & Son Limited, a red meat slaughterhouse based near Launceston, Cornwall, has been ordered to pay almost £9,000 in fines and costs for failing to remove specified risk material (SRM) from sheep carcasses destined for the food chain.
 
SRM is the parts of cattle, sheep and goats most likely to carry BSE. All SRM must be removed in either the slaughterhouse or, in certain circumstances, cutting plant. It must not enter the food chain.
 
The company entered not guilty pleas on six charges and, following a two day hearing, was convicted on all counts.
 
Andrew Rhodes, Chief Operating Officer at the FSA, said: 'The meat industry has worked hard over many years to restore confidence in the safety of UK meat. Unfortunately, some companies occasionally fail to meet the standards which underpin the strict and successful system of controls we have in place to tackle BSE. As the enforcer of those regulations we will take action against any company that potentially puts consumers at risk.'
 
The case was heard at Truro Magistrates’ Court on 29 April 2014.
 
More information about the BSE controls can be found at the link below.
 
 
 
 
Also, at paragraph 17, it is noted that BSE had transmitted to the NPU negative line sheep (please not that as at January 1996, only one of six challenged sheep was clinically affected after oral challenge, four others have since died, and one remains alive. Following intracerebral challenge, three out of six were clinically affected, two confirmed only on pathology, while one was negative.)
 
4. Meeting 16, on 26/1/94 - the update on research (16/5) confirmed that BSE had been transmitted to sheep, and that there was clinical evidence of transmission to mice from the spleen of the affected sheep.
 
snip...
 
IN CONFIDENCE
 
A STUDY AIMED AT DETERMINING WHETHER OR NOT THERE HAVE BEEN SIGNIFICANT CHANGES IN THE NEUROPATHOLOGY OF SCRAPIE IN SHEEP AND GOATS DURING THE LAST TWO DECADES IN MATERIAL SUBMITTED TO CVL PATHOLOGY DEPARTMENT
 
 
NEW URL ;
 
 
 
EXPERIMENTAL TRANSMISSION OF BSE TO SHEEP
 
 
NEW URL ;
 
 
 
THE RISK OF TRANSMISSION OF BSE TO SHEEP VIA FEED
 
 
NEW URL ;
 
 
 
hell, they knew they were screwing up the sheep brains with cow brains in 1992;
 
"The sensitivity of the project may be partially compromised by pooling of brains, but it is considered that the success of transmission to mice with BSE will prove advantageous."
 
'NOT'...tss
 
 
NEW URL ;
 
 
 
Personal $ Confidential -- Addressee only TO ALL MEMBERS OF SEAC
 
THE EXPERIMENTAL TRANSMISSION OF BSE TO SHEEP
 
 
NEW URL ;
 
 
a) Summary of transmission studies. b) Update
 
 
NEW URL ;
 
 
 
 
 
The only circumstance in which infection with the natural isolate produces an higher incidence of disease compared to BSE, is in intracerebrally (and possibly orally) challenged ''positive'' line sheep. Notwithstanding the possibility of indigenous natural scrapie in some of these sheep, there are still sufficient numbers of transmission cases with PrP genotypes which preclude the natural disease developing i.e. those typed as VA136/RR154/QR171.
 
As an extension to this study, it has been possible to recover BSE by passage in mice from brain and spleen taken from ''negative'' line sheep infected with BSAE by ic and oral challenge (Foster and others 1996). The close similarity of incubation periods and pathology from the passage of these tissues in mice to those seen in direct BSE transmission studies from cattle to mice suggests that passaging BSE in sheep does not alter its bilogical properties (Bruce and others 1994). IN FACT, because it has been possible to isolate BSE infectivity from ovine spleens, when this proved impossible from the spleens of naturally infected BSE cows (Fraser and Foster 1993), experimentally-induced BSE in sheep appears to behave more like the natural disease of scrapie.Whether this putative similarity to natural scrapie extends to the possibility of maternal transmission of experimentally-induced BSE in sheep, has till to be elucidated...
 
 
NEW URL ;
 
 
 
*** we have found a link between BSE and CH1641, a C-group of scrapie. Disease susceptibility of sheep to these isolates is associated with different PrP genotypes compared to SSBP/1 scrapie...
 
Transmission of BSE in sheep, goats and mice.
 
snip...
 
BSE has been transmitted in two lines of genetically selected sheep (differeing in their susceptibilities to the SSBP/1 source of scrapie), and to goats by intracerebral injection AND BY ORAL DOSING.
 
snip...
 
Also, intermediate passage of BSE in sheep or goats did not alter these primary transmission properties. Hamsters were susceptible to BSE only after intervening passage through mice...
 
 

NEW URL ;
 
 
 
> *** we have found a link between BSE and CH1641, a C-group of scrapie. ***
 
Wednesday, January 18, 2012
 
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie
 
Journal of Neuropathology & Experimental Neurology:
 
February 2012 - Volume 71 - Issue 2 - p 140–147
 
 
-------- Original Message --------
 
Subject: DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE (similar to exp. BSE in sheep)
 
Date: Wed, 7 Apr 2004 08:56:36 –0500
 
From: "Terry S. Singeltary Sr." flounder@wt.net
 
Reply-To: Bovine Spongiform Encephalopathy BSE-L@uni-karlsruhe.de
 
To: BSE-L@uni-karlsruhe.de
 
######## Bovine Spongiform Encephalopathy #########
 
Date: April 07, 2004 Time: 13:45
 
DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE
 
The Veterinary Laboratories Agency (VLA) have informed Defra, the Devolved Administrations and the Food Standards Agency of a type of scrapie not previously seen in the UK.
 
The VLA and other European laboratories with expertise in scrapie-like diseases have now applied several rapid diagnostic methods to tissue samples from a sheep with suspected scrapie. Some of the methods have indicated that the case does not appear to resemble previously recognised cases of scrapie and, although there were differences, it had some characteristics similar to experimental BSE in sheep and also to an experimental strain of sheep scrapie. More importantly, though, microscopic analysis of brain material showed that the case neither resembled previously recognised types of scrapie or experimental BSE in sheep.
 
A meeting of the scientific experts who performed these analyses, held on the 30th March, concluded that this case could not be considered to be BSE in sheep, although it does not behave like known types of scrapie either. Further investigation will be needed before more can be said about how this unusual result should be described.
 
Defra's Chief Scientific Adviser, Professor Howard Dalton, said "The UK, and especially the VLA, have played an important part in improving the diagnostic methods available for identifying TSEs in sheep. As we continue to assess more samples with these improved methods it is likely that we will continue to find samples, such as this, which fall outside our current knowledge of the disease. Defra, as it does with all research, will continue to consult scientific experts to ensure that we are investigating these cases using the best available techniques and methods."
 
The National Scrapie Plan remains unaffected by this new result and SEAC will be consulted in the near future.
 
Notes to editors
 
1. Scrapie is a fatal neurological sheep disease belonging to a group of diseases called transmissible spongiform encephalopathies (TSEs), including BSE in cattle and CJD in humans. It has been present in the national flock for over 250 years. It is not considered to be transmissible to humans.
 
2. There is a theoretical risk that BSE could be present in sheep, masked by scrapie, but it has not been found naturally occurring in sheep.
 
3. There is as yet no definitive diagnostic method that can rapidly distinguish between different TSEs for example scrapie from BSE. Consequently, from time to time the scrapie surveillance programmes in EU member states throw up unusual results that merit further investigations (Defra press release 371/03 refers http://www.defra.gov.uk/news/2003/030911a.htm)
 
4. The VLA have applied several different methods to the sample to compare it to a wide range of previously detected scrapie cases, experimental BSE in sheep and an experimental strain of scrapie, termed CH1461. Two main methods have been used in this analysis:-
 
a. Western blot (WB) This involves taking a sample of the brain and treating it with an enzyme proteinase k to destroy the normal prion protein (PrPC). The diseased form of the protein (PrPSc) is able to withstand this treatment and is then separated from other cellular material on a gel. A blot is taken of the gel and the PrPSc is visualised using specific antibodies.
 
b. Immunohistochemistry (IHC) This involves taking thin slices of the brain, and by using special (antibody) markers to detect the PrPSc it is possible to see disease specific patterns of PrPSc distribution in the brain under a microscope. The Western blot method found that the sample did not appear to resemble previously recognised cases of scrapie and, although there were some differences, some characteristics were similar to experimental BSE in sheep and also the experimental strain of sheep scrapie, CH1461. IHC found that it neither resembled previously recognised types of scrapie or experimental BSE in sheep
 
5. The tissue sample has now been analysed using a total of 5 different diagnostic methods claiming to be able to differentiate between scrapie and experimental BSE in sheep. Two were performed at the VLA and three were performed in other European laboratories.
 
6. The VLA is the European Reference Laboratory for TSEs and is responsible for co-ordinating such investigations into unusual cases. Their findings will be considered by the European Food Safety Authority's committee of TSE experts and in the UK by the Spongiform Encephalopathy Advisory Committee (SEAC).
 
7. The genotype of the suspect sheep was ARQ/ARQ which is known to be susceptible to some strains of scrapie and, in experiments, to BSE. Background information on scrapie, scrapie genotyping, and the National Scrapie Plan is published on the Defra internet at www.defra.gov.uk/nsp.
 
8. For information and advice on BSE in sheep from the FSA please consult their web site at www.foodstandards.gov.uk
 
Public enquiries 08459 335577; Press notices are available on our website www.defra.gov.uk Defra's aim is sustainable development
 
End
 
Nobel House 17 Smith Square London SW1P 3JR Website www.defra.gov.uk
 
 
TSS
 
 
TSE in Sheep Contingency Planning Assessment of Risk due to BSE Infectivity from Disposal of Sheep A report for DEFRA November 2001
 
Management Summary It has been recognised for a considerable time that sheep in the United Kingdom may have been infected with BSE. To date no evidence has been found to demonstrate that the national flock is actually infected with the disease. DEFRA have prepared a draft contingency plan in the event that BSE were to be identified in UK sheep. The worst case scenario under this plan is the disposal of the entire UK flock, some 40 million sheep and lambs. This study has estimated the potential exposure of the UK population to BSE infectivity present in sheep in the event that this plan had to be put into effect.
 
 
but who would have guessed that such an important experiment/study would have gotton so screwed up, by not being able to tell a sheep brain from a cow brain;
 
© DEFRA 2002 Item 3- Scrapie Brain pool experiments- Update on current position and audits of samples 3.1 Members were updated on experiments conducted at the Institute of Animal Health (IAH) to examine a pool of scrapie brains collected in the early 1990 s for evidence of BSE. SEAC had previously recommended that the material should be examined by DNA analysis to assess whether the pooled brain material may have been contaminated with bovine tissue. The Laboratory of the Government Chemist (LGC) had been asked to perform the work. Their results were completely unexpected as the analysis detected only bovine material in the sample. SEAC had intended to meet on the 19 October to Agreed version consider the experiment in detail. However, in view of the result, the meeting was cancelled.
 
 
Executive Summary An audit of the sample handling procedures at IAH-E was carried out on 24 October 2001 at the request of the Department of the Environment, Food and Rural Affairs (DEFRA), by a team of two UKAS auditors. The scope of the audit was limited to the traceability of cow and sheep brain samples used in several experiments relating to transmissible spongiform encephalopathy (TSE) agents. In particular, the team focused on the audit trail of samples that had been sent to LGC, Teddington, the audit trail of brains collected in 1990/92 by Veterinary Investigation Centres and the audit trail for archived material held by IAH-E. In addition the audit team evaluated the IAH-E procedures against the specific requirements for sampling handling of international standard, ISO 17025 and identified opportunities for improvement. The audit established that there was no formal documented quality system covering this work at IAH-E and that record keeping was inadequate to give confidence in the chain of custody of samples used in the various rendering, genotyping and strain typing experiments audited. It was not possible to establish clear traceability between the samples that had been used in the individual experiments carried out by IAH-E or IAH-C with those analysed at LGC or with those that had been collected in 1990/92. The sample handling procedures covered by this audit at IAH-E did not meet the requirements of ISO 17025.
 
 
explaining the brain mixup blunder;
 
An Investigation of the Substitution of Scrapie Brain Pool Samples A report for DEFRA November 2001
 
Risk Solutions Page 19 Why did the experimenters not notice that they were working with cow brains not sheep brains? The simple answer is because for the most part they were working with brain pool macerate (minced brain material) not brains. It is not credible that staff collecting brains at VICs would have uniformly supplied cow brains or cow brain parts in mistake for sheep. We have interviewed staff at VICs and we understand from the VLA that records do not support the possibility that significant numbers of cow brains were sent to PDM in place of sheep brains. It is also very unlikely that the people preparing the scrapie brain pool would not have noticed if they were for the most part handling cow brains or cow brain parts in place of sheep brains. We cannot rule out the possibility that some cow brain material entered the brain pool at this stage but it is not feasible that the majority of the material was bovine. The substitution, if substitution occurred, must have involved brain pool macerate or rendered products. Why can t the results of the experiments tell us what material was used? The experiments had a number of features that make the results of the mouse bioassay difficult to interpret unambiguously and lead to the possibility that substitution of the samples would be difficult to detect by examining the results of the experiments: 1. The original experiments were not designed to determine whether BSE was present in sheep. Reasonable efforts were taken to ensure that the brain pool remained free from D5055 02 Issue 1 Risk Solutions Page 20 contamination during preparation but the level of control applied during the earlier experiments (272R and 372R) was not to the standard applied later. 2. Mouse bioassay as a method of diagnosing TSEs is not based on a full understanding of biochemical and physical processes. It is an empirical technique that has been widely applied, for example to show v-CJD is similar to BSE and different from scrapie. It is a complex process and the results need to be interpreted by experts. It can take several years to generate a firm result. The principal data collected in the experiments are lesion profiles (patterns of lesions in the mice brains) and incubation period (time from injection of mice to onset of clinical symptoms. The type of TSE is identified by comparing the results with those of known provenance. There is no good agreed test of sameness of lesion profile , so in marginal cases we are reduced to using subjective observations of the form somewhat similar and interpretation is difficult. The incubation times in principle give a more objective signal, but the effect of concentration has to be controlled. The mouse bioassay data that we understand has been collected and analysed at each stage of the experiments is summarised in Table 4.1. Several features of these experiments are not commonly encountered in mouse bioassay of TSEs and this makes determining the origin of the original material from the experimental results extremely difficult. They include: a. Mouse bioassay is generally carried out on individual brains; experience of working with brain pools is very limited. b. The BBP exhibited a low titre of infectivity, which can confound interpretation of results. c. The BBP comprised bovine brains with the hindbrains removed. By contrast most of the BSE strain typing has been carried out on the hindbrains, which may give a different pattern of results. d. The 272R titrations used a different strain of mice than the 372R titrations, so direct comparison of the resulting lesion profiles cannot be made. e. The 246 experiments used brain pool which was in an unsatisfactorily autolysed state. f. The strain typing data collected (incubation time and lesion profiles) are very sparse. Judging the sameness or difference of samples is a less challenging task for strain typing than identifying a strain and it may be possible to compare data from the 246 experiments with both the 272R and 372R experiments to determine whether the samples are similar or clearly different. However, the data are sparse and the result is unlikely to be clear cut. Much of this work is currently unpublished.
 
 
Characterisation of an Unusual TSE in a Goat by Transmission in Knock-in Transgenic Mice
 
Rona Wilson, Declan King, Nora Hunter, Wilfred Goldmann and Rona M. Barron1 + Author Affiliations
 
Neurobiology Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian ↵1 E-mail: rona.barron@roslin.ed.ac.uk Received 18 January 2013. Accepted 24 May 2013.
 
Abstract
 
Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disorder of cattle, and its transmission to humans through contaminated food is thought to be the cause of the variant form of Creutzfeldt-Jakob disease (vCJD). BSE is believed to have spread from the recycling in cattle of ruminant tissue in meat and bone meal (MBM), however during this time sheep and goats were also exposed to BSE-contaminated MBM. Both sheep and goats are experimentally susceptible to BSE, and while there have been no reported natural BSE cases in sheep, two goat BSE field cases have been documented. While cases of BSE are rare in small ruminants, the existence of scrapie in both sheep and goats is well established. In the UK, during 2006-2007, a serious outbreak of clinical scrapie was detected in a large dairy goat herd. Subsequently, 200 goats were selected for post-mortem examinations, one of which showed biochemical and immunohistochemical features of the disease associated prion protein (PrPTSE) which differed from all other infected goats. In the present study we investigated this unusual case by performing bioassays into a panel of mouse lines. Following characterisation, we found that strain properties such as the ability to transmit to different mouse lines, lesion profile pattern, degree of PrP deposition in the brain and biochemical features of this unusual goat case were neither consistent with goat BSE nor with a goat scrapie herdmate control. However our results suggest this unusual case has BSE-like properties and highlights the need for continued surveillance.
 
 
 
 
 
Published Date: 2012-01-04 17:44:07
 
Subject: PRO/AH/EDR> Prion Disease update 2012 (01) Archive Number: 20120104.0027
 
PRION DISEASE UPDATE 2012 (01) ******************************
 
[2] UK: caprine BSE
 
Date: Sat 3 Dec 2011
 
Source: Emerging Infectious Diseases 17(12) 12 [edited] http://wwwnc.cdc.gov/eid/article/17/12/11-0333_article.htm Isolation of prion with BSE properties from farmed goat
 
-------------------------------------------------------
 
[Authors: Spiropoulos J, Lockey R, Sallis RE, Terry LA, Thorne L, Holder TM, et al. Animal Health and Veterinary Laboratories Agency, Weybridge, Surrey, UK]
 
Abstract
 
--------
 
Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that include variant Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE) in cattle. Scrapie is not considered a public health risk, but BSE has been linked to variant Creutzfeldt-Jakob disease. Small ruminants are susceptible to BSE, and in 2005 BSE was identified in a farmed goat in France. We confirm another BSE case in a goat in which scrapie was originally diagnosed and retrospectively identified as suspected BSE. The prion strain in this case was further characterized by mouse bioassay after extraction from formaldehyde-fixed brain tissue embedded in paraffin blocks. Our data show that BSE can infect small ruminants under natural conditions and could be misdiagnosed as scrapie. Surveillance should continue so that another outbreak of this zoonotic transmissible spongiform encephalopathy can be prevented and public health safeguarded.
 
Transmissible spongiform encephalopathies (TSEs) are fatal diseases characterized by neurodegenerative changes in the central nervous system that include vacuolation, gliosis, and accumulation of an abnormal isoform (PrPSc) of a naturally occurring host-encoded protein (PrPC) (1). According to the prion hypothesis, PrPSc is the major or the sole infectious agent (1). Although this hypothesis has not received universal acceptance, PrPScis ubiquitous in all known naturally occurring TSEs, and its detection is widely used for their diagnosis.
 
Bovine spongiform encephalopathy (BSE), a TSE of cattle, was first detected in 1986 (2) and has since been linked with emerging TSEs in other species (3,4) including humans (5,6). Because of its ability to cross species barriers and particularly its zoonotic potential, BSE is considered a public health risk, and extensive measures have been established to detect and eliminate the disease.
 
Scrapie, a naturally occurring TSE affecting small ruminants, has been known for centuries (7) and is not considered to pose a public health risk (8). Under experimental conditions, however, small ruminants are susceptible to BSE, with pathogenesis and clinical signs that are not readily distinguishable from scrapie (9-12). Additionally, the fact that small ruminants were exposed to BSE-contaminated food before the exclusion of meat and bone meal from ruminant feedstuffs led to the possibility that sheep and goats on commercial farms could be affected by BSE that could be misdiagnosed as scrapie (13,14). The response to this potential risk was the implementation of extensive statutory active surveillance, elimination, and breeding for resistance programs in the European Union (EU).
 
In 2005, as part of a review of historical TSE-positive cases of sheep and goats in France, a specimen from a goat slaughtered for human consumption in 2002 was reported to be "indistinguishable from a BSE isolate on the basis of all identification criteria available." (15). In response to this report, 2 retrospective studies were initiated in the United Kingdom to analyze archived samples from goat cases that were initially diagnosed as scrapie (16,17). Because only fixed material was available, both studies had to use differential immunohistochemical analysis (D-IHC), a technique that can discriminate scrapie from experimentally induced BSE in sheep (18). These studies identified a single case, originally diagnosed in 1990 as scrapie, that had a D-IHC signature indistinguishable from BSE (16).
 
Given the wide phenotypic variance of scrapie in sheep and our limited knowledge of this variance in goats, the D-IHC result on its own was insufficient for an unequivocal diagnosis. In accordance with EU regulation 36/2005 (19), the case was referred to the EU Reference Laboratory Strain Typing Expert Group, which recommended further investigation by bioassay.
 
Bioassay is conventionally undertaken by using unfixed tissues to prepare inocula. Much historical tissue is available only as formalin fixed or formalin fixed and paraffin wax embedded. TSE infectivity persists in such material but with a lower infectious titer than with unfixed frozen tissue (20). However, the potential effects on biological activity, and therefore strain characterization, of fixation and processing are unknown. Thus, further investigation of this case required an extensive panel of controls. We report the results of the bioassay analysis and confirm the diagnosis of BSE in a goat in the United Kingdom.
 
-- Communicated by: Terry S Singeltary Sr flounder9@verizon.net
 
[Interested readers should access the original text via the source URL above to view the full text an the references cited. The following has been extracted from the Discussion.
 
"The 2 cases of naturally occurring BSE in small ruminants, the one reported here and the one identified in France (15), occurred in different countries, during different time periods, and before strict BSE control measures were fully implemented. Therefore, the most likely origin of these 2 cases would be exposure to BSE-contaminated food supplements. Although in France goats constitute 14.3 percent of the small ruminant population, in the United Kingdom they account for only 0.3 percent of small ruminants. It is intriguing, therefore, that the only naturally occurring BSE cases in small ruminants in France and particularly in the United Kingdom were detected in goats and not in sheep, although they have also been exposed to contaminated food supplements. A possible explanation could be that goats are generally managed more intensively than sheep and thus might have been exposed to higher doses of the infectious agent because of the more frequent use of concentrates in intensive dairy farming. Similar observations have been reported in cattle, in which the incidence of BSE was significantly higher in dairy herds and in which management is much more intensive than in beef herds (34). In the United Kingdom, most of the commercial goat herds are kept for milk production in a typically intensive production system, similar to dairy cattle.
 
The BSE case we have confirmed was 1 of 26 historic goat samples examined in the United Kingdom collected during 1984-2002 (16,17). Since 1993, scrapie in goats has been a notifiable disease in the United Kingdom, and since 2005, samples from all suspected cases of TSE in small ruminants are required to be tested for BSE-like features by using Western blotting (WB) (19). No BSE cases have been identified, although an intermediate case in a goat was reported and is under investigation by bioassay for final resolution (35,36). This screening of brain samples from all small ruminant cases offers reassurance that BSE is not present in the contemporary small ruminant population. However, application of WB to sheep experimentally co-infected with BSE and scrapie detected only the scrapie agent (37). Also, in contrast to BSE, where infectivity is mainly confined to the nervous system, in small ruminants the BSE agent is widely distributed in peripheral tissues and can be transmitted horizontally (11,38). Therefore, feed ban measures alone would be inadequate to control a BSE outbreak in small ruminants. Also, it would be impossible to prevent BSE from entering the human food chain through consumption of food products derived from small ruminants.
 
Because TSEs in goats are still a problem, particularly in Mediterranean countries, our data suggest that extensive surveillance and breeding schemes must remain in place to prevent a BSE outbreak in small ruminants and to safeguard public health. This report also highlights several issues regarding the use of mouse bioassay to identify TSE strains. As governing bodies seek confirmation of equivocal cases that are identified worldwide, they must be aware of the limitations, cost, and timescale demands of confirming such cases." - Mod.CP]
 
[See http://healthmap.org/r/1lNY for the interactive HealthMap/ProMED map of the United Kingdom. - Mod.MPP]
 
******
 
snip...
 
******
 
[5]
 
Switzerland: BSE Date: Fri 16 Dec 2011 Source: Prionics AG, e-scope newsletter [edited] http://escope.prionics.com/issue/2011-december-4/
 
In spring 2011, 2 new cases of BSE were discovered in Switzerland [see ProMED-mail posting Prion disease update 2011 (10) 20111107.3317]. Both cases were detected using the Prionics(R)-Check BSE tests. A report has now been published showing that these cases represent a novel type of BSE. What are the consequences of these new BSE cases?
 
After a period of 4 years without BSE positive cows, in spring this year [2011] Switzerland was shaken by the discovery of 2 new BSE cases detected only one month apart from each other. The cases appeared in different areas of Switzerland and involved animals aged 8 and 15 years, which were tested with the Prionics(R)-Check BSE tests as part of the active disease surveillance program. Bettina Bernhard, Head of the Prionics diagnostic laboratory reported that: "It was the 1st time in 4.5 years that we had found a BSE positive sample in our laboratory. Based on the results from the Prionics(R)-Check WESTERN, we immediately saw that the fingerprint of the prion protein was not that of the classical BSE cases we have detected before. We then informed the Swiss National Reference Laboratory and veterinary authorities and the positive result was confirmed with the Prionics(R)-Check PrioSTRIP."
 
Novel type of BSE?
 
------------------
 
BSE cases that differ from the classical BSE strain have been detected before, however, with low incidence. These atypical strains, designated BASE/L-BSE and H-BSE, were first reported in 2004 in Italy and France. Both strains were detected as part of routine surveillance using the Prionics(R)-Check WESTERN and ELISA tests. The recent publication by Torsten Seuberlich of the Swiss National and OIE [World Organisation for Animal Health] Reference Laboratories for BSE and Scrapie and his colleagues, is showing that these 2 Swiss cases not only differ from classical BSE, but also from the atypical BSE cases found in other countries. It appears that the 2 BSE cases detected in Switzerland seem to represent a novel type of atypical BSE. Dr Seuberlich explains: "We are now undertaking further investigations into these 2 cases and until there is more clarity, surveillance should continue to be carried out at a high level and disease awareness should be increased. Furthermore, we have to ensure that diagnostic techniques are applied that identify such cases."
 
Continued vigilance needed
 
--------------------------
 
Whereas consumption of meat from cows affected by classical BSE has been associated with vCJD, the public health hazard from atypical BSE is unclear. Little is known about its origin and whether it can be transmitted to other animals. These cases show, however, that BSE has not been completely eradicated and that the disease can continue to occur even with current preventive measures (such as the meat-and-bone meal ban) in place. The appearance of new strains of the prion protein could also indicate that BSE is still evolving. Continuous monitoring will be needed to keep these new strains under surveillance.
 
-- Communicated by: Terry S Singeltary Sr flounder9@verizon.net
 
[[See http://healthmap.org/r/1AFv for the interactive HealthMap/ProMED map of Switzerland. - Mod.MPP]
 
 
Wednesday, January 18, 2012
 
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE February 1, 2012
 
 
Saturday, December 3, 2011
 
Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number
 
12—December 2011
 
 
Sunday, October 3, 2010
 
Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America, who's looking ?
 
 
Tuesday, February 01, 2011
 
Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie
 
Research article
 
 
Monday, March 21, 2011
 
Sheep and Goat BSE Propagate More Efficiently than Cattle BSE in Human PrP Transgenic Mice
 
 
 
*** Most recent positive goat confirmed in April 2013.
 
Scrapie Cases in Goats FY 2002 – FY 2013 As of April 30, 2013
 
***SCRAPIE GOATS CALIFORNIA 13 CASES TO DATE ! ***
 
(an unusually high amount of scrapie documented in goats for a happenstance of bad luck, or spontaneous event, THAT DOES NOT HAPPEN IN OTHER STATES ??? )
 
 
Thursday, November 18, 2010
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep
 
 
Thursday, March 29, 2012
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
 
 
 
*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles
 
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA
 
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.
 
Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
 
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.
 
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.
 
Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.
 
The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
 
 
Wednesday, March 28, 2012
 
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $
 
 
 
*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
 
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.
 
 
Thursday, March 29, 2012
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
 
 
Monday, April 25, 2011
 
Experimental Oral Transmission of Atypical Scrapie to Sheep
 
Volume 17, Number 5-May 2011 However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing finding that the biochemical properties of the resulting PrPSc have changed on transmission (40).
 
 
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
 
 
*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.
 
 
*** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
 
 
Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.
 
Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.
 
(i) the unsuspected potential abilities of atypical scrapie to cross species barriers
 
(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier
 
These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.
 
 
Friday, February 11, 2011
 
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
 
 
RESEARCH
 
Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011
 
Experimental Oral Transmission of Atypical Scrapie to Sheep
 
Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos
 
To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specifi c prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These fi ndings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.
 
SNIP...
 
Although we do not have epidemiologic evidence that supports the effi cient spread of disease in the fi eld, these data imply that disease is potentially transmissible under fi eld situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing fi nding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown.
 
How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantifi ed, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confi rmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.
 
Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011
 
 
Suspect symptoms
 
What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?
 
28 Mar 01
 
Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284. Subscribe and get 4 free issues. FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
 
Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.
 
Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.
 
Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.
 
As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.
 
"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.
 
But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.
 
People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.
 
But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."
 
There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.
 
Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.
 
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
(hmmm, this is getting interesting now...TSS)
 
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,
 
see also ;
 
All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
 
 
see full text ;
 
Monday, December 14, 2009
 
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types
 
 
 
why do we not want to do TSE transmission studies on chimpanzees $
 
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
 
snip...
 
R. BRADLEY
 
 
 
1: J Infect Dis 1980 Aug;142(2):205-8
 
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
 
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
 
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
 
snip...
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
 
PMID: 6997404
 
 
Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"
 
Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
 
snip...
 
76/10.12/4.6
 
 
Nature. 1972 Mar 10;236(5341):73-4.
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
 
Gibbs CJ Jr, Gajdusek DC.
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
 
C. J. GIBBS jun. & D. C. GAJDUSEK
 
National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
 
 
 
Wednesday, February 16, 2011
 
IN CONFIDENCE
 
SCRAPIE TRANSMISSION TO CHIMPANZEES
 
IN CONFIDENCE
 
 
 
 
TSS
 

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