Report on the monitoring of ruminants for the presence of Transmissible
Spongiform Encephalopathies (TSEs) in the EU in 2011 Final version 18 October
2012
TABLE OF CONTENTS
1.
SUMMARY.................................................................................................................4
1.1. Bovine
animals.............................................................................................
1.2. Ovine and caprine
animals......................................................................
2. MONITORING PROGRAMMES, SAMPLING AND DIAGNOSTIC
METHODS APPLICABLE IN 2011
.....................................................................
2.1. Legal
basis.....................................................................................................
2.2. BSE monitoring of bovine animals
.......................................................
2.3. TSE monitoring of ovine and caprine
animals.................................
2.4. Sampling and testing for the prion protein genotype determination in
ovine animals.............................................................
3. ANNUAL AND MONTHLY
REPORTS.................................................................
4. SUMMARY OF THE BSE TESTING IN BOVINE ANIMALS DURING
2011.......................................................................................................
4.1. Sampling
......................................................................................................
4.2. BSE positive
cases....................................................................................
4.3. Testing by target
group..........................................................................
4.4. Age distribution of BSE positive cases
.............................................
4.5. Year of birth distribution of BSE positive cases detected since 2001
...................................................................................................
4.6. Prevalence of BSE in different age categories in 2011..............
4.7. BSE in young animals
.............................................................................
4.8. Atypical BSE cases
...................................................................................
5. SUMMARY OF TSE TESTING IN OVINE AND CAPRINE
ANIMALS DURING
2011...................................................................................
5.1. Sampling
......................................................................................................
5.2. Positive cases
.............................................................................................
5.3. Atypical
cases.............................................................................................
5.4. TSE discriminatory tests
........................................................................
5.5. Age distribution of TSE positive
cases..............................................
5.6.
Genotyping..................................................................................................
1. SUMMARY
1.1. Bovine animals
In 2011, a total of 6 361 591 bovine animals were tested in the EU 27 in
the framework of the BSE monitoring programmes. 28 bovine animals turned out
positive.
Out of the 28 BSE cases identified in 2011, 23 were submitted to
discriminatory testing by the Member States, on a voluntary basis. These tests
confirmed 17 cases of classical BSE, 3 cases of atypical H-type BSE and 3 cases
of atypical L-type BSE.
1 090 192 risk bovine animals and 5 270 593 healthy animals slaughtered for
human consumption were tested by rapid tests. 93 animals were tested in the
framework of culling of animals with an epidemiological connection to a BSE
case. In addition, 713 bovine animals were tested in the framework of passive
surveillance (animals reported as official BSE suspects. 100 % of positive cases
were detected by the active monitoring (testing of risk animals, healthy
slaughtered and culled cattle) and 0 % were detected by passive surveillance.
No BSE cases were found in Belgium, Bulgaria, Czech Republic, Denmark,
Germany, Estonia, Greece, Cyprus, Latvia, Lithuania, Luxembourg, Hungary, Malta,
Netherlands, Austria, Romania, Slovenia, Slovakia, Finland and Sweden. The
number of BSE cases and the overall prevalence in tested animals decreased by
respectively 38 % and 27 % in 2011 compared to 2010.
1.2. Ovine and caprine animals
In 2011, a total of 369 417 ovine and 140 843 caprine animals were tested
in the EU 27 in the framework of the TSE monitoring programmes. 1589 ovine and
366 caprine animals turned out positive to classical scrapie.
369 055 ovine animals were tested by active monitoring, while 362 were
animals reported as official TSE suspects and therefore subjected to laboratory
examination. In caprine animals, the numbers of tests in the respective groups
were 139 612 (active monitoring) and 1 231 (TSE suspects). Some 698 and 134 TSE
cases in respectively sheep and goats confirmed in 2011 were subjected to
discriminatory testing. None of them have been confirmed to be BSE.
All Member States submitted information on the the TSE testing of bovine,
ovine and caprine animals. In addition to the Member States, Norway also
submitted information on their TSE testing programmes.
snip...
4. SUMMARY OF THE BSE TESTING IN BOVINE ANIMALS DURING 2011
The information was extracted directly from the electronic submission of
monthly and case reports by Member States. The monthly information is often
updated and/or corrected by the Member States in following reports. The
information shown in the following summaries is updated according to the
information received electronically until 18 October 2012. Information on adult
cattle population in 2011 was obtained from Eurostat.
4.1. Sampling
Comments on the sampling
Sampling decreased in 2011 from about 7.5 million cattle in 2010 to a
little less than 6.4 million in 2011. This drop can be explained by the fact
that 25 Member States were allowed, as of 1 July 2011, to test only healthy
cattle over 72 months of age at the slaughterhouse. A similar drop should be
expected in 2012 when this new age limit will have been applied to the whole
year. Over 102 million cattle have been tested in the EU since 2001.
Chart B1: Total tests performed in the period 2001–2011 in the EU27
snip...
4.2. BSE positive cases
Comments on BSE positive cases
When analysing the evolution of BSE positive cases, it should be kept in
mind that active monitoring was limited before 2001 and has decreased since 2009
for some Member States due to the modification of the age limit for testing. The
expanded active monitoring became fully applicable in July 2001. The annual
number of tests was about 25 % higher in the period 2002-2008 than in 2001 (see
Chart B1). Despite the fact that the number of tests remained stable between
2002 and 2008, and decreased since 2009, the prevalence of BSE in tested animals
(ratio of positives per 10 000 tests) has been steadily dropping since 2002, due
to the decline in positive cases.
Overall the number of cases and the prevalence in tested animals of BSE
dropped by 36% and 27% respectively in the EU in 2011 compared to 2010.
Chart B2: Evolution of the number of BSE positive cases in the 27 EU Member
States since 2001
snip...
Comments on the age distribution of BSE positive animals
The previous tables and charts confirm in 2011 the general trend of the
past years of a regular increase of the average age of the BSE cases detected.
The slight drop of the average age of BSE cases in risk animals observed in 2010
was not confirmed in 2011. The average age of BSE cases in risk animals is now
close to 172 months, and a little over 178 months in healthy slaughtered animals
has actually almost converged in 2011 and is now close to 178 months, almost 15
years.
The overall evolution of the average age of positive cases appears
favourable since 2001. Taking into consideration an average incubation period of
5-6 years, these figures are an indication that measures taken (mainly feed ban)
have been effective.
snip...
(***PLEASE NOTE INCREASE IN ATYPICAL BSE CASES COMPARED TO TYPICAL BSE
CASES...TSS)
Table B35: Proportion in each target group of all BSE cases submitted to
further discriminatory testing and reported atypical BSE cases, by Member State,
from 2001 to 2011
(***ALSO PLEASE NOTE THE AGE COMPARISON WITH TYPICAL C-BSE WITH THE
SUPPOSEDLY OLD AGE CATTLE SYNDROME OF THE ATYPICAL H AND L TYPE BSE, SEEMS
STRANGE THAT THE C-BSE AGE IS GOING UP, HIGHER THAN THE ATYPICAL CASES
NOW???...TSS)
Chart B12: Average age (in months) of the Classical, L and H-type BSE cases
detected in the EU from 2001 to 2011 after discriminatory testing
SNIP...
Comments on atypical BSE
The 2011 annual report provides data on atypical BSE cases for the time.
The TSE regulation does not require the Member States to conduct discriminatory
testing of all BSE cases. The present data reflect the tests conducted by some
Member States on a voluntary basis. In most contributing Member States, only
part of the past BSE cases have been submitted to discriminatory testing. The
present results should therefore not be considered representative of the
national or EU real situation.
These provisional results suggest that approximately a quarter of the BSE
cases in the EU may be atypical cases, splitting relatively evenly in atypical H
and atypical L- type cases. They also suggest that a higher proportion of
atypical BSE may be found in the fallen stock cases than in the healthy
slaughtered cattle cases.
Chart 12 also suggests that the average ages of atypical H and L-type cases
are similar and have been stable since 2001. During the same period of time, the
average age of classical BSE cases has been steadily increasing and appears to
be higher in 2011 than the average age of atypical H and L-type cases.
snip...
Comments on genotyping
Only one case of classical scrapie was submitted in 2011 (Greece) in a
sheep of the ARR/ARR genotype.
No trend in the genetic profile can be identified from 2004 to 2011 in most
of the EU based on the results of the regulatory random genotyping of the ovine
population. However, the results of the exhaustive genotyping of the sheep
population in Cyprus show a very significant increase of the NSP1 and NSP2
groups since 2005.
(see charts at link...tss)
Wednesday, November 28, 2012
Scientific and technical assistance on the provisional results of the study
on genetic resistance to Classical scrapie in goats in Cyprus 1
SCIENTIFIC REPORT OF EFSA
Sunday, September 23, 2012
EU-Approved Rapid Tests for Bovine Spongiform Encephalopathy Detect
Atypical Forms: A Study for Their Sensitivities
Wednesday, December 21, 2011
Potential mad cows that entered food supply without being tested for BSE
2011: UK END OF YEAR REVIEW
Thursday, September 6, 2012
UK Breaches of BSE controls in consignments of beef 2011 communications
missing four reports
Friday, November 30, 2012
PROPOSED DECISION TO STOP BSE TESTING OF HEALTHY CATTLE SLAUGHTERED FOR
HUMAN CONSUMPTION FSA 12/12/04 Open Board – 11 December 2012
Sunday, December 2, 2012
CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE
BLEW IT’
**** Tuesday, November 6, 2012
Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and
Sporadic CJD, November-December 2012 update
Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform
Encephalopathy (BSE) Surveillance Program
An Arizona meat processing company and its owner pled guilty in February
2007 to charges of theft of Government funds, mail fraud, and wire fraud. The
owner and his company defrauded the BSE Surveillance Program when they falsified
BSE Surveillance Data Collection Forms and then submitted payment requests to
USDA for the services. In addition to the targeted sample population (those
cattle that were more than 30 months old or had other risk factors for BSE), the
owner submitted to USDA, or caused to be submitted, BSE obex (brain stem)
samples from healthy USDA-inspected cattle. As a result, the owner fraudulently
received approximately $390,000. Sentencing is scheduled for May 2007.
snip...
Topics that will be covered in ongoing or planned reviews under Goal 1
include:
soundness of BSE maintenance sampling (APHIS),
implementation of Performance-Based Inspection System enhancements for
specified risk material (SRM) violations and improved inspection controls over
SRMs (FSIS and APHIS),
snip...
The findings and recommendations from these efforts will be covered in
future semiannual reports as the relevant audits and investigations are
completed.
4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half
-MORE Office of the United States Attorney District of Arizona FOR
IMMEDIATE RELEASE For Information Contact Public Affairs February 16, 2007 WYN
HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681
CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENTS MAD
COW DISEASE SURVEILLANCE PROGRAM
PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of
Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail
fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel
Knauss stated, The integrity of the system that tests for mad cow disease relies
upon the honest cooperation of enterprises like Farm Fresh Meats. Without that
honest cooperation, consumers both in the U.S. and internationally are at risk.
We want to thank the USDAs Office of Inspector General for their continuing
efforts to safeguard the public health and enforce the law. Farm Fresh Meats
and Farabee were charged by Information with theft of government funds, mail
fraud and wire fraud. According to the Information, on June 7, 2004, Farabee, on
behalf of Farm Fresh Meats, signed a contract with the U.S. Department of
Agriculture (the USDA Agreement) to collect obex samples from cattle at high
risk of mad cow disease (the Targeted Cattle Population). The Targeted Cattle
Population consisted of the following cattle: cattle over thirty months of age;
nonambulatory cattle; cattle exhibiting signs of central nervous system
disorders; cattle exhibiting signs of mad cow disease; and dead cattle. Pursuant
to the USDA Agreement, the USDA agreed to pay Farm Fresh Meats $150 per obex
sample for collecting obex samples from cattle within the Targeted Cattle
Population, and submitting the obex samples to a USDA laboratory for mad cow
disease testing. Farm Fresh Meats further agreed to maintain in cold storage the
sampled cattle carcasses and heads until the test results were received by Farm
Fresh Meats.
Evidence uncovered during the governments investigation established that
Farm Fresh Meats and Farabee submitted samples from cattle outside the Targeted
Cattle Population. Specifically, Farm Fresh Meats and Farabee submitted, or
caused to be submitted, obex samples from healthy, USDA inspected cattle, in
order to steal government moneys.
Evidence collected also demonstrated that Farm Fresh Meats and Farabee
failed to maintain cattle carcasses and heads pending test results and falsified
corporate books and records to conceal their malfeasance. Such actions, to the
extent an obex sample tested positive (fortunately, none did), could have
jeopardized the USDAs ability to identify the diseased animal and pinpoint its
place of origin. On Wednesday, February 14, 2007, Farm Fresh Meats and Farabee
pleaded guilty to stealing government funds and using the mails and wires to
effect the scheme. According to their guilty pleas:
(a) Farm Fresh Meats collected, and Farabee directed others to collect,
obex samples from cattle outside the Targeted Cattle Population, which were not
subject to payment by the USDA;
(b) Farm Fresh Meats 2 and Farabee caused to be submitted payment requests
to the USDA knowing that the requests were based on obex samples that were not
subject to payment under the USDA Agreement;
(c) Farm Fresh Meats completed and submitted, and Farabee directed others
to complete and submit, BSE Surveillance Data Collection Forms to the USDAs
testing laboratory that were false and misleading;
(d) Farm Fresh Meats completed and submitted, and Farabee directed others
to complete and submit, BSE Surveillance Submission Forms filed with the USDA
that were false and misleading;
(e) Farm Fresh Meats falsified, and Farabee directed others to falsify,
internal Farm Fresh Meats documents to conceal the fact that Farm Fresh Meats
was seeking and obtaining payment from the USDA for obex samples obtained from
cattle outside the Targeted Cattle Population; and
(f) Farm Fresh Meats failed to comply with, and Farabee directed others to
fail to comply with, the USDA Agreement by discarding cattle carcasses and heads
prior to receiving BSE test results. A conviction for theft of government funds
carries a maximum penalty of 10 years imprisonment. Mail fraud and wire fraud
convictions carry a maximum penalty of 20 years imprisonment. Convictions for
the above referenced violations also carry a maximum fine of $250,000 for
individuals and $500,000 for organizations. In determining an actual sentence,
Judge Earl H. Carroll will consult the U.S. Sentencing Guidelines, which provide
appropriate sentencing ranges. The judge, however, is not bound by those
guidelines in determining a sentence.
Sentencing is set before Judge Earl H. Carroll on May 14, 2007. The
investigation in this case was conducted by Assistant Special Agent in Charge
Alejandro Quintero, United States Department of Agriculture, Office of Inspector
General. The prosecution is being handled by Robert Long, Assistant U.S.
Attorney, District of Arizona, Phoenix. CASE NUMBER: CR-07-00160-PHX-EHC RELEASE
NUMBER: 2007-051(Farabee) # # #
Friday, May 4, 2012
May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform
Encephalopathy (BSE) in the United States
PAUL BROWN COMMENT TO ME ON THIS ISSUE
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."
........TSS
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 and 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
Tuesday, January 1, 2008
BSE OIE USDA
Subject: OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local
cattle dealers i.e. USDA
Date: May 14, 2007 at 9:00 am PST
OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle
dealers i.e. USDA
STATEMENT BY DR. RON DEHAVEN REGARDING OIE RISK RECOMMENDATION
March 9, 2007
Tuesday, November 02, 2010
IN CONFIDENCE
The information contained herein should not be disseminated further except
on the basis of "NEED TO KNOW".
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only)
diagnostic criteria CVL 1992
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
Comments on technical aspects of the risk assessment were then submitted to
FSIS.
Comments were received from Food and Water Watch, Food Animal Concerns
Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S.
Singeltary.
This document provides itemized replies to the public comments received on
the 2005 updated Harvard BSE risk assessment. Please bear the following points
in mind:
Owens, Julie
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Monday, July 24, 2006 1:09 PM
To: FSIS RegulationsComments
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)
Page 1 of 98
FSIS, USDA, REPLY TO SINGELTARY
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
2012 atypical L-type BSE BASE California reports
Saturday, August 4, 2012
Final Feed Investigation Summary - California BSE Case - July 2012
SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE
INVESTIGATION JULY 2012
Summary Report BSE 2012
Executive Summary
Saturday, August 4, 2012
Update from APHIS Regarding Release of the Final Report on the BSE
Epidemiological Investigation
CENSORSHIP IS A TERRIBLE THING $$$
Canada has had a COVER-UP policy of mad cow disease since about the 17th
case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored
$$$
THIS proves there is indeed an epidemic of mad cow disease in North
America, and it has been covered up for years and years, if not for decades, and
it’s getting worse $$$
Thursday, February 10, 2011
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011
and how to hide mad cow disease in Canada Current as of: 2011-01-31
Wednesday, August 11, 2010
REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM
ENCEPHALOPATHY (BSE) IN CANADA
Thursday, August 19, 2010
REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM
ENCEPHALOPATHY (BSE) IN CANADA
Friday, March 4, 2011
Alberta dairy cow found with mad cow disease
Reasons for the New Regulation Order No. 23 (as well as amending Order No.
149) of the State Committee for Veterinary Medicine name BSE as the reason for
new import requirement. The legal title for Order No. 23 is "On Urgent Measures
Aimed at Prevention and Elimination of BSE and Other Prion Infections in
Cattle”. Neither Order explains how the threat of introduction of BSE can be
addressed through the inspection of producers of all products of animal origin
including fish, dairy products, poultry and pork. It is not clear what other
concerns are addressed through the proposed inspections. Formal Notification of
Trading Partners On August 3rd, Ukraine's Notification and Enquiry Point issued
a legal Notification G/SPS/N/UKR/3/Rev.1 found on the Official WTO Website
(Committee on Sanitary and Phytosanitary Measures)
Thursday, March 29, 2012
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH
CODE
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...
EFSA Journal 2011 The European Response to BSE: A Success Story
This is an interesting editorial about the Mad Cow Disease debacle, and
it's ramifications that will continue to play out for decades to come ;
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 ;
2011 Monday, September 26, 2011
L-BSE BASE prion and atypical sporadic CJD
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
Wednesday, August 01, 2012
Behavioural and Psychiatric Features of the Human Prion Diseases:
Experience in 368 Prospectively Studied Patients
Monday, August 06, 2012
Atypical neuropathological sCJD-MM phenotype with abundant white matter
Kuru-type plaques sparing the cerebellar cortex
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Friday, August 24, 2012
Iatrogenic prion diseases in humans: an update
Monday, July 23, 2012
The National Prion Disease Pathology Surveillance Center July 2012
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases.
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until
now. Overall, 5 voles were positive with survival time between 281 and 596
d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like
PrPSc electrophoretic pattern, characterized by low molecular weight PrPres.
These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative
with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus
and the N-terminus. Second passages are in progress from these first successful
transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in
BvI109.
The discovery of previously unrecognized prion diseases in both humans and
animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion
diseases might be wider than expected and raises crucial questions about the
epidemiology and strain properties of these new forms. We are investigating this
latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
*** The discovery of previously unrecognized prion diseases in both humans
and animals (i.e., Nor98 in small ruminants) demonstrates that the range of
prion diseases might be wider than expected and raises crucial questions about
the epidemiology and strain properties of these new forms. We are investigating
this latter issue by molecular and biological comparison of VPSPr, GSS and
Nor98.
AS OF AUGUST 2012 ;
CJD UPDATE USA
1 Listed based on the year of death or, if not available, on year of
referral; 2 Cases with suspected prion disease for which brain tissue and/or
blood (in familial cases) were submitted; 3 Disease acquired in the United
Kingdom; 4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case; *** 5 Includes 8 cases in which the diagnosis is
pending, and 18 inconclusive cases; *** 6 Includes 10 (9 from 2012) cases with
type determination pending in which the diagnosis of vCJD has been excluded. ***
The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42
cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases of
sporadic Creutzfeldt-Jakob disease (sCJD).
Friday, November 23, 2012
sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA,
AND CANADA
Sunday, December 9, 2012
Prions, prionoids and pathogenic proteins in Alzheimer disease
R.I.P. MOM DOD 12/14/97 hvCJD confirmed...
TSS
