7. Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine
Products; Final Rule
Federal Register / Vol. 78 , No. 233 / Wednesday, December 4, 2013 Rules
and Regulations
DEPARTMENT OF AGRICULTURE
Animal and Plant Health Inspection Service 9 CFR Parts 92, 93, 94, 95, 96,
and 98 Docket No. APHIS-2008-0010 RIN 0579-AC68
AGENCY: Animal and Plant Health Inspection Service, USDA.
ACTION: Final rule.
SUMMARY: We are amending the regulations that govern the importation of
animals and animal products to revise the conditions for the importation of live
bovines and products derived from bovines with regard to bovine spongiform
encephalopathy (BSE). We are basing importation conditions on the inherent risk
of BSE infectivity in specified commodities, as well as on the BSE risk status
of the region in which the commodities originate. We are establishing a system
for classifying regions as to BSE risk that is consistent with the system
employed by the World Organization for Animal Health (OIE), the international
standard-setting organization for guidelines related to animal health. The
conditions we are adopting for the importation of specified commodities are
based on internationally accepted scientific literature, and are, in general,
consistent with guidelines set out in the OIE's Terrestrial Animal Health Code.
We are also classifying certain specified countries as to BSE risk and are
removing BSE restrictions on the importation of cervids and camelids and
products derived from such animals. We are making these amendments after
conducting a thorough review of relevant scientific literature and a
comprehensive evaluation of the issues and concluding that the changes to the
regulations will continue to guard against the introduction of BSE into the
United States, while allowing the importation of additional animals and animal
products into this country.
DATES: This rule is effective March 4, 2014. The incorporation by reference
of the material described in the rule is approved by the Director of the Federal
Register as of March 4, 2014.
Full text:
See Also:
Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy
Federal Register Volume 78, Number 233 (Wednesday, December 4, 2013) Notices
Pages 72859-72860 From the Federal Register Online via the Government Printing
Office [www.gpo.gov] FR Doc No: 2013-28338
DEPARTMENT OF AGRICULTURE
Animal and Plant Health Inspection Service Docket No. APHIS-2013-0064
Concurrence With OIE Risk Designations for Bovine Spongiform
Encephalopathy
AGENCY: Animal and Plant Health Inspection Service, USDA. ACTION:
Notice.
SUMMARY: We are advising the public of our preliminary concurrence with the
World Organization for Animal Health's (OIE) bovine spongiform encephalopathy
(BSE) risk designations for 14 regions. The OIE recognizes these regions as
being of either negligible risk for BSE or of controlled risk for BSE. We are
taking this action based on our review of information supporting the OIE's risk
designations for these regions.
DATES: We will consider all comments that we receive on or before February
3, 2014.
Full text:
Saturday, November 2, 2013
APHIS Finalizes Bovine Import Regulations in Line with International Animal
Health Standards while enhancing the spread of BSE TSE prion mad cow type
disease around the Globe
I AGREE WITH MR. BULLARD, it’s all about trade and money, BSE TSE PRION aka
mad cow type disease and sound science there from, was thrown out the window by
the USDA et al that fateful day in December 23, 2003, when the USDA lost it’s
‘gold card’ of supposedly being BSE FREE, (that was and still is a sad joke
though), that’s when mad cow junk science was adopted by the USDA...
see why below...kind regards, terry
Monday, November 4, 2013
*** R-CALF Bullard new BSE rule represents the abrogation of USDA’s
responsibility to protect U.S. consumers and the U.S. cattle herd from the
introduction of foreign animal disease
Wednesday, October 30, 2013
SPECIFIED RISK MATERIAL (SRM) CONTROL VERIFICATION TASK FSIS NOTICE 70-13
10/30/13
Terry S. Singeltary Sr. 6/12/08
Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live
Bovines and Products Derived from Bovines Commodities APHIS-2006-0041
Tuesday, May 21, 2013
*** Canada, USA, Bad feed, mad cows: Why we know three BSE cases had a
common origin and why the SSS policy is in full force $$$
CFIA, USDA, AND OIE SHOOT, SHOVEL, AND SHUT THE HELL UP SSS BSE TSE PRION
MAD COW TYPE POLICY $$$, and the media is buying it hook, line, and sinker $$$
EDMONTON - Some of former Alberta premier Ralph Klein's most colourful
quotes — and the reactions they elicited:
SNIP...
"This all came about through the discovery of a single, isolated case of
mad cow disease in one Alberta cow on May 20th.
The farmer — I think he was a Louisiana fish farmer who knew nothing about
cattle ranching.
*** I guess any self-respecting rancher would have shot, shovelled and shut
up, but he didn't do that." — Klein recalls how the mad cow crisis started and
rancher Marwyn Peaster's role.
The premier was speaking at the Western Governors Association meeting in
Big Sky, Mont. September 2004.
Wednesday, December 22, 2010.
Manitoba veterinarian has been fined $10,000 for falsifying certification
documents for U.S. bound cattle and what about mad cow disease?
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
CENSORSHIP IS A TERRIBLE THING $$$
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
$$$
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
BSE in USA
Tags: TSE, USA
Source: FDA
A final investigation summary into the Californian case of BSE in April
this year has been published by FDA.
The report concludes that "compliance with BSE feed regulations was
excellent" and that "none of the facilities had used prohibited material in
their feed manufacturing during the entire period of interest". The cow,
obviously, was not infected through feed, but developed the disease from a
'spontaneous mutation'.
As this is certainly good news regarding the effectiveness of the feed ban
system in the US, the fact that this cow had atypical BSE makes it more likely,
not less, that there are more cases like this one in the US dairy herd. If a
spontaneous mutation can develop once, it can develop several times. The
relevance of atypical BSE for public health is unclear at this time, but there
are indications that L-type atypical BSE, as diagnosed in the Californian cow,
is transmissible to primates. The question then is how reliably such cases can
be detected by the US surveillance system. The April case was detected in the
course of "random testing".
Saturday, May 26, 2012
Are USDA assurances on mad cow case 'gross oversimplification'?
SNIP...
What irks many scientists is the USDA’s April 25 statement that the rare
disease is “not generally associated with an animal consuming infected
feed.”
The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown,
one of the world’s experts on this type of disease who retired recently from the
National Institutes of Health.
"(The agency) has no foundation on which to base that statement.”
“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an
official with the USDA during the Clinton Administration now at Mississippi
State.
In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the
origins of atypical cases of BSE,” she said
The argument about feed is critical because if feed is the cause, not a
spontaneous mutation, the California cow could be part of a larger outbreak.
SNIP...
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
in the url that follows, I have posted
SRM breaches first, as late as 2011.
then
MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until
2007, when they ceased posting them.
then,
MAD COW SURVEILLANCE BREACHES.
Friday, May 18, 2012
Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy
(BSE) in the United States Friday May 18, 2012
Thursday, June 21, 2012
MEATINGPLACE.COM WAVES MAGIC WAND AND EXPECTS THE USDA MAD COW FOLLIES BSE
TO BE GONE
Thursday, June 14, 2012
R-CALF USA Calls USDA Dishonest and Corrupt; Submits Fourth Request for
Extension
R-CALF United Stockgrowers of America
Friday, May 25, 2012
R-CALF USDA’s New BSE Rule Eliminates Important Protections Needed to
Prevent BSE Spread
Monday, June 18, 2012
R-CALF Submits Incomplete Comments Under Protest in Bizarre Rulemaking
“Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products”
Monday, August 06, 2012
Atypical neuropathological sCJD-MM phenotype with abundant white matter
Kuru-type plaques sparing the cerebellar cortex
Monday, July 23, 2012
The National Prion Disease Pathology Surveillance Center July 2012
Monday, August 6, 2012
TAFS BSE in USA August 6, 2012
BSE in USA
Saturday, May 2, 2009
APHIS AND WHO PLAN TO EXEMPT THE ATYPICAL SCRAPIE NOR-98 FROM REGULATIONS
AT MEETING THIS MONTH
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH
CODE
Thursday, December 20, 2012
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe
WITH BOVINE MAD COW DISEASE
Tuesday, July 2, 2013
APHIS USDA Administrator Message to Stakeholders: Agency Vision and Goals
Eliminating ALL remaining BSE barriers to export market
I lost my mother to the Heidenhain Variant of Creutzfeldt Jakob Disease DOD
12/14/97 ‘confirmed’, and never could except the science that the USDA et al
brought forth to date i.e. the UKBSEnvCJD only theory. I simply made a promise
to mom, NEVER FORGET, and so soon you all FORGOT $
Seems none of the officials remember, that went through the mad cow
debacle, or mad cow follies as I have termed them, and they are ongoing, for
anyone who thinks mad cow disease is gone, they are only fooling themselves, and
at the same time, making fools out of everyone that goes along with this TSE
prion mad cow junk science that the OIE has brought to every country around the
globe, and we will ALL pay the price in the years and decades to come. ...
IT is of my opinion, that the OIE and the USDA et al, are the soul reason,
and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion
diseases, including typical and atypical BSE, typical and atypical Scrapie, and
all strains of CWD, and human TSE there from, spreading around the globe.
I have lost all confidence of this organization as a regulatory authority
on animal disease, and consider it nothing more than a National Trading
Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i
said before, OIE should hang up there jock strap now, since it appears they will
buckle every time a country makes some political hay about trade protocol,
commodities and futures. IF they are not going to be science based, they should
do everyone a favor and dissolve there organization.
JUST because of low documented human body count with nvCJD and the long
incubation periods, the lack of sound science being replaced by political and
corporate science in relations with the fact that science has now linked some
sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of
CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call
for this organization to be dissolved. ...
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
Aug. 5, 2001, 12:25AM
Mad cow disease: Could it be here?
Man's stubborn crusade attracts experts' notice
By CAROL CHRISTIAN Copyright 2001 Houston Chronicle
Like Paul Revere with e-mail, Terry Singeltary Sr. is on a mission to sound
an alarm: Beware of mad cow disease.
As is true of many crusaders, however, his pleas often fall on deaf ears.
Health officials here and abroad insist that bovine spongiform encephalopathy --
popularly known as mad cow disease, a fatal brain disorder that can make cows
shake uncontrollably -- has been kept out of this country through surveillance
of the cattle industry.
But since his mother's death in December 1997, the Galveston County man has
been obsessed with possible connections between her deadly brain disorder,
sporadic Creutzfeldt-Jakob Disease, and mad cow disease.
And after much persistence on his part, people are taking notice of this
former machinist and high school dropout who jokes that he has a Ph.D. -- a Pool
Hall Degree.
"They called me Chicken Little for four years," he said. "Now they're
calling back, asking for more information."
For the past year he has been U.S. co-coordinator of an international
monitoring group called CJD Watch. He regularly gets e-mail from scientists and
journalists around the world.
Debora MacKenzie, a reporter for the British magazine New Scientist,
described Singeltary, 47, as a "dogged unearther and tabulator of government
documents." Singeltary monitors "every word written about CJD/BSE," said Anita
Manning of USA Today, also by e-mail.
"He's passionate, opinionated and not always tactful, although I like him
because he's such a character and he is so transparent," Manning said. "He is
what he appears to be."
Science and environment writer Jonathan Leake of the Sunday Times in London
said Singeltary has helped him track down families of people with CJD along with
academic research papers.
"I strongly suspect he is right in thinking the USA has had BSE cases,"
Leake said by e-mail.
"The American government is making the same mistake as the British in
putting the short-term commercial interests of its farmers before health
considerations," he added.
"It should start formal and widespread testing of cattle plus compulsory
autopsies for all human CJD victims at the state's expense. If there is BSE,
then leaving it to spread will kill people -- and that would eventually destroy
the industry, too."
Texas Department of Health epidemiologist Julie Rawlings said Singeltary's
careful monitoring of the disease had proven useful.
"Terry has been helpful in providing contact information regarding suspect
CJD cases so that the Health Department can initiate case investigations and
learn more about CJD in Texas," she said.
Noting that the department cannot release records on individual patients,
she added, "I think we learn more from him than he does from us."
Mad cow disease surfaced in England in 1986 and quickly became an epidemic.
It since has been reported in 15 European countries, most recently Greece on
July 2, and the Czech Republic on June 14. Two German-born cows tested positive
for BSE in November.
Singeltary said he became convinced that BSE is here as he watched his
mother, Barbara Poulter of Crystal Beach, dying of sporadic Creutzfeldt-Jakob
Disease. The rare, fatal brain disease is sometimes accompanied by severe
jerking.
"She would jerk so bad at times, it would take three of us to hold her
down," Singeltary said. "They can call it whatever they want, but I know what I
saw, and what she went through. `Sporadic' simply means they don't know."
Poulter, a retired telephone-company field worker, had a form of sporadic
CJD -- Haidenhain variant -- that is even less common than the typical sporadic
case. One of its first symptoms is loss of vision.
She started seeing brown spots in September 1997 and was virtually blind
within two weeks. By the eighth week of the illness Poulter was bedridden, and
in the 10th week she died. Before that she had been in good health.
In many countries and most U.S. states, physicians are not required to
report CJD cases to health officials. Texas made the disease reportable in 1998.
Through 2000, there were 17 probable or confirmed cases, according to the Texas
Department of Health.
In mid-June, a case of sporadic CJD was confirmed through brain biopsy at
Christus Spohn Hospital Shoreline in Corpus Christi, said Jane Bakos, hospital
vice president. The patient has since died, the hospital reported.
CJD and mad cow disease leave their victims' brains full of holes like a
sponge.
Although not contagious, the illnesses are thought to be transmissible
through prions, or nearly indestructible abnormal proteins.
Because the prion protein is not killed by standard sterilization, sporadic
CJD can be spread by contaminated surgical instruments.
In March 1996, the British government announced the discovery of a new
variant of CJD, most likely explained by exposure to bovine spongiform
encephalopathy.
Through June, 101 cases of new-variant CJD have been reported in the United
Kingdom, three in France and one in Ireland. In contrast to sporadic CJD, the
new variant usually affects younger patients and lasts longer.
No cases of new-variant CJD or BSE have been reported in the United States.
No relationship has been shown between sporadic CJD and mad cow disease.
There is no indication that new-variant CJD can be spread through blood
transfusions, but a U.S. Food and Drug Administration advisory committee voted
in June to broaden the categories for excluding potential donors. The
recommendations have not yet been approved by the FDA.
The American Red Cross has announced that on Sept. 17 it will begin
rejecting potential blood donors who, since 1980, have spent at least three
months in the United Kingdom or at least six months in any European country or
combination of countries. Those who have received a blood transfusion in Britain
since 1980 also will be rejected.
The primary collector of local blood donations is the Gulf Coast Regional
Blood Center, which will follow the FDA's guidelines, said Bill Teague,
president and chief executive officer.
Singeltary said it's naive to think that U.S. prevention efforts have kept
mad cow and new-variant CJD out of the United States.
"They haven't found it," he said, "because they haven't looked."
For one thing, he said, too few cows are tested for the disease. In the
first six months of this year, the European Union tested more than 3.2 million
cows, David Byrne of the European Commission said in a speech last month.
By contrast, it took the U.S. Department of Agriculture nearly 10 years to
analyze about 13,000 cow brains, according to the department's Web site.
With more than 68 million cattle slaughtered since 1990 in the United
States, according to the USDA, checking about 13,000 falls far short, Singeltary
said.
Though not a scholar, Singeltary has collected voluminous material on mad
cow and CJD. Disabled from a neck injury, Singeltary never used a computer until
1998.
He now spends hours each day on the Internet while his wife, Bonnie
Singeltary, runs a flower shop in their home in Bacliff, in north Galveston
County.
His challenge to the CJD/BSE establishment is courageous and refreshing,
said Dr. Lynette Dumble, former visiting professor of surgery at University of
Texas Medical School at Houston and a former senior research fellow in the
history and philosophy of science at the University of Melbourne in
Australia.
"I certainly have no problem with Terry's ideas on BSE/CJD," said Dumble,
who coordinates the Global Sisterhood Network, a computer service that posts
media reports on developments affecting women. "His research skills are
excellent, and he is abreast of each and every development in the field."
Among Singeltary's worries now, he said, are widespread violations of an
August 1997 ban on feeding animal products to U.S. cattle. The FDA reported in
January that hundreds of feed manufacturers were not complying with regulations
designed to keep BSE out of this country.
(That same month, a Purina Mills feedlot near San Antonio told the FDA that
a "very low level" of cow parts had been found in cattle feed. The company
voluntarily removed 1,222 animals who had been fed the prohibited
materials.)
He obtained copies of FDA letters to various feed mills that had been found
in violation of the regulations and immediately sent them by e-mail to hundreds
of people around the world.
Singeltary might not be so zealous in getting the word out if he weren't
convinced that someone is covering up the truth.
"They used to say BSE would never transmit to humans," he said, "and it
has. They lied about the feed ban being in place.
"I've lost faith in the whole process. I've discovered too many
things."
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, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH
CODE
Monday, December 1, 2008
When Atypical Scrapie cross species barriers
TSS
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