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NCBA, R-CALF, COOL, USDA (No Politics!)
TAFS1 Position Paper on Atypical scrapie and Atypical BSE
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<blockquote data-quote="flounder" data-source="post: 405170" data-attributes="member: 3519"><p>Subject: TAFS1 Position Paper on Atypical scrapie and Atypical BSE</p><p>Date: July 9, 2007 at 1:38 pm PST</p><p></p><p>TAFS1 Position Paper on Atypical scrapie and Atypical BSE</p><p></p><p></p><p>TAFS</p><p></p><p>INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY</p><p></p><p>a non-profit Swiss Foundation</p><p></p><p>(May 16, 2007)</p><p></p><p>TAFS1 Position Paper on Atypical scrapie and Atypical BSE</p><p></p><p>In recent years there have been a small number of reports in the scientific</p><p>literature that</p><p>unusual isolates of BSE have been detected in cattle in various countries</p><p>around the world. In</p><p>addition, following the introduction of enhanced surveillance programmes for</p><p>scrapie in small</p><p>ruminants in Europe, unusual or unexpected results were also widely</p><p>reported. In both</p><p>instances, the shortage of scientific data at the time did not enable</p><p>scientists to precisely</p><p>identify what they were dealing with. Because of similarities with the</p><p>diseases that they were</p><p>searching for, namely BSE in cattle and scrapie in sheep, the immediate</p><p>response was to call</p><p>the isolates "atypical BSE" and "atypical scrapie" for reasons that will be</p><p>explained below.</p><p>Some additional local terminology was applied in some countries, but for the</p><p>moment the</p><p>term "atypical" is more commonly applied. This paper aims to provide the</p><p>background to</p><p>these findings, and explain their signficance.</p><p></p><p></p><p>snip...</p><p></p><p></p><p>Is it transmissible?</p><p></p><p>?? Experimentally, it has been shown that it can be transmitted to</p><p>genetically modified</p><p>mice(28), and by intracerebral inoculation to sheep (unpublished work in</p><p>progress).</p><p>?? These transmissions do not prove that it will transmit naturally from</p><p>sheep to sheep,</p><p>but studies involving oral infection of sheep are under way.</p><p>?? Although most atypical cases occur singly in flocks, there are some</p><p>instances where</p><p>two affected sheep have been identified in flocks. This may indicate that</p><p>natural</p><p>transmission may occur, or that the sheep were infected from a common</p><p>alternative</p><p>source(22, 29). Possible indications of an association with the feeding of</p><p>vitamins and</p><p>mineral feed supplements were detected in Norway, but remain to be</p><p>proven(22).</p><p></p><p>Does it represent a risk to human health?</p><p></p><p>Does it represent a risk to human health?</p><p></p><p>?? This is currently unknown, but if atypical scrapie is not a new</p><p>phenomenon, and has</p><p>simply been discovered recently, then the lack of epidemiological</p><p>association between</p><p>TAFS</p><p>6</p><p>prion diseases in humans and sheep, or consumption of sheep products,</p><p>suggest that</p><p>atypical scrapie does not represent a risk to humans. This is not however</p><p>demonstration of absolute safety.</p><p>?? This is currently unknown, but if atypical scrapie is not a new</p><p>phenomenon, and has</p><p>simply been discovered recently, then the lack of epidemiological</p><p>association between</p><p>TAFS</p><p>6</p><p>prion diseases in humans and sheep, or consumption of sheep products,</p><p>suggest that</p><p>atypical scrapie does not represent a risk to humans. This is not however</p><p>demonstration of absolute safety.</p><p></p><p></p><p>======================================================</p><p></p><p></p><p>PLEASE NOTE ;</p><p></p><p></p><p>EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE</p><p></p><p>This is provided by the statistically significant increase in the incidence</p><p>of sheep scrape from 1985, as determined from analyses of the submissions</p><p>made to VI Centres, and from individual case and flock incident studies.</p><p>........</p><p></p><p></p><p><a href="http://www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf" target="_blank">http://www.bseinquiry.gov.uk/files/yb/1 ... 002001.pdf</a></p><p></p><p></p><p>1: J Infect Dis 1980 Aug;142(2):205-8</p><p></p><p></p><p></p><p>Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to</p><p>nonhuman primates.</p><p></p><p>Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.</p><p></p><p>Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep</p><p>and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were</p><p>exposed to the infectious agents only by their nonforced consumption of</p><p>known infectious tissues. The asymptomatic incubation period in the one</p><p>monkey exposed to the virus of kuru was 36 months; that in the two monkeys</p><p>exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months,</p><p>respectively; and that in the two monkeys exposed to the virus of scrapie</p><p>was 25 and 32 months, respectively. Careful physical examination of the</p><p>buccal cavities of all of the monkeys failed to reveal signs or oral</p><p>lesions. One additional monkey similarly exposed to kuru has remained</p><p>asymptomatic during the 39 months that it has been under observation.</p><p></p><p>PMID: 6997404</p><p><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Abstract</a></p><p></p><p></p><p>=====================================================</p><p></p><p></p><p>snip...</p><p></p><p></p><p>Where has atypical BSE been found?</p><p></p><p>?? Although the greatest number of cases is in France(12), increasing</p><p>numbers of cases</p><p>have now been identified in other countries – Canada (1), Germany (2), Italy</p><p>(2),</p><p>Japan (2), Netherlands (4), Poland (7), Sweden (1), Switzerland (1), UK (1),</p><p>and USA</p><p>(2). In Sweden and the USA the atypical cases represent the only indigenous</p><p>cases</p><p>detected. In other words – typical BSE has not been detected in native</p><p>cattle in these</p><p>two countries (34).</p><p>?? In France, Poland, Netherlands and Germany both H and L forms of atypical</p><p>BSE</p><p>have been reported(25).</p><p></p><p>Is there anything else unusual about the cases?</p><p></p><p>?? Yes. With the exception of the first Japanese case, others have generally</p><p>occurred in</p><p>old cows – 8 to 18 years reported in France, 11 and 15 in Italy. Most BSE</p><p>cases occur</p><p>in animals between the age of four and six, although very young and very old</p><p>animals</p><p>can be affected too.</p><p>?? In one case, still unpublished, a mutation of the PrP gene has been</p><p>detected, similar to</p><p>one found in one form of CJD in humans.It has to be stressed that this has</p><p>not been</p><p>identified in every case of atypical BSE, although not all have been</p><p>analysed in this</p><p>way.</p><p></p><p></p><p>snip...</p><p></p><p></p><p>Where has atypical BSE been found?</p><p></p><p>?? Although the greatest number of cases is in France(12), increasing</p><p>numbers of cases</p><p>have now been identified in other countries – Canada (1), Germany (2), Italy</p><p>(2),</p><p>Japan (2), Netherlands (4), Poland (7), Sweden (1), Switzerland (1), UK (1),</p><p>and USA</p><p>(2). In Sweden and the USA the atypical cases represent the only indigenous</p><p>cases</p><p>detected. In other words – typical BSE has not been detected in native</p><p>cattle in these</p><p>two countries (34).</p><p>?? In France, Poland, Netherlands and Germany both H and L forms of atypical</p><p>BSE</p><p>have been reported(25).</p><p></p><p>Is there anything else unusual about the cases?</p><p></p><p>?? Yes. With the exception of the first Japanese case, others have generally</p><p>occurred in</p><p>old cows – 8 to 18 years reported in France, 11 and 15 in Italy. Most BSE</p><p>cases occur</p><p>in animals between the age of four and six, although very young and very old</p><p>animals</p><p>can be affected too.</p><p>?? In one case, still unpublished, a mutation of the PrP gene has been</p><p>detected, similar to</p><p>one found in one form of CJD in humans.It has to be stressed that this has</p><p>not been</p><p>identified in every case of atypical BSE, although not all have been</p><p>analysed in this</p><p>way.</p><p></p><p>TAFS</p><p>9</p><p></p><p>Is there more than one strain of atypical BSE?</p><p></p><p>?? At this stage it is too early to say, but there are early indications</p><p>that this may be so.</p><p>Caution is needed because there is a need to be certain that the variations</p><p>in results are</p><p>not artifacts, either generated by differences in test methods between</p><p>countries, or due</p><p>to degradation of samples before they are tested. This has been shown to</p><p>generate</p><p>variations in blotting patterns, but is unlikely to have produced the</p><p>extensive</p><p>variations seen in the Italian cases or the H form detected in France and</p><p>elsewhere.</p><p>?? So the key to confirming whether or not H and L isolates actually</p><p>represent different</p><p>strains will be further characterization following transmission to</p><p>laboratory rodents</p><p>and/or cattle. These are the methods normally used to characterize prion</p><p>strains</p><p>comprehensively.</p><p>?? This will also help to confirm the extent to which the atypical BSE cases</p><p>differ from</p><p>BSE. In the meantime, especially if it proves possible to transmit isolates</p><p>to other</p><p>animals, additional biochemical methods can be used to investigate other</p><p>aspects of</p><p>prion protein biology of the different isolates.</p><p>?? Two publications have already highlighted the difficulties of</p><p>interpreting data on</p><p>biological transmissibility. One demonstrates that BSE and "H-type" BSE are</p><p>different, based upon their behaviour in genetically modified mice,</p><p>examination of</p><p>fixed and unfixed brain tissue, and comparison of incubation periods(6). The</p><p>other,</p><p>studying "L-type" BSE (Italian BASE), and using different mouse models,</p><p>acknowledges apparent differences between it and BSE when first inoculated</p><p>into</p><p>mice, but claims that further transmission from mouse to mouse by</p><p>inoculation</p><p>produces a strain indistinguishable from BSE (by the limited criteria used</p><p>in the study)</p><p>(11).</p><p>?? These findings suggest that it may prove possible to understand the</p><p>relationship</p><p>between BSE and atypical BSE isolates, and between the criteria used to</p><p>classify them</p><p>at present, and the actual strain of prion that infects the animal.</p><p></p><p>Is atypical BSE transmissible?</p><p></p><p>?? Investigations are under way in France, Italy, Germany and Japan.</p><p>Experimental</p><p>transmissibility to cattle and primates has now been demonstrated for L-type</p><p>BSE, and</p><p>to mice for both H and L types (3, 6, 11). Some of this work remains</p><p>incomplete and</p><p>unpublished at the time of writing.</p><p>?? This does not prove that atypical BSE transmits from animal to animal</p><p>naturally.</p><p>Does it represent a risk to human health?</p><p>?? It is too early to tell whether or not it represents a risk to humans.</p><p>For the moment it is</p><p>assumed to be a danger, and is treated like BSE. Results of experimental</p><p>transmission</p><p>to primates remain unpublished. Some scientists suggest that similarities</p><p>between the</p><p>molecular features of H-type BSE and some prion diseases of humans may</p><p>indicate</p><p>that they are related. Care must be exercised in interpreting such</p><p>preliminary data(8)</p><p>specifically with regard to suggestions of a cause and effect.</p><p>?? Transmissibility to cattle has been confirmed, but remains currently</p><p>unpublished as the</p><p>study is incomplete. It may therefore be possible to investigate further, by</p><p>oral</p><p>challenge, whether or not the infectious agent is distributed around the</p><p>body in a</p><p>different way from BSE, possibly infecting tissues that are not</p><p>considered-infectious</p><p>in BSE. This may have implications for risk management and public health.</p><p>?? It is however important to remember that so far only small numbers of</p><p>atypical BSE</p><p>cases have been detected compared to the many thousands of BSE cases</p><p>TAFS</p><p>10</p><p></p><p>?? Depending on how atypical BSE cases arise, they may represent a long term</p><p>problem</p><p>when BSE has been eradicated, or they may disappear along with BSE because</p><p>the</p><p>controls are equally effective in preventing spread of infection. As</p><p>transmissibility to</p><p>cattle has now been partially demonstrated, it can be presumed that atypical</p><p>BSE may</p><p>transmit to cattle orally through feed, in which case rendering and feed</p><p>controls should</p><p>prevent further transmission by that route.</p><p></p><p>What is the impact of this finding on measures to control BSE, and to</p><p>protect</p><p>consumers?</p><p></p><p>?? At the moment all measures in place to protect cattle and humans from</p><p>becoming</p><p>infected with BSE are considered adequate to protect against atypical BSE.</p><p>Tests used</p><p>to detect BSE in cattle have detected the atypical cases too, and on brain</p><p>tissue, which</p><p>is already defined as SRM in countries where controls are in place.</p><p>?? Care will be needed in relaxing such controls, especially if atypical BSE</p><p>proves to be</p><p>transmissible directly between cattle, or to humans via tissues that are not</p><p>currently</p><p>defined as SRM.</p><p>?? Similarly, if atypical BSE is demonstrated to arise spontaneously, rare</p><p>sporadic cases</p><p>may be expected to occur in all countries with significant cattle</p><p>populations. This in</p><p>itself will challenge expectations of total eradication as a result of</p><p>controls.</p><p>Nevertheless, the existence of sporadic cases would indicate potential to</p><p>give rise to</p><p>further large epidemics if some protective measures are not maintained</p><p>indefinitely.</p><p>These may involve prohibitions on the use of certain proteins in animal</p><p>feed, more</p><p>rigorous rendering processes, and possibly continued removal and destruction</p><p>of</p><p>certain SRM from human food and animal feed chains.</p><p></p><p></p><p>snip...</p><p></p><p></p><p></p><p><a href="http://www.tseandfoodsafety.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf" target="_blank">http://www.tseandfoodsafety.org/positio ... 070516.pdf</a></p><p></p><p></p><p>PLEASE NOTE;</p><p></p><p></p><p>USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN</p><p></p><p>18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7</p><p>December 2006 are now available.</p><p></p><p></p><p>snip...</p><p></p><p></p><p>64. A member noted that at the recent Neuroprion meeting, a study was</p><p>presented showing that in transgenic mice BSE passaged in sheep may be more</p><p>virulent and infectious to a wider range of species than bovine derived BSE.</p><p></p><p>Other work presented suggested that BSE and bovine amyloidotic spongiform</p><p>encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the</p><p>prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A</p><p>MUTATION FOUND IN CASES OF SPORADIC CJD.</p><p></p><p></p><p>snip...</p><p></p><p></p><p><a href="http://www.seac.gov.uk/minutes/95.pdf" target="_blank">http://www.seac.gov.uk/minutes/95.pdf</a></p><p></p><p></p><p></p><p>3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse</p><p></p><p>Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western</p><p>Reserve</p><p>University</p><p></p><p>Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain</p><p>discovered recently in Italy, and similar or different atypical BSE cases</p><p>were also reported in other countries. The infectivity and phenotypes of</p><p>these atypical BSE strains in humans are unknown. In collaboration with</p><p>Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have</p><p>inoculated transgenic mice expressing human prion protein with brain</p><p>homogenates from BASE or BSE infected cattle. Our data shows that about half</p><p>of the BASE-inoculated mice became infected with an average incubation time</p><p>of about 19 months; in contrast, none of the BSE-inoculated mice appear to</p><p>be infected after more than 2 years.</p><p></p><p>***These results indicate that BASE is transmissible to humans and suggest</p><p>that BASE is more virulent than</p><p>classical BSE in humans.***</p><p></p><p></p><p>6:30 Close of Day One</p><p></p><p></p><p><a href="http://www.healthtech.com/2007/tse/day1.asp" target="_blank">http://www.healthtech.com/2007/tse/day1.asp</a></p><p></p><p></p><p></p><p>SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM</p><p>1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype</p><p>of 'UNKNOWN' strain growing. ...</p><p></p><p></p><p><a href="http://www.cjdsurveillance.com/resources-casereport.html" target="_blank">http://www.cjdsurveillance.com/resource ... eport.html</a></p><p></p><p></p><p>There is a growing number of human CJD cases, and they were presented last</p><p>week in San Francisco by Luigi Gambatti(?) from his CJD surveillance</p><p>collection.</p><p></p><p>He estimates that it may be up to 14 or 15 persons which display selectively</p><p>SPRPSC and practically no detected RPRPSC proteins.</p><p></p><p></p><p><a href="http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm" target="_blank">http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.htm</a></p><p></p><p></p><p><a href="http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf" target="_blank">http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.pdf</a></p><p></p><p></p><p>Diagnosis and Reporting of Creutzfeldt-Jakob Disease</p><p></p><p>Singeltary, Sr et al. JAMA.2001; 285: 733-734.</p><p></p><p><a href="http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535" target="_blank">http://jama.ama-assn.org/http://www.neu ... /2/176#535</a></p><p></p><p></p><p>BRITISH MEDICAL JOURNAL</p><p></p><p>BMJ</p><p></p><p><a href="http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406" target="_blank">http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406</a></p><p></p><p></p><p>BMJ</p><p></p><p><a href="http://www.bmj.com/cgi/eletters/320/7226/8/b#6117" target="_blank">http://www.bmj.com/cgi/eletters/320/7226/8/b#6117</a></p><p></p><p></p><p>JOURNAL OF NEUROLOGY</p><p></p><p>MARCH 26, 2003</p><p></p><p>RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob</p><p></p><p>disease in the United States</p><p></p><p>Email Terry S. Singeltary:</p><p></p><p><a href="mailto:flounder@wt.net">flounder@wt.net</a></p><p></p><p>I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to</p><p></p><p>comment on the CDC's attempts to monitor the occurrence of emerging</p><p></p><p>forms of CJD. Asante, Collinge et al [1] have reported that BSE</p><p></p><p>transmission to the 129-methionine genotype can lead to an alternate</p><p></p><p>phenotype that is indistinguishable from type 2 PrPSc, the commonest</p><p></p><p>sporadic CJD. However, CJD and all human TSEs are not reportable</p><p></p><p>nationally. CJD and all human TSEs must be made reportable in every</p><p></p><p>state and internationally. I hope that the CDC does not continue to</p><p></p><p>expect us to still believe that the 85%+ of all CJD cases which are</p><p></p><p>sporadic are all spontaneous, without route/source. We have many TSEs in</p><p></p><p>the USA in both animal and man. CWD in deer/elk is spreading rapidly and</p><p></p><p>CWD does transmit to mink, ferret, cattle, and squirrel monkey by</p><p></p><p>intracerebral inoculation. With the known incubation periods in other</p><p></p><p>TSEs, oral transmission studies of CWD may take much longer. Every</p><p></p><p>victim/family of CJD/TSEs should be asked about route and source of this</p><p></p><p>agent. To prolong this will only spread the agent and needlessly expose</p><p></p><p>others. In light of the findings of Asante and Collinge et al, there</p><p></p><p>should be drastic measures to safeguard the medical and surgical arena</p><p></p><p>from sporadic CJDs and all human TSEs. I only ponder how many sporadic</p><p></p><p>CJDs in the USA are type 2 PrPSc?</p><p></p><p><a href="http://www.neurology.org/cgi/eletters/60/2/176#535" target="_blank">http://www.neurology.org/cgi/eletters/60/2/176#535</a></p><p></p><p></p><p>doi:10.1016/S1473-3099(03)00715-1</p><p>Copyright © 2003 Published by Elsevier Ltd.</p><p>Newsdesk</p><p></p><p>Tracking spongiform encephalopathies in North America</p><p></p><p>Xavier Bosch</p><p></p><p>Available online 29 July 2003.</p><p></p><p></p><p>Volume 3, Issue 8, August 2003, Page 463</p><p></p><p></p><p>"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my</p><p>mom to hvCJD (Heidenhain variant CJD)</p><p>and have been searching for answers ever since. What I have found is that we</p><p>have not been told the truth. CWD</p><p>in deer and elk is a small portion of a much bigger problem."</p><p>............................</p><p></p><p></p><p><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" target="_blank">http://www.thelancet.com/journals/lanin ... 1/fulltext</a></p><p></p><p><a href="http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf" target="_blank">http://download.thelancet.com/pdfs/jour ... 007151.pdf</a></p><p></p><p></p><p>see history of cjd questionnaire</p><p></p><p><a href="http://brain.hastypastry.net/forums/showthread.php?t=2408" target="_blank">http://brain.hastypastry.net/forums/sho ... php?t=2408</a></p><p></p><p></p><p>sporadic CJD, the big lie ;</p><p></p><p></p><p>see full text ;</p><p></p><p></p><p><a href="http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276" target="_blank">http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... =0&P=25276</a></p><p></p><p></p><p>hmmmm, spontaneous TSE i.e. scrapie ???</p><p></p><p>NOT!</p><p></p><p>LOOK at OHIO and tell me if scrapie happens spontaneously, why in the hell is OHIO's rate of scrapie always some 20 times that of any other state ???</p><p></p><p></p><p>Subject: MAY SCRAPIE REPORT 2007 (OHIO still topping the list, what about</p><p>CJD?)</p><p>Date: July 9, 2007 at 12:30 pm PST</p><p></p><p>MAY SCRAPIE REPORT 2007 (OHIO still topping the list, what about CJD?)</p><p></p><p></p><p>OHIO has had a serious problem with scrapie for YEARS!</p><p></p><p>DOES anyone have any ideas as to why, IF TSE occur spontaneously (as some</p><p>would wish us to believe),</p><p>why they spontaneously happen more often in OHIO than any other state, in</p><p>regards to scrapie ???</p><p></p><p></p><p>what is the reason for this phenonimum $</p><p></p><p></p><p>MAY SCRAPIE REPORT 2007</p><p></p><p><a href="http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps" target="_blank">http://www.aphis.usda.gov/animal_health ... ie_rpt.pps</a></p><p></p><p></p><p>NOR98-LIKE STRAIN OF SCRAPIE FOUND IN WYOMING</p><p>From: Terry S. Singeltary Sr. <[log in to unmask]> </p><p>Date: Wed, 11 Apr 2007 15:08:15 -0500 </p><p> </p><p> </p><p> </p><p><a href="http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=8315" target="_blank">http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... T=0&P=8315</a></p><p></p><p></p><p></p><p>TSS</p></blockquote><p></p>
[QUOTE="flounder, post: 405170, member: 3519"] Subject: TAFS1 Position Paper on Atypical scrapie and Atypical BSE Date: July 9, 2007 at 1:38 pm PST TAFS1 Position Paper on Atypical scrapie and Atypical BSE TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation (May 16, 2007) TAFS1 Position Paper on Atypical scrapie and Atypical BSE In recent years there have been a small number of reports in the scientific literature that unusual isolates of BSE have been detected in cattle in various countries around the world. In addition, following the introduction of enhanced surveillance programmes for scrapie in small ruminants in Europe, unusual or unexpected results were also widely reported. In both instances, the shortage of scientific data at the time did not enable scientists to precisely identify what they were dealing with. Because of similarities with the diseases that they were searching for, namely BSE in cattle and scrapie in sheep, the immediate response was to call the isolates “atypical BSE” and “atypical scrapie” for reasons that will be explained below. Some additional local terminology was applied in some countries, but for the moment the term “atypical” is more commonly applied. This paper aims to provide the background to these findings, and explain their signficance. snip... Is it transmissible? ?? Experimentally, it has been shown that it can be transmitted to genetically modified mice(28), and by intracerebral inoculation to sheep (unpublished work in progress). ?? These transmissions do not prove that it will transmit naturally from sheep to sheep, but studies involving oral infection of sheep are under way. ?? Although most atypical cases occur singly in flocks, there are some instances where two affected sheep have been identified in flocks. This may indicate that natural transmission may occur, or that the sheep were infected from a common alternative source(22, 29). Possible indications of an association with the feeding of vitamins and mineral feed supplements were detected in Norway, but remain to be proven(22). Does it represent a risk to human health? Does it represent a risk to human health? ?? This is currently unknown, but if atypical scrapie is not a new phenomenon, and has simply been discovered recently, then the lack of epidemiological association between TAFS 6 prion diseases in humans and sheep, or consumption of sheep products, suggest that atypical scrapie does not represent a risk to humans. This is not however demonstration of absolute safety. ?? This is currently unknown, but if atypical scrapie is not a new phenomenon, and has simply been discovered recently, then the lack of epidemiological association between TAFS 6 prion diseases in humans and sheep, or consumption of sheep products, suggest that atypical scrapie does not represent a risk to humans. This is not however demonstration of absolute safety. ====================================================== PLEASE NOTE ; EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........ [url=http://www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf]http://www.bseinquiry.gov.uk/files/yb/1 ... 002001.pdf[/url] 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. PMID: 6997404 [url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract]http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Abstract[/url] ===================================================== snip... Where has atypical BSE been found? ?? Although the greatest number of cases is in France(12), increasing numbers of cases have now been identified in other countries – Canada (1), Germany (2), Italy (2), Japan (2), Netherlands (4), Poland (7), Sweden (1), Switzerland (1), UK (1), and USA (2). In Sweden and the USA the atypical cases represent the only indigenous cases detected. In other words – typical BSE has not been detected in native cattle in these two countries (34). ?? In France, Poland, Netherlands and Germany both H and L forms of atypical BSE have been reported(25). Is there anything else unusual about the cases? ?? Yes. With the exception of the first Japanese case, others have generally occurred in old cows – 8 to 18 years reported in France, 11 and 15 in Italy. Most BSE cases occur in animals between the age of four and six, although very young and very old animals can be affected too. ?? In one case, still unpublished, a mutation of the PrP gene has been detected, similar to one found in one form of CJD in humans.It has to be stressed that this has not been identified in every case of atypical BSE, although not all have been analysed in this way. snip... Where has atypical BSE been found? ?? Although the greatest number of cases is in France(12), increasing numbers of cases have now been identified in other countries – Canada (1), Germany (2), Italy (2), Japan (2), Netherlands (4), Poland (7), Sweden (1), Switzerland (1), UK (1), and USA (2). In Sweden and the USA the atypical cases represent the only indigenous cases detected. In other words – typical BSE has not been detected in native cattle in these two countries (34). ?? In France, Poland, Netherlands and Germany both H and L forms of atypical BSE have been reported(25). Is there anything else unusual about the cases? ?? Yes. With the exception of the first Japanese case, others have generally occurred in old cows – 8 to 18 years reported in France, 11 and 15 in Italy. Most BSE cases occur in animals between the age of four and six, although very young and very old animals can be affected too. ?? In one case, still unpublished, a mutation of the PrP gene has been detected, similar to one found in one form of CJD in humans.It has to be stressed that this has not been identified in every case of atypical BSE, although not all have been analysed in this way. TAFS 9 Is there more than one strain of atypical BSE? ?? At this stage it is too early to say, but there are early indications that this may be so. Caution is needed because there is a need to be certain that the variations in results are not artifacts, either generated by differences in test methods between countries, or due to degradation of samples before they are tested. This has been shown to generate variations in blotting patterns, but is unlikely to have produced the extensive variations seen in the Italian cases or the H form detected in France and elsewhere. ?? So the key to confirming whether or not H and L isolates actually represent different strains will be further characterization following transmission to laboratory rodents and/or cattle. These are the methods normally used to characterize prion strains comprehensively. ?? This will also help to confirm the extent to which the atypical BSE cases differ from BSE. In the meantime, especially if it proves possible to transmit isolates to other animals, additional biochemical methods can be used to investigate other aspects of prion protein biology of the different isolates. ?? Two publications have already highlighted the difficulties of interpreting data on biological transmissibility. One demonstrates that BSE and “H-type” BSE are different, based upon their behaviour in genetically modified mice, examination of fixed and unfixed brain tissue, and comparison of incubation periods(6). The other, studying “L-type” BSE (Italian BASE), and using different mouse models, acknowledges apparent differences between it and BSE when first inoculated into mice, but claims that further transmission from mouse to mouse by inoculation produces a strain indistinguishable from BSE (by the limited criteria used in the study) (11). ?? These findings suggest that it may prove possible to understand the relationship between BSE and atypical BSE isolates, and between the criteria used to classify them at present, and the actual strain of prion that infects the animal. Is atypical BSE transmissible? ?? Investigations are under way in France, Italy, Germany and Japan. Experimental transmissibility to cattle and primates has now been demonstrated for L-type BSE, and to mice for both H and L types (3, 6, 11). Some of this work remains incomplete and unpublished at the time of writing. ?? This does not prove that atypical BSE transmits from animal to animal naturally. Does it represent a risk to human health? ?? It is too early to tell whether or not it represents a risk to humans. For the moment it is assumed to be a danger, and is treated like BSE. Results of experimental transmission to primates remain unpublished. Some scientists suggest that similarities between the molecular features of H-type BSE and some prion diseases of humans may indicate that they are related. Care must be exercised in interpreting such preliminary data(8) specifically with regard to suggestions of a cause and effect. ?? Transmissibility to cattle has been confirmed, but remains currently unpublished as the study is incomplete. It may therefore be possible to investigate further, by oral challenge, whether or not the infectious agent is distributed around the body in a different way from BSE, possibly infecting tissues that are not considered-infectious in BSE. This may have implications for risk management and public health. ?? It is however important to remember that so far only small numbers of atypical BSE cases have been detected compared to the many thousands of BSE cases TAFS 10 ?? Depending on how atypical BSE cases arise, they may represent a long term problem when BSE has been eradicated, or they may disappear along with BSE because the controls are equally effective in preventing spread of infection. As transmissibility to cattle has now been partially demonstrated, it can be presumed that atypical BSE may transmit to cattle orally through feed, in which case rendering and feed controls should prevent further transmission by that route. What is the impact of this finding on measures to control BSE, and to protect consumers? ?? At the moment all measures in place to protect cattle and humans from becoming infected with BSE are considered adequate to protect against atypical BSE. Tests used to detect BSE in cattle have detected the atypical cases too, and on brain tissue, which is already defined as SRM in countries where controls are in place. ?? Care will be needed in relaxing such controls, especially if atypical BSE proves to be transmissible directly between cattle, or to humans via tissues that are not currently defined as SRM. ?? Similarly, if atypical BSE is demonstrated to arise spontaneously, rare sporadic cases may be expected to occur in all countries with significant cattle populations. This in itself will challenge expectations of total eradication as a result of controls. Nevertheless, the existence of sporadic cases would indicate potential to give rise to further large epidemics if some protective measures are not maintained indefinitely. These may involve prohibitions on the use of certain proteins in animal feed, more rigorous rendering processes, and possibly continued removal and destruction of certain SRM from human food and animal feed chains. snip... [url=http://www.tseandfoodsafety.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf]http://www.tseandfoodsafety.org/positio ... 070516.pdf[/url] PLEASE NOTE; USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN 18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available. snip... 64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE. Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD. snip... [url=http://www.seac.gov.uk/minutes/95.pdf]http://www.seac.gov.uk/minutes/95.pdf[/url] 3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. ***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.*** 6:30 Close of Day One [url=http://www.healthtech.com/2007/tse/day1.asp]http://www.healthtech.com/2007/tse/day1.asp[/url] SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ... [url=http://www.cjdsurveillance.com/resources-casereport.html]http://www.cjdsurveillance.com/resource ... eport.html[/url] There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection. He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins. [url=http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm]http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.htm[/url] [url=http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf]http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.pdf[/url] Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. [url=http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535]http://jama.ama-assn.org/http://www.neu ... /2/176#535[/url] BRITISH MEDICAL JOURNAL BMJ [url=http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406]http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406[/url] BMJ [url=http://www.bmj.com/cgi/eletters/320/7226/8/b#6117]http://www.bmj.com/cgi/eletters/320/7226/8/b#6117[/url] JOURNAL OF NEUROLOGY MARCH 26, 2003 RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Email Terry S. Singeltary: [email=flounder@wt.net]flounder@wt.net[/email] I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc? [url=http://www.neurology.org/cgi/eletters/60/2/176#535]http://www.neurology.org/cgi/eletters/60/2/176#535[/url] doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk Tracking spongiform encephalopathies in North America Xavier Bosch Available online 29 July 2003. Volume 3, Issue 8, August 2003, Page 463 “My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.” ............................ [url=http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext]http://www.thelancet.com/journals/lanin ... 1/fulltext[/url] [url=http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf]http://download.thelancet.com/pdfs/jour ... 007151.pdf[/url] see history of cjd questionnaire [url=http://brain.hastypastry.net/forums/showthread.php?t=2408]http://brain.hastypastry.net/forums/sho ... php?t=2408[/url] sporadic CJD, the big lie ; see full text ; [url=http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276]http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... =0&P=25276[/url] hmmmm, spontaneous TSE i.e. scrapie ??? NOT! LOOK at OHIO and tell me if scrapie happens spontaneously, why in the hell is OHIO's rate of scrapie always some 20 times that of any other state ??? Subject: MAY SCRAPIE REPORT 2007 (OHIO still topping the list, what about CJD?) Date: July 9, 2007 at 12:30 pm PST MAY SCRAPIE REPORT 2007 (OHIO still topping the list, what about CJD?) OHIO has had a serious problem with scrapie for YEARS! DOES anyone have any ideas as to why, IF TSE occur spontaneously (as some would wish us to believe), why they spontaneously happen more often in OHIO than any other state, in regards to scrapie ??? what is the reason for this phenonimum $ MAY SCRAPIE REPORT 2007 [url=http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps]http://www.aphis.usda.gov/animal_health ... ie_rpt.pps[/url] NOR98-LIKE STRAIN OF SCRAPIE FOUND IN WYOMING From: Terry S. Singeltary Sr. <[log in to unmask]> Date: Wed, 11 Apr 2007 15:08:15 -0500 [url=http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=8315]http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... T=0&P=8315[/url] TSS [/QUOTE]
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TAFS1 Position Paper on Atypical scrapie and Atypical BSE
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