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
FAX COVER SHEET
DATE: 4-23-98
TO: Mr. Terry Singeltary @ -------
FROM: Gerald Campbell
FAX: (409) 772-5315 PHONE: (409) 772-2881
Number of Pages (including cover sheet):
Message:
*CONFIDENTIALITY NOTICE*
This document accompanying this transmission contains confidential
information belonging to the sender that is legally privileged. This information
is intended only for the use of the individual or entry names above. If you are
not the intended recipient, you are hereby notified that any disclosure, copying
distribution, or the taking of any action in reliances on the contents of this
telefaxed information is strictly prohibited. If you received this telefax in
error, please notify us by telephone immediately to arrange for return of the
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