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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Tuesday, April 28, 2009


-------------------- BSE-L@LISTS.AEGEE.ORG --------------------



Greetings BSE-L,

I find it disturbing that the Nor-98 atypical cases of scrapie in the USA are NOT documented on the APHIS web page. this makes no sense, and i have ask about it before in the past, APHIS et al refuse to post a seperate map showing the documented Nor98-like atypical cases here in the USA, all of them ? said they would look into it years ago. it's like out of sight, out of mind, like the TSE in the USA bovine. THE ONLY mention of the Nor-98 like scrapie that has been documented in the USA to date was on CHART 3, and then it is very very misleading as to the actual number of cases. it states ;

> * Through March 31, 2009 - does not include the Nor-98-like Scrapie cases found through RSSS (2 in FY 2007 and 1 in FY 2008). < href="">


Thank You,
kind regards,


-------------------- BSE-L@LISTS.AEGEE.ORG --------------------


>>BY my records, to date, there have been 6 cases of the Nor-98-like atypical scrapie documented in the USA.<<<

>>Maybe one of the USDA followers here on the BSE-L, that is in the Scrapie department, would be so kind as to help us clear this up?<<<

oh well, so much for the USDA followers here........ however, i did find this ;

Nor98-like Scrapie in the United States of America

was presented by Drs. Christina M. Loiacono, S. Mark Hall, and Bruce V. Thomsen, National Veterinary Services Laboratory, USDA-APHIS-VS.

This paper describes the first six sheep diagnosed with Nor98-like disease in the United States and serves to acknowledge the increased efforts of diagnosticians and the USDA program to control and eradicate scrapie disease. Classical scrapie, a fatal neurodegenerative disease affecting the central nervous system of sheep and goats, is among a number of diseases classified as transmissible spongiform encephalopathies (TSEs). Recently, a distinct strain of scrapie was diagnosed in sheep in Norway1 and has been identified in numerous countries of the European Union (EU). The disease has been identified, among other names, as Nor98 or Nor98-like scrapie. Distinctions between classical scrapie and Nor98-like scrapie are made based on signalment, clinical signs, histopathology and immunodiagnostic results. In the past, the classical scrapie disease was confirmed by examination of the brain tissue for a triad of histopathological signs – vacuolation, loss of neurons and gliosis – and, more recently, by immunohistochemical (IHC) or biochemical detection of abnormal prion protein (PrPSc) in the brain, or lymphoid tissues. In the case of Nor98-like scrapie there is generally little or no vacuolation in the brain and, to date, no lymphoid accumulation of PrPSc has been detected. Classical scrapie typically has the most intense PrPSc immunostaining at the obex (motor nucleus of the vagus), while this area is spared in Nor98-like scrapie. Alternatively, Nor98-like scrapie consistently has PrPSc immunostaining in the spinal nucleus of the trigeminal nerve and variable, but often an intense immunostaining for PrPSc in the cerebellum. Thus the diagnosis of Nor98 and Nor98-like disease can be based on immunohistochemistry identifying abnormal prion protein in regions of the brain not typically associated with classical scrapie. Additionally there is a distinct diagnostic western blot pattern for Nor98 and Nor-98 like disease consisting of three or more protein bands with the unglycosylated band being less than 15 kd, compared to classical scrapie in which the unglycosylated band is greater than 15 kd. Nor98 and Nor-98 like disease is associated with older sheep, usually greater than four years of age, while sheep in the range of three to five years of age are more commonly affected by classical scrapie. Clinical signs are uncommon with Nor98 and Nor98-like disease but when present most often include ataxia without pruritis. Genotypes known to provide sheep with resistance to classical scrapie are not spared from Nor98 and Nor98-like disease. The six U.S. cases had no clinical signs reported. Three cases were detected during slaughter surveillance, two were detected as a result of classical scrapie being found in the flock, one found during testing associated with diagnostic necropsy. Five of the 6 cases had genotypes that are susceptible to classical scrapie and one was AARR. Only one Nor98-like scrapie case was found per flock. 1. Benestad SL, Sarradin P, Thu B, Schönheit J, Tranulis MA, Bratberg B., Cases of scrapie with unusual features in Norway and designation of a new type, Nor98. Vet. Rec.'Rourke,%20Katherine.pdf

NOW, if there have been more of the Nor-98 documented to date, know one would know since NO MAP for the Nor-98 total cases in the USA is posted on the USDA/APHIS scrapie maps monthly report. ...TSS


Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.



R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000422/!; 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as “atypical” scrapie, as opposed to “classical scrapie”. Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.


Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007


Monday, December 1, 2008 When Atypical Scrapie cross species barriers


This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404



A The Present Position with respect to Scrapie A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.

The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.

It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.



Epidemiology of Scrapie in the United States 1977

Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284 FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in ...


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Friday, April 03, 2009

ARS and Partners Set Their Sights on Scrapie in Goats (live test)

ARS and Partners Set Their Sights on Scrapie in Goats

ARS scientists at the Animal Disease Research Unit in Pullman, Washington, are using a new live-animal test for scrapie in goats and studying genes for resistance to the disease. Here, microbiologist Katherine O’Rourke looks over twin goats born to a naturally scrapie-infected mother. The twins have different genotypes that will be studied for disease susceptibility and resistance. (D1362-1)

Goats are tough, spirited animals. But they’re no match for scrapie, a form of transmissible spongiform encephalopathy. In goats—and sheep—the degenerative disease causes tremors, lip-smacking, weight loss, a hopping gait, and other peculiar symptoms. Scrapie-afflicted animals cannot be cured, and they eventually die.

Since 2001, USDA’s Animal and Plant Health Inspection Service (APHIS) has coordinated an accelerated National Scrapie Eradication Program to eliminate the disease from U.S. sheep and goats. But transmission routes, progression, and genetic underpinnings of scrapie in goats are poorly understood. Low occurrence rates, underreporting, and the inconvenience and cost of tissue testing make eradication challenging.

The good news is that a new, live-animal test to detect scrapie is being applied to goats. There’s also an ongoing study of goat genes that might confer resistance to the disease.

Building on Early Successes

The new method, known as the “rectal mucosal-associated lymphoid tissue (RMALT) test,” is based on the currently used third-eyelid test. Developed by the Agricultural Research Service and Washington State University (WSU), the third-eyelid test has been used by APHIS and state veterinarians since 2002 as an official test to detect scrapie in sheep. It involves snipping a tiny piece of lymphoid tissue from the animal’s nictitating membrane, or third eyelid, staining it with antibodies, and examining it under a microscope. Lymphoid tissue is used because it tends to collect malformed proteins called “prions,” which are thought to cause scrapie.

Microbiologist Katherine O’Rourke and molecular biologist Tom Truscott obtain tissue from a goat for use in a new test for scrapie. (D1360-1)

Researchers in Norway and Scotland modified the method for a different site: lymphoid tissue in the lining of the animal’s rectum. The rectal biopsy has also been used in deer, elk, sheep, and now goats. It’s quick to perform and relatively painless to the animal—thanks to a dab of local anesthetic.

“Rectal biopsy also allows for more repeat samples from an individual animal when needed,” says ARS microbiologist Katherine I. O’Rourke, a member of the scrapie research team, which includes APHIS Veterinary Services (VS) and Wildlife Services, the National Park Service, Colorado State University, and the Canadian Food Inspection Agency.

In January 2008, APHIS-VS approved use of the RMALT test as a scrapie-detection tool after large-scale validation trials comparing its accuracy (87 percent) to third-eyelid biopsies and postmortem examinations of tissue from infected sheep. Additional work on the accuracy of the test in goats is under way.

APHIS has incorporated the RMALT test into the National Scrapie Eradication Program. Current users are mainly federal and state veterinary personnel.

Molecular biologist Tom Truscott views a live-animal test for scrapie. The red areas indicate the presence of the scrapie prion protein. (D1364-1)

Setting the Stage for Selective Breeding

A key facet of scrapie prevention in sheep flocks involves use of selective breeding to increase the number of sheep with the version of the prion protein gene (dubbed “R171”) that confers resistance. For now, goat producers don’t have that option. Despite searches by ARS and WSU scientists at Pullman, as well as at other labs, the R171 prion gene version has never been found in goats. But there are several different gene variants in goats, some of which might confer resistance to scrapie. No one has confirmed genetic resistance in goats thus far, but some tantalizing leads are emerging.

“In sheep, the discovery of resistance genes was key to developing a broadly accepted eradication program. If scrapie is found in a flock, only the genetically susceptible sheep are removed, allowing the producer to maintain quality animals,” says O’Rourke. “As we learn more about goat genetic resistance, we hope the same approach can work for them.”

The live-animal test for scrapie requires removal of a small snip of tissue after a local anesthetic is applied. (D1361-1)

Towards that end, ARS Pullman geneticist Stephen White is leading a team of ARS and university scientists to characterize the prion protein gene of goats and identify important gene variants in individuals and breeds. The Pullman team has so far examined the prion protein gene sequences from 446 goats representing 10 breeds, 8 of which have never been genetically characterized for their potential scrapie response.

The team’s analysis found four gene variants (R143, S146, H154, and K222) in the genes of Boer, Nubian, Saanen, Toggenburg, and a few other goat breeds. These gene variants were relatively rare or absent in animals that developed scrapie in previous outbreaks, which suggests these gene variants might help the animals in some way. A fifth variant (M142) was found mainly in Alpine and Toggenburg goats, and it is known to delay incubation of scrapie from infection to clinical disease. More work is needed to demonstrate true resistance for any of these genetic variations, notes the team in a paper published in the September-October 2008 issue of Genetic Selection Evolution.

Geneticist Stephen White studies goats with a version of the prion gene that might confer increased resistance to scrapie. (D1363-3)

Putting Together the Pieces

In related work, the Pullman scientists are monitoring six goats from a Michigan farm with a known history of scrapie infection. In March, two of the infected goats, named “Nutmeg” and “Meeko,” gave birth to three kids, providing the scientists with an unprecedented look into parent-to-offspring transmission of scrapie and inheritance of genes affecting the animal’s response to the disease.

“We learned that—unlike in sheep—the goat placenta is not a very reliable indicator of the scrapie status of the dam,” says O’Rourke. “We’ll do some more research to determine why this is, and how it affects transmission in a herd.”

Molecular biologist Jim Reynolds extracts DNA for genetic testing to see which goats might have increased resistance to scrapie. (D1365-1)

Genetic testing revealed that Meeko’s kid is genetically susceptible, which allows for monitoring of scrapie’s onset and development during the animal’s life. Nutmeg gave birth to twins—one genetically susceptible and the other with a variation that may eventually prove to confer resistance.

Besides their university colleagues, ARS’s Pullman team, led by Donald P. Knowles, has collaborated closely with APHIS-VS to formulate a strategy aimed at helping the U.S. goat industry to eliminate scrapie. The current effort takes a multipronged approach that includes early detection through slaughter surveillance and reporting of clinical suspects, flock management and selective breeding in sheep, scrapie-free flock certification, and producer outreach and education.

“In support of the eradication effort led by APHIS and industry, ARS will continue to do research on genetic resistance, diagnostic testing, and transmission modes,” says O’Rourke. “Prevention is always a more desirable route than removal of infected animals or exposed animals. So research on resistance genetics and transmission modes will be especially important contributions.”—By Jan Suszkiw, Agricultural Research Service Information Staff.

This research is part of Animal Health, an ARS national program (#103) described on the World Wide Web at

To reach scientists mentioned in this article, contact Jan Suszkiw, USDA-ARS Information Staff, 5601 Sunnyside Ave., Beltsville, MD 20705-5129; phone (301) 504-1630, fax (301) 504-1486.

"ARS and Partners Set Their Sights on Scrapie in Goats" was published in the April 2009 issue of Agricultural Research magazine.

Wednesday, January 28, 2009

TAFS1 Position Paper on BSE in small ruminants (January 2009)


NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007


Monday, September 1, 2008


September 1, 2008

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