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From: TSS ()
Subject: Re: UK's first case of H-type BSE confirmed by DEFRA
Date: March 12, 2007 at 10:46 am PST

In Reply to: UK's first case of H-type BSE confirmed by DEFRA posted by TSS on March 9, 2007 at 11:03 am:


Nobel House, 17 Smith Square, London SW1P 3JR
Out of hours telephone 020 7270 8960 Ref: 71/07
Date: 9 March 2007

2005 case of H-type BSE in a cow

During routine research carried out by the Veterinary Laboratories Agency (VLA), test results have revealed a single case of H-type Bovine Spongiform Encephalopathy (BSE) in the UK in 2005. This form of BSE has already been identified in several other European countries in addition to Japan, Canada and the United States of America.

The cow died on a farm in Dumfries and Galloway at 13 years of age. The carcass was sampled and tested for BSE in accordance with EU requirements for testing all fallen stock aged over 24 months. Test results confirmed the presence of BSE. The carcass was incinerated and the offspring from this cow were culled in line with current BSE regulations.

In August 2006 the VLA began a retrospective examination of brain samples taken from previous BSE cases as part of a research programme designed to analyse different types of BSE in the national herd. The aim of the study is to determine whether unusual cases of BSE, had in fact occurred in the UK in the past. The studies are ongoing however this case has been identified as H-type. All forms of BSE are treated identically therefore no additional action would have been required in this case.

The Spongiform Encephalopathy Advisory Committee (SEAC) will examine all the available data on this case during a discussion of different forms of BSE worldwide at their next meeting on 10 th May 2007.

Notes to editors
1) SEAC briefly discussed information on the different forms of BSE worldwide, at their February 2007 meeting. The background papers for that meeting are available at

2) Japan, the USA, Canada and several European countries (including France, the Netherlands, Sweden, Switzerland, and Germany) have reported H-type BSE. Japan and several European countries (including Italy, Denmark, Poland, Belgium and Germany) have reported L-type BSE. Both types have been reported predominantly in cattle over 10 years old. In both types, the abnormal prion protein (PrP Sc ) in these animals has different biochemical characteristics to that seen in BSE. Mice experimentally infected with the brains of these animals develop a disease which differs from that seen in BSE infected mice. In two L-type cases where the intact whole brain was studied, the pattern of deposition of PrP Sc in the brain differed from that in BSE.

QUESTIONs FIRST PLEASE, maybe someone can answer this for me.

DOES H-type BSE found now in the U.K., and other EU countries, Japan, Canada and the USA, does this pathology look like sporadic CJD also, like the L-type BSE found in Japan and several European countries (including Italy, Denmark, Poland, Belgium and Germany), as opposed to the nvCJD pathology being like that of typical BSE ???

IN other words, does both H-type and L-type BSE differ from the BSE in relations to the nvCJD pathology in humans, and does both H-type and L-type BSE pathology look like some sporadic CJD phenotypes ???

FINALLY, in the paper submitted here, i found this very interesting ;

9. Atypical Bovine Spongiform Encephalopathy case associated with a prion protein ?gent mutation


HERE we report a bovine BSE case associated with a novel prion protein gene (Prnp) polymorphism resulting in an amino acid substitution. The animal carrying this mutation was positive for the abnormal prion protein, PrPBSE, in brainstem tissue. Western blot characterization of the PrPBSE of this animal revealed a high molecular weight phenotype of BSE when compared to PrPBSE from typical BSE isolates. AN IDENTICAL MUTATION IN THE HUMAN PRION PROTEIN SEQUENCE HOMOLOGUE HAS BEEN PREVIOUSLY DESCRIBED AS THE MOST COMMON CAUSE OF THE GENETIC HUMAN PRION DISEASES (gCJD, FFI and GSS). 8...end

confusious is a bit confused here.....

thank you, kind regards,




molecular similarities have been described between two case of BASE found in Italy and a subtype of sporadic CJD...


Our observations underline the high susceptibility of a primate species to the BASE prion strain and provide a biochemical basis for the identification of a potential occurence in man. 9...end



SUCH a glycosylation-ratio is distinct from that of the typical BSE agent in which the di-glycosylated form is dominant (approx. 70%) but, INTRIGUINGLY, SIMILAR TO THAT OF THE TYPE-2 SPORADIC CJD AGENT.


JUDGING from the glycosylation-ration, BSE prion herein is different from the typical BSE prion, and the atypical BSE prion found previously in a Holstein steer in Japan. INSTEAD, its molecular feature is close, IF NOT IDENTICAL, to PrPSc found in the cattle succumbed to bovine amyloidtic spongiform encephalopathy, and to the sporadic CJD-like PrPSc in the mice inoculated with BSE agent. 12...end

Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock

2006 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 the report for the parent project 3625-32000-073-00D, Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies (TSEs).

The aim of the cooperative research project is (i) to compare the U.S. Bovine Spongiform Encephalopathy (BSE) isolates and the atypical BSE isolates identified in Italy and (ii) to determine whether diagnostic methods routinely used at the USDA are able to identify atypical BSE cases.

Within FY06, formalin fixed and frozen brain materials from animals with typical and atypical Italian (BASE) BSE have been sent by CEA to the USDA-ARS-NADC laboratory in Ames, IA. The serial brain material sections of BSE and BASE (consecutively numbered) will be analyzed in Ames using the USDA immunohistochemistry (IHC) protocol. To evaluate its reproducibility in Italian laboratories and to standardize the method, the USDA IHC protocol is being established at the CEA neuropathology laboratory. As soon as the Ventana NexES IHC Staining System is available to the CEA, the same samples (different cut numbers) will be examined by the CEA using the CEA in-house and the USDA IHC protocol.

In order to evaluate the USDA Western blot method, about 2 gram of typical Italian BSE and about 2 gram of atypical BASE brain tissue have been sent to the USDA-ARS-NADC laboratory. These samples and U.S. typical and atypical BSE samples have been analyzed in parallel using both the USDA and CEA Western blot methods and three different monoclonal antibodies (6H4, P4, SAF 84). These studies were performed during the visit by a CEA collaborator to the USDA-ARS-NADC. The latter studies revealed that both the Italian and USDA extraction and Western blot methods allowed the identification of the typical and atypical BSE samples tested.


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 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.

NEW findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease

Atypical BSE
Because of its contemporary nature, the study of atypical BSE is very much a work in progress, with comparatively little published data and many unknowns. The first 2 cases to be identified were a serendipitous discovery made in the course of an unrelated experimental study that required a detailed neuropathologic and immunochemical examination of the entire brain (5). The absence of clinical signs in these older animals, the unusual distribution of PrPTSE, together with amyloid plaques, and a Western blot pattern that differed from the stereotypic pattern seen in typical BSE left little doubt about the probability that a new "atypical strain" had been identified (bovine amyloidotic spongiform encephalopathy[BASE]).

Although no further cases were found in over 100 cattle examined in Italy, the initiation of Western blot studies of animals in other countries with screening test programs began to yield additional atypical patterns (Table 2, Figure 3) (6–14; P. Lind, pers. comm.). Two major patterns have been described, named L (resembling the original Italian case pattern with a lower molecular weight than typical BSE) and H (for a distinct pattern first seen in France with a higher molecular weight than typical BSE). It is not yet clear whether other mixed patterns result from technical procedures in different laboratories or whether a more complicated scheme of classification will evolve as more atypical patterns are discovered.

In addition, Western blots of PrPTSE are a fragile basis on which to define a BSE phenotype. Little or no information is available about either the clinical status or neuropathologic features of these animals. We know that cases have occurred in different breeds and PrP genotypes, and we also know that very few of the animals have had the typical clinical picture of BSE (behavioral disturbances, sensory signs, ataxia, and tremors), but a cloud of ambiguity surrounds the clinical picture even in those animals for which an extensive post-hoc investigation was undertaken. The fact that few detailed neuropathologic results are available is explained by the need to preserve at least a full half brain for examination, which is presently not done in any of the various countries that have screening test programs. In the future, the brain as well as the carcass must be retained in cold storage until the test results are known.

The frequency of atypical cases is another unknown. Published (7,12) and unpublished (11,13) observations indicate that in some countries it might be as high as 5%–10% of the total number of older animals diagnosed by rapid screening tests (e.g., 2/27 in Germany, and 1/9 in Canada), which would seem to be a surprisingly high proportion of spontaneously occurring cases. However, data are not yet sufficient to estimate the overall prevalence of atypical BSE, i.e., cases per million tested animals of all ages.

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

mad cow in TEXAS they rendered without any test at ALL.


IN TEXAS, cattle on feed for decades, fda says 5.5 grams ruminant protein,
if tainted with TSE, is not enough to kill a cow. actually, it's enough to
kill 100+ cows ;-)

and add these in ;

UPI previously reported that from 2001 to 2003 the USDA collected the wrong part of the brain in more than 200 cows that were being screened as part of its BSE surveillance program.

The USDA documents also indicate the agency never was able to identify or test 52 cows that came into the United States in 2001 along with the Washington cow that tested positive in 2003. Of these, 11 were considered to be "high risk" because they were born within a year and on the same premises as the infected cow.

These cows may have gone into the food supply and been consumed by people. The concern is humans can contract a fatal brain disease from eating beef products contaminated with the mad cow pathogen.

NOT to forget what Paul Brown TSE expert at CDC said ;



The U.S. Department of Agriculture was quick to assure the public earlier
this week that the third case of mad cow disease did not pose a risk to
them, but what federal officials have not acknowledged is that this latest
case indicates the deadly disease has been circulating in U.S. herds for at
least a decade.

The second case, which was detected last year in a Texas cow and which USDA
officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a
picture of the disease having been here for 10 years or so, since it is
thought that cows usually contract the disease from contaminated feed they
consume as calves. The concern is that humans can contract a fatal,
incurable, brain-wasting illness from consuming beef products contaminated
with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of
other undetected cases," Dr. Paul Brown, former medical director of the
National Institutes of Health's Laboratory for Central Nervous System
Studies and an expert on mad cow-like diseases, told United Press
International. "The question was, 'How many?' and we still can't answer

Brown, who is preparing a scientific paper based on the latest two mad cow
cases to estimate the maximum number of infected cows that occurred in the
United States, said he has "absolutely no confidence in USDA tests before
one year ago" because of the agency's reluctance to retest the Texas cow
that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005
suspect," Brown said. ...snip...end

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ...
Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central
Nervous System ... Address for correspondence: Paul Brown, Building 36, Room
4A-05, ...


Tuesday, September 12, 2006 11:10 AM

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years,
and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the
USDA and the Canadian Food Agency."

OR, what the Honorable Phyllis Fong of the OIG found ;

Audit Report

Animal and Plant Health Inspection Service

Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II


Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain


TEXAS ATYPICAL BSE IN BOVINE FINALLY CONFIRMED ALMOST ONE YEAR AFTER FACT BY OIG DOING END AROUND JOHANNS ET AL, or that cow would have never been confirmed. 48 HOUR turnaround for confirmation of suspect BSE case by BSE guidelines flaunted until OIG finally stepped in. ...tss


WASHINGTON, June 24, 2005 -- Agriculture Secretary Mike Johanns today announced that the U.S. Department of Agriculture has received final test results from The Veterinary Laboratories Agency in Weybridge, England, confirming that a sample from an animal that was blocked from the food supply in November 2004 has tested positive for bovine spongiform encephalopathy (BSE). Johanns also directed USDA scientists to work with international experts to thoughtfully develop a new protocol that includes performing dual confirmatory tests in the event of another "inconclusive" BSE screening test.


The animal was selected for testing because, as a non-ambulatory animal, it was considered to be at higher risk for BSE. An initial screening test on the animal in November 2004 was inconclusive, triggering USDA to conduct the internationally accepted confirmatory IHC tests. Those test results were negative. Earlier this month, USDA's Office of the Inspector General recommended further testing of the seven-month-old sample using another internationally recognized confirmatory test, the Western blot. Unlike the IHC, the Western blot was reactive, prompting USDA to send samples from the animal to the Weybridge laboratory for further analysis.!ut/p/_s.7_0_A/7_0_1OB/.cmd/ad/.ar/sa.retrievecontent/.c/6_2_1UH/.ce/7_2_5JM/.p/5_2_4TQ/.d/1/_th/J_2_9D/_s.7_0_A/7_0_1OB?PC_7_2_5JM_contentid=2005/06/0232.xml&PC_7_2_5JM_navtype=RT&PC_7_2_5JM_parentnav=LATEST_RELEASES&PC_7_2_5JM_navid=NEWS_RELEASE#7_2_5JM!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2006/03/0084.xml

The investigation identified one feed which contained an animal protein source that could not be identified. The investigation also found one feed mill that supplied feed to the farm that had used ruminant MBM in feed formulations for non-ruminant species after the BSE/Ruminant Feed rule went into effect, which is permitted under the rule, and that several feed mills had used ruminant MBM in feeds prior to the feed ban. Although the investigation did not identify a specific feed source as the likely cause of this animal’s infection, it is probable that the most likely route of exposure for this animal was consumption of an animal feed containing mammalian protein prior to the implementation of the BSE/Ruminant Feed rule in 1997.

Texas BSE Investigation
Final Epidemiology Report
August 2005


The owner did keep some replacement heifers and, although he was relatively sure that he
had not kept any offspring from the yellow cow because of her excitable demeanor, DNA
analysis of the herd revealed several animals in the herd that may have been older offspring
of the index cow. While the owner sold 12 calves at the sale with the index cow on
11/11/04, her last calf was not in that group. According to the owner, the index cow’s last
calf was born either in Fall 2003 or Spring 2004, weaned early, and sold through the
livestock market some time between February and October 2004. The calf prior to that
would have been born either in Fall 2002 or Spring 2003 and was sold at the livestock
market sometime between January and December 2003.
Birth Cohort
The owner of Farm A kept very few herd records; this made finding documentation on this
cow’s birth cohort difficult. The birth cohort, by definition, included all cattle born on the
positive animal’s birth premises within 1 year, before or after, the positive animal’s date of
birth. The index cow was approximately 12 years of age in November 2004, but there was
no exact birth date in the herd records. A potential age range of 11 to 13 years was used to
sufficiently cover the animal’s most likely age. Using this range, all cattle born on the
index premises between 1990 and 1995 were considered part of the birth cohort.


Trace Herd 1
The owner of Trace Herd 1 was identified as having received one of the adult COI from the
index herd. Trace Herd 1 contained 909 head of cattle in multiple pastures and was placed
under hold order on 7/21/05. Upon completion of herd inventory, the animal of interest
was not found within the herd. A GDB search of all recorded herd tests conducted on
Trace Herd 1 and all market sales by the owner failed to locate the identification tag of the
animal of interest and she was subsequently classified as untraceable. The hold order on
Trace Herd 1 was released on 8/8/05.
Trace Herd 2
Trace Herd 2 was identified as having received one of the adult COI from the index herd.
Trace Herd 2 contained 19 head of cattle on one pasture and was placed under hold order
on 7/25/05. The owner of Trace Herd 2 identified the animal of interest by her eartag while
he was feeding his cattle out of a bucket and individually penned her for inspection by field
personnel. While the cow was identified as one of the animals that had left the index farm,
her age by dentition was estimated to be only 5 years old, which was too young to have
placed her as part of the birth or feed cohort of the index animal. She was classified as
found alive but determined not to be one of the COI; the hold order on Trace Herd 2 was
released on 7/26/05.
Trace Herd 3
The owner of Trace Herd 3 was identified as possibly having received an animal of
interest. The herd was placed under hold order on 7/27/05. The herd inventory was
conducted on 7/28/05. The animal of interest was not present within the herd, and the hold
order was released on 7/28/05. The person who thought he sold the animal to the owner of
Trace Herd 3 had no records and could not remember who else he might have sold the cow
to. Additionally, a search of GDB for all cattle sold through the markets by that individual
did not result in a match to the animal of interest. The animal of interest traced to this herd
was classified as untraceable because all leads were exhausted.
Trace Herd 4
The owner of Trace Herd 4 was identified as having received one of the COI through an
order buyer. Trace Herd 4 was placed under hold order on 7/29/05. A complete herd
inventory was conducted on 8/22/05 and 8/23/05. There were 233 head of cattle that were
examined individually by both State and Federal personnel for all man-made identification
and brands. The animal of interest was not present within the herd. Several animals were
reported to have died in the herd sometime after they arrived on the premises in April 2005.
A final search of GDB records yielded no further results on the eartag of interest at either
subsequent market sale or slaughter. With all leads having been exhausted, this animal of
interest has been classified as untraceable. The hold order on Trace Herd 4 was released on
Trace Herd 5
The owner of Trace Herd 5 was identified as having received two COI and was placed
under hold order on 8/1/05. Trace Herd 5 is made up of 67 head of cattle in multiple
pastures. During the course of the herd inventory, the owner located records that indicated
that one of the COI, a known birth cohort, had been sold to Trace Herd 8 where she was
subsequently found alive. Upon completion of the herd inventory, the other animal of
interest was not found within the herd. A GDB search of all recorded herd tests conducted
on Trace Herd 5 and all market sales by the owner failed to locate the identification tag of
the animal of interest and she was subsequently classified as untraceable due to all leads
having been exhausted. The hold order on Trace Herd 5 was released on 8/8/05.
Trace Herd 6
The owner of Trace Herd 6 was identified as possibly having received an animal of interest
and was placed under hold order on 8/1/05. This herd is made up of 58 head of cattle on
two pastures. A herd inventory was conducted and the animal of interest was not present
within the herd. The owner of Trace Herd 6 had very limited records and was unable to
provide further information on where the cow might have gone after he purchased her from
the livestock market. A search of GDB for all cattle sold through the markets by that
individual did not result in a match to the animal of interest. Additionally, many of the
animals presented for sale by the owner of the herd had been re-tagged at the market
effectually losing the traceability of the history of that animal prior to re-tagging. The
animal of interest traced to this herd was classified as untraceable due to all leads having
been exhausted. The hold order on Trace Herd 6 was released on 8/3/05.
Trace Herd 7
The owner of Trace Herd 7 was identified as having received an animal of interest and was
placed under hold order on 8/1/05. Trace Herd 7 contains 487 head of cattle on multiple
pastures in multiple parts of the State, including a unit kept on an island. The island
location is a particularly rough place to keep cattle and the owner claimed to have lost 22
head on the island in 2004 due to liver flukes. Upon completion of the herd inventory, the
animal of interest was not found present within Trace Herd 7. A GDB search of all
recorded herd tests conducted on Trace Herd 7 and all market sales by the owner failed to
locate the identification tag of the animal of interest. The cow was subsequently classified
as untraceable. It is quite possible though that she may have died within the herd,
especially if she belonged to the island unit. The hold order on Trace Herd 7 was released
on 8/8/05.
Trace Herd 8
Trace Herd 8 received an animal of interest, which happened to be a known birth cohort of
the index cow, from Trace Herd 5. Trace Herd 8 consists of 146 head of cattle that were
placed under hold order on 8/4/05. A herd inventory was conducted, the birth cohort was
found alive in the herd, and she was purchased and euthanized. The hold order on Trace
Herd 8 was released on 8/4/05. The cow was sampled on 8/5/05 and BSE tested by ELISA
at NVSL. Results were negative (as reported on 8/6/05); carcass disposal was completed
by alkaline digestion.



Jim Rogers (202) 690-4755
USDA Press Office (202) 720-4723

Statement by USDA Chief Veterinary Officer John Clifford (DVM) Regarding Positive BSE Test Results
March 13, 2006

“We received a positive result on a Western blot confirmatory test conducted at the USDA laboratories in Ames, Iowa, on samples from an animal that had tested “inconclusive” on a rapid screening test performed on Friday, March 10.

“The samples were taken from a non-ambulatory animal on a farm in Alabama. A local private veterinarian euthanized and sampled the animal and sent the samples for further testing, which was conducted at one of our contract diagnostic laboratories at the University of Georgia. The animal was buried on the farm and it did not enter the animal or human food chains.


Alabama BSE Investigation
Final Epidemiology Report
May 2, 2006


Despite a thorough investigation of two farms that were known to contain the index cow,
and 35 other farms that might have supplied the index cow to the farms where the index
case was known to have resided, the investigators were unable to locate the herd of
origin. The index case did not have unique or permanent identification, plus, the size and
color of the cow being traced is very common in the Southern United States. Due to the
unremarkable appearance of solid red cows, it is not easy for owners to remember
individual animals. In the Southern United States, it is common business practice to buy
breeding age cows and keep them for several years while they produce calves. Most
calves produced are sold the year they are born, whereas breeding cows are sold when
there is a lapse in breeding, which can occur multiple times in cows’ lives. For all of
these reasons, USDA was unable to locate the herd of origin.


AFTER this, it was just to much risk for USDA/APHIS/FSIS et al to do anymore extensive TSE testing of any sort in the USA bovine. so they reduced BSE testing drastically ;


WASHINGTON, July 20, 2006-Agriculture Secretary Mike Johanns announced today that the U.S. Department of Agriculture will soon begin transitioning to an ongoing Bovine Spongiform Encephalopathy (BSE) surveillance program that corresponds to the extremely low prevalence of the disease in the U.S.

"It's time that our surveillance efforts reflect what we now know is a very, very low level of BSE in the United States," said Johanns. "This ongoing surveillance program will maintain our ability to detect BSE, provide assurance that our interlocking safeguards are successfully preventing BSE, while continuing to exceed science-based international guidelines."

The ongoing BSE surveillance program will sample approximately 40,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected, similar to the enhanced surveillance program procedures.


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.

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

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models 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.


MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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




THE rest is history, GWs and the OIE BSE MRR policy that will spread these different strains of TSE around the globe,
all for a buck, cattle futures and commodities rule, to hell with human health. OF which due to the long incubation period,
ramifications of this blunder will not be felt for a decade or more later down the road. ...TSS

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