Sunday, August 28, 2016

Transmissible Spongiform Encephalopathy TSE Prion and how Politics and Greed by the Industry spread madcow type diseases from species to species and around the globe

Transmissible Spongiform Encephalopathy TSE Prion and how Politics and Greed by the Industry spread madcow type diseases from species to species and around the globe
 
TSE PRIONS AKA MAD COW TYPE DISEASE, LIONS AND TIGERS AND BEARS, OH MY!
 
Please be assured, the USA does NOT have any clue as to what the real perspective on the TSE prion disease in domestic feline and canine, much less our big wild cats, OR any other species including humans for that matter, but one thing for sure, the studies and history of the mad cow debacle below are deeply concerning with regards, to humans and wild big cats like mountain lions, cougars, lynx, Jaguar, and such, that feed on cervids that are infected with CWD. one thing for sure, don’t kid yourselves, all are very much susceptible to the TSE Prion disease, and if you don’t look, you don’t find, problems solved$$$
 
are we as humans, not only witnessing the 6th extinction, but more importantly, are we as humans the cause?
 
I would say yes to both...imo.
 
and please, before going any further, please remember this ;
 
2015
 
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
 
 
 
Subject: The species barriers and public health threat of CWD and BSE prions
 
WDA 2016 NEW YORK
 
We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions.
 
Student Presentations Session 2
 
The species barriers and public health threat of CWD and BSE prions
 
Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr. Edward Hoover1 1Colorado State University
 
Chronic wasting disease (CWD) is spreading rapidly through cervid populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease) arose in the 1980s because cattle were fed recycled animal protein. These and other prion diseases are caused by abnormal folding of the normal prion protein (PrP) into a disease causing form (PrPd), which is pathogenic to nervous system cells and can cause subsequent PrP to misfold. CWD spreads among cervids very efficiently, but it has not yet infected humans. On the other hand, BSE was spread only when cattle consumed infected bovine or ovine tissue, but did infect humans and other species. The objective of this research is to understand the role of PrP structure in cross-species infection by CWD and BSE. To study the propensity of each species’ PrP to be induced to misfold by the presence of PrPd from verious species, we have used an in vitro system that permits detection of PrPd in real-time. We measured the conversion efficiency of various combinations of PrPd seeds and PrP substrate combinations. We observed the cross-species behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD.
 
Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders
 
Ms. Alyssa Wetterau1, Dr. Krysten Schuler1, Dr. Elizabeth Bunting1, Dr. Hussni Mohammed1 1Cornell University
 
Chronic wasting disease (CWD) is a fatal disease of North American Cervidae. New York State (NYS, USA) successfully managed an outbreak of CWD in 2005 in both captive and wild white-tailed deer (Odocoileus virginianus) with no reoccurrence of the disease as of 2015. To attain maximum compliance and efficacy of management actions for prevention of CWD entry, understanding the varied risk perceptions will allow for targeted, proactive communication efforts to address divergences between expert-derived risk assessments and stakeholder risk perceptions. We examined perceived risks associated with CWD introduction and exposure among agricultural and wildlife agency professionals within and outside of NYS, as well as stakeholder groups (e.g., hunters and captive cervid owners). We measured perceived risk using a risk assessment questionnaire online via Qualtrics survey software and evaluated similarities within, as well as differences in, perception among participant groups. New York State biologists employed by the Department of Environmental Conservation and independent non-NYS wildlife and agricultural professionals thought CWD risks associated with captive cervids were high; captive cervid owners thought risks for wild and captive cervids were low. Agriculture and wildlife professional groups agreed on general risk perceptions. We ranked 15 individual risk hazards into high and low medium categories based on all responses. Differences between groups were most evident in hypothetical disease pathways. Any pathway involving inter-state import of live cervids received high ranking for all groups except captive cervid owners. Comparatively low risk perceptions by captive cervid operators may stem from misinformation, lack of understanding of testing programs, and indemnity payments for animal depopulation. Communication and education directed at areas of disagreement may facilitate effective disease prevention and management.
 
 
*** We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. *** We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD. ***
 
72 | Detection of Prions on Plants Collected from Rocky Mountain National Park
 
Aimee Ortega, Jan Leach, and Mark Zabel
 
Chronic Wasting Disease (CWD) affects cervids such as elk, deer, and moose and since its discovery in 1967 has become endemic in certain areas. Prevalence in captive herds have reached as high as 90%, and by measuring a large herd within Rocky Mountain National Park (ROMO) we have found that most recent estimates reach up to 19%. CWD is one of many transmissible spongiform encephalopathies which occur due to the accumulation of an abnormally folded, proteinase K resistant, form of the normal cellular prion protein PrPC. This abnormally folded form, PrPCWD, seeds conversion of PrPC into PrPCWD and eventually forms amyloid fibrils. Spread of CWD occurs through horizontal, vertical, and indirect/environmental routes. PrPCWD has been found in both soil and water. Additionally, PrPCWD is very resistant to degradation which makes it stable in the environment for long periods of time. A study has shown that the abnormal prion protein can remain viable in the environment for as long as 16 years. Wanting to explore environmental transmission of CWD we surveyed three sites within Rocky Mountain National Park and collected a total of 32 plants. Plants were collected from both outside and inside exclosures that serve to keep wildlife out and allow for restoration and regrowth of the flora. Plant samples were assayed via the Protein Misfolding Cyclic Amplification assay for detection of PrPCWD. Here we show novel evidence of PrPCWD on the surface of a number of plants collected.
 
Graduate Student/ Microbiology, Immunology and Pathology
 
wasting disease (CWD) is the transmissible spongiform encephalopathy (TSE), or prion disease of free-ranging and captive cervids (deer, elk and moose). CWD was first reported in a captive mule deer herd in 1967. Clinical signs include weight loss, polydipsia, polyphagia and gait impairment. CWD is recognized as the most readily transmitted TSE and since its discovery has been detected in cervid populations in 23 states, 2 Canadian provinces, and the Republic of Korea. The presence of infectious prions in the tissues, bodily secretions/ excreta and environments of CWD-infected animals is thought to account for its high transmission efficiency. Recently it has been recognized that transmission from mother to offspring may contribute to this facile transmission. Although the mechanism of maternal transmission has yet to be elucidated, the extended asymptomatic TSE carrier phase, lasting years to decades, suggests that maternal transmission may have implications in the spread of prions. Our work aims to identify whether prions are transmitted from a CWD-positive mother to her offspring, and identify whether they are sufficient to transmit disease. We employed a transgenic mouse that expresses cervid prion protein to elucidate the role of mother to offspring CWD transmission. Females were inoculated with CWD-positive material and subsequently bred with CWD-naïve males at various timepoints post inoculation. Resultant offspring were monitored over a period of 500 days for clinical signs, and at this time sacrificed for tissue analysis seeking CWD-prion deposition. We have demonstrated that CWD-infected transgenic females successfully breed and bear offspring irrespective to TSE disease stage. PrPCWD was detected in brain, reproductive tissue and spleens from these females. While offspring born to CWD-infected females did not exhibit signs of TSE disease and lacked detectible PrPres via conventional methodologies, conversion competent prions were identified in the brains of offspring by highly sensitive amyloid seeding assays.
 
Graduate Student/ Microbiology, Immunology and Pathology
 
 
No evaluation of determination of CWD risk is required for alternative livestock or captive wildlife shipped directly to slaughter or to a biosecure facility approved by the Division and the Dept. of Agriculture.
 
 
Predator-Prey dynamics in relation to chronic wasting disease and scavenging interactions at cougar kill sites
 
Joe Halseth, Matt Strauser, and Mat Alldredge (CPW)
 
The current Colorado Parks and Wildlife (CPW) cougar (Puma concolor) research on the Front- range is utilizing GPS radio collar technology allowing researchers to track cougar movements on a real time basis. With up to seven uploads a day, the roughly 20 current active project collars give researchers the ability to identify possible kill sites quickly, sometimes as soon as 6 to I~ hours after a kill is made. This provides the opportunity to explore previously un-researched facets of cougar behavior during the relatively short time interval from the point a cougar makes a kill, to the point at which it abandons the carcass. Feeding behavior, intraspecific kill site interaction, and scavenger competition can now be investigated.
 
Similar data to that collected in Krumm et al.' s (2005) and Miller et al.' s (2008) cougar studies, which examined cougar selection of Chronic Wasting Disease (CWD) positive mule deer (Odocoileus hemionus), can now be collected with a greater degree of efficiency. The study areas of each of the two prior CWD cougar projects lie within the more broad boundaries of the current Front-range cougar project, and a larger number of known cougars will increase sample sizes ofCWD tissues from cougar- killed mule deer. Additionally, much of the field work from the two previous studies is nearly a decade old which justifies another project to compare to past results. The ability to collect a potentially larger sample size will yield more accurate findings, identify gaps in need of further study, and/or detect developing trends in regards to possible temporal patterns.
 
The ongoing cougar project's available technology and resources, and the relatively minor additional project costs, provide the opportunity to initiate a camera study to explore cougar feeding behavior and scavenger interaction in the period immediately following a cougar kill. Site visitation of fresh cougar kills also allows for the collection of adequate tissue samples to test for CWD, in order to further explore if cougars are selecting for CWD positive mule deer or other ungulates. Objectives:
 
I. Document sharing and/or abandonment rates of cougars occupying kill sites in response to presence of other cougars and/or scavengers
 
2. Document time from kill until presence of competing scavengers
 
3. Document feeding patterns and length of individual feeding sessions.
 
4. Compare CWO infection rates from cougar-killed deer and elk to existing CPW CWD infection rates to determine if cougars are selecting for CWD positive deer and elk.
 
Scavenging and Kill Site Interactions
 
Placing cameras at kill sites was completed in January 2014 wrapping up 25 months of data collection. Over the course of the study we placed cameras on 225 kill sites recording over 400,000 photos. Pictures have been identified once and are currently in the process of a second round of identification.
 
Timely approaches to kill sites continued to be successful in 2013 and early 2014, usually occurring within 24 hours of a cougars first GPS location at a kill site. This allowed technicians to evaluate the prey item to ensure the estimated time of death matched the carcass condition in order to rule out other possible causes of death (road kill, hunting loss, etc). Cougars were often present at the kill site upon approach but usually retreated as the researcher neared the site. There were several situations where a cougar had been unwilling to move from a kill. In these situations technicians left the area, and if time allowed, returned at a later time.
 
We documented 6 instances throughout the study where carcasses were abandoned following camera placement. Four of these abandonments were due to the cougar occupying a second kill site and never returning to the first, and not likely a result of human visitation and camera placement on the first
 
41
 
carcass. Cameras continued to document bear visitation in both scavenging and direct competition situations and photo sequences continue to be analyzed to determine frequency of these scenarios. Red fox (Vulpes vulpes) were commonly observed scavenging at cougar kill sites. Other scavengers documented include striped skunk (Mephitis mephitis), spotted skunk (Spilogale gracilis), raccoon (Procyon lotor}, ringtail cat (Bassariscus astutus), grey fox (Urocyon cinereoargenteus), coyote (Canis /atrans), domestic dog (Canis lupus familiaris), bobcat (Lynx rufus), golden eagle (Aquila chrysaetos), red-tailed hawk (Buteo jamaicensis), great-homed owl (Bubo virginianus) and a variety of Corvidae bird species.
 
Over the course of the study there have been at least 12 camera sites where we have identified multiple cougars simultaneously occupying a kill site. These observations include two 'sharing' situations involving two cougar family groups and multiple sharing situations involving an adult male and female. Other interactions include two instances of female cougars stealing food items from another female, three unrelated adult females, and one instance of an adult male feeding on a prey item occupied by a female and three young kittens. There have also been several instances where non-focal cougars scavenge on the remains of prey items already consumed and abandoned by the focal cougar.
 
CWD sample collections from cougar-killed ungulates were completed in April 2014 wrapping up 30 months of data collection. In 2013 and 2014, there were no problems with obtaining tissue samples to test for CWD except in rare situations where tissues have been consumed by the cougar. Samples collected in the field were issued a head tag and transferred to the CPW Wildlife Health Lab in Fort Collins for testing. Throughout the course of the study, we collected 192 samples from cougar-killed ungulates of which 190 were testable. Of these, 163 were adult mule deer (65M, 98F), 11 were adult elk and the rest comprised fawn mule deer (n=14), an elk calf (n=l), and an adult white-tailed deer (n=I), Table 1 shows the breakdown of species, age and test results within each deer DAU from adult mule deer sampled within the broad boundary ofthe front-range cougar project. Tables 2 and 3 show mule deer sampling by sex and figure 1 shows the sampling breakdown by month throughout the entire study.
 
Table 1. Total CWD results Total Total %
 
DAD GMU Sampled Positive Positive
 
D-IO 20 28 4 14.29%
 
D-27 29 78 17 21.79%
 
D-27 38 45 13 28.89%
 
D-17 39 2 0 0.00%
 
D-17 391 10 3 30.00%
 
Total 163 37 22.70%
 
Table 2. Male mule deer CWD results Males Males %
 
DAD GMU Sampled Positive Positive
 
D-I0 20 8 1 12.50%
 
D-27 29 32 10 31.25%
 
D-27 38 18 8 44.44%
 
D-17 39 2 0 0.00%
 
D-17 391 5 1 20.00%
 
Total 65 20 30.77%
 
Table 3. Female mule deer CWD results Females Females %
 
DAD GMU Sampled Positive Positive
 
D-IO 20 20 3 15.00%
 
D-27 29 46 7 15.22%
 
D-27 38 27 5 18.52%
 
D-17 39 0 0 0.00%
 
D-17 391 5 2 40.00%
 
Total 98 17 17.35%
 
43
 
 
J Virol. 2013 February; 87(4): 1947–1956. doi: 10.1128/JVI.02592-12 PMCID: PMC3571486
 
Susceptibility of Domestic Cats to Chronic Wasting Disease
 
Candace K. Mathiason,corresponding authora Amy V. Nalls,a Davis M. Seelig,a Susan L. Kraft,b Kevin Carnes,b Kelly R. Anderson,a Jeanette Hayes-Klug,a and Edward A. Hoovera aDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA bDepartment of Environmental and Radiological Health Sciences, Colorado State University, Fort Collins, Colorado, USA corresponding authorCorresponding author. Address correspondence to Candace K. Mathiason, Email: ude.etatsoloc@nosaihtam.ecadnac. C.K.M. and A.V.N. contributed equally to this article as first authors. Author information ► Article notes ► Copyright and License information ► Received 2012 September 21; Accepted 2012 December 4. Copyright © 2013, American Society for Microbiology. All Rights Reserved. This article has been cited by other articles in PMC. Go to:
 
Abstract Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated intracerebrally (i.c.) or orally (p.o.) with CWD-infected deer brain. At 40 and 42 months postinoculation, two i.c.-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and the cats progressed to terminal disease within 5 months. Brains from these two cats were pooled and inoculated into cohorts of cats by the i.c., p.o., and intraperitoneal and subcutaneous (i.p./s.c.) routes. Upon subpassage, feline CWD was transmitted to all i.c.-inoculated cats with a decreased incubation period of 23 to 27 months. Feline-adapted CWD (FelCWD) was demonstrated in the brains of all of the affected cats by Western blotting and immunohistochemical analysis. Magnetic resonance imaging revealed abnormalities in clinically ill cats, which included multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyperintensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 i.p./s.c.- and 2 of 4 p.o. secondary passage-inoculated cats have developed abnormal behavior patterns consistent with the early stage of feline CWD. These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to-feline transmission in nature.
 
Go to: INTRODUCTION Trans-species transmission of prion diseases is unpredictable and remains poorly understood, yet has obvious public health implications. These facts drive continued appraisal of present concepts regarding both transmissible spongiform encephalopathy (TSE) spread and intervention strategies. It is clear that variant Creutzfeldt-Jakob disease (vCJD) (1) and feline spongiform encephalopathy (FSE) (2) can be attributed to the ingestion of meat and bone from bovine spongiform encephalopathy (BSE)-infected cattle. Transmissible mink encephalopathy (TME) in the United States is also attributed to the consumption of scrapie- or BSE-infected meat products (3). It has been hypothesized that sheep scrapie may have crossed the species barrier from scrapie-infected sheep to cattle as BSE, and possibly from sheep to deer as chronic wasting disease (CWD) (4), yet this remains far from certain, and spontaneous generation cannot be excluded. Thus, there is accruing evidence for the trans-species transmission of prions, with potentially grave consequences for animals and humans.
 
FSE, the fatal TSE of domestic and nondomestic cats, was first described shortly after the BSE epidemic (5) and strain typed to be the result of the consumption of BSE-contaminated beef (1). This provided support for the adaptation and transmission of orally consumed prions, as well as evidence that felids are susceptible to prion infection. Chronic wasting disease has been shown to be readily transmitted via multiple means, including direct contact with a CWD-infected cervid (6–8), exposure to infectious CWD prions in the environment (9, 10), and ingestion of bodily fluids and tissues from a CWD-infected cervid (11–15). Recent research by Miller and colleagues (16) performed in an area of Colorado where CWD is endemic, where nearly one-fourth of the adult mule deer are prion infected, confirmed the assumption that afflicted animals become prey to mountain lions more often than healthy ones. In addition, experimental studies have demonstrated that prions infect and cause disease in several rodent scavenger species (i.e., voles and deer mice) overlapping in distribution with CWD and scrapie outbreaks (17, 18). Such scavenging rodents are known to cannibalize and are also an important food source for many larger predators and scavengers in nature, hence providing a reservoir and a potential bridge for cross-species transmission of prions from the natural host to predator/scavenger species.
 
Thus, the facile transmission of CWD, coupled with its growing geographical distribution (19) and propensity to persist in the environment (20–22), provide ample opportunity for the bioavailability of CWD prions to many species coexisting with CWD in nature, including felids.
 
We undertook this study to determine (i) whether felids are susceptible to deer origin CWD prion infection and disease progression and (ii) whether disease course and transmission efficiency are altered upon subsequent passage of feline-adapted CWD in cats.
 
From: J Virol. 2013 February; 87(4): 1947–1956. doi: 10.1128/JVI.02592-12 Copyright/License ►Request permission to reuse Copyright © 2013, American Society for Microbiology. All Rights Reserved.
 
Table 2
 
Pairwise genetic distances between domestic cats and 7 other mammalian species sharing a home range in North America
 
Species Genetic distance (%) froma:
 
--------------------------------------------------------------------------------
 
Domestic cats Mountain lions Bobcats Canadian lynx White-tailed deer Mule deer Elk Humans Cattle
 
Domestic cats 100 94.9 98.6 99.1 92.1 92.1 92.5 89.7 87.7
 
Mountain lions — 100 96.2 96.5 88 88 88.4 84 84.6
 
Bobcats — — 100 99.6 90.4 90.4 90.8 86.4 86.9
 
Canadian lynx — — — 100 90.8 90.8 91.2 86.8 87.3
 
White-tailed deer — — — — 100 100 99.6 90.2 94.7
 
Mule deer — — — — — 100 99.6 90.2 94.7
 
Elk — — — — — — 100 89.8 94.3
 
Humans — — — — — — — 100 87.9
 
Cattle 100
 
View it in a separate window
 
a—, repeat comparison.
 
 
snip...
 
DISCUSSION Transmission of deer origin CWD to domestic cats. CWD, the only prion disease in a wildlife population, exhibits high transmission rates among cervids in their natural environment. Because of the facile transmission between cervids, questions arise regarding interspecies transmission and alternate host reservoirs in scavenger/predator species sympatric with CWD in nature. In this study, we have demonstrated transmission of deer origin CWD (DeerCWD) to one such scavenger species, the domestic cat, via the classical i.c. route of inoculation.
 
Transmission of cat-adapted CWD to domestic cats. The successful transmission of prions to alternate hosts is determined by the species barrier, the strength of which, for prions, is dependent upon interactions between host PrPC and the infecting prion strain (30). Transmission across a species barrier requires adaptation by both the host and the infectious agent, as the original host PrPRES templates its conformation onto the new host PrPC (31). The new host PrPRES thus formed takes on strain-specific properties in the tertiary structure. Therefore, subsequent passage into this new host requires less time for efficient templating, conversion, rogue prion deposition, and progression to terminal clinical disease (31, 32). The results of this study support this host adaptation hypothesis, as upon secondary passage of cat-adapted CWD (FelCWD), 100% of FelCWD i.c.-inoculated cats developed clinical disease in about half the time required for DeerCWD-inoculated cats. In conjunction with an appreciably shortened course of clinical disease, FelCWD deposition and distribution were more extensive and diffuse (Fig. 2), suggesting a broadening of host cell tropism, i.e., the ability of feline-adapted CWD prions to infect and amplify in or on a greater variety of feline cell types.
 
Clinical disease in peripherally inoculated domestic cats. A plausible route for interspecies transmission of prion diseases is via breach of mucosal surfaces during ingestion of infectious prions found in carcasses, their by-products, and in the environment. Precedence for prion trans-species transmission has been well documented with vCJD, FSE, and TME (4, 33). Studies have also demonstrated that prion diseases can be orally transmitted to many species: i.e., CWD to voles (34), Peromyscus mice (34), and ferrets (35), scrapie to squirrel monkeys (36) and hamsters (37–40), BSE to sheep (41–43), goats (41), cynomolgus macaques (44, 45), and lemurs (46), and CJD and Kuru to squirrel monkeys (36), with some requiring prior in vivo or in vitro adaptation.
 
In our studies, p.o. inoculation of DeerCWD was not sufficient to induce TSE. Western blotting, IHC, and RT-QuIC failed to show the presence of FelCWD in these animals, demonstrating the existence of a considerable species barrier. However, early signs of disease are currently observed in second passage p.o.- and i.p./s.c FelCWD-inoculated cats, suggesting that the ingestion of and/or open wound exposure to CWD-contaminated material could cause feline TSE disease. Naïve cats cohoused with the i.c.-inoculated FelCWD-positive cohort continue to serve as horizontal/environmental shedding test subjects and are currently housed with the i.p./s.c.-inoculated cohort. These remaining cats will continue to be monitored for further development of clinical disease, and terminally harvested tissues will be analyzed for the presence of FelCWD.
 
Detection of MRI abnormalities in clinically ill cats. Cerebral MRI has been used to detect specific abnormalities between sporadic CJD (sCJD), vCJD, and nonprion rapidly progressive dementias (47–52). In sCJD patients, T2 hyperintensities are observed in the cortex and deep gray matter, in particular the striatum, whereas in vCJD, T2 hyperintensities are most commonly seen in posterior pulvinar and medial thalamus (47, 52–56). The histological basis of the T2 hyperintensities is not certain, but for at least certain subtypes of CJD, it may be related to vacuolar fluid accumulation (57). Cerebral atrophy can also become evident on MRI in CJD patients (58). DW-MRI is physiologically based and is a highly sensitive and specific marker of restricted brain diffusion in CJD patients (59, 60) and has improved the imaging diagnosis over conventional MRI. Restricted diffusion and DW-MRI signal hyperintensity have been found in the basal ganglia, thalamus, and cortex of CJD patients (60). Again, the proposed explanations for restricted diffusion vary with the CJD subtype but may be related to intraneuronal microvacuolation (57), prion protein deposition, and spongiform change (61). The combination of DW-MRI with FLAIR imaging has sensitivity and specificity reported at >91 to 98% (59, 62). Those sequences are important for early detection to inform appropriate diagnostic testing (61) and to enable timely intervention as new therapies are developed (59). Although only a small number of studies have used MRI to investigate animal models of prion disease, it has been shown that focal blood-brain barrier disruption occurs in a hamster scrapie model (63), that there are increases in T2 measurements in the septum, hippocampus, thalamus, and cortex of mice inoculated with 139A scrapie (64), and that the diffusion of tissue water was significantly reduced in the late preclinical period in mice inoculated with ME7 scrapie (65). Furthermore, a recent MRI study in sheep naturally exposed to scrapie demonstrates a generalized cerebral atrophy (66).
 
In this study, MRIs were performed on cats inoculated with deer or cat origin CWD. Abnormalities resembling those found in aforementioned MRI studies in CJD patients (47, 52–56, 58), including nonspecific T2 and T2 FLAIR hyperintense signal changes and ventricular enlargement consistent with cerebral atrophy, were present in the symptomatic cat inoculated with DeerCWD, whereas only ventricular enlargement was found in symptomatic cats inoculated with FelCWD. Based on the limited MRI data in other prion animal models, it appears that FelCWD most closely resembles the atrophy demonstrated in sheep scrapie. How or whether these signal abnormalities correlate with histopathological data is currently being assessed.
 
Comparison of CWD and BSE transmission to domestic cats. Striking similarities are seen in the clinical presentation and duration of TSEs (4, 55). In cats, both BSE and CWD are marked by changes in behavior and gait, hyperaesthesia, ataxia, and tremors, and both progress to terminal-phase disease 3 weeks to 5 months postobservation of initial TSE signs (67–69).
 
BSE appears to be more efficiently transmitted to domestic cats by oral exposure than CWD. In this study, while we have shown that cats are susceptible to CWD infection by the classical intracranial route, oral transmission was seen only after subpassage into a second cohort of cats that had received lingual abrasions known to enhance transmission potential (23, 24). While the presence or absence of oral lesions was not reported in FSE cases observed by Wyatt (68, 69) and Legget (67), it is hypothesized that the affected cats consumed BSE-contaminated meat and bone in commercial cat foods.
 
Implications associated with trans-species transmission of CWD prions. It has been determined that, once a prion strain has been adapted to a new host species, the prions from this new host species propagate more efficiently in a third host. Specifically, the passage of cow BSE prions in sheep or goats markedly increased the transmission efficiency into human transgenic mice (70). Although a substantial species barrier appears to exist between deer and cats, barring an invasive route of inoculation, we must consider the epidemiologic and ecologic implications associated with CWD transmission to felids, which could potentially result in the generation of prion strains adapted for natural (mucosal) routes of transmission to felids and/or other noncervid species. The high genetic identity homology observed between domestic and nondomestic cats compared to 5 other mammalian species sharing a home range with CWD-infected cervids, while warranting further analysis of protein structure and protein-protein interactions, suggests that nondomestic cats may be a susceptible reservoir species in nature (Fig. 5). If CWD has the ability to infect and establish alternate host reservoirs in nature or to initiate the adaptations necessary for trans-species transmission, this will impact not only wildlife, but also domestic species, which can lead to serious consequences for human health. Here, we have demonstrated that one scavenger/predator species (the domestic cat) that cohabitates with the natural hosts of CWD could become an intermediate host for this prion disease in nature.
 
 
Monday, August 8, 2011
 
Susceptibility of Domestic Cats to CWD Infection
 
Oral.29: Susceptibility of Domestic Cats to CWD Infection
 
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†
 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu
 
Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness. Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.
 
 
http://felinespongiformencephalopathyfse.blogspot.com/2011/08/susceptibility-of-domestic-cats-to-cwd.html In October 1998 the simultaneous occurrence of spongiform encephalopathy in a man and his pet cat was reported.
 
The report from Italy noted that the cat did not display the same clinical features as FSE cases previously seen. Indeed, the presence of a new type of FSE was suggested. The man was diagnosed as having sporadic CJD, and neither case (man nor cat) appeared to be affected by a BSE-related condition.
 
 
Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy
 
Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, Sergio Ferrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, Salvatore Monaco
 
Transmissible spongiform encephalopathies (TSE) encompass inherited, acquired, and sporadic mammalian neurological disorders, and are characterised by the conversion of the cellular prion protein (PrP) in an insoluble and protease-resistant isoform (PrPres). In human TSE, four types of PrPres have been identified according to size and glycoform ratios, which may represent different prion strains. Type-1 and type-2 PrPres are associated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 with iatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence that variant CJD is caused by the bovine spongiform encephalopathy (BSE)-prion strain.2-4 The BSE strain has been identified in three cats with feline spongiform encephalopathy (FSE), a prion disease which appeared in 1990 in the UK.5 We report the simultaneous occurrence of sporadic CJD in a man and a new variety of FSE in his cat.
 
A 60-year-old man, with no unusual dietary habits, was admitted in November, 1993, because of dysarthria, cerebellar ataxic gait, visual agnosia, and myoclonus. An electroencephalogram (EEG) showed diffuse theta-delta activity. A brain magnetic resonance imaging scan was unremarkable. 10 days later, he was speechless and able to follow only simple commands. Repeat EEGs showed periodic triphasic complexes. 2 weeks after admission, he was mute, akinetic, and unable to swallow. He died in early January, 1994.
 
His 7-year-old, neutered, female shorthaired cat presented in November, 1993, with episodes of frenzy, twitching of its body, and hyperaesthesia. The cat was usually fed on canned food and slept on its owner's bed. No bites from the cat were recalled. In the next few days, the cat became ataxic, with hindquarter locomotor dysfunction; the ataxia got worse and there was diffuse myoclonus. The cat was killed in mid-January, 1994.
 
No pathogenic mutations in the patient's PrP gene were found. The patient and the cat were methionine homozygous at codon 129. Histology of the patient's brain showed neocortical and cerebellar neuronal loss, astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed a punctate pattern and paralleled spongiform changes (figure B). The cat's brain showed mild and focal spongiosis in deeper cortical layers of all four lobes (figure C), vacuolated cortical neurons (figure D), and mild astrogliosis. The cerebellar cortex and the dentate nucleus were gliosed. Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, and caudate nucleus (figure E). Western blot analysis of control and affected human and cat brain homogenates showed 3 PrP bands of 27-35 kDa. After digestion with proteinase K and deglycosylation, only samples from the affected patient and cat showed type-1 PrPres, with PrP glycoform ratios comparable to those observed in sporadic CJD1 (details available from author).
 
[Image]
 
Microscopic sections of patient and cat brains
 
A: Occipital cortex of the patient showing moderate spongiform degeneration and neuronal loss (haematoxylin and eosin) and B: punctate perineuronal pattern of PrP immunoreactivity; peroxidase immunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortex showing mild spongiform degeneration (haematoxylin and eosin).D: vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidase immunohistochemistry with antibody 3F4 shows punctate perineuronal deposition of PrP in temporal cortex.
 
This study shows a spatio-temporal association between human and feline prion diseases. The clinical features of the cat were different from previously reported cases of FSE which were characterised by gradual onset of behavioural changes preceding locomotor dysfunction and ataxia.5 Neuropathological changes were also at variance with the diffuse spongiosis and vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern of PrP deposition, similar in the cat and in the patient, was atypical for a BSE-related condition. Evidence of a new type of FSE was further provided by the detection of a type-1 PrPres, other than the BSE-associated type 4.2 Taken together, our data suggest that the same agent strain of sporadic CJD was involved in the patient and in his cat.
 
It is unknown whether these TSE occurred as the result of horizontal transmission in either direction, infection from an unknown common source, or the chance occurrence of two sporadic forms.
 
1 Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variablity in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767-78 [PubMed].
 
2 Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].
 
3 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 498-501 [PubMed].
 
4 Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448-50 [PubMed].
 
5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiform encephalopathy: a review. Vet Annual 1993; 33: 1-10.
 
------------------------------------------------------------------------
 
Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e della Visione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy (S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto Zooprofilattico Sperimentale della Lombardia e dell' Emilia, Brescia
 
 
 
COVER SHEET FOR DFA 23 24 JANUARY 2000
 
No update has been prepared for this DFA. Further relevant evidence and corrections are noted below.
 
Further relevant evidence
 
Further relevant evidence may be found in:
 
YB90/5.15/8.1 YB90/6.11/6.1-6.7
 
Corrections
 
Please note the following corrections:
 
para 2 “PS/PUSS” should read “PS/PUS” para 2 “advised that first thoughts suggested” should read “advised that her first thoughts were” para 2 “Her own assessment was:” should read “Her briefing included the following:” para 3 “about the cat” should read “with more details about the cat. The minute was copied to several other DH officials, including Dr McInnes (PS/CMO)”
 
DFA 23
 
MAFF statements in 1990 about a cat with a spongiform encephalopathy
 
Draft Factual Accounts 16 November 1999
 
This is one of a series of documents intended to provide an account as at the date of publication of the factual evidence received by the Inquiry. The documents do not make any judgements about the implications of the facts or point to any conclusions. They are simply working drafts seeking in a neutral way to set out relevant evidence. They do not contain any expressions of opinions by the Secretariat or the Committee of the Inquiry. The series will only cover certain areas of the evidence.
 
The DFAs may contain inaccuracies and omissions. The purpose of publishing them is to invite corrections, additions and comments. The Inquiry has received suggestions for such corrections and additions in relation to DFAs already published. This is helpful in furthering the work of the Inquiry; all suggestions are considered and used to update the Secretariat’s working papers which will form the basis of the Committee’s Report in due course. The DFAs should not be treated as setting out a complete and accurate appreciation of the relevant facts.
 
You are invited to let the Secretariat know of any errors, inaccuracies or material omissions in this DFA. It would be helpful if you could distinguish suggested amendments to the DFA from more general comments which would not involve such amendment. Please write to:
 
The Secretary The BSE Inquiry 6th Floor Hercules House Hercules Road London SE1 7DU Email to : inquiry@bse.org.uk
 
MAFF statements in 1990 about a cat with a spongiform encephalopathy
 
1. On 9 May 1990, Mr Lowson (Head of Animal Health Division - MAFF) put a submission to the Minister following the confirmation of SE in a cat. The submission was also copied to Mr Bradley (Pathologist - CVL) and Mr Wells (Head of Neuropathology - CVL). Mr Lowson said:
 
‘2. Although this is the first firm recorded instance of a cat being infected by apparently natural means, it is not particularly surprising; it has been known for some time that cats are susceptible to CJD by experimental inoculation, and greater public awareness may have meant that cases that previously went unnoticed will now be suspected based on clinical symptoms. Nevertheless once this information becomes public (as is inevitable) it is bound to excite comment and a connection will be made with BSE, scrapie and possibly even with CJD.
 
3. After discussion with Mr Dugdale we recommend that, rather then wait for the story to emerge, we should take the initiative in releasing the information. This would be done by means of a letter to the Veterinary Record, but as the first issue in which it could be published would be that on Friday week, 18 May, it would be better to release the text in advance. If ministers agree, this release could be accompanied by a press briefing by officials, possibly on Friday of this week.
 
4. …We [MAFF] will … concert our approach with DOH and the British Veterinary Association, and recommend that they should both participate in the Press Briefing…
 
5. … Detailed further enquiries will be needed; it is not certain that this is a transmissible spongiform encephalopathy. More needs to be known about the circumstances in which this animal might have been infected; although it is most unlikely that further enquiries will lead to any firm conclusions.
 
6. … Clearly this case will bring into sharp relief all the various issues that have been troubling the media for some time… Above all it will be important not to encourage the public to jump to conclusions about the likely origin of this case…’[1]
 
2. On 9 May 1990, Dr Pickles minuted Mrs Baxter (PS/PUSS, Mr Dorrell), with copies to Sir Donald Acheson and Dr Metters, regarding the news from MAFF about the cat. Dr Pickles stated that DH were in close contact with MAFF and were ‘asking them to discuss this urgently with our joint outside experts [SEAC]’. As regards the DH’s concerns, she advised that first thoughts suggested that the case ‘does not affect our views on the lack of hazard of BSE to man… However, the implications of this new information needs to be considered by the full Tyrrell Committee’. She identified the following concerns for MAFF:[2]
 
‘* BSE has spread to yet another species: how many more?
 
* Whether it should still be permissible to include offal in pet food.
 
* Renewed pressure to ban the feeding of processed sheep and cow offal to pigs and poultry.
 
* Could there be spread between cats or to other species from cats?’
 
Her own assessment was:
 
‘…there are pressures to extend the ruminant protein ban: at present pigs and poultry receive this sort of feed. Publicity about the infected cat could increase these pressures. Such action, which would be hard to justify scientifically, would increase costs for the industry and cause perhaps insurmountable problems for abattoirs, who would find renderers no longer willing to accept offal. Many 1000s of tons of offal need to be disposed of daily’.
 
3. On 10 May 1990, Dr Pickles minuted Dr Metters about the cat. Dr Pickles offered two alternative explanations:[3]
 
‘The implications have to be considered at leisure by the new Tyrrell Committee. Dr Tyrrell is on holiday but the others are being informed on the phone. Assuming further investigation does not question the diagnosis or reveal unusual routes by which this cat could have been infected, there are two main possibilities. Firstly, that cats have been susceptible to ingestion of infected meat for years and cases previously have gone unrecognised. Secondly, a new agent, presumably BSE, is virulent in a way in which previous agents such as scrapie have not been. The second possibility is most unlikely, but more worrying since it challenges the assumptions we have made for humans by analogy with scrapie…
 
…the public may start asking again about pig and poultry feed. We must be fully involved in discussions, particularly as some essential pharmaceuticals are made in pigs. The press, on being reassured catgut does not come from cats, could question where it does come from; perhaps [company name deleted] should be warned’.
 
4. On 10 May 1990, Mr Gummer (Secretary of State - MAFF) met Mr David Maclean (Parliamentary Secretary - MAFF), Mr Meldrum, Mr Lowson and others to discuss how to make public the information that a cat had been diagnosed with SE.[4] A note of the meeting prepared by Mr Gummer’s private office and copied to Mr Bradley and Mr Wells amongst others, records:
 
‘[The CVO] confirmed the Minister’s assumption that there was no likely connection between this case and BSE. Nor was the illness necessarily linked with the cat’s diet. We knew that cats could be infected by the injection of high doses of CJD virus, but it had not been possible to inject them with the scrapie virus. No experiments had been done on cats using BSE virus. This was the first discovered case of a spongiform encephalopathy in a domestic cat although there had been a case involving tigers in the 1970s where it was alleged the animals had an encephalopathy. As regards the present case, the cat had fed on a wide range of pet foods including cooked fresh meat bought from a local shop. The Minister concluded from this that, if it weren’t for the general public interest in encephalopathies, the death of this cat would not be a matter of significance. However, given the public interest, it was desirable to make the information available as soon as possible.’[5]
 
5. In a supplemental statement, Mr Meldrum stated that he did not believe that he would have used the words “no likely connection”:
 
‘Mr Lebrecht's note of the meeting on 10th May, 1990 with the Minister (Mr Gummer) and the Parliamentary Secretary (Mr Maclean) records that I "confirmed the Minister's assumption that there was no likely connection between this case [of a spongiform encephalopathy in a cat] and BSE" (YB90/5.10/10.1). I do not believe that I would have used the words "no likely connection". I believe that it is more probable that I said that there was no known connection between the case and BSE. This was the wording that I used in my letter to the Veterinary Record which was attached to MAFF's Press Notice dated 10th May, 1990 (YB90/5.10/2.1-2.3). In truth it is the letter to the Veterinary Record that must carry the greatest weight since that was the information that was being given to the public and not the note of the meeting which was essentially for internal use within MAFF.’[6]
 
6. In a supplemental statement, Mr Meldrum noted that Mr Wells and Mr Bradley received copies of Mr Lowson’s minute of 9 May 1990 and Mr Lebrecht’s note of the meeting on 10 May 1990. Mr Meldrum continued:
 
‘The points were made in Mr Lowson's minute that it was not certain that this was a case of a TSE, that it was important not to encourage the public to jump to conclusions about the origin of the case, ‘defensive’ briefings would be prepared and there was nothing further to be done apart from bringing it to the attention of the veterinary profession. Mr Lebrecht's note contained the record that I ‘confirmed the Minister's assumption that there was no likely connection’ between the case and BSE (although as I have already commented, ‘likely’ is not a term that I believe I would have used nor was it a term that I used at the Press Briefing) and the Minister's conclusion that, if it wasn't for the general public interest at the time, the death of the cat would not have been a matter of significance. Yet neither Mr Wells nor Mr Bradley (on behalf of Mr Wells) nor any more senior officer at the Central Veterinary Laboratory appear to have raised any concerns as a result of the points made in these two documents about the approach being taken to making the information public.’[7]
 
7. A News Release was issued by MAFF on 10 May 1990, which stated:
 
‘Pathologists at the Bristol Veterinary School and MAFF's Central Veterinary Laboratory at Weybridge have diagnosed a sub-acute spongiform encephalopathy in a five-year old Siamese cat originating from the Bristol area. Keith Meldrum the Ministry's Chief Veterinary Officer has written to the 'Veterinary Record'’. [8]
 
8. Attached to the 10 May press release was a letter from Mr Meldrum to the Veterinary Record[9]. In this letter Mr Meldrum stated:
 
‘Inquiries into the case will continue, but there is no evidence that the condition is transmissible nor is there any known connection with the other animal encephalopathies.’
 
9. A supplemental statement from Mr Meldrum includes the following:
 
‘As my letter in the Veterinary Record (attached to the MAFF Press Release of 10th May, 1990; YB90/5.10/2.1-2.2) stated, "[i]nquiries into the case will continue, but there is no evidence that the condition is transmissible nor is there any known connection with the other animal encephalopathies". This most certainly does not discount possible transmission of BSE to cats but clearly states the true position; that it was not known. A quote similar to that in the Veterinary Record letter was reported in the article in The Daily Telegraph on 11th May, 1990 (YB90/5.11/12.1) and, in addition to reporting my comments at the Press Briefing, the article in The Times on the same day (YB90/5.11/12.1) also referred to the fact that it was not yet known if the disease was transmissible and experiments were being carried out. A perhaps more balanced report of the 10th May, 1990 Press Briefing was published in Nature on 17th May, 1990 (J/N/345/194). The article notes my comment that ‘there is no evidence for a connection with similar diseases in other animals’ and goes on to explain that MAFF ‘is to commission research to see if the cat spongiform encephalopathy can be passed to laboratory mice. This should reveal if the cat infection behaves similarly to BSE and scrapie…’.’[10]
 
10. In recalling his press briefing of 10 May 1990, Mr Meldrum states:[11]
 
‘…the position at that time was that the first case ever of a naturally occurring spongiform encephalopathy in a cat had been confirmed, the disease had not been confirmed as being transmissible (i.e. as being a TSE the same as BSE and scrapie), it was not known how the cat had been infected and whether any more cases could be expected and this was only one cat death out of seven million cats in the U.K. As stated in paragraph 36 of the section above on “Ministers’ meeting on 10th May, 1990”, it would have been singularly unwise for me to conclude on the basis of the laboratory examination of the brain of one cat that BSE had crossed the species barrier from cattle to cats. It was not until 1991 that it was indicated by the University of Bristol experiments that the spongiform encephalopathy in cats was transmissible (verbally notified in June 1991 and the written interim report in September 1991). Whilst some scientists (for example, Mr Wilesmith; T52, Vol. T6, tab 2 page 117) might suggest that it could have been considered in May 1990 that it was "highly likely" that the disease was a TSE, this was not communicated to me as far as I can recall. Until scientific confirmation of transmissibility was obtained it would not have been sensible for me to publicly state that this one case was a TSE; no one knew this to be the case at the time. In the event that this had not proved to be the case, such a statement would have caused unnecessary concern and damaged both my and MAFF's credibility at what was a very difficult time.’
 
11. On 10 May 1990, comments made by Mr Meldrum about the discovery of spongiform encephalopathy in a cat were reported on BBC1 News at Six. Mr Meldrum said[12]:
 
‘This is only one cat death out of seven million cats in the UK, and there is no reason or cause for concern at all. If we hadn’t got the other encephalopathies in animals in this country, this report would have been published without comment’.
 
12. A supplementary statement from Mr Meldrum commented on the above quote:[13]
 
‘The draft DFA includes a quote taken from an extract from the ITN ‘News at 10’ on 10th May, 1990, which appears in the television programme ‘Mad Cows and Englishmen’. The full extract in ‘Mad Cows and Englishmen’ includes this introductory sentence from Julia Somerville (presenting the ITN News) before cutting to a clip of me speaking: ‘[e]xperts at the Ministry of Agriculture are trying to find out if there is a link between the illness of a pet cat and the Mad Cow Disease BSE’. In any event, the ‘Mad Cows and Englishmen’ extract is in itself a highly selective and edited presentation of the ITN ‘News at 10’ on 10th May, 1990, with the opening titles for the ‘News at 10’ following with the single sentence from Julia Somerville and cutting straight to a clip of me at the Press Briefing on 10th May, 1990. In actual fact the report on the ‘pet cat’ only appeared about half way through ‘News at 10’ after reports on Michael Heseltine, the poll tax, the inquiry into the Kegworth air crash and safety problems in the Channel Tunnel. So the document uses a selective quotation from a television programme containing a highly edited clip from another television programme which only contained a shortened version of an entire interview given by me at a full Press Briefing.’
 
13. On Friday 11 May 1990, the story of the Bristol cat was covered in the national press.[14] The Times published an article entitled ‘Mad cow symptoms found in pet cat’ on 11 May. The article reported that it was not yet known how the cat had become infected or whether the feline form of the spongiform encephalopathy could be transmitted between different species and that scientists were investigating whether it could be passed to laboratory mice. Mr Meldrum was reported to have said:
 
‘There is no need for pet owners to change their pet food or to consider putting their cats down. The risk to man is no greater than it was before diagnosis.’[15]
 
Mr Meldrum was also reported to have said:
 
‘Such findings have not previously in domestic cats at this stage, this is the only known case in Britain.’
 
14. Also on 11 May, the Daily Telegraph published an article entitled ‘Mad cow disease check as cat dies’. The article stated that according to Mr Meldrum, the spongiform encephalopathy diagnosed in the cat had not been seen to occur naturally in cats before.[16] The article stated:
 
‘Mr Meldrum ruled out having the other animals in the affected house put down. He stressed that there is no danger to owners of cats and said that there is no known dog encephalopathy.’[17]
 
Mr Meldrum was reported as saying:
 
‘There is no need to consider putting one’s cat down because of one case of encephalopathy in Bristol.’[18]
 
15. The article reported that ‘results of the transmission will take more than a year to emerge.’ The article quoted Mr Meldrum as saying: ‘at present there is no evidence that the condition is transmissible, nor is there any known connection with the other animal encephalopathies, be they in sheep (scrapie) or in cattle.’ Mr Meldrum was reported as emphasising that it emphasising that it was “one cat in a population of seven million” and had was reported as having added that “only time will tell” whether it was a one off event or more cases would be diagnosed in cats.[19]
 
16. On 11 May 1990, Mark Hawkins (MAFF Animal Health Division) sent Mr Lowson’s submission dated 9 May 1990 to officials in the agricultural departments in Scotland, Wales and Northern Ireland. He also enclosed a document entitled ‘A spongiform encephalopathy in a cat - Speaking note for K C Meldrum’. The speaking note included the following:
 
‘Pathologists at the Bristol Veterinary School and the Central Veterinary Laboratory at Weybridge have diagnosed a subacute spongiform encephalopathy in a 5 year old siamese cat originating from the Bristol area. This is the first known recorded case of a spongiform encephalopathy occurring in a cat.
 
The animal was referred to the Bristol Veterinary School by a veterinary surgeon in the area, and had symptoms of a nervous disturbance including unsteadiness on the feet and incoordination. Typical lesions of a spongiform encephalopathy were found in the brain following post-mortem examination. Preliminary inquiries have revealed that the cat had been fed on a wide variety of food.
 
Inquiries into the case will continue, but at this time there is no reason to associate this case with any other animal encephalopathy that occurs in Great Britain. Transmission studies with material from this cat will commence as soon as possible but results are unlikely to be available for at least a year.
 
This case is not entirely surprising, bearing in mind that some cats have been found to be susceptible to some encephalopathies under challenging laboratory conditions and that there is a heightened awareness of the animal encephalopathies in the UK at this time and therefore laboratory examination of material will now be undertaken that would not have been undertaken previously.
 
This case should be seen for what it is. A single case of a spongiform encephalopathy in a population of some 7 million cats. There is no evidence that the condition is transmissible and there is no known connection with the other transmissible animal encephalopathies. Detailed investigations will continue and the results will be published in due course.’ [20]
 
17. A supplemental statement from Mr Meldrum to the Inquiry included the following:
 
‘I used the speaking note as the basis for the comments that I made to the press on 10th May, 1990. This is borne out by the fact that the reports in the press on the 11th May, 1990 followed the thrust of the note fairly accurately. Notably, the tone of the speaking note was tentative and nothing was ruled out. In addition, nothing is contained in the speaking note on the human health implications of the case, this being the responsibility of the Department of Health.’[21]
 
18. A supplemental statement from Mr Meldrum also includes the following:
 
‘It is noteworthy that the press reports of 11th May, 1990 (YB90/5.11/3.1-3.2; YB90/5.11/4.1; YB90/5.11/5.1; YB90/5.11/6.1; YB90/5.11/12.1) do not reach the conclusion that this single case increased the risk to man. Clearly, however, when further cases were confirmed in the coming weeks it did necessitate a re-evaluation of the relevance of the cases of SEs in cats. This was the subject of considerable discussion by the press arising from the concerns that were subsequently expressed by the German Government leading up to the lengthy discussions in Brussels on the safety of British beef later in May 1990.’[22]
 
19. The following comment was also provided by Mr Meldrum in a supplemental statement to the Inquiry:
 
‘As I have been asked to consider press reports which have used selective quotes from me, I feel that it would be fair to comment that the press cannot always be relied upon to quote anybody fully, accurately or in the correct context. This problem is compounded when single or part sentences are then extracted from an entire report. Unfortunately, I have no control over how the press choose to report either my, or anyone else's, public statements. In any event the Press Briefing referred to in paragraph 1 above was the source of the information in the broadsheets and dealt with the animal health aspects of the spongiform encephalopathy in the cat. My speaking note did not rule out the possibility that this could have been the first case of a TSE in a cat and thus a further example of the transmission of a ruminant TSE. In the Press Briefing I ruled nothing out.’[23]
 
20. Mr Wells drafted a minute to Mr Bradley on 11 May 1990 commenting on the way that the finding of FSE had been ‘trivialised’ in representations to the media. His minute read:
 
‘SCRAPIE-LIKE ENCEPHALOPATHY IN A SIAMESE CAT
 
The comments made by the CVO on BBC1 News at Six, 10 May 1990, on this subject were unfortunate, inappropriate and provocative.
 
The current situation requires a guarded public statement. The findings are preliminary but have potential agreed importance and should not, from virtually all viewpoints, have been represented as inconsequential.
 
Even at this early stage of the investigation the indications are: that this is unlikely to be an isolated incident; that the cat is susceptible to a scrapie-like disease by a route other than the intracerebral; that the origin of infection is likely to be cattle or sheep and that the possible vehicles of implications include products of the rendering industry, prepared pet food and fresh meat trades. The temporal occurrence of this incident is also consistent with possible exposure to scrapie or BSE agents during the period of recycling of carcases of clinically affected cattle in addition to the continuance recycling of sheep material.
 
Reassurance regarding this incident from both the CVO and the BVA in the media is at present an over optimistic response which may well, in a very short time, result in a loss of credibility for the veterinary profession in this whole sensitive subject area.
 
Furthermore the trivialization of the occurrence in the public statement made has incensed the research workers in the Departments of Veterinary Pathology and Veterinary Medicine, the University of Bristol Veterinary School whose professionalism, foresight and co-operation in informing MAFF in confidence, of their findings provided a valuable forewarning which less sympathetic individuals and organisations may well have relished circumventing. This has potentially comprised further collaboration on the present material and subsequent communication of the subject.
 
In the interests of all concerned please could you advise the CVO appropriately.’ [24]
 
It appears that this minute was not sent. Mr Wells later told Mr Meldrum his ‘personal view that discovery of FSE was probably of profound significance in relation to BSE and should lead to a complete ban on meat and bonemeal entering the animal food chain’.[25]
 
21. In a supplementary statement to the Inquiry, Mr Meldrum referred to his previous evidence to the Inquiry regarding the conversation between Mr Wells and Mr Meldrum, and commented:
 
‘As I stated in that evidence, I believe that the conversation took place later in the summer of 1990, and this ties in with a request I made on 25th June, 1990 to Dr Cawthorne and Mr K. Taylor to obtain information on archives of cat brains (YB90/6.25/15.1;YB90/6.25/21.1-21.2). By this time there had been four confirmed cases in cats and as I noted in my previous statement, ‘[n]ow that further cases of FSE were being confirmed I sought further information to determine whether this was a new problem in cats or whether it was a pre-existing condition’ (see paragraph 22 of Section L of WS 184A).’ [26]
 
22. A supplemental statement from Mr Meldrum included the following futher comment on the views expressed by Mr Wells in his draft minute of 11 May 1990:
 
‘In any event, the views expressed by Mr Wells in his draft minute of 11th May, 1990, which he did not appear to send out in either draft or final form (YB90/5.11/1.1-1.2), relate more to the question as to whether the case of spongiform encephalopathy in the one cat was an example of BSE having transmitted to cats than to whether such a transmission had direct implications on the risks to humans from BSE. As discussed in paragraphs 6 to 8 above, it did not follow that if I believed BSE had crossed the species barrier to cats it should be the case that the risk to humans was greater. In any event there was no evidence at the time that the case of SE in the cat was a TSE.’[27]
 
23. On 11 May the Daily Telegraph published an article titled ‘Mad cow disease check as cat dies’. The article stated that according to Mr Meldrum, the spongiform encephalopathy diagnosed in the cat had not been seen to occur naturally in cats before.[28] The article stated:
 
‘Mr Meldrum ruled out having the other animals in the affected house put down. He stressed that there is no danger to owners of cats and said that there is no known dog encephalopathy.’[29]
 
Mr Meldrum was quoted as saying:
 
‘There is no need to consider putting one’s cat down because of one case of encephalopathy in Bristol.’[30]
 
24. The article reported that ‘results of the transmission will take more than a year to emerge.’ The article quoted Mr Meldrum as saying: ‘at present there is no evidence that the condition is transmissible, nor is there any known connection with the other animal encephalopathies, be they in sheep (scrapie) or in cattle.’[31]
 
25. On 13 May, 1990 Professor Lacey, once a Government health advisor, and at the time Professor of Clinical Microbiology at Leeds University, was reported in The Sunday Times as saying that “the risks of humans catching mad cow disease are now so great that 6m cattle need to be slaughtered” and that “people should not eat beef until half the herds in Britain, each of which had at least one infected cow, had been destroyed and beef had been proven to be safe again”. Professor Lacey called for a “package of urgent safety measures” with proposals that included a total ban on the £350 million exports of British cattle and beef each year. He said, “ we need to put the British Isles in quarantine ourselves before someone else does”. He went on, “we need authoritative advice from medical doctors instead of all these ministers, vets and civil servants who are telling us that everything is safe,” and that “we now have two new mammals – cattle and cats – infected naturally for the first time by this agent. The likelihood is increased of the possibility of transmission to man from cattle.” Professor Lacey also urged pet owners worried by the cat’s death to heat canned pet food in a pressure cooker or switch to butcher’s scraps.[32]
 
26. In the same article, Mr Meldrum was reported as accusing Professor Lacey of “pure supposition, over-reaction and scare-mongering”. He said, “to suggest that the discovery of a spongiform encephalopathy in a cat increases the risk to man is absolute nonsense. The basis on which we are saying beef is safe is that the agent of scrapie has not been detected in the muscle of sheep affected with scrapie”. Also in the article, the Parliamentary Secretary (Mr Maclean) was reported as saying “Professor Lacey is good at popping up in the media with scare stories. Even the most elementary scientist knows that this disease cannot be passed from cow to cow like an infection. As far as telling people not to eat beef if they are under 50, if anyone is daft enough to believe his doomsday scenario, then the age of 50 seems plucked out of thin air and is nonsensical.[33]
 
27. In a supplementary statement, Mr Meldrum commented on the reported comments attributed to him:[34]
 
‘The reported comments attributed to me to the effect that the risk to man was ‘no greater’ or had not ‘increased’ do not amount to stating that BSE had not transmitted to cats. Even if at the time it was known to be a case of a TSE in cats due to BSE it did not follow that the risk of transmission of BSE to man had changed. It should be remembered that at this time the ruminant feed ban had been in place since July 1988, clinically affected cattle were being slaughtered and destroyed, ante-mortem inspection of adult cattle was being introduced and the SBOs of all clinically healthy cattle had been prohibited from entering the human food chain since November 1989. With these control measures already in place to protect man from the remote risk of transmission of BSE, what I was saying was that the discovery of this one case could not, on its own and in the absence of further investigations into the cause of the case, be stated as being evidence that the risk of transmission of BSE to man had increased. In fact the risk would not have increased in any such event but the appreciation of the possible risk to man may have changed. The same could be said of the cases of SE occurring in exotic species, none of which put the human population at any increased risk.’
 
28. Mr Meldrum also noted in his supplementary statement that there is evidence on this point from various witnesses to the BSE Inquiry:[35]
 
‘For example, in a letter dated 16th May, 1990 to Mr Wilkinson (Department of Education and Science), Professor Bourne (then Director of the Institute of Animal Health) stated that ‘[t]he finding of encephalopathy in a cat did not change the situation in relation to susceptibility of man, …We are aware that transmissible encephalopathies can be experimentally transmitted between species and that there are species differences… But what is significant is the mass of epidemiological evidence that shows that man is not susceptible to scrapie… It is possible, as a result of heightened interest in the transmissible encephalopathies, that they are subsequently found to cause a natural disease in other species including the dog and cat’ (YB90/5.16/2.2-2.9). A similar view was expressed by Dr MacOwan in his oral evidence (T39, Vol. T4, Tab 9, page 115) when he was asked whether the conclusion that the case was an example of BSE crossing the species barrier into cats had implications on the risk that BSE might be transmissible to humans. Dr MacOwan replied as follows: ‘I do not think so… It is true it had crossed a species barrier to different species and that was unusual… I do not think there is a simple logic that can be applied to species susceptibility. So while I found it a very interesting situation, and a confirmation… that probably BSE was spreading through food, I did not immediately think that it was therefore an increased hazard for people.’ This was explored further in the oral evidence of Dr Bostock (T44, Vol. T5, Tab 4, pages 131 and 132). After explaining the unpredictability of transmission of agents, Dr Bostock was asked whether when finding BSE is transmissible to a cat was that ground for concern that there is a possibility that it is transmissible to a human. Dr Bostock replied, ‘I do not think it alters the position of whether or not it is transmissible to humans. The concern should have been there in the first place.’ Of course, it was because of the ‘concern’ referred to by Dr Bostock (albeit a concern of a remote risk of transmission of BSE to man) that the control measures, and more particularly the slaughter and destruction of affected cattle and the SBO ban, had been put in place. In any event on 10th May 1990 it was simply not known that the spongiform encephalopathy in the first cat was a TSE.’
 
29. Press reports on 15 May 1990 suggested that many education officers were considering taking British beef off school menus because they were dissatisfied with answers to their questions about its safety. The newspaper Today reported confusion as to the source of advice on this issue: [36]
 
‘a) The Education Department was reported as referring local authorities to MAFF and the Department of Health;
 
b) A MAFF spokesperson was reported as saying this was a matter for the Department of Health;
 
c) Department of Health spokesperson was reported as saying that, ‘if a warning was necessary, it should come from the Ministry of Agriculture.’
 
30. On 15 May 1990, several authorities followed Humberside County Council in imposing a complete or partial ban on the serving of beef and beef products in schools. The action taken varied considerably between authorities. For example:
 
a) Staffordshire Council said it would ban beef sausages, beefburgers and mince, but would allow cuts from joints to remain on the menu. b) Liverpool City Council banned all beef, both UK and foreign, and said it was considering a ban on lamb because of concern about infection from scrapie. c) Richmond-Upon-Thames Council withdrew beef from primary and junior schools, but not from secondary or tertiary schools. [37]
 
31. A supplemental statement from Mr Meldrum includes the following in relation to the press reports on the actions by Local Authorities in taking beef and beef products off school menus:
 
‘I do not believe that my public comments on or around 10th May, 1990 when looked at in their correct context discounted the possibility that BSE had been transmitted naturally to a cat. Further, I do not believe that the public were misled by reports of those comments. If anything, the actions of Local Authorities in taking beef and beef products off school menus demonstrate that reports in the press of my comments did not mislead the public into thinking that ‘BSE posed no risk to human health’, but had quite the opposite effect.’[38]
 
32. On 15 May 1990, MAFF issued a news release entitled ‘British Beef is Safe, Gummer’. This news release included text from Mr Gummer’s written answer to a Parliamentary Question from William Hague MP and includes the following:
 
‘British beef is perfectly safe to eat John Gummer, Food Minister, announced today.
 
In a written answer to a Parliamentary Question from to William Hague, MP, (Richmond, Yorks) Mr Gummer said:
 
“The wide ranging measures adopted by the Government have been based on the best scientific expertise available. These experts concluded that the risk to humans is remote but action has been taken;
 
1. to slaughter and destroy all affected cattle so that no part can enter the human food chain;
 
2. specified offals, including the brain and spinal cord, must be removed from healthy cattle slaughtered for human consumption;
 
3. banning of the use of these offals as food or in food products.
 
“The removal and banning for food of these offals is a precautionary measure which goes beyond the scientific advice. These actions fully protect the public from what is a remote and theoretical risk.
 
“The situation is however being kept under constant review and hence the Committee under Dr Tyrrell has been constituted as a permanent committee to examine new information and concerns and to ensure that the advice on which the Government acts is kept fully up-to-date.
 
“A major research programme has been in progress since the disease was first identified in 1986. Over £2 million has already been spent. A further £12 million, has been allocated to projects, devised on the basis of advice from the Tyrrell Committee, for the next 3 years. All the results of this research will be made publicly available.
 
“The source of the disease has been cut off with the ban, from July 1988, on the feeding of ruminant material to ruminants. Because of the long incubation period cases will continue to occur form some years. We intend to eradicate this disease in cows but this will obviously take time. In the meantime the action taken demonstrates the Government’s absolute commitment to protecting and reassuring the public.
 
“Local Education Authorities can therefore continue to provide beef in school meals with complete confidence. As the Minister responsible for food safety for the whole nation let me reiterate the British beef is perfectly safe to eat. This is the view not only of our top scientists but also European Community experts.
 
“The Ministry of Agriculture, Fisheries and Food is committed to making public all the information that we have about these matters. That is why we gave out the information about the one cat (out of Britains’ 7 million) diagnosed to have an encephalopathy. There is nothing to link that encephalopathy with BSE or scrapie or to suggest that it is transmissible. However, we were determined that all this should be in the public domain and we shall continue our investigations, the results of which will also be announced.”’[39]
 
33. Also on 15 May 1990, Mr Gummer wrote an open letter to the Chairman of the National Consumer Council, Lady Wilcox. This letter included the following:
 
‘In view of the considerable current discussion about BSE, I am sure it would be useful to give further clear advice to consumers on this subject. I am accordingly setting out below comprehensive advice which I do hope you will feel able to bring to the attention of interested organisations.
 
The disease
 
BSE is a brain disease of cattle which belongs to the same group of diseases as scrapie, which has been in our sheep population for over 250 years. The most likely cause of BSE was the feeding to cattle of compound rations which contained protein material derived from sheep, some of which were infected with scrapie. The practice of feeding ruminant protein to ruminants, including cattle, sheep and deer, was banned in July 1988 so the source has been cut off.
 
Human health
 
There is a very rare disease in humans called Creutzfeldt Jacob Disease (CJD) which has an incidence of less than 1 in a million people and world wide distribution. Despite scientific studies over many years, a link has never been found between scrapie and the human disease CJD, which occurs in countries where scrapie does not exist and which has been known to affect a lifelong vegetarian. There is no reason to believe BSE will be any different from scrapie and independent experts have concluded that BSE is most unlikely to have any implications for human health.
 
Other species
 
Some ruminant zoo animals have been reported with a scrapie-like disease which they aquired from the same food source as cattle. There have been cases reported outside the UK in mink, again traced to infected feedingstuff fed on a continuous basis. There is no record of pigs or poultry ever getting a similar disease. A recent post mortem of a cat in the UK showed a brain condition which is being further investigated. There is at present no evidence that it is transmissible or connected with other animal brain diseases of the scrapie family.
 
Action taken
 
Tough measures have been taken to ensure that the consumer is reassured and protected:-
 
- All cattle suspected of having BSE are slaughtered on farm and destroyed so that no part can enter the food chain. Farmers receive full compensation for their animals.
 
- As an additional safeguard, tissues that might contain the agent which causes the disease (brain, spinal cord, spleen, thymus, tonsils and intestines) in healthy cattle are also banned from any use in human food. Cattle under 6 months are exempt since they have not been fed ruminant protein and no cases of scrapie have been found in very young animals.
 
Research
 
From the beginning extensive research has been undertaken. In fact the Government will be spending more than £12 million over the next three years on BSE and its related diseases. The results will contribute to our knowledge about these diseases and will help in our efforts to eradicate BSE.
 
Beef is safe
 
The precautionary measures taken go further than experts thought necessary to deal with any BSE risk, however remote and theoretical. Added to which studies on scrapie show that the agent which causes the disease is not found in meat. British beef in therefore not a public health risk and can be eaten with complete confidence – a view endorsed by the European Community’s top scientists.
 
In view of the public interest in this matter I am releasing the text of this letter to the Press.’[40]
 
34. In a supplemental statement, Mr Meldrum referred to press reports on the actions by Local Authorities in taking beef and beef products off school menus and statements made by the Minister (Mr Gummer) and the CMO on the safety of beef and on the launch of the Agriculture Select Committee Inquiry. Mr Meldrum stated:
 
‘Although the first case of a spongiform encephalopathy in a cat may have renewed public and media interest in general and gave rise to the need for the statements on the safety of beef to be made, these were not events which can be said to be public pronouncements by me. In fact I left for a meeting of the OIE in Paris on 12th May, 1990 and was not in the country at the time that Mr Gummer and the CMO made their statements on the safety of beef, so I was not involved in any public pronouncements at that time. So far as I can recall I only briefed the press on the 10th and 11th May 1990.’[41]
 
35. On 17 May 1990 SEAC held an emergency meeting.[42] It discussed, among other things, the first case of a spongiform encephalopathy in a cat. The possibilities identified by SEAC were:
 
(A) this was a feline disorder in its own right with no association with BSE or scrapie. (B) It was feline scrapie, neither (A) or (B) having been recognised previously in view of the rarity of cat neuropathological studies. (C) It was feline BSE.
 
36. SEAC felt that, as at that time there had only been one case, it would be premature to draw conclusions without further data being available. The secretariat (Dr Pickles) reassured SEAC that there were no parenteral human medicinal products of feline origin and the Health & Safety Executive had been alerted to the potential for occupational exposure to cat nervous tissue.
 
37. A supplemental statement supplied by Mr Meldrum contains the following comments in relation to the seeking of advice from SEAC on the implications of the diagnosis of a spongiform encephalopathy in a cat:
 
‘As Mr Lowson's minute of 9th May, 1990 (YB90/5.9/3.1-3.2) records, consideration was being given to asking the newly formed SEAC to consider the implications of the case. However, as Dr Pickles' minute to Dr Metters of 10th May, 1990 (YB90/5.10/4.1-4.2) explains, the newspapers already knew about the case and as a result MAFF had to release details on 10th May, 1990. Dr Pickles' minute also records that Dr Tyrrell was on holiday at the time but that other members of SEAC ‘are being informed on the phone’. The emergency meeting of SEAC could not take place until 17th May, 1990 and in the meantime MAFF, in accordance with the policy of openness, had to release the information and hold a Press Briefing. It would have been totally unacceptable for me to have refused to comment on the case at all in the face of the intense media interest. It seems likely that such ‘secrecy’ would have been interpreted and reported in the press in an even more alarmist manner than occurred in any event, although despite attempts to be open on the issue of the feline SE there were still unfounded allegations of secrecy in the media. For example, the Inquiry is referred to the letter dated 20th July, 1994 from the University of Bristol to the Editor of The Sunday Times. In the letter the University of Bristol stated, ‘[t]here is no conspiracy of silence in this matter. On the contrary, every channel of scientific publicity has been used. MAFF has never objected to publication. We have also taken steps through the Press Association to see that the public are adequately informed’. Further, if MAFF had decided to wait until SEAC had reviewed the discovery and information on it had been made public by the press in the mean time (as occurred on 10th May, 1990), this would have been even more damaging. In the event this would have achieved nothing since SEAC made little comment and no recommendations and in a general sense followed the line that I had taken at the Press Briefing.’[43]
 
38. When Mr Gummer, Mr Meldrum, Mrs Attridge (MAFF) and Dr Pickles gave evidence to the Agriculture Select Committee on 23 May 1990, the following exchange took place:
 
‘(Mr Martlew) Minister, you are well aware there is a considerable degree of concern amongst pet owners in the country with regard to the safety of their domestic animals, especially cats and dogs. I wonder if you could give me an update on the situation? I wonder if you could tell the Committee how many dead cats are actually suspected of dying of the equivalent of BSE and whether any tests are being carried out at the moment on dogs?
 
(Mr Gummer) Well, there are two confirmed cases of cats who have died of encephalopathy and one which is being investigated at this moment. [I] have announced that information already, I have no further information at this point, although some newspapers have suggested some other things I have to tell you that is the information I have got. As you know I published the results of the first cat investigation and I shall publish these results, as I said, once they are confirmed generally. There is a real problem about these encephalopathy, we know that cats are susceptible to encephalopathy, you can experimentally show that, but what you cannot tell is whether they have always had encephalopathies. This is a difficult issue. We do know that cats have been found to have diseases, everybody has then rushed about saying: ‘Gosh, there is this new disease’ and then later it has been discovered that in fact the cats have, as far as we know, always had that disease. Of course, cats are not normally investigated. Dead cats, if I may say so, are not very often post-mortemed, if that is the right word. I questioned this advice because I said: ‘What about a cat suspected of rabies, would you not pick it up while you were doing the rabies?’ and I am told the mechanism for looking at a rabid cat is such that you would not necessarily, indeed you would be unlikely to find the encephalopathy you now find when you are looking for it. Indeed, each of the three cats that have so far been either suspected or confirmed of having an encephalopathy were only suspected because of the arguments about BSE and because we asked vets to look out for these things in any case, certainly after the first one. The vet who found the first one said that he was drawn to it by thinking of what he had seen on the television and the rest of it. We are in the difficulty of first not knowing whether cats have always had this and, therefore, that we caught not to be looking for some sort of new cause of it. Secondly, there is certainly no evidence whatsoever that it is connected with feed. Thirdly, I must say the connection with feed is difficult to see at this moment because of the home background of the different cats whose feeding habits are very different indeed and the fact that the feed industry has taken as to 95 per cent of its members, and probably very much nearer 100 per cent because the smaller firms have followed that advice, particular measures to protect its feed from any possible contamination. I find it very difficult to say other than we have had these cases, that the reason that we have had these cases is largely because of the concern about BSE but there is absolutely no evidence at all that it is connected with BSE and may be another line of these things, like other separate lines, and we shall have to find out.’[44]
 
39. An annex to a memorandum submitted to the Agriculture Select Committee in May 1990 by Professor Richard Lacey and Dr Stephen Dealler includes the following:
 
‘Cats appear to be now at risk of a spongiform encephalopathy (possibly to be called feline spongiform encephalopathy). This was not recorded before the appearance of BSE and sheep offal had been fed to cats from any years. It would be likely that FSE and BSE are linked in some way. Prospective and retrospective controlled (if possible) studies should take place in which cats fed with scrapie and BSE infected meat should be examined using histopathological and PrP isolation techniques to test for infection. These experiments should be funded and organised independently from the food, animal food, or farming industries and the Government. Carnivores were not previously thought to be at risk of catching a prion encephalopathy by mouth and this was thought to be reasonable because of Darwinian selection theory. If cats were found to be at risk of catching FSE from cattle then this would confirm that BSE has a different infective spectrum from scrapie; a very serious finding.’[45]
 
40. When Sir Richard Southwood gave oral evidence to the Agriculture Select Committee, he said:
 
‘The case of the cat is the first case of spongiform encephalopathy ever in a cat. Well, it appears to be – but if poor moggie got a bit of the staggers, you took it along to the vet and said it was not very happy, it would be put to sleep in the past and no one would have paid £300 to have a post mortem undertaken. So it is just possible that these were occurring before and were in fact caught from moggie being fed scrapie offal from sheep. I cannot say.’[46]
 
41. When Dr Tyrrell gave oral evidence to the Agriculture Select Committee, the following exchange took place:
 
‘(Mr Marland) Dr Tyrrell, can you tell us what was the significance of the emergence of spongiform encephalopathy in cats?
 
(Dr Tyrrell) As a scientist, I say I do not know the exact significant, but there are several aspects that one can speak about. Firstly, it could be that cats had this sort of disease all along, and it is an increased awareness of these diseases which has meant that they have been found now. It may not be true. But I am aware of the fact that it has been discovered that cats get Aids, and nobody made this interpretation (though I am sure the disease has been occurring for many years) until awareness of human Aids made the pathologists think and the clinicians think ‘Well, could this be the equivalent?’ So it could be just recent awareness of a long continuing disease in cats. I understand informally that expert veterinary pathologists do not think that this is likely; they think that they have not seen it before. But I think this needs investigation. It would be possible, I believe, to check back over the brains collected from cats in the past and see whether somebody has missed it. It is possible. Secondly, it could be that this is what one might call a parallel epidemic to the BSE epidemic. After all, we know about animals in the zoo which were fed similar food derived from presumably similar materials, which got a disease like BSE, scrapie of Eland and so on. So this could be the parallel thing happening in cats. If you ask why, one might argue that it is clear that BSE seems to have followed the fact that these animals are now getting more and more scrapie in their diet, or they were doing, and it is a dose effect. The more that they get, the more likely that they are to get disease, the more likely it is to be noticed. The third possibility is that the agent, having passed through cattle – and in the early days it was passing from cattle back into the feed stock – has actually changed and now is capable of causing disease in cats, which it did not do before. All these things can be examined clinically and by experiment, and I believe it is extremely important that these experiments and tests are done urgently. It is my understanding that they are being set up already even though it is only a matter of weeks since the first cat was detected. I think it is going beyond the evidence to say that we really know exactly what it means, but seen in context with the other things, I think it just reinforces the wisdom of saying that the policy should be to exclude this type to ruminant material from the human food chain, which is – of course, the policy.
 
(Mr Marland) But do you think there is any evidence that this is actually the cause of it?
 
(Dr Tyrrell) No.’[47]
 
42. On 24 May 1990 Dr Pickles minuted Dr McInnes, providing an ‘update on events since the meeting with CMO yesterday morning.’[48] The minute was copied to Dr Metters, Miss Pease, Dr Jones and Mr Otley. Attached to the minute is a transcript of a radio interview Mr Meldrum had given on the Wednesday 23 May.[49] According the transcript, when asked whether he believed there was a positive link between BSE and the death of some of the cats, Mr Meldrum responded as follows:
 
‘No, I don’t. No, I don’t at all. In fact there is no evidence yet that the two cases of spongiform encephalopathy in cats is related at all, to either BSE or scrapie in sheep and goats.’[50]
 
43. When Mr Meldrum was asked during this radio interview about when the public might expect to see the results of the investigations into FSE, he stated:
 
‘The investigations on transmission will take some time, up to a year. So it’ll be some time before we have a definitive answer. But at the moment we must assume this is a problem for cats and there is no association with BSE.’[51]
 
44. At its meeting on 13 June 1990,[52] SEAC had before it a paper prepared by the Animal Health Division and entitled ‘Spongiform Encephalopathies and other Species: Pigs and Poultry’. The paper noted that: ‘The Government has resisted calls to extend the scope of the ban in the firm belief that there is no scientific justification for such action’ and referred to the conclusions of the Southwood Report on susceptibility of non-mammalian species and to the absence of any record of pigs ever getting a TSE anywhere in the world. Further information was offered on feeding practices and on transmission studies specific to pigs in the scientific literature. It also stated that a literature search for spongiform encephalopathies was in progress but the vast number of references found and the vagueness of most titles had so far prevented the identification of transmission studies specific to pigs and poultry; although they were aware of two such studies. Finally, the paper described current transmission studies: pigs had been inoculated with BSE in February 1989 and oral exposure of pigs began in May/June 1990. SEAC agreed that the issue needed further study. Points to be considered were that pigs would have received the same exposure to the BSE agent as cattle; that most pigs were slaughtered before the disease was likely to express itself; and that bovine material in the gut contents of slaughtered pigs might be used in MBM fed to cattle, but the probability of the agent being recycled back into cattle was small.[53] SEAC also considered the possible human health implications from the recent diagnosis of a spongiform encephalopathy in three cats, noting that specialists at Bristol believed this to be a new condition rather than one which had hitherto not been recognised. The minutes of SEAC’s meeting include the following:
 
“At present there was simply no way of knowing whether the condition was a species adapted encephalopathy which had not previously been observed; a scrapie-based disease which had spread to cats in the same way as BSE had spread to cattle; or a BSE-based disease acquired as a result of consuming bovine material. The Committee was in no position to offer advice on the implications for human health until more was known about the condition.”[54]
 
45. A supplemental statement from Mr Meldrum includes the following:
 
‘Whilst at subsequent meetings SEAC continued to receive updates on the FSE situation and discussed issues of surveillance and research, no specific observations or recommendations on the risk to humans were made. That remains the position to the present day.’[55]
 
46. A supplemental statement from Mr Meldrum includes comment on the University of Bristol results (see paragraph 10 above). [56]
 
[1] YB 90/5.9/3.1-3.2, paras 2 - 6 [2] YB90/5.09/2.1-2.4 [3] YB90/5.10/4.1 [4] YB 90/05.10/7.1 [5] YB 90/05.10/7.1 [6] S Meldrum 7 (WS No. 184E) Section F para 36 [7] S Meldrum 7 (WS No. 184E) Section J para 9 [8] YB 90/5.10/2.1 [9] YB90/5.10/2.1-2.3 [10] S Meldrum 7 (WS No. 184E) Section J para 6 [11] S Meldrum 7 (WS No. 184 E) Section J. para 4. [12] BBC 1 Six O’ Clock News on 10 May 1990, time stamped 18.18.14 [13] S Meldrum 7 (WS No. 184 E) Section J, para 2 (c) [14] YB 90/5.11/3.1-3.2; YB 90/5.11/4.1; YB 90/5.11/5.1 [15] YB90/5.11/12.1 [16] YB90/5.11/12.1 [17] YB90/5.11/12.1 [18] YB90/5.11/12.1 [19] YB90/5.11/12.1 [20] YB90/5.11/10.1-10.5 [21] S Meldrum 7 (WS No. 184E) Section J para 1 [22] S Meldrum 7 (WS No. 184E) Section J para 1 [23] S Meldrum 7 (WS No. 184E) Section J para 3 [24] YB90/5.11/1.1-1.2 [25] S 65A Wells para 103 [26] S Meldrum 7 (WS No. 184 E) Section J, para 2 (d) [27] S Meldrum 7 (WS No. 184 E) Section J, para 10 [28] YB90/5.11/12.1 [29] YB90/5.11/12.1 [30] YB90/5.11/12.1 [31] YB90/5.11/12.1 [32] YB90/05.13/1.1 [33] YB90/05.13/1.1 [34] S Meldrum 7 (WS No. 184 E) Section J, para 7. [35] S Meldrum 7 (WS No. 184 E) Section J, para 8. [36] YB90/5.15/28.1 [37] These actions were reported in The Guardian newspaper on 16th May 1990; YB 90/5.16/31.1 [38] S Meldrum 7 (WS No. 184 E) Section J, para 5 [39] YB90/5.15/14.1-14.2 [40] YB 90/5.15/16.1-16.3 [41] S Meldrum 7 (WS No. 184E) Section J para 2(e) [42] YB90/5.17/1.1 – 1.4, S 184A Meldrum, para L6 [43] S Meldrum 7 (WS No. 184E) Section J para 11 [44] IBD7, pages 14-15 [45] IBD7, page 24 [46] IBD7, page 62 [47] IBD7, pages 78-79 [48] YB90/05.24/3.1-3.2 [49] YB90/5.24/3.3-3.6 [50] YB90/5.24/3.3 [51] YB90/5.24/3.6 [52] SEAC 3/4 [53] YB90/6.13/1.3 [54] YB90/6.13/1.2 [55] S Meldrum 7 (WS No. 184E) Section J para 13 [56]S Meldrum 7 (WS No. 184E) Section J para 4
 
=============================================
 
 
Feline Spongiform Encephalopathy A total of 89 cases of spongiform encephalopthy have been confirmed in domestic cats, the last one in 2001.
 
Year 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 Cases 12 12 10 11 16 8 6(1) 6(2) 4(2) 2(1) 1(1) 1(1)
 
Figures in brackets indicate the number of animals that can be clearly identified as having been born after September 1990 (i.e. the date the ban on the use of specified bovine offals was extended to any animal feed).
 
 
PUBLIC PRONOUNCEMENTS BY THE CMO,
 
MAY 1990
 
1. On 9 May 1990, Dr Pickles put a submission to the Parliamentary Under Secretary, Mr Stephen Dorrell, to inform him that spongiform encephalopathy had been confirmed in a domestic Siamese cat.1 The submission was copied to the CMO (Sir Donald Acheson), DCMO (Dr Metters), Mr Baker of the Information Division and other health officials. The covering minute stated:
 
‘First thoughts are that this new information does not affect our views on the lack of hazard of BSE to man. Although humans are in close contact with cats these disorders are not spread by intimate contact. However, the implications of this new information needs [sic] to be considered by the full Tyrrell Committee.’2
 
2. Dr Pickles said the concerns for MAFF included:
 
* BSE has spread to yet another species: how many more?
 
* Whether it should still be permissible to include offal in pet food.
 
* Renewed pressure to ban the feeding of processed sheep and cow offal to pigs and poultry.
 
* Could there be spread between cats or to other species from cats?’3
 
3. Dr Pickles noted:
 
‘Details of the case will be published next week. They [MAFF] plan a low key press statement in the next day or two in anticipation of the news breaking. We are in close contact with MAFF and are asking them to discuss this urgently with our joint outside experts.’4
 
1 YB 90/5.09/2.1-2.4
 
2 YB 90/5.09/2.1
 
3 YB 90/5.09/2.1
 
4 YB 90/5.09/2.1
 
3
 
4. On 10 May 1990 Dr Pickles sent a minute to Mr Baker of the Department of Health Information Division.5 She said:
 
‘As requested, I provide a Q&A brief to cover the human aspects of the cat with spongiform encephalopathy. MAFF should be asked to handle detailed enquiries on the implications for cats and other species.’ This brief was copied to Dr Metters, Mr Maslin and others. It considered the routes for transfer of FSE to humans.
 
5. A more detailed minute was sent by Dr Pickles to Dr Metters and copied to the CMO on 10 May 1990.6 Dr Pickles said ‘[t]he Siamese cat in Bristol presented with ataxia and postural changes but no behaviour changes (so not “mad mog” disease after all). The neuropathologist at the vet school was surprised to find spongiform changes.’ Dr Pickles also noted that the case was being written up for ‘next week’s Veterinary Record’ but as the Sun newspaper already knew about it, MAFF were to release details that afternoon. She went on to say:
 
‘Previously there have been no natural cases of spongiform encephalopathies described in cats. Experimenters have succeeded in transmitting – although infrequently – CJD by intracerebral inoculation to cats, but have not succeeded with scrapie. The Bristol Veterinary School are particularly interested in spongiform encephalopathies and may have picked up lesions that would have been missed by others. It is quite likely that previous cases in cats – particularly if the clinical presentation found with this case is typical – would have been missed.
 
The implications have to be considered at leisure by the new Tyrrell Committee. Dr Tyrrell is on holiday but the others are being informed on the phone. Assuming further investigation does not question the diagnosis or reveal unusual routes by which this cat could have been infected, there are two main possibilities. Firstly, that cats have been susceptible to ingestion of infected meat for years and cases previously have gone unrecognised. Secondly, a new agent, presumably BSE, is virulent in a way in which previous agents such as scrapie have not been. The second possibility is most unlikely, but more worrying since it challenges the assumptions we have made for humans by analogy with scrapie.’
 
Dr Pickles stated that it was ‘most unlikely’ that a new agent ‘presumably BSE’ was virulent in the way in which previous agents such as scrapie had not been.
 
6. In his statement to the Inquiry, Sir Donald Acheson stated:
 
‘I heard of the first recorded case of FSE in a report from Dr Pickles dated 9 May 19907. Although I was aware that scrapie material had transmitted to mink I nevertheless remained deeply concerned about the possible
 
5 YB 90/5.10/3.1-3.3
 
6 YB 90/5.10/4.1-4.2
 
7 YB 90/5.9/2.1-2.4
 
4
 
implications of a further transpecies 'jump' of BSE. On the basis of the information in Dr Pickles' report and her second report dated 10 May8 it was not immediately clear whether this represented a new disease, possibly caused by BSE infected pet food, or was a naturally occurring case of SE, previously unrecognised in cats. In either case it was important to get expert advice as soon as possible. I therefore gave instructions that although the first meeting of SEAC had just occurred and a second had been arranged for 2 July, an additional emergency meeting must be called. The first practicable date was 17 May. It was my earnest hope that I would not have to make a public statement on the significance of the case of FSE before having the advantage of the advice of SEAC on that date.’9
 
SNIP...
 
 
14. On 4 August 1992 Dr Wight, the Senior Medical Officer in HEF(M)1, minuted Dr Roger Skinner and Ms Lockyer. Dr Wight stated that the Department of Health had recently been made aware on a medical in confidence basis of a case of probable CJD in a 55 year old farmer who had had a dairy cow with BSE on his farm. In her minute Dr Wight stated: ‘I must emphasis that the diagnosis has not been confirmed and this is the line we would need to take. There is no direct evidence that the two events (BSE and CJD) are linked and Dr Will feels they are probably a
 
15 S Calman 1 (WS No. 179) para 44
 
16 S Calman 1 (WS No. 179) para 45
 
17 IBD15 (vol IBD2 tab 4 page 16)
 
7
 
coincidence. However we cannot be sure, and the media could well seize upon it before or after the patient dies.’ 18
 
15. On 13 August 1992 Dr Wight minuted the CMO and asked him to consider speaking to the CVO personally about this development given the need for confidentiality.19 This minute also set out a suggested line to be adopted, which was that there was no scientific evidence to link BSE with CJD and that the information about the dairy farmer did not change that: ‘We are aware of the development of probable CJD in a farmer, though the diagnosis has not been confirmed. To date, there is no scientific evidence to link BSE with the human illness CJD and the information available in this case does not change this situation.’20
 
16. In his statement to the Inquiry, Sir Kenneth Calman recalled: ‘The National CJD Surveillance Unit had already been notified and had advised that, if the case was confirmed, it was probably inevitable from a statistical point of view that this would happen sooner or later. … At this time consideration was being given as to whether there may be greater occupational risks to those employed in the farming and meat industry. However, SEAC considered this particular case and their views remained unchanged. It was confirmed subsequently that this was a typical sporadic case of CJD.’21
 
17. On 13 August 1992 Sir Kenneth alerted the Press Office, Ministers, MAFF and the International Division of the Department of Health to this development.22
 
18. On 23 October 1992 Ms Diana Dunstan, Director of Research Management (MRC), presented Sir Kenneth with a paper outlining the results of a series of ongoing experiments that revealed the transmissibility to marmosets of two degenerative diseases: Alzheimer’s Disease and Gerstmann-Straussler-Scheinker Syndrome.23 The paper, prepared by Dr Ridley, stated:
 
‘SAFETY At this point we would like to stress again the lack of evidence relating Alzheimer’s disease to exposure to brain tissue through neurosurgery or occupation. Nevertheless it is appropriate that the proper bodies should consider whether the results of [the] experiments have any implications for human health.’
 
18 YB 92/8.04/3.1
 
19 YB 92/8.13/2.1-2.2
 
20 YB 92/8.13/2.1-2.2, para 4
 
21 S Calman 1 (WS No. 179) para 49.
 
22 YB 92/8.13/2.1 – 2.2; S Calman 1 (WS No. 179) para 49
 
23 YB 92/10.23/1.1-1.5; S Calman 1 (WS No. 179) para 50.
 
8
 
19. Sir Kenneth directed that further consideration be given to the scientific issues and the implications for the Department of Health in view of continuing public concern and persistent media interest over the BSE epidemic.24
 
1993
 
20. On 26 February 1993 the CMO was informed of the forthcoming publication of details of the dairy farmer’s case in the Lancet 25 within ‘the next couple of weeks’.26 Administrators within the Department of Health repeated the line to take which had been suggested following the confirmation of the diagnosis of CJD in the farmer.27
 
21. On 1 March 1993 Sir Kenneth was told that the case report would be published in the Lancet medical journal on 6 March 1993.28
 
22. Mr Robert Crighton, of Mrs Bottomley’s private office, advised MAFF on 4 March 1993 that the Minister had agreed that the CMO should deal with press enquiries relating to the dairy farmer with CJD, and that it was believed that this was already in hand.29
 
23. In the Lancet article published on 6 March 1993 Dr Will et al reported: ‘This is the first report of CJD in an individual with direct occupational contact with a case of BSE and raises the possibility of a causal link. About 120 000 individuals work in dairy farming in England and Wales and over one-third of farms have had at least one case of BSE. The national incidence of CJD is about 0.5 cases per million per year and a crude calculation suggests that in the 2 ½ years since the start of our survey, we would have expected about 0.05 cases in dairy farmers with a BSE-affected herd. This calculation takes no account of other groups with increased exposure to affected animals and we have found no case of CJD in other potentially “at-risk” groups, such as abattoir workers or veterinarians. We have identified individuals with occupations (eg, vicar, art teacher) that are statistically less likely to have occurred by chance than potentially less “at-risk occupations.
 
The course of symptoms and signs in out case, the investigations (including electroencephalography) and the necropsy findings are consistent with previous experience in CJD. Risk factors for CJD, including iatrogenic transmission and genetic predisposition, have been largely excluded by the history and gene analysis. The Southwood Committee recommended surveillance of specific occupational groups
 
24 S Calman 1 (WS No. 179) para 51
 
25 S Calman 1 (WS No. 179) para 53.
 
26 YB 93/2.26/1.1-1.2
 
27 YB 93/2.26/1.1-1.2
 
28 YB 93/3.1/2.1
 
29 YB 93/3.4/2.1
 
9
 
because of the risk of direct inoculation of bovine tissue. The history suggests no such occurrence in our case and the only possible direct route of cross-contamination was by drinking milk. Milk does not contain detectable titres of infectivity, even from animals clinically affected with natural diseases and epidemiological evidence (eg, the absence of vertical transmission in kuru after breastfeeding) largely precludes milk as a route of transmission in spongiform encephalopathies.
 
CJD in our case is most likely to have been a chance finding and a causal link with BSE is at most conjectural.’30
 
24. Sir Kenneth Calman became aware through MAFF that there remained continued speculation that the BSE agent may be transmissible in milk: ‘This issue was subsequently considered by SEAC on 22 April 1993 who confirmed that epidemiological evidence from beef and dairy herds and from the human population did not suggest milk transmits the disease, and that this was supported by experimental data and therefore concluded that they were in a position to endorse the view that milk does not pose a risk.’31
 
25. Press coverage of the death of the farmer increased on 9 March32 and 10 March 1993.33 In an article in the Telegraph on 9 March 1993, Dr Will was reported as saying that this case ‘could just be a coincidence’, while Kevin Taylor (the assistant Chief Veterinary Officer) said he did not think a link between BSE and the death of the farmer was ‘even conjectural’. The Ministry of Agriculture was reported as saying ‘there are no implications for human or animal health’, in the farmer’s death.
 
26. Sir Kenneth Calman met Mr Gummer to discuss a possible press release. Sir Kenneth said in his statement to the Inquiry: ‘In view of the fact that this event resulted in intense media interest, I met with Mr Gummer to discuss the release of a Press statement to allay public fears. … We both agreed that it was necessary to reassure the public that previous advice had been reconsidered and remained unaltered…’ 34
 
27. Mr Gummer also referred to this meeting in his statement to the Inquiry: ‘I subsequently met with the CMO, Dr Kenneth Calman, to discuss these reports. We agreed that given the press speculation it was important that the CMO should issue a press release making it plain that the Tyrrell committee had considered this case, which had been reported to the
 
30 J/L/341/642
 
31 S Calman 1 (WS No. 179) para 54.
 
32 See articles in the Daily Telegraph and The Times at YB 93/3.9/1.1 and YB 93/3.9/2.1
 
33 See articles in the Daily Express, Today and the Daily Mail, on 10th March 1993; YB 93/3.10/4.1, YB 93/3.10/5.1 and YB 93/3.10/6.1
 
34 S Calman 1 (WS No. 179) para 54.
 
10
 
National CJD Surveillance Unit in August 1992, and had advised that it did not alter the advice that had previously been given.’35
 
28. On 10 March 1993 a briefing on the case of CJD in a farmer was prepared for the Prime Minister and the Lord President by Department of Health administrators.36 The briefing had been cleared with medical colleagues in HEF(M)1 and copied to Mr Maslin at MAFF. The briefing repeated the line to take which had been suggested in previous minutes relating to the death of the farmer:
 
‘The Government are aware of a confirmed case of CJD in a farmer, who had a cow with BSE. To date there is no scientific evidence to link BSE with the human illness CJD and the Tyrrell Committee have advised that the information available in this case does not change this situation.’37
 
29. In the afternoon of 11 March 1993, Sir Kenneth Calman made a statement, which repeated the assurance about the safety of beef given by his predecessor, Sir Donald Acheson, on 16 May 1990.38 Dr Metters was involved in discussing drafts of this statement and agreed with the wording selected by the CMO.39 The statement read:
 
‘Following the death of a farmer from CJD I am aware of media reports that have speculated about a link between this rare human condition and BSE.
 
I wish to emphasise that there is no scientific evidence of a causal link between BSE in cattle and CJD in humans. The Tyrrell Committee have considered the details of this case and have advised that this does not alter the advice that has previously been given.
 
That advice issued on 16 May 1990 by my predecessor, Sir Donald Acheson that beef can be eaten safely by everyone, both adults and children, including patients in hospital, remains valid.
 
The Department continues to monitor developments in this area closely and will carefully consider all new evidence as it emerges. I am confident that all necessary action has been taken to safeguard public health.
 
NOTES FOR EDITORS
 
 
*** Singeltary Submission 2016 ***
 
Alzheimer-type brain pathology may be transmitted by grafts of dura mater 26/01/2016
 
 
 
 
5.195 Among occupational groups exposed to BSE, farmers remain unusual in having such an excess over the incidence of CJD for the population as a whole. No cases of CJD have been reported amount veterinarians exposed to BSE. Four people in the meat industry (butchers, abattoirs, rendering plants, etc) have been reported to have vCJD.386 The present evidence has been accepted by some as reassuring in that such occupations may not pose as serious a risk as might have been expected.
 
 
This was not simply another farmer but the third farmer......
 
 
suspect case of CJD in a farmer who has had a case of BSE in his beef suckler herd.
 
 
cover-up of 4th farm worker ???
 
 
 
CONFIRMATION OF CJD IN FOURTH FARMER
 
 
now story changes from;
 
SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.
 
to;
 
This is not unexpected...
 
was another farmer expected?
 
 
4th farmer, and 1st teenager
 
 
2. snip...
 
Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.
 
3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...
 
 
CJD FARMERS WIFE 1989
 
 
 
20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....
 
 
THE COVER UP OF MAD COW DISEASE IN FARMERS, FARMERS WIVES, AND VICKY RIMMER, THE DAY MAD COW SCIENCE CHANGED $$$
 
Monday, May 19, 2008
 
*** SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS ***
 
 
DOES ANYONE BESIDES ME SEE A PATTERN YET ???
 
Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.
 
SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.
 
Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.
 
and there have been 16 year old die from sporadic CJD in the USA as well.
 
snip...
 
I have interviewed Mrs Rimmer at my constituency surgery
 
IF there is nothing to hide, why is there so much SECRECY? WHY is the Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are taken, surely the problem could be contained, however, as it stands the lack of investigation and interest of the possibility of B.S.E. and C.J.D. being linked is open for speculation and surely someone has to account for peoples lives! WHY is so much trouble being taken to convice people that B.S.E. and C.J.D. are not linked? Guilty Conscience perhaps ? - or cover up?
 
HOUSE OF COMMONS
 
FROM BARRY JONES, M.P.
 
22 FEBRUARY 1994
 
 
Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.
 
(now story changes that biopsy shows she does not have CJD...tss)
 
 
now story changes to ;
 
Advice
 
7. The Parliamentary Secretary is invited to note the recent statements made on __________ and the present position which remains that CJD cannot be confirmed, in this case at this stage.
 
 
3. The Medical Director at ___________________ Hospital advised the Department on 6 June that the results of ___________________ brain biopsy had been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital subsequently issued a statement to the press to this effect and this was publicised widely in the press (doc 1). News coverage which followed suggested that the statement made by ________________ Hospital had been misleading (doc 2). Enquires have been made of the Medical Director at _______________ Hospital who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR. The facts are that two pathology reports on the same piece of brain tissue were recieved. The first report indicated that CJD was unlikely, The second report indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive diagnosis could be made before a post mortem was undertaken.
 
 
MAD COW MEAL DESTROYED MY DAUGHTERS LIFE
 
A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating a contaminated burger it was claimed last night.
 
VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).
 
 
GIVE ME BACK MY LIFE
 
THEY BEGGED ME TO HUSH IT UP – GRAN’S AGONY
 
 
HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''
 
 
 
WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY
 
 
 
ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM SPORADIC CJD,
snip...
 
full text ;
 
 
 
 
 
 
Wednesday, October 09, 2013
 
*** WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, £41,078,281 in compensation ***
 
 
Thursday, July 30, 2015
 
Professor Lacey believes sporadic CJD itself originates from a cattle infection number of cattle farmers falling victim to Creutzfeld-Jakob Disease is much too high to be mere chance
 
 
MAFF statements in 1990 about a cat with a spongiform encephalopathy
 
1. On 9 May 1990, Mr Lowson (Head of Animal Health Division - MAFF) put a submission to the Minister following the confirmation of SE in a cat. The submission was also copied to Mr Bradley (Pathologist - CVL) and Mr Wells (Head of Neuropathology - CVL). Mr Lowson said:
 
‘2. Although this is the first firm recorded instance of a cat being infected by apparently natural means, it is not particularly surprising; it has been known for some time that cats are susceptible to CJD by experimental inoculation, and greater public awareness may have meant that cases that previously went unnoticed will now be suspected based on clinical symptoms. Nevertheless once this information becomes public (as is inevitable) it is bound to excite comment and a connection will be made with BSE, scrapie and possibly even with CJD.
 
3. After discussion with Mr Dugdale we recommend that, rather then wait for the story to emerge, we should take the initiative in releasing the information. This would be done by means of a letter to the Veterinary Record, but as the first issue in which it could be published would be that on Friday week, 18 May, it would be better to release the text in advance. If ministers agree, this release could be accompanied by a press briefing by officials, possibly on Friday of this week.
 
4. …We [MAFF] will … concert our approach with DOH and the British Veterinary Association, and recommend that they should both participate in the Press Briefing…
 
5. … Detailed further enquiries will be needed; it is not certain that this is a transmissible spongiform encephalopathy. More needs to be known about the circumstances in which this animal might have been infected; although it is most unlikely that further enquiries will lead to any firm conclusions.
 
6. … Clearly this case will bring into sharp relief all the various issues that have been troubling the media for some time… Above all it will be important not to encourage the public to jump to conclusions about the likely origin of this case…’1
 
SNIP...
 
2. On 9 May 1990, Dr Pickles minuted Mrs Baxter (PS/PUSS, Mr Dorrell), with copies to Sir Donald Acheson and Dr Metters, regarding the news from MAFF about the cat. Dr Pickles stated that DH were in close contact with MAFF and were ‘asking them to discuss this urgently with our joint outside experts [SEAC]’. As regards the DH’s concerns, she advised that first thoughts suggested that the case ‘does not affect our views on the lack of hazard of BSE to man… However, the implications of this new information needs to be considered by the full Tyrrell Committee’. She identified the following concerns for MAFF:2
 
* BSE has spread to yet another species: how many more?
 
* Whether it should still be permissible to include offal in pet food.
 
* Renewed pressure to ban the feeding of processed sheep and cow offal to pigs and poultry.
 
* Could there be spread between cats or to other species from cats?’ Her own assessment was:
 
…there are pressures to extend the ruminant protein ban: at present pigs and poultry receive this sort of feed. Publicity about the infected cat could increase these pressures. Such action, which would be hard to justify scientifically, would increase costs for the industry and cause perhaps insurmountable problems for abattoirs, who would find renderers no longer willing to accept offal. Many 1000s of tons of offal need to be disposed of daily’.
 
2016 PIGS TSE PRION UPDATE
 
WS-02
 
Scrapie in swine: A diagnostic challenge
 
Justin J Greenlee1, Robert A Kunkle1, Jodi D Smith1, Heather W. Greenlee2
 
1National Animal Disease Center, US Dept. of Agriculture, Agricultural Research Service, United States; 2Iowa State University College of Veterinary Medicine
 
A naturally occurring prion disease has not been recognized in swine, but the agent of bovine spongiform encephalopathy does transmit to swine by experimental routes. Swine are thought to have a robust species barrier when exposed to the naturally occurring prion diseases of other species, but the susceptibility of swine to the agent of sheep scrapie has not been thoroughly tested.
 
Since swine can be fed rations containing ruminant derived components in the United States and many other countries, we conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Scrapie inoculum was a pooled 10% (w/v) homogenate derived from the brains of clinically ill sheep from the 4th passage of a serial passage study of the U.S scrapie agent (No. 13-7) through susceptible sheep that were homozygous ARQ at prion protein residues 136, 154, and 171, respectively. Pigs were inoculated intracranially (n=19) with a single 0.75 ml dose or orally (n=24) with 15 ml repeated on 4 consecutive days. Necropsies were done on a subset of animals at approximately six months post inoculation (PI), at the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of TSE until study termination at 80 months PI or when removed due to intercurrent disease (primarily lameness). Brain samples were examined by immunohistochemistry (IHC), western blot (WB), and enzyme-linked immunosorbent assay (ELISA). Brain tissue from a subset of pigs in each inoculation group was used for bioassay in mice expressing porcine PRNP.
 
At six-months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more methods: IHC (n=4), WB (n=3), or ELISA (n=5). Interestingly, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study).
 
Swine inoculated with the agent of scrapie by the intracranial and oral routes do not accumulate abnormal prion protein (PrPSc) to a level detectable by IHC or WB by the time they reach typical market age and weight. However, strong support for the fact that swine are potential hosts for the agent of scrapie comes from positive bioassay from both intracranially and orally inoculated pigs and multiple diagnostic methods demonstrating abnormal prion protein in intracranially inoculated pigs with long incubation times.
 
Curriculum Vitae
 
Dr. Greenlee is Research Veterinary Medical Officer in the Virus and Prion Research Unit at the National Animal Disease Center, US Department of Agriculture, Agricultural Research Service. He applies his specialty in veterinary anatomic pathology to focused research on the intra- and interspecies transmission of prion diseases in livestock and the development of antemortem diagnostic assays for prion diseases. In addition, knockout and transgenic mouse models are used to complement ongoing experiments in livestock species. Dr. Greenlee has publications in a number of topic areas including prion agent decontamination, effects of PRNP genotype on susceptibility to the agent of sheep scrapie, characterization of US scrapie strains, transmission of chronic wasting disease to cervids and cattle, features of H-BSE associated with the E211 K polymorphism, and the development of retinal assessment for antemortem screening for prion diseases in sheep and cattle. Dr. Greenlee obtained his DVM degree and completed the PhD/residency program in Veterinary Pathology at Iowa State University. He is a Diplomate of the American College of Veterinary Pathologists.
 
 
Prion
 
Volume 9, Issue 4, 2015
 
Porcine prion protein amyloid
 
DOI:10.1080/19336896.2015.1065373Per Hammarströma & Sofie Nyströma*
 
pages 266-277
 
Received: 1 Jun 2015 Accepted: 17 Jun 2015 Accepted author version posted online: 28 Jul 2015
 
© 2015 The Author(s). Published with license by Taylor & Francis Group, LLC Additional license information
 
ABSTRACT
 
Mammalian prions are composed of misfolded aggregated prion protein (PrP) with amyloid-like features. Prions are zoonotic disease agents that infect a wide variety of mammalian species including humans. Mammals and by-products thereof which are frequently encountered in daily life are most important for human health. It is established that bovine prions (BSE) can infect humans while there is no such evidence for any other prion susceptible species in the human food chain (sheep, goat, elk, deer) and largely prion resistant species (pig) or susceptible and resistant pets (cat and dogs, respectively). PrPs from these species have been characterized using biochemistry, biophysics and neurobiology. Recently we studied PrPs from several mammals in vitro and found evidence for generic amyloidogenicity as well as cross-seeding fibril formation activity of all PrPs on the human PrP sequence regardless if the original species was resistant or susceptible to prion disease. Porcine PrP amyloidogenicity was among the studied. Experimentally inoculated pigs as well as transgenic mouse lines overexpressing porcine PrP have, in the past, been used to investigate the possibility of prion transmission in pigs. The pig is a species with extraordinarily wide use within human daily life with over a billion pigs harvested for human consumption each year. Here we discuss the possibility that the largely prion disease resistant pig can be a clinically silent carrier of replicating prions.
 
SNIP...
 
CONCLUDING REMARKS Should the topic of porcine PrP amyloid be more of a worry than of mere academic interest? Well perhaps. Prions are particularly insidious pathogens. A recent outbreak of peripheral neuropathy in human, suggests that exposure to aerosolized porcine brain is deleterious for human health.43,44 Aerosolization is a known vector for prions at least under experimental conditions.45-47 where a mere single exposure was enough for transmission in transgenic mice. HuPrP is seedable with BoPrP seeds and even more so with PoPrP seed (Fig. 1), indicating that humans could be infected by porcine APrP prions while neurotoxicity associated with spongiform encephalopathy if such a disease existed is even less clear. Importantly transgenic mice over-expressing PoPrP are susceptible to BSE and BSE passaged through domestic pigs implicating that efficient downstream neurotoxicity pathways in the mouse, a susceptible host for prion disease neurotoxicity is augmenting the TSE phenotype.25,26 Prions in silent carrier hosts can be infectious to a third species. Data from Collinge and coworkers.21 propose that species considered to be prion free may be carriers of replicating prions. Especially this may be of concern for promiscuous prion strains such as BSE.19,48 It is rather established that prions can exist in both replicating and neurotoxic conformations.49,50 and this can alter the way in which new host organisms can react upon cross-species transmission.51 The na€ıve host can either be totally resistant to prion infection as well as remain non-infectious, become a silent non-symptomatic but infectious carrier of disease or be afflicted by disease with short or long incubation time. The host can harbor and/or propagate the donor strain or convert the strain conformation to adapt it to the na€ıve host species. The latter would facilitate infection and shorten the incubation time in a consecutive event of intra-species transmission. It may be advisable to avoid procedures and exposure without proper biosafety precautions as the knowledge of silence carrier species is poor. One case of iatrogenic CJD in recipient of porcine dura mater graft has been reported in the literature.52 The significance of this finding is still unknown. The low public awareness in this matter is exemplified by the practice of using proteolytic peptide mixtures prepared from porcine brains (Cerebrolysin) as a nootropic drug. While Cerebrolysin may be beneficial for treatment of severe diseases such as vascular dementia,53 a long term follow-up of such a product for recreational use is recommended.
 
 
Friday, August 21, 2015
 
Porcine prion protein amyloid or mad pig disease PSE Porcine Spongiform Encephalopathy ?
 
 
 
 
3. On 10 May 1990, Dr Pickles minuted Dr Metters about the cat. Dr Pickles offered two alternative explanations:3
 
‘The implications have to be considered at leisure by the new Tyrrell Committee. Dr Tyrrell is on holiday but the others are being informed on the phone. Assuming further investigation does not question the diagnosis or reveal unusual routes by which this cat could have been infected, there are two main possibilities. Firstly, that cats have been susceptible to ingestion of infected meat for years and cases previously have gone unrecognised. Secondly, a new agent, presumably BSE, is virulent in a way in which previous agents such as scrapie have not been. The second possibility is most unlikely, but more worrying since it challenges the assumptions we have made for humans by analogy with scrapie… …the public may start asking again about pig and poultry feed. We must be fully involved in discussions, particularly as some essential pharmaceuticals are made in pigs. The press, on being reassured catgut does not come from cats, could question where it does come from; perhaps [company name deleted] should be warned’.
 
2 YB90/5.09/2.1-2.4
 
3 YB90/5.10/4.1
 
SNIP...
 
4. On 10 May 1990, Mr Gummer (Secretary of State - MAFF) met Mr David Maclean (Parliamentary Secretary - MAFF), Mr Meldrum, Mr Lowson and others to discuss how to make public the information that a cat had been diagnosed with SE.4 A note of the meeting prepared by Mr Gummer’s private office and copied to Mr Bradley and Mr Wells amongst others, records:
 
‘[The CVO] confirmed the Minister’s assumption that there was no likely connection between this case and BSE. Nor was the illness necessarily linked with the cat’s diet. We knew that cats could be infected by the injection of high doses of CJD virus, but it had not been possible to inject them with the scrapie virus. No experiments had been done on cats using BSE virus. This was the first discovered case of a spongiform encephalopathy in a domestic cat although there had been a case involving tigers in the 1970s where it was alleged the animals had an encephalopathy. As regards the present case, the cat had fed on a wide range of pet foods including cooked fresh meat bought from a local shop. The Minister concluded from this that, if it weren’t for the general public interest in encephalopathies, the death of this cat would not be a matter of significance. However, given the public interest, it was desirable to make the information available as soon as possible.’5
 
5. In a supplemental statement, Mr Meldrum stated that he did not believe that he would have used the words “no likely connection”:
 
‘Mr Lebrecht's note of the meeting on 10th May, 1990 with the Minister (Mr Gummer) and the Parliamentary Secretary (Mr Maclean) records that I "confirmed the Minister's assumption that there was no likely connection between this case [of a spongiform encephalopathy in a cat] and BSE" (YB90/5.10/10.1). I do not believe that I would have used the words "no likely connection". I believe that it is more probable that I said that there was no known connection between the case and BSE. This was the wording that I used in my letter to the Veterinary Record which was attached to MAFF's Press Notice dated 10th May, 1990 (YB90/5.10/2.1- 2.3). In truth it is the letter to the Veterinary Record that must carry the greatest weight since that was the information that was being given to the public and not the note of the meeting which was essentially for internal use within MAFF.’6
 
6. In a supplemental statement, Mr Meldrum noted that Mr Wells and Mr Bradley received copies of Mr Lowson’s minute of 9 May 1990 and Mr Lebrecht’s note of the meeting on 10 May 1990. Mr Meldrum continued: ‘The points were made in Mr Lowson's minute that it was not certain that this was a case of a TSE, that it was important not to encourage the public to jump to conclusions about the origin of the case, ‘defensive’ briefings would be prepared and there was nothing further to be done apart from bringing it to the attention of the veterinary profession. Mr Lebrecht's note
 
4 YB 90/05.10/7.1
 
5 YB 90/05.10/7.1
 
6 S Meldrum 7 (WS No. 184E) Section F para 36
 
6
 
contained the record that I ‘confirmed the Minister's assumption that there was no likely connection’ between the case and BSE (although as I have already commented, ‘likely’ is not a term that I believe I would have used nor was it a term that I used at the Press Briefing) and the Minister's conclusion that, if it wasn't for the general public interest at the time, the death of the cat would not have been a matter of significance. Yet neither Mr Wells nor Mr Bradley (on behalf of Mr Wells) nor any more senior officer at the Central Veterinary Laboratory appear to have raised any concerns as a result of the points made in these two documents about the approach being taken to making the information public.’7
 
7. A News Release was issued by MAFF on 10 May 1990, which stated: ‘Pathologists at the Bristol Veterinary School and MAFF's Central Veterinary Laboratory at Weybridge have diagnosed a sub-acute spongiform encephalopathy in a five-year old Siamese cat originating from the Bristol area. Keith Meldrum the Ministry's Chief Veterinary Officer has written to the 'Veterinary Record'’. 8
 
8. Attached to the 10 May press release was a letter from Mr Meldrum to the Veterinary Record9. In this letter Mr Meldrum stated:
 
‘Inquiries into the case will continue, but there is no evidence that the condition is transmissible nor is there any known connection with the other animal encephalopathies.’
 
9. A supplemental statement from Mr Meldrum includes the following: ‘As my letter in the Veterinary Record (attached to the MAFF Press Release of 10th May, 1990; YB90/5.10/2.1-2.2) stated, "[i]nquiries into the case will continue, but there is no evidence that the condition is transmissible nor is there any known connection with the other animal encephalopathies". This most certainly does not discount possible transmission of BSE to cats but clearly states the true position; that it was not known. A quote similar to that in the Veterinary Record letter was reported in the article in The Daily Telegraph on 11th May, 1990 (YB90/5.11/12.1) and, in addition to reporting my comments at the Press Briefing, the article in The Times on the same day (YB90/5.11/12.1) also referred to the fact that it was not yet known if the disease was transmissible and experiments were being carried out. A perhaps more balanced report of the 10th May, 1990 Press Briefing was published in Nature on 17th May, 1990 (J/N/345/194). The article notes my comment that ‘there is no evidence for a connection with similar diseases in other animals’ and goes on to explain that MAFF ‘is to commission research to see if the cat spongiform encephalopathy can be passed to laboratory mice. This should reveal if the cat infection behaves similarly to BSE and scrapie…’.’10
 
7 S Meldrum 7 (WS No. 184E) Section J para 9
 
8 YB 90/5.10/2.1
 
9 YB90/5.10/2.1-2.3
 
10 S Meldrum 7 (WS No. 184E) Section J para 6
 
7
 
10. In recalling his press briefing of 10 May 1990, Mr Meldrum states:11 ‘…the position at that time was that the first case ever of a naturally occurring spongiform encephalopathy in a cat had been confirmed, the disease had not been confirmed as being transmissible (i.e. as being a TSE the same as BSE and scrapie), it was not known how the cat had been infected and whether any more cases could be expected and this was only one cat death out of seven million cats in the U.K. As stated in paragraph 36 of the section above on “Ministers’ meeting on 10th May, 1990”, it would have been singularly unwise for me to conclude on the basis of the laboratory examination of the brain of one cat that BSE had crossed the species barrier from cattle to cats. It was not until 1991 that it was indicated by the University of Bristol experiments that the spongiform encephalopathy in cats was transmissible (verbally notified in June 1991 and the written interim report in September 1991). Whilst some scientists (for example, Mr Wilesmith; T52, Vol. T6, tab 2 page 117) might suggest that it could have been considered in May 1990 that it was "highly likely" that the disease was a TSE, this was not communicated to me as far as I can recall. Until scientific confirmation of transmissibility was obtained it would not have been sensible for me to publicly state that this one case was a TSE; no one knew this to be the case at the time. In the event that this had not proved to be the case, such a statement would have caused unnecessary concern and damaged both my and MAFF's credibility at what was a very difficult time.’
 
11. On 10 May 1990, comments made by Mr Meldrum about the discovery of spongiform encephalopathy in a cat were reported on BBC1 News at Six. Mr Meldrum said12:
 
‘This is only one cat death out of seven million cats in the UK, and there is no reason or cause for concern at all. If we hadn’t got the other encephalopathies in animals in this country, this report would have been published without comment’.
 
12. A supplementary statement from Mr Meldrum commented on the above quote:13
 
‘The draft DFA includes a quote taken from an extract from the ITN ‘News at 10’ on 10th May, 1990, which appears in the television programme ‘Mad Cows and Englishmen’. The full extract in ‘Mad Cows and Englishmen’ includes this introductory sentence from Julia Somerville (presenting the ITN News) before cutting to a clip of me speaking: ‘[e]xperts at the Ministry of Agriculture are trying to find out if there is a link between the illness of a pet cat and the Mad Cow Disease BSE’. In any event, the ‘Mad Cows and Englishmen’ extract is in itself a highly selective and edited presentation of the ITN ‘News at 10’ on 10th May, 1990, with the opening titles for the ‘News at 10’ following with the single sentence from Julia Somerville and cutting straight to a clip of me at the
 
11 S Meldrum 7 (WS No. 184 E) Section J. para 4.
 
12 BBC 1 Six O’ Clock News on 10 May 1990, time stamped 18.18.14
 
13 S Meldrum 7 (WS No. 184 E) Section J, para 2 (c)
 
8
 
Press Briefing on 10th May, 1990. In actual fact the report on the ‘pet cat’ only appeared about half way through ‘News at 10’ after reports on Michael Heseltine, the poll tax, the inquiry into the Kegworth air crash and safety problems in the Channel Tunnel. So the document uses a selective quotation from a television programme containing a highly edited clip from another television programme which only contained a shortened version of an entire interview given by me at a full Press Briefing.’
 
13. On Friday 11 May 1990, the story of the Bristol cat was covered in the national press.14 The Times published an article entitled ‘Mad cow symptoms found in pet cat’ on 11 May. The article reported that it was not yet known how the cat had become infected or whether the feline form of the spongiform encephalopathy could be transmitted between different species and that scientists were investigating whether it could be passed to laboratory mice. Mr Meldrum was reported to have said:
 
‘There is no need for pet owners to change their pet food or to consider putting their cats down. The risk to man is no greater than it was before diagnosis.’15
 
Mr Meldrum was also reported to have said:
 
‘Such findings have not previously in domestic cats at this stage, this is the only known case in Britain.’
 
14. Also on 11 May, the Daily Telegraph published an article entitled ‘Mad cow disease check as cat dies’. The article stated that according to Mr Meldrum, the spongiform encephalopathy diagnosed in the cat had not been seen to occur naturally in cats before.16 The article stated: ‘Mr Meldrum ruled out having the other animals in the affected house put down. He stressed that there is no danger to owners of cats and said that there is no known dog encephalopathy.’17
 
SNIP...
 
 
HOUND STUDY
 
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
 
snip...
 
 
Date: Tue, 27 May 2003 08:07:58 –0500
 
Reply-To: Bovine Spongiform Encephalopathy
 
Sender: Bovine Spongiform Encephalopathy
 
From: "Terry S. Singeltary Sr."
 
Subject: FDA BSE Update - Pet Food from Canadian Manufacturer & MAD DOG DATA
 
######## Bovine Spongiform Encephalopathy #########
 
Statement
 
FOR IMMEDIATE RELEASE Statement May 26, 2003
 
Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA
 
FDA BSE Update - Pet Food from Canadian Manufacturer
 
The Food and Drug Administration (FDA) has learned from the government of Canada that rendered material from a Canadian cow that last week tested positive for bovine spongiform encephalopathy (BSE, also known as mad cow disease ) may have been used to manufacture pet food, specifically dry dog food, some of which was reported to have been shipped to the United States. The Canadian government prevented the BSE positive cow from being processed for human food. Therefore, consumers can be assured that their food does not contain any remnants of the BSE positive cow.
 
It is also important to stress that there is no scientific evidence to date that dogs can contract BSE or any similar disease. In addition there is no evidence that dogs can transmit the disease to humans.
 
FDA notified the U.S. pet food firm, The Pet Pantry International, of Carson City, Nevada, when FDA learned that the pet food that the firm received may have included rendered material from the BSE positive cow. The manufacturer of the pet food is Champion Pet Food, Morinville, Alberta. Even though there is no known risk to dogs from eating this dog food, as a prudent measure to help assure that the U.S. stays BSE free The Pet Pantry International is asking its customers who may have purchased the suspect product to hold it for pickup by the distributor so that the dog food will not mistakenly be mixed into cattle or other feeds if any of the dog food is discarded or otherwise not used to feed dogs. The suspect dog food was produced by Champion Pet Food between February 4, 2003, and March 12, 2003.
 
The Pet Pantry products were packaged in 50 lb bags, distributed to franchises around the country, and sold by home delivery only. There was no retail distribution of the product. Consumers purchase Pet Pantry products by phone or email orders. The product is then delivered by the nearest franchisee directly to the consumer s home.
 
The product subject to this notification includes Maintenance Diet labeled with a use by date of 17FEB04 and Beef with Barley with a use by date of 05MAR04 . Consumers who have purchased dog food from The Pet Pantry since February of this year are asked to check their present supplies and see if any match the description of the product being removed. If so, consumers are asked to contact The Pet Pantry at 1-800-381-7387 for further information on how to return the product to The Pet Pantry for proper disposal. Consumers are asked not to destroy or discard the product themselves. The Pet Pantry will also use its sales records to contact consumers who purchased the affected product.
 
FDA is working closely with the Pet Pantry International to assure for proper disposal of the recovered product.
 
FDA will continue to provide updates on this case of BSE in Canada as additional information becomes available.
 
 
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
 
snip...
 
 
cases have been reported in domestic cats), are characterised by long asymptomatic incubation periods followed by progressive symptoms and signs of degeneration of the brain, leading eventually to death.
 
 
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
 
worse still, there is serious risk the media could get to hear of such a meeting...
 
snip...
 
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
 
 
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
 
snip...
 
YOU explained that imported crushed heads were extensively used in the petfood industry...
 
 
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
 
 
 
some 100+ _documented_ TSE cats of all types later...tss
 
on occassions, materials obtained from slaughterhouses will be derived from sheep affected with scrapie or cattle that may be incubating BSE for use in petfood manufacture...
 
 
Meldrum's notes on pet foods and materials used
 
 
 
IN CONFIDENCE CJD TO CATS...
 
‘’It should be noted that under experimental conditions cats succumb to an encephalopathy after intracerebral inoculation of material derived from patients affected with Creutzfeldt-Jakob Disease.’’
 
 
Confidential BSE and __________________
 
 
1st case natural FSE
 
 
FSE and pharmaceuticals
 
 
CONFIDENTIAL
 
BSE
 
CONFIDENTIAL
 
CMO
 
FROM: DR J S METTERS DCMO
 
International, Prevention and Community Services
 
14 June 1990
 
THE TYRRELL COMMITTEE
 
1. I attended the Tyrrell Committee meeting on 13 June. This was also Professor Ingrid Allen’s first attendance.
 
2. Dr Pickles will be providing a note about the Scientific discussion. My purpose is to draw attention to toher problems facing Dr Tyrrell and his Committee.
 
3. First, I am very uneasy about the relative lack of urgency and and interest that MAFF appear to hold for getting the necessary research programme on BSE and related encephalopathies started, and getting it going fast. For example, Mr Bradley of CVL said that there were difficulties in organising transmission experiments from the brain of the cat which died of an encephalopathy in Bristol. There were arguments going on about who should pay for this weork. Should it be MAFF, the Bristol Veterinary School or someone else? Dr. Tyrell was clearly exasperated.
 
4. Next we were told that...
 
snip...see ;
 
 
 
 
DOH’iOI'i 0013
 
8. These and other remarks about the lack of policy and co-ordination of the animal—related research were criticised by Committee members and led Dr Tyrrell, who was equally irritated, to ask the CVL observer and the MAFF member of the Secretariat to get these organizational problems sorted out. r
 
9. Frankly, I had not realised the situation was so unsatisfactory. While I needed little convincing that a Directed Programme would be a better way to proceed, from what I heard at the Tyrrell Committee convinces me even more of the need for one. However, it appears that without a major change of attitude at “high levels“ in MAFF, this will not happen.
 
10. The Committee discussed the two questions you had particularly asked them to-address. On the question of an epidemic of encephalopathies in cats, the consensus was that an increase in the numbers was likely and would not materially change the Committee's perception. At present, there is no data about how common a spongiform encephalopathy is among cats with neuro— logical distrubances. There is no information on whether the cat encephalopathy is trasmissible to other species, or indeed to other cats. Further work needs to be done to review retrospec- tively the brains of cats that have died of neurological disorders to see if changes similar to that observed in the Bristol and Belfast cats can be identified.
 
11. The Committee were even less forthcoming on what their reaction might be if an encephalopathy is found in another species, perhaps in dogs. Their first reaction was that, as with the cats, the first step would be to investigate whether this was really a new disease, or simply one that had not previously been recognized and to see whether it has any links to ESE, scrapie or other transmissible encephalopathies. Indeed, some members of the Committee seem to regard the whole question of another species as a hypothetical question to be addressed only when it happened. A rather unsatisfactory posture.
 
12. In advance of your meeting with Dr Tyrrell on Monday morning, I have not voiced my anxieties about the support the Committee is receiving from MAFF to anyone other than Dr Pickles. This minute does little more than confirm a position we already suspected, but I am sure MAFF Ministers would be very disturbed if they were aware of lack of action to follow through the Committee‘s requests.
 
imthMW/Jfim ‘
 
J S METTE RS Room 509 Richmond House
 
lll/YdeS
 
90l6.1416.2 \ i ‘
 
 
 
can't forget about the mad man and his mad cat;
 
Deaths of CJD man and cat linked
 
 
In October 1998 the simultaneous occurrence of spongiform encephalopathy in a man and his pet cat was reported. The report from Italy noted that the cat did not display the same clinical features as FSE cases previously seen. Indeed, the presence of a new type of FSE was suggested. The man was diagnosed as having sporadic CJD, and neither case (man nor cat) appeared to be affected by a BSE-related condition.
 
 
indeed there have been 4 documented cases of TSE in Lions to date.
 
Lion 32 December 98 Born November 86
 
Lion 33 May 1999 (euthanased) Born November 81.
 
Lion 36 Euthanased August 2000 Born July 87. Deteriorating hind limb ataxia.
 
Lion 37 Euthanased November 2001 Male, 14 years. Deteriorating hind limb ataxia since September 2001. (Litter mate to Ref. 36.)
 
 
go to the url above, on the bar at the top, click on _statistics_, then in middle of next page, click on _other TSEs_.
 
or go here;
 
 
and
 
 
 
also;
 
Reports on the clinical symptoms presented by these cats give a relatively homogeneous picture: Affected cats show a lack of coordination with an ataxia mainly of the hind limbs, they often fall and miss their target when jumping. Fear and increased aggressiveness against the owner and also other animals is often seen. They do not longer tolerate to be touched (stroked) and start hiding. These behavioural chances might be the result of a hypersensibility to touch and noise, but also to increased fear. Excessive salivation is another more frequently seen symptom. Cats with FSE in general show severe behavioural disturbances, restlessness and depression, and a lack of coat cleaning. Symptoms in large cats in general are comparable to those in domestic cats. A report on FSE (in german) has been presented in 2001 in the Swiss FVO Magazin. A paper on the first FSE case in a domestic cat in Switzerland is currently in press in the Journal Schweizer Archiv für Tierheilkunde (SAT).
 
 
Genetic Predictions of Prion Disease Susceptibility in Carnivore Species Based on Variability of the Prion Gene Coding Region
 
Paula Stewart1, Lauren Campbell1, Susan Skogtvedt2, Karen A. Griffin3, Jon M. Arnemo4, Morten Tryland5,6, Simon Girling7, Michael W. Miller3, Michael A. Tranulis2, Wilfred Goldmann1* 1 Neurobiology Division, The Roslin Institute & amp; R(D)SVS, University of Edinburgh, Roslin, United Kingdom, 2 Norwegian School of Veterinary Science, Dept. Basic Sciences & Aquatic Medicine, Oslo, Norway, 3 Wildlife Research Center, Colorado Division of Parks and Wildlife, Fort Collins, Colorado, United States of America, 4 Department of Forestry & Wildlife Management, Faculty of Applied Ecology and Agricultural Sciences, Hedmark University College, Campus Evenstad, Elverum, Norway & Department of Wildlife, Fish and Environmental Studies, Faculty of Forest Sciences, Swedish University of Agricultural Sciences, Umea°, Sweden, 5 Norwegian School of Veterinary Science, Section of Arctic Veterinary Medicine, Tromsø, Norway, 6 GenØk - Centre for Biosafety, Tromsø, Norway, 7 Royal Zoological Society of Scotland, Edinburgh Zoo, Edinburgh, United Kingdom
 
Abstract
 
Mammalian species vary widely in their apparent susceptibility to prion diseases. For example, several felid species developed prion disease (feline spongiform encephalopathy or FSE) during the bovine spongiform encephalopathy (BSE) epidemic in the United Kingdom, whereas no canine BSE cases were detected. Whether either of these or other groups of carnivore species can contract other prion diseases (e.g. chronic wasting disease or CWD) remains an open question. Variation in the host-encoded prion protein (PrPC) largely explains observed disease susceptibility patterns within ruminant species, and may explain interspecies differences in susceptibility as well. We sequenced and compared the open reading frame of the PRNP gene encoding PrPC protein from 609 animal samples comprising 29 species from 22 genera of the Order Carnivora; amongst these samples were 15 FSE cases. Our analysis revealed that FSE cases did not encode an identifiable disease-associated PrP polymorphism. However, all canid PrPs contained aspartic acid or glutamic acid at codon 163 which we propose provides a genetic basis for observed susceptibility differences between canids and felids. Among other carnivores studied, wolverine (Gulo gulo) and pine marten (Martes martes) were the only non-canid species to also express PrP-Asp163, which may impact on their prion diseases susceptibility. Populations of black bear (Ursus americanus) and mountain lion (Puma concolor) from Colorado showed little genetic variation in the PrP protein and no variants likely to be highly resistant to prions in general, suggesting that strain differences between BSE and CWD prions also may contribute to the limited apparent host range of the latter.
 
Citation: Stewart P, Campbell L, Skogtvedt S, Griffin KA, Arnemo JM, et al. (2012) Genetic Predictions of Prion Disease Susceptibility in Carnivore Species Based on Variability of the Prion Gene Coding Region. PLoS ONE 7(12): e50623. doi:10.1371/journal.pone.0050623
 
Editor: Mark D. Zabel, Colorado State University, College of Veterinary Medicine and Biomedical Sciences, United States of America Received August 29, 2012; Accepted October 23, 2012; Published December 7, 2012
 
Copyright: 2012 Stewart et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
 
Funding: PS, LC and WG were supported by Roslin Institute Strategic Grant funding from the Biotechnology and Biological Sciences Research Council, United Kingdom. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Competing Interests: The authors have declared that no competing interests exist. * E-mail: wilfred.goldmann@roslin.ed.ac.uk
 
This study has shown that FSE cases were not associated with PRNP genetics and that wild populations of carnivores have very little genetic variation in the PRNP gene which could result in unrestricted susceptibility to TSEs. Genetic uniformity of PRNP in American black bear and mountain lion could result in homogeneous susceptibility to a compatible prion strain within these species - should such a strain emerge –.
 
 
Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay
 
Christopher J. Johnson1, Christina M. Carlson2, Aaron R. Morawski3, Alyson Manthei4, Neil R. Cashman5
 
1USGS National Wildlife Health Center, 2Department of Soil Science, University of Wisconsin–Madison, 3Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 4Merial Veterinary Scholars Program, School of Veterinary Medicine, University of Wisconsin–Madison, 5Department of Neurology, University of British Columbia
 
Summary
 
Measuring the barrier to the interspecies transmission of prion diseases is challenging and typically involves animal challenges or biochemical assays. Here, we present an in vitro prion protein conversion assay with the ability to predict species barriers.
 
Date Published: 3/10/2015, Issue 97; doi: 10.3791/52522
 
Keywords: Medicine, Issue 97, Prion, species barrier, conversion, immunoblotting, transmissible spongiform encephalopathy, interspecies transmission Cite this Article
 
Johnson, C. J., Carlson, C. M., Morawski, A. R., Manthei, A., Cashman, N. R. Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay. J. Vis. Exp. (97), e52522, doi:10.3791/52522 (2015). Abstract
 
Studies to understanding interspecies transmission of transmissible spongiform encephalopathies (TSEs, prion diseases) are challenging in that they typically rely upon lengthy and costly in vivo animal challenge studies. A number of in vitro assays have been developed to aid in measuring prion species barriers, thereby reducing animal use and providing quicker results than animal bioassays. Here, we present the protocol for a rapid in vitro prion conversion assay called the conversion efficiency ratio (CER) assay. In this assay cellular prion protein (PrPC) from an uninfected host brain is denatured at both pH 7.4 and 3.5 to produce two substrates. When the pH 7.4 substrate is incubated with TSE agent, the amount of PrPC that converts to a proteinase K (PK)-resistant state is modulated by the original host’s species barrier to the TSE agent. In contrast, PrPC in the pH 3.5 substrate is misfolded by any TSE agent. By comparing the amount of PK-resistant prion protein in the two substrates, an assessment of the host’s species barrier can be made. We show that the CER assay correctly predicts known prion species barriers of laboratory mice and, as an example, show some preliminary results suggesting that bobcats (Lynx rufus) may be susceptible to white-tailed deer (Odocoileus virginianus) chronic wasting disease agent.
 
 
*** PLEASE NOTE, no update on the CWD Mountain Lion Study since ;
 
From: Miller, Mike Sent:
 
Saturday, November 17, 2012 5:06 PM
 
To: Terry S. Singeltary Sr.
 
Subject: Re: Hello Dr. Miller
 
Thank you for your interest in our ongoing research. To date our mountain lions are doing just fine.
 
On Sat, Nov 17, 2012 at 11:23 AM, Terry S. Singeltary Sr. wrote:
 
any update on the cwd to mountain lion transmission studies to date ???
 
any positive ???
 
thanks, kind regards, terry
 
-------- Original Message --------
 
Subject: STUDY PLAN - Susceptibility of mountain lions to chronic wasting disease
 
Date: Tue, 5 Apr 2005 09:25:25 -0500
 
From: "Terry S. Singeltary Sr." flounder
 
snip...end
 
*** and nobody seems to want to talk about it now???
 
Subject: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
 
Date: Thu, 17 Oct 2002 17:04:51 –0700
 
From: "Terry S. Singeltary Sr."
 
Reply-To: Bovine Spongiform Encephalopathy
 
To: BSE-L
 
Greetings BSE-L,
 
is there any other CWD surveys/testing in the UK on their deer? what sort of testing has been done to date on UK/EU deer? any input would be helpful... thank you
 
DEER SPONGIFORM ENCEPHALOPATHY SURVEY
 
CVO BSE 1 28
 
DEER BRAIN SURVEY
 
1. The Parliamentary Secretary will wish to be aware of a survey of deer brains for signs of a spongiform encephalopathy.
 
2. The purpose of the study will be to gather evidence of freedom from any spongiform encephalopathy in the UK deer- population to support our efforts to resume trade in deer with countries with which BSE has disrupted it.
 
3. This will be a low key study with no publicity to avoid unnecessary media interest. It will be carried out in two stages:
 
(i) A small scale examination of around 30 deer brains to establish the normal histology of the healthy brain; and
 
(ii) A larger scale random examination of 300 or more adult deer brains drawn from both deer farms and parks to establish whether there is any evidence of a cervine spongiform encephalopathy.
 
4. The industry have agreed to cooperate with the study and will supply the necessary brain material. No compensation will be paid. The only cost to Government will be for the laboratory examinations which will be borne within existing resources.
 
*** 5. Although the sample size would be too small to provide scientifically valid evidence that there is no cervine spongiform encephalopathy in the UK (4,000 odd brains would be necessary for that), a negative result would aid our efforts to have trade in deer resumed.
 
ROBERT LOWSON
 
Animal Health (Disease Control) Division
 
081 330 8042 - GTN 3836
 
Fax: 081 330 7862
 
Room 28A TOLB
 
20 November 1991
 
Mr Tanner - PS/Mr Maclean
 
c.c. PS/Minister Dr P Dawson Mr Lawrence PS/Permanent Secretary Mr Dugdalé Dr Matthews Mr Capstick Mr Bradley — CVL Mr Maslin Mr MeldrumL Mr Wilesmith - CVL Mr Thomson SOAFD Mr Haddon Mr Bell Mr Shannon DANI Mr X Taylor Mr Robertson Mr Podmore WOAD
 
91/11.20/4.1
 
 
 
***5. Although the sample size would be too small to provide scientifically valid evidence that there is no cervine spongiform encephalopathy in the UK (4,000 odd brains would be necessary for that), a negative result would aid our efforts to have trade in deer resumed. ...end...tss see 2016 ;
 
Tuesday, August 02, 2016
 
Chronic wasting disease of deer – is the battle to keep Europe free already lost?
 
 
Tuesday, April 12, 2016
 
The first detection of Chronic Wasting Disease (CWD) in Europe free-ranging reindeer from the Nordfjella population in South-Norway.
 
 
Tuesday, June 14, 2016
 
*** Chronic Wasting Disease (CWD) in a moose from Selbu in Sør-Trøndelag Norway ***
 
 
Thursday, July 07, 2016
 
Norway reports a third case Chronic Wasting Disease CWD TSE Prion in 2nd Norwegian moose
 
14/06/2016 - Norway reports a third case
 
 
Saturday, July 16, 2016
 
Chronic wasting Disease in Deer (CWD or Spongiform Encephalopathy) The British Deer Society 07/04/2016
 
Red Deer Ataxia or Chronic Wasting Disease CWD TSE PRION?
 
could this have been cwd in the UK back in 1970’S ???
 
 
 
 
 
SEE FULL TEXT ;
 
 
 
hope they did not go by the wayside as the hound study;
 
 
 
37.Putative TSE in hounds - work started 1990 -(see para 41)
 
Robert Higgins, a Veterinary Investigation Officer at Thirsk, had been working on a hound survey in 1990. Gerald Wells and I myself received histological sections from this survey along with the accompanying letter (YB90/11.28/1.1) dated November 1990. This letter details spongiform changes found in brains from hunt hounds failing to keep up with the rest of the pack, along with the results of SAF extractions from fresh brain material from these same animals. SAFs were not found in brains unless spongiform changes were also present. The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.
 
38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.
 
39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.
 
40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.
 
41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.
 
Histopathological support to various other published MAFF experiments
 
42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).
 
 
nothing to offer scientifically;
 
 
maddogs and Englishman
 
 
kind regards, terry
 
###########bse-l ############
 
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
 
Date: Fri, 18 Oct 2002 23:12:22 +0100
 
From: Steve Dealler
 
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
 
To: BSE-L@ References: <3daf5023 .4080804="" wt.net="">
 
Dear Terry, An excellent piece of review as this literature is desparately difficult to get back from Government sites.
 
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!
 
Steve Dealler
 
===============
 
Incubation periods for BSE are proportional to the life expectancy of the animal affected. The disease's incubation period is 18% of a cow's life expectancy and would be expected to about double when crossing to another species [---] that is, to 36% of 70 years in humans.
 
Steve Dealler, consultant in medical microbiology. Burnley General Hospital, Burnley BB10 2PQ deal@airtime.co.uk
 
TSS
 
 
Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2 [PART 1]
 
 
Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -11 Accepted - Volume 2 [PART 2]
 
 
ABOUT THAT CAT GUT FOR SUTURES AND TSE PRION ???
 
‘’The press, on being reassured catgut does not come from cats, could question where it does come from; perhaps [company name deleted] should be warned’.’’
 
INDEED, PLEASE BE WARNED...TSS
 

Other US BSE risks: the imported products picture

24 Jul 00 Trade Statistics: UK to US
Compiled by Terry S.Singeltary Sr of Bacliff, Texas
Opinion (webmaster): The US has focused for years on tracing, containing, and eradicating live animal imports from the UK or other countries with acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep. This strategy does not acknowledge imports of rendered bovine products from England during the BSE period nor secondary products such as surgical catgut, which is to say surgical cowgut, or dairy cattle embryos, vaccines for veterinarian and human medicines. What has become of these? 
Mr. Singeltary, who lost his mother to CJD of unexplained origin a few years back and went on to became a well-known TSE activist, has tracked down voluminous pertinent import data through correspondence with UK officials and searches of government web sites. Imports of such products are frequently cited by Europeans in rating BSE risks in the US and in shutting out US exports. Many people's eyes glaze over when reviewing reams of sometimes older trade statistics. There is no proof that any of the imported products was contaminated with BSE nor if so, any evidence that any BSE product lead to infection in US livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr. Singeltary establish that an appalling variety and tonnage of products that were imported by the US from the UK and othr BSE-affected countries during the peak of the BSE epidemic years.]
10 January 1990 COMMERCIAL IN CONFIDENCE

NOT FOR PUBLICATION

COMMITTEE ON SAFETY OF MEDICINES
WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY

SURGICAL CATGUT SUTURES
2.1     At the first meeting of the Working Party on Bovine
        Spongiform Encephalopathy on 6 September 1989, detailed
        consideration was given to XXXXX Surgical Catgut. This
        arose from the Company's response to the Letter to Licence
        Holders, indicating that the bovine small intestine source
        material was derived from UK cattle, unlike 8 other
        licenced catgut sutures. In contrast XXXXX Surgical
        Catgut was stated to hold over 90% share of the market for
        catgut sutures, and to constitute approximately 83% of all
        sutures used in U.K.
IMPORTS OF SUTURES FROM THE KNOWN BSE COUNTRY;


3006.10.0000: STERILE SURGICAL CATGUT, SIMILAR STERILE SUTURE MATERIALS 
AND STERILETISSUE ADHESIVES FOR SURGICAL WOUND CLOSURE; AND SIMILAR 
STERILE MATERIAL 
U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date
(Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) 

                           <--- ---="" 1998="" dec="">  <--- ---="" 1998="" ytd="">
Country                    Quantity     Value  Quantity     Value
=================================================================
WORLD TOTAL . . . . . . .    10,801     3,116   143,058    40,068

Belgium . . . . . . . . .       ---       ---       107        14
France  . . . . . . . . .        81        49     2,727     1,132
Switzerland . . . . . . .       ---       ---     1,357     1,693
United Kingdom  . . . . .     1,188       242    35,001     5,564

   U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date
   Subheading 300210: ANTISERA AND OTHER BLOOD FRACTIONS, AND MODIFIED
   IMMUNOLOGICAL PRODUCTS
 
   3002.10.0010: HUMAN BLOOD PLASMA
   U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date
   (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)
   
                              <--- ---="" ...="" .="" 05="" 10="" 11="" 12="" 131="" 134="" 13="" 145="" 14="" 15="" 162="" 16="" 17="" 18="" 192="" 1998="" 1999="" 199="" 19="" 1="" 20="" 218="" 22="" 233="" 23="" 248="" 250="" 25="" 26="" 270="" 27="" 2="" 309="" 329="" 32="" 335="" 353="" 36="" 374="" 378="" 38="" 39="" 3="" 41="" 440="" 44="" 461="" 464="" 47="" 4="" 53="" 550="" 584="" 588="" 597="" 5="" 60="" 62="" 643="" 6="" 716="" 718="" 743="" 756="" 817="" 82="" 86="" 87="" 88="" 8="" 92="" 944="" 9="" and="" animal="" animals="" atest-december="" atest-month="" austria="" belgium="" blood="" bovine="" canada="" cattle="" chapter="" consumption="" country="" cultures="" dairy="" dead="" dec="" december="" dollars="" elsewhere="" embryos="" equine="" etc.="" fetal="" for="" fractions="" france="" freeze-dried="" glue="" goats="" hides="" horses="" html="" human="" immune="" imports="" in="" included="" industry="" kilograms="" kingdom="" mar="" march="" medicine="" micro-organisms="" mports="" nesoi="" netherlands="" nits="" normal="" not="" number="" of="" or="" otea="" other="" parings="" poultry="" pre="" prepared="" products="" quantity="" rade-detail="" raw="" sera="" serum="" sheep="" similar="" skins="" specified="" stock="" subheading="" switzerland="" therapeutic="" thousands="" total="" toxins="" u.s.="" unfit="" united="" uses="" ustoms="" vaccines="" value="================================================================" waste="" whether="" world="" www.ita.doc.gov="" year-to-date="" yeasts="" ytd="">
 
SNIP...SEE FULL TEXT ;
 
 
August 22, 2003 5:11 PM
 
Mad cat disease
 
A second case of feline spongiform encephalopathy (FSE), a disease affecting the brain tissue of cats, has been recorded in Switzerland. The veterinary authorities said the likely cause of the infection, which is similar to mad cow disease, was contaminated pet food. A first case of FSE was reported two years ago. Experts say the disease poses no health risk for people.
 
swissinfo
 
 
Un document de 1991 indiqué dans la liste BSE-L par Terry S. Singeltary A CONTRIBUTION TO THE NEUROPATHOLOGY OF THE RED-NECKED OSTRICH (STRUTHIO CAMELUS) - SPONGIFORM ENCEPHALOPATHY
 
 
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE: 13 JUNE 1990
 
snip...
 
More cat brains are being examined and news of a cat 'epidemic' is expected soon in the media. CMO wants advice from the committee on how we should respond to this. The MAFF line to date has been to play down any suggestion of a link with BSE, and this may be more difficult (as well as probably incorrect) if/when several more recent cases are described.
 
(vii) the mink work suggests poultry may retain infective agent, at least TME.
 
(viii) recycling of pig and poultry remains to these species may serve to select pathogenic strains as a result of repeated passage.
 
 
 
 
 
Veterinary Pathology Onlinevet.sagepub.com Published online before print February 27, 2014, doi: 10.1177/0300985814524798 Veterinary Pathology February 27, 2014 0300985814524798
 
Lesion Profiling and Subcellular Prion Localization of Cervid Chronic Wasting Disease in Domestic Cats
 
D. M. Seelig1⇑ A. V. Nalls1 M. Flasik2 V. Frank1 S. Eaton2 C. K. Mathiason1 E. A. Hoover1 1Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA 2Department of Biomedical Sciences, Colorado State University, Fort Collins, CO, USA D. M. Seelig, University of Minnesota, Department of Veterinary Clinical Sciences, Room 339 VetMedCtrS, 6192A (Campus Delivery Code), 1352 Boyd Ave, St Paul, MN 55108, USA. Email address: dseelig@umn.edu
 
Abstract
 
Chronic wasting disease (CWD) is an efficiently transmitted, fatal, and progressive prion disease of cervids with an as yet to be fully clarified host range. While outbred domestic cats (Felis catus) have recently been shown to be susceptible to experimental CWD infection, the neuropathologic features of the infection are lacking. Such information is vital to provide diagnostic power in the event of natural interspecies transmission and insights into host and strain interactions in interspecies prion infection. Using light microscopy and immunohistochemistry, we detail the topographic pattern of neural spongiosis (the “lesion profile”) and the distribution of misfolded prion protein in the primary and secondary passage of feline CWD (FelCWD). We also evaluated cellular and subcellular associations between misfolded prion protein (PrPD) and central nervous system neurons and glial cell populations. From these studies, we (1) describe the novel neuropathologic profile of FelCWD, which is distinct from either cervid CWD or feline spongiform encephalopathy (FSE), and (2) provide evidence of serial passage-associated interspecies prion adaptation. In addition, we demonstrate through confocal analysis the successful co-localization of PrPD with neurons, astrocytes, microglia, lysosomes, and synaptophysin, which, in part, implicates each of these in the neuropathology of FelCWD. In conclusion, this work illustrates the simultaneous role of both host and strain in the development of a unique FelCWD neuropathologic profile and that such a profile can be used to discriminate between FelCWD and FSE.
 
prion chronic wasting disease immunohistochemistry interspecies cat feline spongiform encephalopathy transmissible spongiform encephalopathy adaptation species barrier
 
 
Monday, August 8, 2011 Susceptibility of Domestic Cats to CWD Infection
 
Oral.29: Susceptibility of Domestic Cats to CWD Infection
 
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†
 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu
 
Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness.
 
*** Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.
 
 
 
AD.63:
 
Susceptibility of domestic cats to chronic wasting disease
 
Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN USA
 
Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from these two cats were pooled and inoculated into cohorts of cats by IC, PO, and intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the symptomatic cats by western blotting and immunohistochemistry and abnormalities were seen in magnetic resonance imaging, including multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns consistent with the early stage of feline CWD.
 
*** These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to- feline transmission in nature.
 
 
www.landesbioscience.com
 
PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)
 
 
 
FELINE SPONGIFORM ENCEPHALOPATHY FSE
 
 
 
A CONTRIBUTION TO THE NEUROPATHOLOGY OF THE RED-NECKED OSTRICH (STRUTHIO CAMELUS) - SPONGIFORM ENCEPHALOPATHY
 
 
4.21 Three cases of SE’s with an unknown infectious agent have been reported in ostriches (Struthio Camellus) in two zoos in north west Germany (Schoon @ Brunckhorst, 1999, Verh ber Erkeg Zootiere 33:309-314). These birds showed protracted central nervous symptoms with ataxia, disturbances of balance and uncoordinated feeding behaviour. The diet of these birds had included poultry meat meal, some of which came from cattle emergency slaughter cases.
 
 
SE1806
 
TRANSMISSION STUDIES OF BSE TO DOMESTIC FOWL BY ORAL EXPOSURE TO BRAIN HOMOGENATE
 
1 challenged cock bird was necropsied (41 months p.i.) following a period of ataxia, tremor, limb abduction and other neurological signs. Histopathological examination failed to reveal any significant lesions of the central or peripheral nervous systems...
 
1 other challenged cock bird is also showing ataxia (43 months p.i.).
 
snip...
 
94/01.19/7.1
 
 
A notification of Spongiform Encephalopathy was introduced in October 1996 in respect of ungulates, poultry and any other animal.
 
4.23 MAFF have carried out their own transmission experiments with hens. In these experiments, some of the chickens exposed to the BSE agent showed neurological symptoms. However MAFF have not so far published details of the symptoms seen in chickens. Examination of brains from these chickens did not show the typical pathology seen in other SE’s. 4.24 A farmer in Kent in November 1996 noticed that one of his 20 free range hens, the oldest, aged about 30 months was having difficulty entering its den and appeared frightened and tended to lose its balance when excited. Having previously experienced BSE cattle on his farm, he took particular notice of the bird and continued to observe it over the following weeks. It lost weight, its balance deteriorated and characteristic tremors developed which were closely associated with the muscles required for standing. In its attempts to maintain its balance it would claw the ground more than usual and the ataxia progressively developed in the wings and legs, later taking a typical form of paralysis with a clumsy involuntary jerky motion. Violent tremors of the entire body, particularly the legs, became common, sparked off by the slightest provocation. This is similar to that seen in many BSE cases where any excitement may result in posterior ataxia, often with dropping of the pelvis, kicking and a general nervousness. Three other farmers and a bird breeder from the UK are known to have reported having hens with similar symptoms. The bird breeder who has been exhibiting his birds for show purposes for 20 years noticed birds having difficulty getting on to their perch and holding there for any length of time without falling. Even though the bird was eating normally, he noticed a weight loss of more than a pound in a bird the original weight of which was 5 pounds. 4.25 Histological examination of the brain revealed degenerative pathological changes in hens with a minimal vacuolation. The presence of PrP immunostaining of the brain sections revealed PrP-sc positive plaques and this must be regarded as very strong evidence to demonstrate that the hens had been incubating Spongiform Encephalopathy.
 
 
OPINION on : NECROPHAGOUS BIRDS AS POSSIBLE TRANSMITTERS OF TSE/BSE ADOPTED BY THE SCIENTIFIC STEERING COMMITTEE AT ITS MEETING OF 7-8 NOVEMBER 2002
 
OPINION
 
1. Necrophagous birds as possible transmitters of BSE. The SSC considers that the evaluation of necrophagous birds as possible transmitters of BSE, should theoretically be approached from a broader perspective of mammals and birds which prey on, or are carrion eaters (scavengers) of mammalian species. Thus, carnivorous and omnivorous mammals, birds of prey (vultures, falcons, eagles, hawks etc.), carrion eating birds (crows, magpies etc.) in general could be considered possible vectors of transmission and/or spread of TSE infectivity in the environment. In view also of the occurrence of Chronic Wasting Disease (CWD) in various deer species it should not be accepted that domestic cattle and sheep are necessarily the only source of TSE agent exposure for carnivorous species. While some information is available on the susceptibility of wild/exotic/zoo animals to natural or experimental infection with certain TSE agents, nothing is known of the possibility of occurrence of TSE in wild animal populations, other than among the species of deer affected by CWD in the USA.
 
1 The carrion birds are animals whose diet regularly or occasionally includes the consumption of carcasses, including possibly TSE infected ruminant carcasses.
 
C:\WINNT\Profiles\bredagi.000\Desktop\Necrophagous_OPINION_0209_FINAL.doc
 
 
snip...
 
skroll down to the bottom ;
 
 
Sunday, July 07, 2013
 
Could avian scavengers translocate infectious prions to disease-free areas initiating new foci of chronic wasting disease?
 
Prion. 2013 Jul 3;7(4). [Epub ahead of print]
 
 
 
 
Below, the entire scientific literature of 46 papers on zoo TSE, many obscure and expensive to obtain, are summarized from full text. The overall picture that emerges is appalling -- the British zoo cover-up has not only affected animals in their own zoos but also other zoos worldwide through the sale of contaminated speciality chows and through export and exchange of rare and endangered species involved in conservation programs. All the zoos involved are named by name here (unlike in the journal articles). Why protect a zoo that feeds cheetahs split spinal cords from cattle throughout the BSE epidemic? (Better to have tossed them the zoo veterinarian.) Names are important for zoos which would not want to export their healthy animals to these facilities or import possibly preclinical animals for their own endangered species breeding programs or release into wild populations. Medical scientists doing unrelated research want to know if animals in their programs are already incubating prion disease.
 
Ravensden, Marwell, Chester, Port Lympne, London, Whipsnade, Woburn, and Edinburgh are 8 known BSE affected British zoos. Woburn Safari Park apparently killed the lion by feeding it split cattle spinal cords and skulls.
 
 
The table below summarizes results in the 1999 PNAS paper. Penetrance of the disease is very high and many animals did not yet display symptoms . This paper was the first (and only one) to look at non-symptomatic zoo animals for prion infection (shown below in red). In the TSE column of the table, '+' signs indicate confirmed, 'p' indicates suspicious/probable, '-' means CNS study negative for TSE.(shown as brown), 'pc' means positive diagnosis in preclinical animal.
 
PNAS 96:4046-4051 199 30 Mar 1999 full text see comment PNAS 96[9] 4738-4739, April 27, 1999 by Will and Ironside C R Acad Sci III 1997 Dec;320(12):971-9 N Bons et al. C R Acad Sci III 1996 Aug;319(8):733-6 Lancet Volume 348, Number 9019 6 July 1996
 
The 82 zoo animals with BSE:
 
Id TSE Genus Species Subsp Birth Origin Death Place of Death 654 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier 656 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier 481 + Eulemur fulvus mayottensis 1974 Madagascar 1992 Montpellier zoo 474 + Eulemur fulvus mayottensis 1974 Madagascar 1990 Montpellier zoo 584 - Eulemur fulvus mayottensis 1984 Montpellier 1991 Montpellier zoo 455 + Eulemur fulvus mayottensis 1983 Montpellier 1989 Montpellier zoo - + Eulemur fulvus mayottensis 1988 Montpellier 1992 Montpellier zoo - + Eulemur fulvus mayottensis 1995 Montpellier 1996 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo 456 + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo 586 + Eulemur mongoz - 1979 Madagascar 1998 Montpellier zoo - p Eulemur mongoz - 1989 Mulhouse 1991 Montpellier zoo - p Eulemur mongoz - 1989 Mulhouse 1990 Montpellier zoo - p Eulemur macaco - 1986 Montpellier 1996 Montpellier zoo - p Lemur catta - 1976 Montpellier 1994 Montpellier zoo - p Varecia variegata variegata 1985 Mulhouse 1990 Montpellier zoo - p Varecia variegata variegata 1993 xxx 1994 Montpellier zoo 455 + Macaca mulatta - 1986 Ravensden UK 1992 Montpellier zoo - p Macaca mulatta - 1986 Ravensden UK 1993 Montpellier zoo - p Macaca mulatta - 1988 Ravensden UK 1991 Montpellier zoo - p Saimiri sciureus - 1987 Frejus France 1990 Frejus zoo 700 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 701 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 702 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 703 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 704 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 705 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 706 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 707 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 708 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 709 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 710 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 711 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 712 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 713 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 714 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 715 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 716 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 717 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo x p genus species - - Lille zoo 1996 Lille zoo y p genus species - - Lille zoo 1996 Lille zoo z p genus species - - Lille zoo 1996 Lille zoo
 
1 + Actinonyx jubatus cheetah 1986 Marwell zoo 1991 Pearle Coast AU Duke + Actinonyx jubatus cheetah 1984 Marwell zoo 1992 Colchester zoo? UK Saki + Actinonyx jubatus cheetah 1986 Marwell zoo 1993 unknown UK Mich + Actinonyx jubatus cheetah 1986 Whipsnade 1993 Whipsnade UK Fr1 + Actinonyx jubatus cheetah 1987 Whipsnade 1997 Safari de Peaugres FR Fr2 + Actinonyx jubatus cheetah 1991 Marwell zoo 1997 Safari de Peaugres Fr xx + Actinonyx jubatus cheetah 19xx xxx zoo 199x Fota zoo IR yy + Actinonyx jubatus cheetah 19xx yyy zoo 1996+ yyyy zoo UK zz + Actinonyx jubatus cheetah 19xx zzz zoo 1996+ yyyy zoo UK
 
aaa + Felis concolor puma 1986 Chester zoo 1991 Chester zoo UK yy + Felis concolor puma 1980 yyy zoo 1995 yyyy zoo UK zz + Felis concolor puma 1978 zzz zoo 1995 zzzz zoo UK
 
xxx + Felis pardalis ocelot 1987 xxx 1994 Chester zoo UK zzz + Felis pardalis ocelot 1980 zzz 1995 zzzz zoo UK
 
85 + Felis catus cat 1990+ various 1999+ various UK LI NO 19 + Canis familia. dog 1992+ various 1999+ various UK
 
Fota + Panthera tigris tiger 1981 xxx zoo 1995 xxxx zoo UK yy + Panthera tigris tiger 1983 yyy zoo 1998 yyyy zoo UK
 
Lump + Panthera leo lion 1986 Woburn SP 1998 Edinburgh zoo UK [since 1994]
 
1 + Taurotragus oryx eland 1987 Port Lympne 1989 Port Lympne zoo UK Moll + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK Nedd + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK Elec + Taurotragus oryx eland 1990 xx UK 1992 not Port Lympne Uk Daph p Taurotragus oryx eland 1988 xx UK 1990 not Port Lympne UK zzz + Taurotragus oryx eland 1991 zz UK 1994 zzz UK yyy + Taurotragus oryx eland 1993 yy UK 1995 yyy UK
 
Fran p Tragelaphus strepsi. kudu 1985 London zoo 1987 London zoo UK Lind + Tragelaphus strepsi. kudu 1987 London zoo 1989 London zoo UK Karl + Tragelaphus strepsi. kudu 1988 London zoo 1990 London zoo UK Kaz + Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK Bamb pc Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK Step - Tragelaphus strepsi. kudu 1984 London zoo 1991 London zoo UK 346 pc Tragelaphus strepsi. kudu 1990 London zoo 1992 London zoo UK 324 + Tragelaphus strepsi. kudu 1989 Marwell zoo 1992 London zoo UK
 
xxx + Tragelaphus angasi nyala 1983 Marwell zoo 1986 Marwell zoo UK
 
yy + Oryx gazella gemsbok 1983 Marwell zoo 1986 Marwell zoo UK zz + Oryx gazella gemsbok 1994+ zzz zoo 1996+ zzzz zoo UK
 
xx + Oryx dammah scim oryx 1990 xxxx zoo 1993 Chester zoo UK
 
yy + Oryx leucoryx arab oryx 1986 Zurich zoo 1991 London zoo UK
 
yy + Bos taurus ankole cow 1987 yyy zoo 1995 yyyy zoo UK zz + Bos taurus ankole cow 1986 zzz zoo 1991 zzzz zoo UK
 
xx + Bison bison Eu bison 1989 xxx zoo 1996 xxxx zoo UK
 
 
TSE - UK: EXOTIC ANIMALS Sat, 7 Jun 1997 a HREF="dpreslar@fas.org">Dorothy Preslar Briefing to the TSE conference hosted by the New Zealand MAFF
 
In a written reply to the House of Commons, Agriculture Minister of State Jeff Rooker has provided details of Transmissible Spongiform Encephalopathy in animals other than livestock. His report includes confirmed cases of TSE in 2 ankole cows, 1 bison, 3 cheetah, 6 eland, 1 gemsbok, 6 kudu, 1 nyala, 2 ocelot, 1 Arabian oryx, 1 scimitar horned oryx, 3 pumas and 1 tiger, 77 domestic cats.
 
SE Diagnoses In Exotic Species UK MAFF site as it appeared in August 1997
 
kudu 6 gemsbok 1 nyala 1 oryx 2 eland 6 cat (domestic) 78 cheetah 4 + 1 Australia + 1 France + 1 Ireland puma 3 tiger 1 ocelot 2 bison (bison bison) 1 ankole 2 BSE in Great Britain: A Progress Report published twice yearly dated May 1996.
 
kudu 6 gemsbok 1 nyala 1 oryx 2 eland 6 cat 70 cheetah 2 UK + 1 AU + 1 ROI puma 3 tiger 1 ocelot 2 ankole cow 2 http://www.mad-cow.org/may99_zoo_news.html TSEs in Exotic Ruminants TSEs have been detected in exotic ruminants in UK zoos since 1986. These include antelopes (Eland, Gemsbok, Arabian and Scimitar oryx, Nyala and Kudu), Ankole cattle and Bison. With hindsight the 1986 case in a Nyala was diagnosed before the first case of BSE was identified. The TSE cases in exotic ruminants had a younger onset age and a shorter clinical duration compared to that in cattle with BSE. All the cases appear to be linked to the BSE epidemic via the consumption of feed contaminated with the BSE agent. The epidemic has declined as a result of tight controls on feeding mammalian meat and bone meal to susceptible animals, particularly from August 1996.
 
References: Jeffrey, M. and Wells, G.A.H, (1988) Spongiform encephalopathy in a nyala (Tragelaphus angasi). Vet.Path. 25. 398-399
 
Kirkwood, J.K. et al (1990) Spongiform encephalopathy in an Arabian oryx (Oryx leucoryx) and a Greater kudu (Tragelaphus strepsiceros) Veterinary Record 127. 418-429.
 
Kirkwood, J.K. (1993) Spongiform encephalopathy in a herd of Greater kudu (Tragelaphus strepsiceros): epidemiological observations. Veterinary Record 133. 360-364
 
Kirkwood, J. K. and Cunningham, A.A. (1994) Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Veterinary Record. 135. 296-303.
 
Food and Agriculture Organisation (1998) Manual on Bovine Spongiform Encephalopathy.
 
 
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
 
worse still, there is serious risk the media could get to hear of such a meeting...
 
snip...
 
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
 
 
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
 
snip...
 
YOU explained that imported crushed heads were extensively used in the petfood industry...
 
 
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
 
 
 
some 100+ _documented_ TSE cats of all types later...tss
 
on occassions, materials obtained from slaughterhouses will be derived from sheep affected with scrapie or cattle that may be incubating BSE for use in petfood manufacture...
 
 
*** Meldrum's notes on pet foods and materials used
 
 
*** BSE & Pedigree Petfoods ***
 
 
FELINE SPONGIFORM ENCEPHALOPATHY FSE
 
 
 
 
TSE & HOUNDS
 
GAH WELLS (very important statement here...TSS)
 
HOUND STUDY
 
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
 
snip...
 
 
76 pages on hound study;
 
snip...
 
 
The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.
 
38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.
 
39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.
 
40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.
 
41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.
 
Histopathological support to various other published MAFF experiments
 
42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).
 
 
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
 
snip...
 
 
SEE NEW URL ;
 
 
2005
 
DEFRA Department for Environment, Food & Rural Affairs
 
Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk
 
GTN: FAX:
 
Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518
 
21 November 2001
 
Dear Mr Singeltary
 
TSE IN HOUNDS
 
Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.
 
As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.
 
Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.
 
Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less
 
 
As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.
 
Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK
 
You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.
 
I hope this is helpful
 
Yours sincerely 4
 
HUGH MCDONAGH BSE CORRESPONDENCE SECTION
 
======================================
 
HOUND SURVEY
 
I am sorry, but I really could have been a co-signatory of Gerald's minute.
 
I do NOT think that we can justify devoting any resources to this study, especially as larger and more important projects such as the pathogenesis study will be quite demanding.
 
If there is a POLITICAL need to continue with the examination of hound brains then it should be passed entirely to the VI Service.
 
J W WILESMITH Epidemiology Unit 18 October 1991
 
Mr. R Bradley
 
cc: Mr. G A H Wells
 
 
3.3. Mr R J Higgins in conjunction with Mr G A Wells and Mr A C Scott would by the end of the year, indentify the three brains that were from the ''POSITIVE'' end of the lesion spectrum.
 
 
 
 
OR-09: Canine spongiform encephalopathy—A new form of animal prion disease
 
Monique David, Mourad Tayebi UT Health; Houston, TX USA
 
It was also hypothesized that BSE might have originated from an unrecognized sporadic or genetic case of bovine prion disease incorporated into cattle feed or even cattle feed contaminated with prion-infected human remains.1 However, strong support for a genetic origin of BSE has recently been demonstrated in an H-type BSE case exhibiting the novel mutation E211K.2 Furthermore, a specific prion protein strain causing BSE in cattle is believed to be the etiological agent responsible for the novel human prion disease, variant Creutzfeldt-Jakob disease (vCJD).3 Cases of vCJD have been identified in a number countries, including France, Italy, Ireland, the Netherlands, Canada, Japan, US and the UK with the largest number of cases. Naturally occurring feline spongiform encephalopathy of domestic cats4 and spongiform encephalopathies of a number of zoo animals so-called exotic ungulate encephalopathies5,6 are also recognized as animal prion diseases, and are thought to have resulted from the same BSE-contaminated food given to cattle and humans, although and at least in some of these cases, a sporadic and/or genetic etiology cannot be ruled out. The canine species seems to display resistance to prion disease and no single case has so far been reported.7,8
 
Here, we describe a case of a 9 week old male Rottweiler puppy presenting neurological deficits; and histological examination revealed spongiform vacuolation characteristic of those associated with prion diseases.9 Initial biochemical studies using anti-PrP antibodies revealed the presence of partially proteinase K-resistant fragment by western blotting. Furthermore, immunohistochemistry revealed spongiform degeneration consistent with those found in prion disease and displayed staining for PrPSc in the cortex.
 
Of major importance, PrPSc isolated from the Rottweiler was able to cross the species barrier transmitted to hamster in vitro with PMCA and in vivo (one hamster out of 5). Futhermore, second in vivo passage to hamsters, led to 100% attack rate (n = 4) and animals displayed untypical lesional profile and shorter incubation period.
 
In this study, we show that the canine species might be sensitive to prion disease and that PrPSc isolated from a dog can be transmitted to dogs and hamsters in vitro using PMCA and in vivo to hamsters.
 
If our preliminary results are confirmed, the proposal will have a major impact on animal and public health and would certainly lead to implementing new control measures for ‘canine spongiform encephalopathy’ (CSE).
 
References
 
1. Colchester AC, Colchester NT. The origin of bovine spongiform encephalopathy: the human prion disease hypothesis. Lancet 2005; 366:856-61; PMID:16139661; http:// dx.doi.org/10.1016/S0140-6736(05)67218-2.
 
2. Richt JA, Hall SM. BSE case associated with prion protein gene mutation. PLoS Pathog 2008; 4:e1000156; PMID:18787697; http://dx.doi.org/10.1371/journal. ppat.1000156.
 
3. Collinge J. Human prion diseases and bovine spongiform encephalopathy (BSE). Hum Mol Genet 1997; 6:1699-705; PMID:9300662; http://dx.doi.org/10.1093/ hmg/6.10.1699.
 
4. Wyatt JM, Pearson GR, Smerdon TN, Gruffydd-Jones TJ, Wells GA, Wilesmith JW. Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats. Vet Rec 1991; 129:233-6; PMID:1957458; http://dx.doi.org/10.1136/vr.129.11.233.
 
5. Jeffrey M, Wells GA. Spongiform encephalopathy in a nyala (Tragelaphus angasi). Vet Pathol 1988; 25:398-9; PMID:3232315; http://dx.doi.org/10.1177/030098588802500514.
 
6. Kirkwood JK, Wells GA, Wilesmith JW, Cunningham AA, Jackson SI. Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros). Vet Rec 1990; 127:418-20; PMID:2264242.
 
7. Bartz JC, McKenzie DI, Bessen RA, Marsh RF, Aiken JM. Transmissible mink encephalopathy species barrier effect between ferret and mink: PrP gene and protein analysis. J Gen Virol 1994; 75:2947-53; PMID:7964604; http://dx.doi.org/10.1099/0022-1317- 75-11-2947.
 
8. Lysek DA, Schorn C, Nivon LG, Esteve-Moya V, Christen B, Calzolai L, et al. Prion protein NMR structures of cats, dogs, pigs, and sheep. Proc Natl Acad Sci U S A 2005; 102:640-5; PMID:15647367; http://dx.doi.org/10.1073/pnas.0408937102.
 
9. Budka H. Neuropathology of prion diseases. Br Med Bull 2003; 66:121-30; PMID:14522854; http://dx.doi.org/10.1093/bmb/66.1.121.
 
 
Monday, March 26, 2012
 
CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE
 
OR-09 15:10 - 15:25 CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE
 
 
 
PRION 2016 TOKYO
 
EP-021 Canine Prions: A New Form of Prion Disease
 
Mourad Tayebi1, Monique A David2, Brian Summers3
 
1 University of Melbourne, Veterinary Sciences, Australia; 2Ausbiologics, Sydney, Australia; 3Royal Veterinary College, London, UK
 
The origin of bovine spongiform encephalopathy (BSE), which rapidly evolved into a major epidemic remains unresolved and was initially widely attributed to transmission of sheep scrapie to cattle with contaminated feed prepared from rendered sheep carcasses. Alternative transmission hypotheses also include feed contaminated with unrecognized subclinical case(s) of bovine prion disease or with prion-infected human remains. However, following the demonstration of a BSE case exhibiting the novel mutation E211 K, similar to the E200K mutation associated with most genetic CJD in humans, support for a genetic origin of prion disease in cattle is gaining momentum. In contrast to other animal species such as feline, the canine species seems to be resistant to prion disease as no canine prion cases were previously reported.
 
We describe here three cases of Rottweiler puppy (called RWD cases) with neurological deficits and spongiform change. We used animal bioassays and in vitro studies to show efficient interspecies transmission of this novel canidae prion isolate to other species.
 
Biochemical studies revealed the presence of partially proteinase K (PK)-resistant fragment and immunohistochemistry displayed staining for PrPSc in the cerebral cortex. Importantly, interspecies transmission of canine PrPSc derived from RWD3 brain homogenates following inoculation of hamsters led to signs of prion disease and replication of PrPSc in brains, spinal cords and spleens of these animals.
 
These findings if confirmed by further cases of prion disease in canidae and regardless of the origin of the disease would have a major impact on animal and public health.
 
PRION 2016 TOKYO
 
 
 
-------- Original Message --------
 
Subject: PETFOODS, DOGS, CATS AND TSEs
 
Date: Fri, 12 Sep 2003 21:06:35 –0500
 
From: "Terry S. Singeltary Sr."
 
X-Virus-Scanner: Found to be clean Message-ID: <>
 
Date: Tue, 27 May 2003 08:07:58 –0500
 
Reply-To: Bovine Spongiform Encephalopathy
 
Sender: Bovine Spongiform Encephalopathy
 
From: "Terry S. Singeltary Sr."
 
Subject: FDA BSE Update - Pet Food from Canadian Manufacturer & MAD DOG DATA
 
######## Bovine Spongiform Encephalopathy #########
 
Statement
 
FOR IMMEDIATE RELEASE Statement May 26, 2003
 
Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA
 
FDA BSE Update - Pet Food from Canadian Manufacturer
 
The Food and Drug Administration (FDA) has learned from the government of Canada that rendered material from a Canadian cow that last week tested positive for bovine spongiform encephalopathy (BSE, also known as mad cow disease ) may have been used to manufacture pet food, specifically dry dog food, some of which was reported to have been shipped to the United States. The Canadian government prevented the BSE positive cow from being processed for human food. Therefore, consumers can be assured that their food does not contain any remnants of the BSE positive cow.
 
It is also important to stress that there is no scientific evidence to date that dogs can contract BSE or any similar disease. In addition there is no evidence that dogs can transmit the disease to humans.
 
FDA notified the U.S. pet food firm, The Pet Pantry International, of Carson City, Nevada, when FDA learned that the pet food that the firm received may have included rendered material from the BSE positive cow. The manufacturer of the pet food is Champion Pet Food, Morinville, Alberta. Even though there is no known risk to dogs from eating this dog food, as a prudent measure to help assure that the U.S. stays BSE free The Pet Pantry International is asking its customers who may have purchased the suspect product to hold it for pickup by the distributor so that the dog food will not mistakenly be mixed into cattle or other feeds if any of the dog food is discarded or otherwise not used to feed dogs. The suspect dog food was produced by Champion Pet Food between February 4, 2003, and March 12, 2003.
 
The Pet Pantry products were packaged in 50 lb bags, distributed to franchises around the country, and sold by home delivery only. There was no retail distribution of the product. Consumers purchase Pet Pantry products by phone or email orders. The product is then delivered by the nearest franchisee directly to the consumer s home.
 
The product subject to this notification includes Maintenance Diet labeled with a use by date of 17FEB04 and Beef with Barley with a use by date of 05MAR04 . Consumers who have purchased dog food from The Pet Pantry since February of this year are asked to check their present supplies and see if any match the description of the product being removed. If so, consumers are asked to contact The Pet Pantry at 1-800-381-7387 for further information on how to return the product to The Pet Pantry for proper disposal. Consumers are asked not to destroy or discard the product themselves. The Pet Pantry will also use its sales records to contact consumers who purchased the affected product.
 
FDA is working closely with the Pet Pantry International to assure for proper disposal of the recovered product.
 
FDA will continue to provide updates on this case of BSE in Canada as additional information becomes available.
 
 
Friday, March 8, 2013
 
Dogs may have been used to make Petfood and animal feed
 
 
 
MAD DOGS AND ENGLISHMAN
 
 
 
Chronic Wasting Disease Susceptibility of Four North American Rodents
 
Chad J. Johnson1*, Jay R. Schneider2, Christopher J. Johnson2, Natalie A. Mickelsen2, Julia A. Langenberg3, Philip N. Bochsler4, Delwyn P. Keane4, Daniel J. Barr4, and Dennis M. Heisey2 1University of Wisconsin School of Veterinary Medicine, Department of Comparative Biosciences, 1656 Linden Drive, Madison WI 53706, USA 2US Geological Survey, National Wildlife Health Center, 6006 Schroeder Road, Madison WI 53711, USA 3Wisconsin Department of Natural Resources, 101 South Webster Street, Madison WI 53703, USA 4Wisconsin Veterinary Diagnostic Lab, 445 Easterday Lane, Madison WI 53706, USA *Corresponding author email: cjohnson@svm.vetmed.wisc.edu
 
We intracerebrally challenged four species of native North American rodents that inhabit locations undergoing cervid chronic wasting disease (CWD) epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles (Myodes gapperi). The inocula were prepared from the brains of hunter-harvested white-tailed deer from Wisconsin that tested positive for CWD. Meadow voles proved to be most susceptible, with a median incubation period of 272 days. Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the brains of all challenged meadow voles. Subsequent passages in meadow voles lead to a significant reduction in incubation period. The disease progression in red-backed voles, which are very closely related to the European bank vole (M. glareolus) which have been demonstrated to be sensitive to a number of TSEs, was slower than in meadow voles with a median incubation period of 351 days. We sequenced the meadow vole and red-backed vole Prnp genes and found three amino acid (AA) differences outside of the signal and GPI anchor sequences. Of these differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is particularly intriguing due its postulated involvement in "rigid loop" structure and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5 years post-inoculation, but appear to be exhibiting a high degree of disease penetrance. White-footed mice have an even longer incubation period but are also showing high penetrance. Second passage experiments show significant shortening of incubation periods. Meadow voles in particular appear to be interesting lab models for CWD. These rodents scavenge carrion, and are an important food source for many predator species. Furthermore, these rodents enter human and domestic livestock food chains by accidental inclusion in grain and forage. Further investigation of these species as potential hosts, bridge species, and reservoirs of CWD is required.
 
 
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
 
Emmanuel Comoy, Jacqueline Mikol, Val erie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France
 
Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. *** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, ***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), ***is the third potentially zoonotic PD (with BSE and L-type BSE), ***thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.
 
===============
 
***thus questioning the origin of human sporadic cases...TSS
 
===============
 
 
==========================================
 
***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.
 
==========================================
 
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
 
*** Title: Transmission of scrapie prions to primate after an extended silent incubation period
 
Authors
 
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire, Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron, Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item Deslys, Jean-Philippe -
 
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015 Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 2015. Transmission of scrapie prions to primate after an extended silent incubation period. Scientific Reports. 5:11573.
 
Interpretive Summary:
 
The transmissible spongiform encephalopathies (also called prion diseases) are fatal neurodegenerative diseases that affect animals and humans. The agent of prion diseases is a misfolded form of the prion protein that is resistant to breakdown by the host cells. Since all mammals express prion protein on the surface of various cells such as neurons, all mammals are, in theory, capable of replicating prion diseases. One example of a prion disease, bovine spongiform encephalopathy (BSE; also called mad cow disease), has been shown to infect cattle, sheep, exotic undulates, cats, non-human primates, and humans when the new host is exposed to feeds or foods contaminated with the disease agent. The purpose of this study was to test whether non-human primates (cynomologous macaque) are susceptible to the agent of sheep scrapie. After an incubation period of approximately 10 years a macaque developed progressive clinical signs suggestive of neurologic disease. Upon postmortem examination and microscopic examination of tissues, there was a widespread distribution of lesions consistent with a transmissible spongiform encephalopathy. This information will have a scientific impact since it is the first study that demonstrates the transmission of scrapie to a non-human primate with a close genetic relationship to humans. This information is especially useful to regulatory officials and those involved with risk assessment of the potential transmission of animal prion diseases to humans.
 
Technical Abstract:
 
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease that also causes variant Creutzfeldt-Jakob disease in humans. Over the past decades, c-BSE's zoonotic potential has been the driving force in establishing extensive protective measures for animal and human health. In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
 
***This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
 
 
Tuesday, December 16, 2014
 
Evidence for zoonotic potential of ovine scrapie prions
 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics
 
Abstract
 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
 
Subject terms: Biological sciences• Medical research At a glance
 
 
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
 
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
 
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
 
 
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
 
Taylor & Francis
 
Prion 2016 Animal Prion Disease Workshop Abstracts
 
WS-01: Prion diseases in animals and zoonotic potential
 
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,
 
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
 
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France
 
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.
 
To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.
 
These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.
 
Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
 
 
 
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
 
 
2015
 
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
 
 
 
SCRAPIE AND CWD ZOONOSIS
 
PRION 2016 CONFERENCE TOKYO
 
Saturday, April 23, 2016
 
*** SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016 ***
 
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
 
 
Transmission of scrapie prions to primate after an extended silent incubation period
 
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
 
 
Wednesday, June 29, 2016
 
CWD, SCRAPIE, ZOONOSIS, it’s for real folks, the risk factors have increased greatly, and science has spoken, cwd and scrapie to humans as sporadic cjd may have already happened.
 
Transmission of scrapie prions to primate after an extended silent incubation period
 
Emmanuel E. Comoy , Jacqueline Mikol , Sophie Luccantoni-Freire , Evelyne Correia , Nathalie Lescoutra-Etchegaray , Valérie Durand , Capucine Dehen , Olivier Andreoletti , Cristina Casalone , Juergen A. Richt , Justin J. Greenlee , Thierry Baron , Sylvie L. Benestad , Paul Brown & Jean-Philippe Deslys
 
Abstract
 
Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.
 
snip...
 
In addition to previous studies on scrapie transmission to primate1,8,9 and the recently published study on transgenic humanized mice13, our results constitute new evidence for recommending that the potential risk of scrapie for human health should not be dismissed. Indeed, human PrP transgenic mice and primates are the most relevant models for investigating the human transmission barrier. To what extent such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. During the past decades, many protective measures have been successfully implemented to protect cattle from the spread of c-BSE, and some of these measures have been extended to sheep and goats to protect from scrapie according to the principle of precaution. Since cases of c-BSE have greatly reduced in number, those protective measures are currently being challenged and relaxed in the absence of other known zoonotic animal prion disease. We recommend that risk managers should be aware of the long term potential risk to human health of at least certain scrapie isolates, notably for lymphotropic strains like the classical scrapie strain used in the current study. Relatively high amounts of infectivity in peripheral lymphoid organs in animals infected with these strains could lead to contamination of food products produced for human consumption. Efforts should also be maintained to further assess the zoonotic potential of other animal prion strains in long-term studies, notably lymphotropic strains with high prevalence like CWD, which is spreading across North America, and atypical/Nor98 scrapie (Nor98)50 that was first detected in the past two decades and now represents approximately half of all reported cases of prion diseases in small ruminants worldwide, including territories previously considered as scrapie free. Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
 
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
 
 
 
PRION 2016 TOKYO
 
Zoonotic Potential of CWD Prions: An Update
 
Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6
 
1Department of Pathology, 3National Prion Disease Pathology Surveillance Center, 5Department of Neurology, 6National Center for Regenerative Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.
 
4Department of Biological Sciences and Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada,
 
2Encore Health Resources, 1331 Lamar St, Houston, TX 77010
 
Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions.
 
PRION 2016 TOKYO
 
In Conjunction with Asia Pacific Prion Symposium 2016
 
PRION 2016 Tokyo
 
Prion 2016
 
 
Prion 2016
 
Purchase options Price * Issue Purchase USD 198.00
 
 
Cervid to human prion transmission
 
Kong, Qingzhong
 
Case Western Reserve University, Cleveland, OH, United States
 
Abstract
 
Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that:
 
(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;
 
(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;
 
(3) Reliable essays can be established to detect CWD infection in humans;and
 
(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.
 
Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3.
 
Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1.
 
Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.
 
Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans.
 
Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans.
 
Funding Agency Agency National Institute of Health (NIH)
 
Institute National Institute of Neurological Disorders and Stroke (NINDS)
 
Type Research Project (R01)
 
Project # 1R01NS088604-01A1
 
Application # 9037884
 
Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND)
 
Program Officer Wong, May
 
Project Start 2015-09-30
 
Project End 2019-07-31
 
Budget Start 2015-09-30
 
Budget End 2016-07-31
 
Support Year 1
 
Fiscal Year 2015
 
Total Cost $337,507
 
Indirect Cost $118,756
 
Institution
 
Name Case Western Reserve University
 
Department Pathology
 
Type Schools of Medicine
 
DUNS # 077758407
 
City Cleveland
 
State OH
 
Country United States
 
Zip Code 44106
 
 
===========================================================
 
We hypothesize that:
 
(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;
 
(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;
 
(3) Reliable essays can be established to detect CWD infection in humans;and
 
(4) *** CWD transmission to humans has already occurred. *** We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.
 
============================================================
 
Key Molecular Mechanisms of TSEs
 
Zabel, Mark D.
 
Colorado State University-Fort Collins, Fort Collins, CO, United States Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative diseases affecting humans, cervids, bovids, and ovids. The absolute requirement of PrPC expression to generate prion diseases and the lack of instructional nucleic acid define prions as unique infectious agents. Prions exhibit species-specific tropism, inferring that unique prion strains exist that preferentially infct certain host species and confront transmission barriers to heterologous host species. However, transmission barriers are not absolute. Scientific consensus agrees that the sheep TSE scrapie probably breached the transmission barrier to cattle causing bovine spongiform encephalopathy that subsequently breached the human transmission barrier and likely caused several hundred deaths by a new-variant form of the human TSE Creutzfeldt-Jakob disease in the UK and Europe. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease of cervids, overcoming similar human transmission barriers. A groundbreaking discovery made last year revealed that mice infected with heterologous prion strains facing significant transmission barriers replicated prions far more readily in spleens than brains6. Furthermore, these splenic prions exhibited weakened transmission barriers and expanded host ranges compared to neurogenic prions. These data question conventional wisdom of avoiding neural tissue to avoid prion xenotransmission, when more promiscuous prions may lurk in extraneural tissues. Data derived from work previously funded by NIH demonstrate that Complement receptors CD21/35 bind prions and high density PrPC and differentially impact prion disease depending on the prion isolate or strain used. Recent advances in live animal and whole organ imaging have led us to generate preliminary data to support novel, innovative approaches to assessing prion capture and transport. We plan to test our unifying hypothesis for this proposal that CD21/35 control the processes of peripheral prion capture, transport, strain selection and xenotransmission in the following specific aims. 1. Assess the role of CD21/35 in splenic prion strain selection and host range expansion. 2. Determine whether CD21/35 and C1q differentially bind distinct prion strains 3. Monitor the effects of CD21/35 on prion trafficking in real time and space 4. Assess the role of CD21/35 in incunabular prion trafficking
 
Public Health Relevance Transmissible spongiform encephalopathies, or prion diseases, are devastating illnesses that greatly impact public health, agriculture and wildlife in North America and around the world. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease (CWD) of cervids, overcoming similar human transmission barriers. Early this year Canada reported its first case of BSE in over a decade audits first case of CWD in farmed elk in three years, underscoring the need for continued vigilance and research. Identifying mechanisms of transmission and zoonoses remains an extremely important and intense area of research that will benefit human and other animal populations.
 
Funding Agency Agency National Institute of Health (NIH)
 
Institute National Institute of Allergy and Infectious Diseases (NIAID)
 
Type High Priority, Short Term Project Award (R56)
 
Project # 1R56AI122273-01A1
 
Application # 9211114
 
Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND)
 
Program Officer Beisel, Christopher E
 
Project Start 2016-02-16
 
Project End 2017-01-31
 
Budget Start 2016-02-16
 
Budget End 2017-01-31
 
Support Year 1
 
Fiscal Year 2016
 
Total Cost
 
Indirect Cost Institution Name Colorado State University-Fort Collins
 
Department Microbiology/Immun/Virology
 
Type Schools of Veterinary Medicine
 
DUNS # 785979618 City Fort Collins
 
State CO
 
Country United States
 
Zip Code 80523
 
 
PMCA Detection of CWD Infection in Cervid and Non-Cervid Species
 
Hoover, Edward Arthur
 
Colorado State University-Fort Collins, Fort Collins, CO, United States Abstract Chronic wasting disease (CWD) of deer and elk is an emerging highly transmissible prion disease now recognized in 18 States, 2 Canadian provinces, and Korea. We have shown that Infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces, and in the tissues generating those body fluids and excreta, thereby leading to facile transmission by direct contact and environmental contamination. We have also shown that CWD can infect some non-cervid species, thus the potential risk CWD represents to domestic animal species and to humans remains unknown. Whether prions borne in blood, saliva, nasal fluids, milk, or excreta are generated or modified in the proximate peripheral tissue sites, may differ in subtle ways from those generated in brain, or may be adapted for mucosal infection remain open questions. The increasing parallels in the pathogenesis between prion diseases and human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, add relevance to CWD as a transmissible protein misfolding disease. The overall goal of this work is to elucidate the process of CWD prion transmission from mucosal secretory and excretory tissue sites by addressing these questions: (a) What are the kinetics and magnitude of CWD prion shedding post-exposure? (b) Are excreted prions biochemically distinct, or not, from those in the CNS? (c) Are peripheral epithelial or CNS tissues, or both, the source of excreted prions? and (d) Are excreted prions adapted for horizontal transmission via natural/trans-mucosal routes? The specific aims of this proposal are: (1) To determine the onset and consistency of CWD prion shedding in deer and cervidized mice; (2); To compare the biochemical and biophysical properties of excretory vs. CNS prions; (3) To determine the capacity of peripheral tissues to support replication of CWD prions; (4) To determine the protease- sensitive infectious fraction of excreted vs. CNS prions; and (5) To compare the mucosal infectivity of excretory vs. CNS prions. Understanding the mechanisms that enable efficient prion dissemination and shedding will help elucidate how horizontally transmissible prions evolve and succeed, and is the basis of this proposal. Understanding how infectious misfolded proteins (prions) are generated, trafficked, shed, and transmitted will aid in preventing, treating, and managing the risks associated with these agents and the diseases they cause.
 
Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an emergent highly transmissible prion disease now recognized throughout the USA as well as in Canada and Korea. We have shown that infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces thereby leading to transmission by direct contact and environmental contamination. In that our studies have also shown that CWD can infect some non-cervid species, the potential risk CWD may represents to domestic animal species and humans remains unknown. The increasing parallels in the development of major human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and prion diseases add relevance to CWD as a model of a transmissible protein misfolding disease. Understanding how infectious misfolded proteins (prions) are generated and transmitted will aid in interrupting, treating, and managing the risks associated with these agents and the diseases they cause.
 
Funding Agency Agency National Institute of Health (NIH)
 
Institute National Institute of Neurological Disorders and Stroke (NINDS)
 
Type Research Project (R01)
 
Project # 4R01NS061902-07
 
Application # 9010980
 
Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND)
 
Program Officer Wong, May Project Start 2009-09-30
 
Project End 2018-02-28
 
Budget Start 2016-03-01
 
Budget End 2017-02-28
 
Support Year 7
 
Fiscal Year 2016
 
Total Cost $409,868
 
Indirect Cost $134,234 Institution Name Colorado State University-Fort Collins
 
Department Microbiology/Immun/Virology
 
Type Schools of Veterinary Medicine
 
DUNS # 785979618 City Fort Collins
 
State CO
 
Country United States
 
Zip Code 80523
 
 
LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$
 
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
 
 
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS
 
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***
 
O18
 
Zoonotic Potential of CWD Prions
 
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas, USA
 
*** These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.
 
==================
 
***These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.***
 
==================
 
P.105: RT-QuIC models trans-species prion transmission
 
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA
 
Conversely, FSE maintained sufficient BSE characteristics to more efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was competent for conversion by CWD and fCWD.
 
***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.
 
================
 
***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.***
 
================
 
 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
 
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
 
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
 
 
 
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********
 
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
 
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)
 
These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
 
Table 9 presents the results of an analysis of these data.
 
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
 
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
 
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
 
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
 
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
 
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
 
snip...
 
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
 
snip...
 
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
 
snip...
 
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
 
snip...see full report ;
 
 
CJD9/10022
 
October 1994
 
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
 
Dear Mr Elmhirst,
 
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
 
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
 
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
 
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
 
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
 
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
 
 
Monday, May 02, 2016
 
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
 
 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD
 
 
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
 
*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,
 
*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.
 
PPo2-27:
 
Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions
 
*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.
 
PPo2-7:
 
Biochemical and Biophysical Characterization of Different CWD Isolates
 
*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.
 
 
Envt.07:
 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
 
 
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<
 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
 
Wednesday, January 01, 2014
 
Molecular Barriers to Zoonotic Transmission of Prions
 
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
 
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
 
 
 
Thursday, August 25, 2016
 
TPWD Action Disease Detection and Response – Chronic Wasting Disease TPW Commission Adopts New CWD Zones, Deer Movement Rules August 25, 2016 This map shows the recently imposed Surveillance Zone for CWD in portions of Bandera, Medina and Uvalde counties.
 
 
Saturday, April 23, 2016
 
*** SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016 ***
 
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
 
 
Monday, May 02, 2016
 
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
 
 
======================================================
 
Wednesday, June 29, 2016
 
*** NIH awards $11 million to UTHealth researchers to study deadly CWD prion diseases Claudio Soto, Ph.D. ***
 
Public Release: 29-Jun-2016
 
 
Monday, August 22, 2016
 
*** CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES ***
 
 
Transmission of scrapie prions to primate after an extended silent incubation period
 
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
 
 
Thursday, August 18, 2016
 
*** PROCEEDINGS ONE HUNDRED AND Nineteenth ANNUAL MEETING of the USAHA BSE, CWD, SCRAPIE, PORCINE TSE PRION October 22 28, 2015 ***
 
 
Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie Experiment 1964
 
How Did CWD Get Way Down In Medina County, Texas?
 
Confucius ponders...
 
Could the Scrapie experiments back around 1964 at Moore Air Force near Mission, Texas, could this area have been ground zero for CWD TSE Prion (besides the CWD cases that have waltzed across the Texas, New Mexico border near WSMR Trans Pecos region since around 2001)?
 
Epidemiology of Scrapie in the United States 1977
 
snip...
 
Scrapie Field Trial Experiments Mission, Texas
 
A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission, Texas. It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.
 
The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds. They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed. Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary. The station was divided into 2 areas: (1) a series of pastures and-pens occupied by male animals only, and (2) a series of pastures and pens occupied by females and young progeny of both sexes. ...
 
snip...see full text ;
 
 
Thursday, June 09, 2016
 
Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie TSE Prion Experiment 1964
 
How Did CWD Get Way Down In Medina County, Texas?
 
 
 
Thursday, August 04, 2016
 
MEETING ON THE FEASIBILITY OF CARRYING OUT EPIDEMIOLOGICAL STUDIES ON CREUTZFELDT JAKOB DISEASE 1978 THE SCRAPIE FILES IN CONFIDENCE CONFIDENTIAL SCJD
 
 
Tuesday, August 9, 2016
 
*** Concurrence with OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2015-0055]
 
 
Saturday, July 23, 2016
 
*** BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
 
 
Tuesday, July 26, 2016
 
*** Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016
 
 
Saturday, July 16, 2016
 
*** Importation of Sheep, Goats, and Certain Other Ruminants [Docket No. APHIS-2009-0095]RIN 0579-AD10
 
WITH great disgust and concern, I report to you that the OIE, USDA, APHIS, are working to further legalize the trading of Transmissible Spongiform Encephalopathy TSE Pion disease around the globe.
 
THIS is absolutely insane. it’s USDA INC.
 
 
Thursday, October 22, 2015
 
*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened those mad cows in Texas ***
 
 
Monday, June 20, 2016
 
*** Specified Risk Materials SRMs BSE TSE Prion Program ***
 
 
Thursday, April 14, 2016
 
Arizona 22 year old diagnosed with Creutzfeldt Jakob Disease CJD
 
 
Thursday, January 15, 2015
 
41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report
 
 
Saturday, January 17, 2015
 
*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease
 
 
Saturday, December 12, 2015
 
CREUTZFELDT JAKOB DISEASE CJD TSE PRION REPORT DECEMBER 14, 2015
 
 
Sunday, August 21, 2016
 
Kay Ellen Roedl Schwister Deceased August 7, 2016 at the age of 53 with Creutzfeldt-Jakob disease CJD TSE Prion spontaneous sporadic, zoonosis, or iatrogenic?
 
 
Monday, August 22, 2016
 
*** CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES ***
 
 
Using in vitro prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.
 
Claudio Soto
 
Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.
 
Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.
 
=========================
 
***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.
 
========================
 
Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.
 
 
see ;
 
with CWD TSE Prions, I am not sure there is any absolute yet, other than what we know with transmission studies, and we know tse prion kill, and tse prion are bad. science shows to date, that indeed soil, dirt, some better than others, can act as a carrier. same with objects, farm furniture. take it with how ever many grains of salt you wish, or not. if load factor plays a role in the end formula, then everything should be on the table, in my opinion. see ;
 
***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.
 
Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.
 
 
see ;
 
 
Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil Particles
 
Author Summary
 
Transmissible spongiform encephalopathies (TSEs) are a group of incurable neurological diseases likely caused by a misfolded form of the prion protein. TSEs include scrapie in sheep, bovine spongiform encephalopathy (‘‘mad cow’’ disease) in cattle, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Scrapie and chronic wasting disease are unique among TSEs because they can be transmitted between animals, and the disease agents appear to persist in environments previously inhabited by infected animals. Soil has been hypothesized to act as a reservoir of infectivity and to bind the infectious agent. In the current study, we orally dosed experimental animals with a common clay mineral, montmorillonite, or whole soils laden with infectious prions, and compared the transmissibility to unbound agent. We found that prions bound to montmorillonite and whole soils remained orally infectious, and, in most cases, increased the oral transmission of disease compared to the unbound agent. The results presented in this study suggest that soil may contribute to environmental spread of TSEs by increasing the transmissibility of small amounts of infectious agent in the environment.
 
 
tse prion soil
 
 
 
 
 
Saturday, May 28, 2016
 
*** Infection and detection of PrPCWD in soil from CWD infected farm in Korea Prion 2016 Tokyo ***
 
 
Wednesday, December 16, 2015
 
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
 
 
The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.
 
 
>>>Particle-associated PrPTSE molecules may migrate from locations of deposition via transport processes affecting soil particles, including entrainment in and movement with air and overland flow. <<<
 
Fate of Prions in Soil: A Review
 
Christen B. Smith, Clarissa J. Booth, and Joel A. Pedersen*
 
Several reports have shown that prions can persist in soil for several years. Significant interest remains in developing methods that could be applied to degrade PrPTSE in naturally contaminated soils. Preliminary research suggests that serine proteases and the microbial consortia in stimulated soils and compost may partially degrade PrPTSE. Transition metal oxides in soil (viz. manganese oxide) may also mediate prion inactivation. Overall, the effect of prion attachment to soil particles on its persistence in the environment is not well understood, and additional study is needed to determine its implications on the environmental transmission of scrapie and CWD.
 
 
P.161: Prion soil binding may explain efficient horizontal CWD transmission
 
Conclusion. Silty clay loam exhibits highly efficient prion binding, inferring a durable environmental reservoir, and an efficient mechanism for indirect horizontal CWD transmission.
 
 
>>>Another alternative would be an absolute prohibition on the movement of deer within the state for any purpose. While this alternative would significantly reduce the potential spread of CWD, it would also have the simultaneous effect of preventing landowners and land managers from implementing popular management strategies involving the movement of deer, and would deprive deer breeders of the ability to engage in the business of buying and selling breeder deer. Therefore, this alternative was rejected because the department determined that it placed an avoidable burden on the regulated community.<<<
 
Wednesday, December 16, 2015
 
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
 
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
 
Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1
 
1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK
 
Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.
 
snip...
 
Discussion
 
Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).
 
Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing. Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building. Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9). The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture. When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.
 
This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep. Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease. It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled. Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases. Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.
 
PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice. In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals. In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay. False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28). This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm. This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc. In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc. Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material. In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12). A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30). This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model. Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.
 
In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.
 
Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification
 
 
Wednesday, December 16, 2015
 
*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***
 
 
*** Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle ***
 
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
 
snip...
 
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
 
 
 
 
In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells
 
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...
 
 
”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” ...page 26.
 
 
*** Calling Canadian beef unsafe is like calling your twin sister ugly," Dopp said.
 
Thursday, August 25, 2016
 
*** FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA MAD COW TYPE DISEASE ***
 
 
Friday, August 26, 2016
 
Journal Journal of Toxicology and Environmental Health, Part A Volume 79, 2016 - Issue 16-17 Prion Research in Perspective IV CANADA BSE CWD SCRAPIE CJD TSE Prion Disease
 
 
to be continued...
 
Terry S. Singeltary Sr, Bacliff, Texas USA 77518
 

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