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From: TSS ()
Subject: Bovine Spongiform Encephalopathy: what is "Atypical BSE" and can we detect it?
Date: November 26, 2006 at 6:03 pm PST

Title: Bovine Spongiform Encephalopathy: what is "Atypical BSE" and can we detect it?


Richt, Juergen

Submitted to: Animal Health Research Reviews
Publication Type: Abstract
Publication Acceptance Date: October 17, 2006
Publication Date: N/A

Technical Abstract: Transmissible spongiform encephalopathy (TSE) agents induce fatal neurodegenerative diseases in humans and in some other mammalian species. Human TSEs include Creutzfeldt¿Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, Kuru and Fatal Familial Insomnia (FFI). In animals, several distinct TSE diseases are recognized: scrapie in sheep and goats, transmissible mink encephalopathy (TME) in mink, chronic wasting disease (CWD) in cervids, and bovine spongiform encephalopathy (BSE) in cattle. BSE was first detected in 1986 in the United Kingdom and is the most likely cause of variant CJD in humans. BSE in cattle is a neurological disease with a characteristic molecular pattern of the protease-resistant prion protein, PrP**res. This BSE 'signature' has also been identified in BSE-induced TSEs of both domestic cats and exotic ruminant species. Since 2004, some cases of prion diseases in cattle have been described which show unusual or atypical features as assessed by the molecular characterization of PrP**res and/or histopathology, when compared to the unique features of previously described BSE. These atypical BSE cases have been characterized by Western blot and have been referred to as H- (i.e., high molecular weight) or L-type (i.e., low molecular weight type). These atypical BSE cases have been found mainly in cattle older than 8 years. In the U.S., three cases of BSE have been diagnosed so far. Case 1 represented a typical BSE isolate, identified in an animal imported from Canada. Cases 2 and 3 were identified in animals raised in the U.S. and revealed an unusual molecular PrP**res pattern, consistent with atypical BSE cases described as H-type in Europe. It should be noted that the Western Blot method applied for BSE confirmatory tests in the U.S. has been able to detect both H-type and L-type BSE cases when using known positive European samples.

Title: Identification and Characterization of U.S. Bse Cases


Richt, Juergen

Submitted to: Meeting Abstract
Publication Type: Abstract
Publication Acceptance Date: April 25, 2006
Publication Date: May 28, 2006
Citation: Hall, S.M., Richt, J., Davis, A., Kluge, J., Simmons, M., Stack, M., Spencer, Y. 2006. Identification and characterization of U.S. BSE cases [abstract]. Prion Diseases of Domestic Livestock. p. 25.

Technical Abstract: Bovine Spongiform Encephalopathy (BSE) surveillance has been ongoing in the USA since the early 1990¿s and initial testing was done at the USDA, National Veterinary Services Laboratories (NVSL) utilizing routine histopathology exclusively. In 1995, the immunohistochemistry (IHC) test was incorporated into surveillance testing in addition to routine histopathology. By 1999 virtually all BSE screening was performed by IHC and by 2001 the NVSL had switched to an automated IHC procedure. In 2002 and 2003 the NVSL tested about 20,000 high risk animals each year by IHC. In December, 2003 an animal was identified by IHC as positive for BSE (Case 1); this animal was determined to be imported from Canada. After this animal was identified, in June 2004 the USDA began its enhanced surveillance program as a shared effort between selected state veterinary diagnostic laboratories and NVSL, as part of the National Animal Health Laboratory Network. The plan called for testing as many targeted high risk animals as possible in a 12-18 month period. From June 1, 2004 through March 21, 2006, over 650,000 animals have been tested (Bio-Rad ELISA). Of those tested, two animals (Cases 2 and 3) have been identified as positive for BSE. While all three cases were strongly positive by Bio-Rad ELISA, Cases 2 and 3 have common features which are distinct from Case 1. Definitive spongiform changes in the obex, strong immunohistochemical reactions, and Western blot patterns similar to European BSE cases were observed in Case 1. In contrast, Cases 2 and 3 did not contain definitive histological lesions of BSE and the IHC staining was less intense than Case 1. In addition, Cases 2 (approximately 12 years) and Case 3 (approximately ten years) were older animals while Case 1 was 6.5 years old. Western blot analysis, PrP**Sc from Case 1 showed molecular features similar to typical BSE isolates, whereas PrP**Sc from Cases 2 and 3 revealed an unusual molecular PrP**Sc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates. Case 1 contained more PrP**Sc per brain tissue mg equivalent compared with Cases 2 and 3 using antibody 6H4. In Western Blot analysis, Case 2 and Case 3 were strongly positive with antibody P4, while Case 1 was negative or weakly positive with P4.

2005 Annual Report

4d.Progress report.
This report serves to document research conducted under a specific cooperative agreement between ARS and the Italian Reference Centre for Animal TSE (CEA) at the Istituto Zooprofilattico Sperimentale, Turin, Italy. Additional details of research can be found in then report for the parent project 3625-32000-073-00D, Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies. The aim of the cooperative research project conducted by the CEA and ARS is to compare the U.S. bovine spongiform encephalopathy (BSE) isolate and the bovine amyloidotic spongiform encephalopathy isolates (BASE) identified in Italy. The first objective was to determine whether diagnostic methods routinely used by USDA are able to identify the Italian BASE cases. For this purpose, CEA received the immunohistochemistry (IHC) protocol developed by APHIS-USDA. The IHC protocol was reproduced and standardized in the CEA laboratory and will be applied to the Italian BSE and BASE cases. Furthermore, fixed brainstem sections and frozen brainstem material from Italian BSE and BASE cases will be sent to ARS for analysis using USDA IHC and Western blot (WB) methods. These studies will enable us to determine whether the present diagnostic tools (IHC and WB) employed at the USDA will be able to detect the Italian BASE cases and also enable us to compare Italian BSE and BASE with the U.S. BSE cases.

Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-073-07
Project Type: Specific C/A

Start Date: Sep 15, 2004
End Date: Sep 14, 2007

The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.

This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.

2005 Annual Report

4d.Progress report.
This report serves to document research conducted under a specific cooperative agreement between ARS and Veterinary Laboratories Agency (VLA)-Weybridge, New Haw, Addlestone, Surrey, UK. Additional details of research can be found in the report for the parent project 3625-32000-073-00D, Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies. The objective of the cooperative research project conducted by ARS and the VLA is to obtain material from an oral BSE pathogenesis study performed at the VLA-Weybridge in the United Kingdom. The BSE materials will be used to identify PrP**res tissue distribution and migration in BSE infected cattle. In order to obtain BSE-infected tissues from the TSE Archive at the VLA, a request including a scientific justification has to be made to an Independent Archive Advisory Group (IAAG) which oversees the TSE Archive at the VLA. Two requests have been made in 2005 and the approval for obtaining BSE-infected materials is pending at the time of writing.


On the Question of Sporadic

or Atypical Bovine SpongiformEncephalopathy and

Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

Strategies to investigate the possible existence of sporadic

bovine spongiform encephalopathy (BSE) require

systematic testing programs to identify cases in countries

considered to have little or no risk for orally acquired disease,

or to detect a stable occurrence of atypical cases in

countries in which orally acquired disease is disappearing.

To achieve 95% statistical confidence that the prevalence

of sporadic BSE is no greater than 1 per million (i.e., the

annual incidence of sporadic Creutzfeldt-Jakob disease

[CJD] in humans) would require negative tests in 3 million

randomly selected older cattle. A link between BSE and

sporadic CJD has been suggested on the basis of laboratory

studies but is unsupported by epidemiologic observation.

Such a link might yet be established by the discovery

of a specific molecular marker or of particular combinations

of trends over time of typical and atypical BSE and various

subtypes of sporadic CJD, as their numbers are influenced

by a continuation of current public health measures that

exclude high-risk bovine tissues from the animal and

human food chains. ......


3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years. These results indicate that BASE is
transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.

6:30 Close of Day One

1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)



9 December 2005
Division of Dockets Management (RFA-305)

James J. Kramer, Ph.D.
Vice President, Corporate Operations

Embassy of Japan

Dockets Entered on December 22, 2005
2005D-0330, Guidance for Industry and FDA Review Staff on Collection of
by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...

03-025IF 03-025IF-631 Linda A. Detwiler [PDF]
Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4.
Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12.

03-025IF 03-025IF-634 Linda A. Detwiler [PDF]
Page 1. 03-025IF 03-025IF-634 Linda A. Detwiler Page 2.
Page 3. Page 4. Page 5. Page 6. Page 7. Page 8.

Page 1 of 17 9/13/2005 [PDF]
... 2005 6:17 PM To: Subject: [Docket
No. 03-025IFA]
FSIS Prohibition of the Use of Specified Risk Materials for Human Food ...

03-025IFA 03-025IFA-6 Jason Frost [PDF]
... Zealand Embassy COMMENTS ON FEDERAL REGISTER 9 CFR Parts 309 et al
[Docket No. 03-
025IF] Prohibition of the Use of Specified Risk Materials for Human Food and

In its opinion of 7-8 December 2000 (EC 2000), the SSC ... [PDF]
Page 1. Linda A. Detwiler, DVM 225 Hwy 35 Red Bank, New Jersey 07701 Phone:
Cell: 732-580-9391 Fax: 732-741-7751 June 22, 2005 FSIS Docket Clerk US ...

Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.




your only fooling yourselves with this stupid ukbsenvcjd only theory, and

BSE methology of the OIE. most any coutnry that went by those same OIE

BSE guidelines all went down with BSE.

THE OIE has now shown they are nothing more than a National Trading
Brokerage for all strains of animal TSE.
AS i said before, OIE should hang up there jock strap now, since it appears
they will buckle every time a country

makes some political hay about trade protocol, commodities and futures. IF
they are not going to be science based, they

should do everyone a favor and dissolve there organization. ...

O.K., let me get this straight. after the first documented case of BSE in the USA,

the rest i.e (the next two documented TSE cows) became atypical, of which USDA

now wants us to believe are of a spontaneous nature, that feed did not cause this?

r i g h t ............

IF typical BSE spread via feed, why can't atypical BSE or BASE spread the same way ???

STUDIES in Mission Texas of USA sheep scrapie to USA produced a TSE unlike BSE.

THE USA has been rendering and feeding that same TSE back into feed for animals for human and animal consumption for decades.

WHY would we have not documented these supposedly 'spontaneous' BASE cases previously ???

spontaneous TSE in any species is a myth. ...

It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

1: J Infect Dis. 1994 Apr;169(4):814-20.

Intracerebral transmission of scrapie to cattle.

Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM, Robinson MM.

USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA 50010.

To determine if sheep scrapie agent(s) in the United States would induce a disease in cattle resembling bovine spongiform encephalopathy, 18 newborn calves were inoculated intracerebrally with a pooled suspension of brain from 9 sheep with scrapie. Half of the calves were euthanatized 1 year after inoculation. All calves kept longer than 1 year became severely lethargic and demonstrated clinical signs of motor neuron dysfunction that were manifest as progressive stiffness, posterior paresis, general weakness, and permanent recumbency. The incubation period was 14-18 months, and the clinical course was 1-5 months. The brain from each calf was examined for lesions and for protease-resistant prion protein. Lesions were subtle, but a disease-specific isoform of the prion protein was present in the brain of all calves. Neither signs nor lesions were characteristic of those for bovine spongiform encephalopathy.

MeSH Terms: Animals Brain/microbiology* Brain/pathology Cattle Cattle Diseases/etiology* Cattle Diseases/pathology Encephalopathy, Bovine Spongiform/etiology* Encephalopathy, Bovine Spongiform/pathology Immunoblotting/veterinary Immunohistochemistry Male Motor Neurons/physiology Prions/analysis Scrapie/pathology Scrapie/transmission* Sheep Sleep Stages Time Factors

Substances: Prions


Intracerebral transmission of scrapie to cattle FULL TEXT PDF;



WE conclude that American sources of sheep scrapie are transmissible to cattle by direct intracerebral inoculation but the disease induced is NOT identical to BSE as seen in the United Kingdom. While there were similarities in clinical signs between this experimental disease and BSE, there was no evidence of aggressiveness, hyperexcitability, hyperesthesia (tactile or auditory), or hyperemetria of limbs as has been reported for BSE (9). Neither were there extensive neurologic lesions, which are primary for BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis and gliosis. Although some vacuolation of neuropil, chromotolysis in neurons, and gliosis were seen in the brains of some affected calves, these were industinguishable from those of controls. Vacuolated neurons in the red nucleus of both challenged and normal calves were considered normal for the bovines as previously described (50).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, and the amount of PrP-res positively related to the length of the incubation. ...


WE also conclude from these studies that scrapie in cattle MIGHT NOT BE RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGEST THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is necessary to make a definitive diagnosis. THUS, undiagnosed scrapie infection could contribute to the ''DOWNER-COW'' syndrome and could be responsible for some outbreaks of transmissible mink encephalopathy proposed by Burger and Hartsough

(8) and Marsh and harsough (52). ...


Multiple sources of sheep affected with scrapie and two breeds of cattle from several sources were used inthe current study in an effort to avoid a single strain of either agent or host. Preliminary results from mouse inoculations indicate multiple strains of the agent were present in the pooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by using intracerebral, intramuscular, subcutaneous, and oral routes of inoculation of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1 affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48 months after inoculation. Signs were disorientation, incoordination, a stiff-legged stilted gait, progressive difficulty in rising, and finally in terminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattle similarly inoculated with brain tissue from a goat with scrapie exhibited similar signs 27 and 36 months after incoluation. Clinical courses were 43 an 44 days. Brain lesions of mild gliosis and vacuolation and mouse inoculation data were insufficient to confirm a diagnosis of scrapie. This work remained controversial until recent examination of the brains detected PrP-res in all 3 cattle with neurologic disease but in none of the unaffected cattle (62). Results of these studies are similar to ours and underscore the necessity of methods other than histopathology to diagnose scrapie infection in cattle. We believe that immunologic techniques for detecting PrP-res currently provide the most sensitive and reliable way to make a definitive diagnosis...

Visit to USA ... info on BSE and Scrapie


Title: Experimental Transmission of Transmissible Mink Encephalopathy (Tme) to Cattle by Intracerebral Inoculation


Hamir, Amirali
Kunkle, Robert
Miller, Janice - ARS RETIRED
Greenlee, Justin
Richt, Juergen

Submitted to: International Veterinary Vaccines and Diagnostics Conference
Publication Type: Abstract
Publication Acceptance Date: March 15, 2006
Publication Date: June 25, 2006
Citation: Hamir, A.N., Kunkle, R.A., Miller, J.M., Greenlee, J.J., Richt, J.A. 2006. Experimental transmission of transmissible mink encephalopathy (TME) to cattle by intracerebral inoculation [abstract]. 4th International Veterinary Vaccines and Diagnostics Conference. p. 89. Paper No. PO53.

Technical Abstract: To compare clinicopathological findings of transmissible mink encephalopathy (TME) with other transmissible spongiform encephalopathies (TSE, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie, and chronic wasting disease, CWD), 2 groups of calves (n = 4 each) were intracerebrally inoculated with TME agents from 2 different sources (mink with TME and a bovine with TME). Two uninoculated calves served as controls. Within 15.3 months post inoculation (PI), all animals from both inoculated groups developed clinical signs of central nervous system (CNS) abnormality; their CNS tissues had microscopic spongiform encephalopathy (SE); and PrP**res was detected in their CNS tissues by immunohistochemistry (IHC) and Western blot (WB) techniques. These findings demonstrate that intracerebrally inoculated cattle not only amplify TME PrP**res but also develop clinical CNS signs and extensive lesions of SE. The latter has not been shown with other TSE agents (scrapie and CWD) similarly inoculated into cattle. The findings also suggest that the diagnostic techniques currently used for confirmation of bovine spongiform encephalopathy (BSE) would detect TME in cattle should it occur naturally. However, it would be a diagnostic challenge to differentiate TME in cattle from BSE. Our recent preliminary results indicate that WB may be able to differentiate between bovine TME and BSE.

Date: July 15, 2006 at 5:52 am PST

Pelt Production Up 3 Percent

Mink pelt production in the United States in 2005 totaled
2.63 million pelts, up 3 percent from 2004. Wisconsin, the largest
mink producing State, produced 778,000 pelts. Utah the second
largest producing State, produced 600,000 pelts.

The number of pelts by color class as a percent of the total U.S.
production in 2005 is as follows: Black at 47.6 percent, Mahogany
at 20.9 percent, Blue Iris at 11.3 percent, Demi/Wild at
6.3 percent, Sapphire at 4.0 percent, and White at 3.8 percent.
The remaining color classes accounted for 6.1 percent.

Value of Pelt Production Up 33 Percent

Mink pelts produced during the 2005 crop year were valued at
$160 million, up 33 percent from $120 million a year ago. The
average price per pelt for the 2005 crop year was $60.90, up from
$47.10 in 2004. .....snip.......end


3.9.11 Mink Producers

Mink offal is now rendered with other species and will decline in value under the first four

regulatory options.


Mink are raised for their pelts and oil. Most mink farmers kill and pelt their own animals

once a year near the end of November or in early December. Once the pelts are removed, the fat is

then scrapped from the hide. This fat is used to manufacture mink oil that is sought for cosmetic

uses because of its hypoallergenic qualities and in leather treatments. The total value of mink

production in 1995 was $143 million, an increase of 72 percent from 1994.

In 1995, 446 mink farms produced a total of 2.69 million pelts (NASS, 1996b). Mink

producers vary in size but most are small operations. Mink farming is concentrated in Utah (130


farms), Wisconsin (77 farms), and Minnesota (52 farms). There has been recent consolidation

within the industry, with the number of farms decreasing by 8 percent from 1993 to 1994 and 3

percent from 1994 to 1995. The market price for mink pelts is subject to wide demand fluctuations

based on fashion and weather.

Once the pelt and fat are removed, the entire carcass is then rendered. Mink carcasses sent

to rendering (minus the pelt and fat) weigh an average of 2.5 pounds, so the total estimated offal

produced per year is 6.7 million pounds. Mink farmers are reported to have difficulty with getting

renderers to pick-up their material because of its low volume and the infrequency of offal


WHAT sort of TME surveillance program is in place now, if any???

DO they test for TSE in Mink and what are these figures if so ???

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

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