National Scrapie Eradication Program October 2013 Monthly Report Fiscal 
Year 2014 TSE PRION REPORT
 
 
PROGRAM SUMMARY
 
At the end of FY 2013, the percent of cull sheep found positive at 
slaughter and adjusted for face color was 0.015 percent (Chart 1). This measure 
of prevalence has decreased by 90 percent since slaughter surveillance started 
in FY 2003.
Eight source flocks (including two goat herds) and three infected flocks 
were designated in FY 2013. This is compared to eight new infected or source 
flocks that were designed in FY 2012. (Chart 2).
 
INTRODUCTION
 
Surveillance (Part 1)
 
Regulatory Scrapie Slaughter Surveillance (RSSS)
 
RSSS started April 1, 2003. It is a targeted slaughter surveillance program 
which is designed to identify infected flocks. Samples have been collected from 
399,940 animals since April 1, 2003. There have been 470 NVSL confirmed positive 
animals (462 classical cases and 8 Nor98-like cases) since the beginning of 
RSSS. As of October 31, 2013, 2,240 samples have been collected in FY 2014, 655 
of which were from goats. One white-faced sheep has tested positive for scrapie 
in FY 2014. The percentage of samples that have tested positive for each face 
color from FY 2003 through FY 2014 is depicted in Chart 3. Cumulative regional 
sample collection numbers are shown in Chart 4 and are based upon the State in 
which the animal was tagged. The number of animals collected for FY 2014 by 
month and by region where collected is shown in Chart 5. A monthly comparison of 
RSSS collections by fiscal year is displayed in Chart 6. Chart 7 is a 
retrospective 6-month rolling average of the percent positive, black-faced sheep 
sampled at RSSS collection sites.
 
*RSSS positives are reported based on collection date and may have been 
confirmed after October 31, 2013.
 
 
Surveillance (Part 2) On-Farm Surveillance Testing of animals in the field 
has always been part of scrapie surveillance (regulatory field cases and 
live-animal testing). As the National Scrapie Eradication Program moves closer 
towards meeting the goal of identifying the last remaining cases of classical 
scrapie by 2017, finding and testing all sheep and goats meeting targeted 
sampling criteria is even more important. As of October 31, 2013, 315 sheep and 
35 goats have been tested on-farm for FY 2014. No animals have tested positive. 
The number of animals tested on-farm by month and by species for FY 2014 is 
shown in Chart 8. Total Animals Sampled for Scrapie Testing As of October 31, 
2013, 2,590 animals have been sampled for scrapie testing:
 
•2,240 RSSS samples and 350 on-farm samples [includes regulatory testing 
(necropsy and live-animal) and on-farm surveillance] (Chart 9);
 
•Of which 1,900 were sheep and 690 were goats. Distribution of sampling by 
type (RSSS or on-farm) and by species is shown in Chart 10.
 
 
Positive Cases and New Infected/Source Flocks
 
Positive Scrapie Cases
 
One positive white-faced sheep tested through RSSS has been reported in FY 
2014* (Table 1 and (Figure 1).
 
The number of confirmed positive cases in goats since FY 2002 is 34; the 
most recent case was a Nigerian dwarf goat, confirmed positive in July 2013. 
(Figure 2).
 
Infected and Source Flocks
 
As of October 31, 2013, there were ten flocks with an open infected or 
source status
(Figure 3). Three new infected flocks have been designated in 2014 (Figure 
4). The ratio of infected and source flocks released to newly identified 
infected and source flocks for FY 2014 = 0 : 3. New infected and source statuses 
from FY 1997 to FY 2014 are depicted in Chart 2.
 
* Samples collected between October 1, 2013 and October 31, 2013 and 
confirmed by
November 15, 2013.
 
 
snip...see full text ;
 
 
 
 
Sunday, November 17, 2013 
 
L-BSE in Genetically Susceptible and Resistant Sheep: Changes in Prion 
Strain or Phenotypic Plasticity of the Disease-Associated Prion Protein?
 
 
 
Saturday, November 02, 2013 
 
OREGON DETECTS SCRAPIE 
 
 
 
Friday, July 26, 2013
Voluntary 
Scrapie Program USA UPDATE July 26, 2013 increase in FY 2013 is not 
statistically meaningful due to the sample size 
http://scrapie-usa.blogspot.com/2013/07/voluntary-scrapie-program-usa-update.html
 
 
Friday, May 10, 2013 
Evidence 
of effective scrapie transmission via colostrum and milk in 
sheep
http://scrapie-usa.blogspot.com/2013/05/evidence-of-effective-scrapie.html
 
 
 
 
why do we not want to do TSE transmission 
studies on chimpanzees $ 
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. 
snip... 
The successful transmission of kuru, 
Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys 
that we have reported provides further grounds for concern that scrapie-infected 
meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. 
PMID: 6997404 
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract 
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. 
snip... 
76/10.12/4.6 
http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf 
Nature. 1972 Mar 10;236(5341):73-4. 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). 
Gibbs CJ Jr, Gajdusek DC. 
Nature 236, 73 - 74 (10 March 1972); 
doi:10.1038/236073a0 
Transmission of Scrapie to the Cynomolgus Monkey 
(Macaca fascicularis) 
C. J. GIBBS jun. & D. C. GAJDUSEK 
National Institute of Neurological Diseases and Stroke, National 
Institutes of Health, Bethesda, Maryland 
SCRAPIE has been transmitted to 
the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation 
period of more than 5 yr from the time of intracerebral inoculation of 
scrapie-infected mouse brain. The animal developed a chronic central nervous 
system degeneration, with ataxia, tremor and myoclonus with associated severe 
scrapie-like pathology of intensive astroglial hypertrophy and proliferation, 
neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie 
virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of 
scrapie obtained as ninth intracerebral passage of the agent in goat brain, from 
Dr R. L. Chandler (ARC, Compton, Berkshire). 
 
 
 
 
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine 
(reticular) deposits,
 
see also ;
 
All of the Heidenhain variants were of the methionine/ methionine type 1 
molecular subtype.
 
 
 
Tuesday, July 29, 2008
 
Heidenhain Variant Creutzfeldt Jakob Disease Case Report
 
snip...
 
Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 
'MOM'
 
DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 
McCullough Bldg. Galveston, Texas 77555-0785
 
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original documents. -------------------------- Patient Account: 90000014-518 
Med. Rec. No.: (0160)118511Q Patient Name: POULTER, BARBARA Age: 63 YRS DOB: 
10/17/34 Sex: F Admitting Race: C
 
Attending Dr.: Date / Time Admitted : 12/14/97 1228 Copies to:
 
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 
772-1238 Fax (409) 772-5683 Pathology Report
 
FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858
 
Autopsy NO.: AU-97-00435
 
AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown Residence: 
Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97 
15:00 Pathologist/Resident: Pencil/Fernandez Service: Private Restriction: Brain 
only
 
FINAL AUTOPSY DIAGNOSIS
 
I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.
 
snip...see full text ;
 
 
 
P.5.21
 
Parallels between different forms of sheep scrapie and types of 
Creutzfeldt-Jakob disease (CJD)
 
Wiebke M. Wemheuer1, Sylvie L. Benestad2, Arne Wrede1, Wilhelm E. 
Wemheuer3, Tatjana Pfander1, Bjørn Bratberg2, Bertram Brenig3,Walter J. 
Schulz-Schaeffer1 1University Medical Center Goettingen, Germany; 2Institute of 
Veterinary Medicine Oslo, Norway; 3Institute of Veterinary Medicine Goettingen, 
Germany
 
Background: Scrapie in sheep and goats is often regarded as the archetype 
of prion diseases. In 1998, a new form of scrapie - atypical/Nor98 scrapie - was 
described that differed from classical scrapie in terms of epidemiology, Western 
blot profile, the distribution of pathological prion protein (PrPSc) in the body 
and its stability against proteinase K. In a similar way, distinct disease types 
exist in sporadic Creutzfeldt-Jakob disease (CJD). They differ with regard to 
their clinical outcome, Western blot profile and PrPSc deposition pattern in the 
central nervous system (CNS).
 
Objectives: The comparison of PrPSc deposits in sheep scrapie and human 
sporadic CJD.
 
Methods: Tissues of the CNS of sheep with classical scrapie, sheep with 
atypical/Nor98 scrapie and 20 patients with sporadic CJD were examined using the 
sensitive Paraffin Embedded Tissue (PET) blot method. The results were compared 
with those obtained by immunohistochemistry. With the objective of gaining 
information on the protein conformation, the PrPSc of classical and 
atypical/Nor98 sheep scrapie and sporadic CJD was tested for its stability 
against denaturation with guanidine hydrochloride (GdnHCl) using a Membrane 
Adsorption Assay.
 
Results: The PrPSc of atypical/Nor98 scrapie cases and of CJD prion type 1 
patients exhibits a mainly reticular/synaptic deposition pattern in the brain 
and is relatively sensitive to denaturation with GdnHCl. In contrast classical 
scrapie cases and CJD prion type 2 patients have a more complex PrPSc deposition 
pattern in common that consists of larger PrPSc aggregates and the PrPSc itself 
is comparatively stable against denaturation.
 
Discussion: The similarity between CJD types and scrapie types indicates 
that at least two comparable forms of the misfolded prion protein exist beyond 
species barriers and can elicit prion diseases. It seems therefore reasonable to 
classify classical and atypical/Nor98 scrapie - in analogy to the existing CJD 
types - as different scrapie types.
 
 
 
Monday, December 1, 2008
 
When Atypical Scrapie cross species barriers
 
 
 
Thursday, December 20, 2012 
OIE GROUP RECOMMENDS THAT SCRAPE PRION 
DISEASE BE DELISTED, WISHES TO CONTINUE SPREADING IT AROUND THE GLOBE
 
 
Wednesday, December 4, 2013 
 
Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine 
Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December 
4, 2013 
 
TO ALL IMPORTING COUNTRIES THAT IMPORTS FROM THE USA, BE WARNED, NEW MAD 
COW BSE REGULATIONS USDA, AND OIE, not worth the paper the regulations were 
wrote on, kind of like the mad cow feed ban of August 1997, nothing but ink on 
paper $$$
 
full text ;
 
 
 
IN A NUT SHELL ; 
 
(Adopted by the International Committee of the OIE on 23 May 2006) 
 
11. Information published by the OIE is derived from appropriate 
declarations made by the official Veterinary Services of Member Countries. The 
OIE is not responsible for inaccurate publication of country disease status 
based on inaccurate information or changes in epidemiological status or other 
significant events that were not promptly reported to the Central Bureau, 
 
 
 
 
Thursday, May 30, 2013 
 
World Organization for Animal Health (OIE) has upgraded the United States' 
risk classification for mad cow disease to "negligible" from "controlled", and 
risk further exposing the globe to the TSE prion mad cow type disease 
 
U.S. gets top mad-cow rating from international group and risk further 
exposing the globe to the TSE prion mad cow type disease 
 
 
 
 
 
 
Saturday, November 2, 2013 
 
Exploring the risks of a putative transmission of BSE to new species
 
 
 
Sunday, November 13, 2011 
 
California BSE mad cow beef recall, QFC, CJD, and dead stock downer 
livestock 
 
 
 
Sunday, August 11, 2013 
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report 
August 2013 
 
Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing 
an extreme increase of 48% between 2008 and 2010 
 
 
 
Sunday, October 13, 2013 
 
CJD TSE Prion Disease Cases in Texas by Year, 2003-2012 
 
 
 
Saturday, September 21, 2013 
 
Westland/Hallmark: 2008 Beef Recall A Case Study by The Food Industry 
Center January 2010 THE FLIM-FLAM REPORT 
 
 
 
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH 
RISK FOR MAD COW DISEASE ??? 
 
this recall was not for the welfare of the animals. ...tss 
 
you can check and see here ; 
 
(link now dead, does not work...tss)
 
 
 
 
try this link ; 
 
 
 
 
Thursday, November 28, 2013 
 
U.S., suppliers settle over school lunch beef linked to recall 
 
 
 
 
Monday, October 10, 2011 
 
EFSA Journal 2011 The European Response to BSE: A Success Story 
 
snip... 
 
EFSA and the European Centre for Disease Prevention and Control (ECDC) 
recently delivered a scientific opinion on any possible epidemiological or 
molecular association between TSEs in animals and humans (EFSA Panel on 
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical 
BSE prions as the only TSE agents demonstrated to be zoonotic so far but the 
possibility that a small proportion of human cases so far classified as 
"sporadic" CJD are of zoonotic origin could not be excluded. Moreover, 
transmission experiments to non-human primates suggest that some TSE agents in 
addition to Classical BSE prions in cattle (namely L-type Atypical BSE, 
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic 
wasting disease (CWD) agents) might have zoonotic potential. 
 
snip... 
 
 
 
 
 
 
see follow-up here about North America BSE Mad Cow TSE prion risk factors, 
and the ever emerging strains of Transmissible Spongiform Encephalopathy in many 
species here in the USA, including humans ; 
 
 
 
 
Thursday, August 12, 2010 
 
Seven main threats for the future linked to prions 
 
First threat 
 
The TSE road map defining the evolution of European policy for protection 
against prion diseases is based on a certain numbers of hypotheses some of which 
may turn out to be erroneous. In particular, a form of BSE (called atypical 
Bovine Spongiform Encephalopathy), recently identified by systematic testing in 
aged cattle without clinical signs, may be the origin of classical BSE and thus 
potentially constitute a reservoir, which may be impossible to eradicate if a 
sporadic origin is confirmed. 
 
***Also, a link is suspected between atypical BSE and some apparently 
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases 
constitute an unforeseen first threat that could sharply modify the European 
approach to prion diseases. 
 
Second threat 
 
snip... 
 
 
 
 
 
 
 
 
WHAT about the sporadic CJD TSE proteins ?
 
WE now know that some cases of sporadic CJD are linked to atypical BSE and 
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all 
it’s sub-types $$$ 
 
 
Monday, June 27, 2011 
 
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive 
Prionopathy and Gerstmann-Sträussler-Scheinker Disease 
 
 
 
 
Monday, October 14, 2013 
 
Researchers estimate one in 2,000 people in the UK carry variant CJD 
proteins 
 
 
 
 
Tuesday, October 29, 2013 
 
VARIANT CJD PRESENTS DIFFERENTLY IN OLDER PATIENTS 
 
 
 
 
Wednesday, October 09, 2013 
 
*** WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, 
£41,078,281 in compensation REVISED 
 
 
 
 
Thursday, October 10, 2013 
 
*** CJD REPORT 1994 increased risk for consumption of veal and venison and 
lamb 
 
 
 
 
Friday, August 16, 2013 
 
*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and 
Contaminated blood products induce a highly atypical prion disease devoid of 
PrPres in primates 
 
 
 
 
Saturday, November 16, 2013 
 
*** Management of neurosurgical instruments and patients exposed to 
creutzfeldt-jakob disease 2013 December 
 
Infect Control Hosp Epidemiol. 
 
 
 
 
Friday, November 29, 2013 
 
*** Identification of Misfolded Proteins in Body Fluids for the Diagnosis 
of Prion Diseases 
 
International Journal of Cell Biology
 
 
 
 
Monday, December 02, 2013 
 
*** A parliamentary inquiry has been launched today into the safety of 
blood, tissue and organ screening following fears that vCJD – the human form of 
‘mad cow’ disease – may be being spread by medical procedures 
 
 
 
 
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001 
 
 
 
 
 
 
Romans 12:19 ESV 
 
Beloved, never avenge yourselves, but leave it to the wrath of God, for it 
is written, “Vengeance is mine, I will repay, says the Lord.” 
 
 
Tuesday, November 26, 2013
 
Transmission of multiple system atrophy prions to transgenic mice 
 
 
 
 
just saying...TSS
 
 
TSS