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From: TSS ()
Subject: Recalled beef from Chino slaughterhouse was used in 466 food products
Date: March 21, 2008 at 11:07 am PST

Recalled beef from Chino slaughterhouse was used in 466 food products


10:05 PM PDT on Wednesday, March 19, 2008

By JANET ZIMMERMAN
The Press-Enterprise

PDF: Businesses that may have received the meat

http://www.pe.com/multimedia/pdf/2008/WestlandRecallConsolidatedRetailDistributionforWeb3-18-08.pdf


PDF: Recalled products

http://www.pe.com/multimedia/pdf/2008/AdditionalProductsContainingWestlandRecalledBeef03-14-08.pdf


From Slim Jim jerky and Jenny Craig meatloaf to Farmer John salami and Kids
Cuisine frozen tacos, the list of products containing recalled meat from the
now-closed Chino slaughterhouse continues to grow.

The California Department of Public Health now lists 466 types of foods sold
to markets, restaurants, grocery chains, catering businesses, workplace
cafeterias and other food services. The state expects the list to get
longer, department spokeswoman Lea Brooks said.

"We're identifying more products in which the recalled beef was an
ingredient," she said.

When the list debuted in late February, it contained products from three
manufacturers. Now there are 16. The recall may not involve entire product
lines. The links to affected lot numbers and retail distributors are listed
on the California Department of Public Health Web site: www.cdph.ca.gov

Also growing is the list of California restaurants, markets and other
retailers that may have received some of the 143 million pounds of beef from
Westland/Hallmark Meat Co.

The recall, the largest beef recall in U.S. history, was triggered by an
undercover video shot by the Humane Society of the United States showing
employees at the plant abusing cattle and violating federal slaughter rules.

Still at issue is who will pay for the losses. Industry experts have said
the value of the affected foods could reach hundreds of millions of dollars.

Westland/Hallmark President Steve Mendell testified before a congressional
subcommittee last week that his company is broke and won't be able to
reimburse distributors or the many schools that received his meat products
through the National School Lunch Program.

The recall, which covered meat from cattle slaughtered at the plant between
Feb. 1, 2006, and Feb. 2, 2008, is a class II recall, which means the chance
of getting sick from the meat is remote.

In Riverside County, one restaurant was found to have some recalled meat.
None has been found by public health officials in San Bernardino County, and
the chances lessen as time passes, they said. Most has been consumed,
destroyed or returned to the manufacturer, said Steve Van Slocum, Riverside
County's deputy director of environmental health on Wednesday.

As recently as last week, Ralphs and Food 4 Less, which have 400 stores in
Southern California, were removing items from shelves, said Terry O'Neill,
the chains' spokesman. The stores don't carry all items on the list, and
O'Neill couldn't say which ones were pulled.

"The recall is so vast," he said.

The products are being immediately removed from shelves based on
notification from the manufacturer or the grocery chain's parent company,
Cincinnati-based Kroger Co., and being destroyed or held for return, he said

O'Neill said all customers will be reimbursed for products that are on the
list or that they are worried may be affected.

On Wednesday, three restaurants were removed from the list of retailers in
the state -- which grew from 5,000 to 7,900 in the past three weeks. But
some distributors turned over names of all their customers, so the state's
list included some stores and restaurants that never received recalled meat.

In all, six restaurants have been deleted from the list: P.H. Wood's Brewery
in Moreno Valley, the Yellow Basket restaurants in Temecula and Santa Ana,
and three others in Orange County.

P.H. Wood's received a small amount of the meat from supplier American Meats
in 2002. It was probably a sample, said Scott Diehl, the brewery's general
manager and part owner.

A couple of customers notified him that he was on the list, and it took
about a week to get the documentation, mostly letters from his suppliers of
six years, to prove he wasn't receiving meat from Westland/Hallmark, Diehl
said.

"The frustrating thing is having that bad name of being on the list," he
said. "It looks like (the distributor) gave a blanket list."

Reach Janet Zimmerman at 951-368-9586 or jzimmerman@PE.com


http://www.pe.com/localnews/sbcounty/stories/PE_News_Local_D_recall20.3cf1153.html


Recalled beef from Chino slaughterhouse was used in 466 food products

http://downercattle.blogspot.com/2008/03/recalled-beef-from-chino-slaughterhouse.html


QUESTIONS AND ANSWERS HALLMARK/WESTLAND MEAT PACKING CO.

March 6, 2008

Consumer Concerns

Q. My child/school recently consumed Hallmark/Westland products. What is the
risk to children's health?


snip...


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

Our prior report identified a number of inherent problems in identifying and
testing high-risk cattle. We reported that the challenges in identifying the
universe of high-risk cattle, as well as the need to design procedures to
obtain an appropriate representation of samples, was critical to the success
of the BSE surveillance program. The surveillance program was designed to
target nonambulatory cattle, cattle showing signs of CNS disease (including
cattle testing negative for rabies), cattle showing signs not inconsistent
with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS
condemned cattle were sampled and made a concerted effort for outreach to
obtain targeted samples, industry practices not considered in the design of
the surveillance program reduced assurance that targeted animals were tested
for BSE.


snip...


Inherent Limitations in Identifying and Testing High-Risk Cattle APHIS obtained significantly more samples for testing than they originally anticipated would be needed to achieve its stated level of confidence in estimating the prevalence of BSE in the U.S. herd. Because of the voluntary nature of its program, however, we could not determine how successful APHIS was in obtaining a representative proportion of high-risk cattle for testing. Our prior report recognized the significant challenges for APHIS to obtain samples from the high-risk population because of the inherent problems with obtaining voluntary compliance and transporting carcasses for testing. APHIS took steps to obtain facilitated pathways, by entering into over 100 agreements, to collect and test brain samples for BSE. However, using USDA published data that estimates the distribution of the cattle population, as well as those that died or became nonambulatory, we could not determine whether APHIS achieved either geographical representation or representation of the desired surveillance stream (clinical suspects, fallen stock, casualty slaughter fallen stock, and routine slaughter). Findings 1 and 2 present the conditions noted that impact this evaluation. USDA Testing Protocols and Quality Assurance Procedures In November 2004, USDA announced that its rapid screening test produced an inconclusive BSE test result. A contract laboratory ran its rapid screening test on a brain sample collected for testing and produced three high positive reactive results. As required, the contract laboratory forwarded the inconclusive sample to APHIS’ National Veterinary Services Laboratories (NVSL) for confirmation. NVSL repeated the rapid screening test, which again produced three high positive reactive results. Following established protocol, NVSL ran its confirmatory test, an immunohistochemistry (IHC) test, which was interpreted as negative for BSE. Faced with conflicting results between the rapid screening and IHC tests, NVSL scientists recommended additional testing to resolve the discrepancy but APHIS headquarters officials concluded that no further testing was necessary since testing protocols were followed and the confirmatory test was negative. In our discussions with APHIS officials, they justified their decision to not do additional testing because the IHC test is internationally recognized as the “gold standard” of testing. Also, they believed that

conducting additional tests would undermine confidence in USDA’s testing protocols. OIG obtained evidence that indicated additional testing was prudent. We came to this conclusion because the rapid screening tests produced six high positive reactive results, the IHC tests conflicted, and various standard operating procedures were not followed. Also, our review of the relevant scientific literature, other countries’ protocols, and discussions with experts led us to conclude that additional confirmatory testing should be considered in the event of conflicting test results. To maintain objectivity and independence, we requested that USDA’s Agricultural Research Service (ARS) perform the Office International des Epizooties (OIE) Scrapie-Associated Fibrils (SAF) immunoblot test. The additional testing produced positive results. To confirm, the Secretary of Agriculture requested that an internationally recognized BSE laboratory in Weybridge, England (Weybridge) perform additional testing. Weybridge conducted various tests, including their own IHC tests and three Western blot tests. The tests confirmed that the cow was infected with BSE. The Secretary immediately directed USDA scientists to work with international experts to develop new protocols that include performing dual confirmatory tests in the event of an inconclusive BSE screening test. We attribute the failure to identify the BSE positive sample to rigid protocols, as well as the lack of adequate quality assurance controls over its testing program. Details of our concerns are discussed in Findings 3 and 4.

snip...

Controls (Firewalls) to Prevent BSE in the Food Supply USDA instituted proactive procedures to prevent tissues and products that could possibly contain the infective agent for BSE from entering the food supply. FSIS performs inspections on cattle before slaughter (ante mortem) to observe clinical signs that may indicate a central nervous system disorder or other signs that may be associated with BSE. Such animals are condemned and prohibited from slaughter for human consumption. FSIS also identified high-risk beef tissue and products as SRMs, and banned them from the food supply. FSIS inspects slaughter processes to verify that slaughterhouses have incorporated controls for handling SRMs into their operational plans; adequate procedures must be in place for removing, segregating, and disposing of SRMs. OIG reviewed the SRM plans of several establishments, observed FSIS inspection procedures, and evaluated the effectiveness of controls during the slaughter process. We did not identify SRMs entering the food supply. However, due to the lack of adequate records, we could not determine whether SRM procedures were followed and/or were adequate in 9 of

12 establishments visited during the audit. There is no requirement in the United States for the age of animals to be recorded, therefore, APHIS and FSIS rely on meat establishments to determine the age of cattle slaughtered using documentation or dentition. SRM restrictions apply predominantly to cattle 30 months of age or older. FSIS periodically checks the accuracy of age determinations through dentition; however, we could not determine how often these checks are made. We found that improvements can be made in the following areas. • FSIS approved an alternate ante mortem inspection procedure that limited the number of cattle subject to inspection. FSIS discontinued this procedure during the audit. • FSIS does not have an information system capable of readily identifying the scope of, and trends in, noncompliance violations relating to SRMs. • Most of the establishments reviewed did not have adequate SRM plans, and FSIS did not always identify these deficiencies. • Several of the establishments did not comply with their SRM plans and/or maintain records to support that they follow their plans. FSIS has addressed the specific cases of noncompliance identified during the audit. Findings

snip...


Downers and Cattle that Died on the Farm

Our prior audit recognized the significant challenge for APHIS to obtain samples from some high-risk populations because of the inherent problems with obtaining voluntary compliance and transporting the carcasses for testing. USDA issued rules to prohibit nonambulatory animals (downers) from entering the food supply at inspected slaughterhouses. OIG recommended, and the International Review Subcommittee33 emphasized, that USDA should take additional steps to assure that facilitated pathways exist for dead and nonambulatory cattle to allow for the collection of samples and proper disposal of carcasses. Between June 1, 2004, and May 31, 2005, the APHIS database documents 27,617 samples were collected showing a reason for submission of nonambulatory and 325,225 samples were collected with reason of submission showing “dead.”

snip...


We also disagree with APHIS/FSIS’ contention that because they have tested over 375,000 of their 446,000 estimate of high risk cattle, few in the high-risk population are being missed, including those that might be pre-screened before entering a slaughter facility’s property. In our prior audit, we reported that APHIS underestimated the high-risk population;


*** we found that this estimate should have been closer to 1 million animals (see Finding 1).


snip...


The policy stated in the preamble to 9 CFR 309.2(b)104 states that FSIS has excluded all nonambulatory disabled cattle from the human food supply, regardless of the reason for their nonambulatory status or the time at which they became nonambulatory (emphasis added). If an animal becomes nonambulatory in route to the establishment due to an acute injury, it must be humanely removed from the truck, humanely euthanized, and the carcass properly disposed of. Likewise, cattle that become nonambulatory on the establishment premises, such as an animal that breaks its leg as it is unloaded from the truck, are also required to be humanely moved, humanely euthanized, and the carcass disposed of properly. However, an FSIS notice105 states that if cattle are ambulatory at ante mortem inspection and become nonambulatory disabled prior to slaughter, the VMO should verify that the animal suffered an acute injury and allow the animal to proceed to slaughter and post mortem inspection. FSIS would expect such situations to be extremely rare because cattle, when handled and moved under proper humane handling conditions, should not be injured while being moved in pens. For cattle that become nonambulatory disabled after ante

mortem inspection, if the VMO cannot determine that a specific, acute injury occurred that caused the animal to become nonambulatory disabled, the animal is to be condemned and cannot enter the slaughter establishment. There appears to be inconsistent USDA policies related to slaughtering downers/nonambulatory cattle. Regarding animals for slaughter, it is clear that downers will not be slaughtered. In fact, one report106 states: “The U.S. Policy is to condemn all cattle that are nonambulatory or disabled when presented for slaughter." The Department has widely publicized that one of the firewalls put in place to prevent the spread of BSE is the prevention of downers from entering the food supply. Our review at the 12 plants visited showed the following variations in application of the policy for condemning or passing nonambulatory cattle for slaughter.

This was the only documentation of the condition of the cattle available at the plants. Plant inspection personnel believed that FSIS Notice 5-04 allowed the slaughter of nonambulatory cattle if the cattle had passed ante mortem inspection and then went down as the result of an acute injury. Therefore, they had allowed the plant to slaughter these cattle for human consumption. We observed use of a forklift and a rail above the pens to transport nonambulatory cattle to the slaughter area.

snip...see full text 130 pages ;

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


snip...


Science 23 November 2001:
Vol. 294. no. 5547, pp. 1726 - 1728
DOI: 10.1126/science.1066838

Reports

Estimation of Epidemic Size and Incubation Time Based on Age Characteristics
of vCJD in the United Kingdom

Alain-Jacques Valleron,1 Pierre-Yves Boelle,1 Robert Will,2 Jean-Yves
Cesbron3


SNIP...


The distribution of the vCJD incubation period that best fits the data
within the framework of our model has a mean of 16.7 years, with a standard
deviation of 2.6 years. The 95% upper percentile of this distribution is
21.4 years. The 95% confidence interval (CI) of the estimates of the mean
and standard deviation is relatively narrow: The 95% CI for the estimate of
the mean incubation period is 12.4 to 23.2 years, and the 95% CI of the
standard deviation is 0.9 to 8 years (10). The decrease in susceptibility to
infection in exposed subjects older than 15 years, as estimated from the
parameter , was found to be very sharp: 16% per year of age (CI: 12 to 23%).
This means that, under the best fitting hypothesis, an individual aged 20
years in 1981 had 55% less risk of becoming infected than a child aged 15
years (99.9% for an individual aged 70).

http://www.sciencemag.org/

http://downercattle.blogspot.com/2008/03/usda-questions-and-answers.html

SPECIFIED RISK MATERIALS

http://madcowspontaneousnot.blogspot.com/2008/02/specified-risk-materials-srm.html

March 16, 2008


MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or
Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Communicated by:
Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. - Mod.CP]

[see also:

snip...


************************************************************
Become a ProMED-mail Premium Subscriber at

************************************************************
Visit ProMED-mail's web site at .


http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

Original Paper

Association between Deposition of Beta-Amyloid and Pathological Prion
Protein in Sporadic Creutzfeldt-Jakob Disease

Laura Debatina, Johannes Strefferb, Markus Geissenc, Jakob Matschkec,
Adriano Aguzzia, Markus Glatzela, c

aInstitute of Neuropathology, and
bDivision of Psychiatry Research, University Hospital Zurich, Zurich,
Switzerland;
cInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf,
Hamburg, Germany


Address of Corresponding Author

Neurodegenerative Dis (DOI: 10.1159/000121389)


----------------------------------------------------------------------------
----

Key Words

Sporadic Creutzfeldt-Jakob disease
Alzheimer's disease
Deposition of -amyloid
Prion protein

----------------------------------------------------------------------------
----

Abstract

Background: Alzheimer's disease (AD) and prion diseases such as sporadic
Creutzfeldt-Jakob disease (sCJD) share common features concerning their
molecular pathogenesis and neuropathological presentation and the
coexistence of AD and CJD in patients suggest an association between the
deposition of the proteolytically processed form of the amyloid precursor
protein, -amyloid (A), which deposits in AD, and the abnormal form of the
prion protein, PrPSc, which deposits in sCJD. Methods: We have characterized
sCJD patients (n = 14), AD patients (n = 5) and nondemented controls (n = 5)
with respect to the deposition of PrPSc and A morphologically, biochemically
and genetically and correlated these findings to clinical data. Results:
sCJD-diseased individuals with abundant deposits of A present with a
specific clinicopathological profile, defined by higher age at disease
onset, long disease duration, a genetic profile and only minimal amounts of
PrPSc in the cerebellum. Conclusion: The co-occurrence of pathological
changes typical for sCJD and AD in combination with the inverse association
between accumulation of A and PrPSc in a subgroup of sCJD patients is
indicative of common pathways involved in the generation or clearance of A
and PrPSc in a subgroup of sCJD patients.

Copyright © 2008 S. Karger AG, Basel


----------------------------------------------------------------------------
----

Author Contacts

Markus Glatzel
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf
Martinistrasse 52, DE-20246 Hamburg (Germany)
Tel. +49 40 42 803 2218, Fax +49 40 42 803 4929
E-Mail m.glatzel@uke.uni-hamburg.de

http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000121389


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


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Since this article does not have an abstract, we have provided the first 150
words of the full text and any section headings.


To the Editor:

In their Research Letter, Dr Gibbons and colleagues1 reported that the
annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable
since 1985. These estimates, however, are based only on reported cases, and
do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number
of persons with a diagnosis of Alzheimer disease in fact may have CJD,
although only a small number of these patients receive the postmortem
examination necessary to make this diagnosis. Furthermore, only a few states
have made CJD reportable. Human and animal transmissible spongiform
encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr
Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL
TEXT


http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

should be drastic measures to safeguard the medical and surgical arena

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

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


Regarding Alzheimer's disease

(note the substantial increase on a yearly basis)

http://www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf


snip...

The pathogenesis of these diseases was compared to Alzheimer's disease at a
molecular level...

snip...

http://www.bseinquiry.gov.uk/files/yb/1990/03/12003001.pdf


And NONE of this is relevant to BSE?

There is also the matter whether the spectrum of ''prion disease'' is wider
than that recognized at present.

http://www.bseinquiry.gov.uk/files/yb/1990/07/06005001.pdf


Human BSE

snip...

These are not relevant to any possible human hazard from BSE nor to the much
more common dementia, Alzheimers.

snip...

http://www.bseinquiry.gov.uk/files/yb/1990/07/09001001.pdf


IN STRICT CONFIDENCE

TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES

http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf


Subject: Re: Hello Dr. Manuelidis Date: Fri, 22 Dec 2000 17:47:09 -0500
From: laura manuelidis Reply-To:
laura.manuelidis@yale.edu Organization: Yale Medical School To: "Terry S.
Singeltary Sr."

References: <

Dear Terry,

One of our papers (in Alzheimer's Disease Related Disord. 3:100-109, 1989)
in text cites 6 of 46 (13%) of clinical AD as CJD. There may be a later
paper from another lab showing the same higher than expected incidence but I
can't put my hands on it right now. We also have a lot of papers from 1985
on stating that there are likely many silent (non-clinical) CJD infections,
i.e. much greater than the "tip of the iceberg" of long standing end-stage
cases with clinical symptoms. Hope this helps.

best wishes for the new year laura manuelidis

"Terry S. Singeltary Sr." wrote: > > Hello again Dr. Manuelidis, > > could
you please help me locate the 2 studies that were > done on CJD where it
showed that up to 13% of the people > diagnosed as having Alzheimer's
actually had CJD. > trying to find reference... > > thank you, > Terry S.
Singeltary Sr.


==================================
http://neurotalk.psychcentral.com/thread13175.html

http://neurotalk.psychcentral.com/showthread.php?p=156015


TSE UPDATE (SEE LINKS BELOW)

Thursday, March 13, 2008

DOWNER COW BLUES SENATORS WANT CRACKDOWN

http://downercattle.blogspot.com/2008/03/downer-cow-blues-senators-want.html


Thursday, March 6, 2008

House committee subpoenas Hallmark/Westland CEO - i call for an
investigation of the investigators


http://downercattle.blogspot.com/2008/03/house-committee-subpoenas.html

Friday, March 7, 2008

QUESTIONS AND ANSWERS HALLMARK/WESTLAND MEAT PACKING CO.

March 6, 2008

Consumer Concerns

Q. My child/school recently consumed Hallmark/Westland products. What is the
risk to children's health?


SEE FULL TEXT ;


http://downercattle.blogspot.com/2008/03/usda-questions-and-answers.html

March 16, 2008


MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or
Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html


IN FY 2007 TWO FIELD CASES, ONE VALIDATION CASE, AND TWO RSSS CASES WERE
CONSISTENT WITH NOR-98 SCRAPIE. ...


(BRINGS A TOTAL OF 5 NOR-98 CASES DOCUMENTED IN 2007 IN USA. ...TSS)


http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps

http://nor-98.blogspot.com/


http://scrapie-usa.blogspot.com/


CWD


http://chronic-wasting-disease.blogspot.com/


TME


http://transmissible-mink-encephalopathy.blogspot.com/


Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

Emmanuelle Uro-Coste1#, Hervé Cassard2#, Stéphanie Simon3, Séverine Lugan2,
Jean-Marc Bilheude4, Armand Perret-Liaudet5, James W. Ironside6, Stéphane
Haik7,8, Christelle Basset-Leobon1, Caroline Lacroux2, Katell Peoch'9,
Nathalie Streichenberger5, Jan Langeveld10, Mark W. Head6, Jacques Grassi3,
Jean-Jacques Hauw8, Francois Schelcher2, Marie Bernadette Delisle1, Olivier
Andréoletti2*

1 INSERM U858, Institut de Médecine Moléculaire de Rangueil and Service
d'Anatomie Pathologique et Histologie-Cytologie, C.H.U. Rangueil, Toulouse,
France2 UMR Institut National de la Recherche Agronomique (INRA)/Ecole
Nationale Vétérinaire de Toulouse (ENVT) 1225, Interactions Hôtes Agents
Pathogènes, ENVT, Toulouse, France3 Commissariat à l'Energie Atomique (CEA),
Service de Pharmacologie et d'Immunologie, DRM, CEA/Saclay, Gif sur Yvette,
France4 Bio-Rad, Research and Development Department, Marnes-la-Coquette,
France5 Hôpital Neurologique, Services de Neurochimie et de Pathologie,
Bron, France6 National Creutzfeldt-Jakob Disease Surveillance Unit, Division
of Pathology, University of Edinburgh, Western General Hospital, Edinburgh,
United Kingdom7 INSERM, Equipe Avenir, Maladies à Prions chez l'Homme,
Paris, France8 Neuropathology Laboratory, Salpêtrière Hospital, AP-HP,
Paris, France9 Service de Biochimie et Biologie Moléculaire, Hôpital
Lariboisière, Paris (Laboratoire associé au CNR “ATNC”) et EA 3621 Faculté
de Pharmacie, Paris, France10 Central Institute for Animal Disease Control
CIDC-Lelystad, Lelystad, The Netherlands

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified
according to the methionine/valine polymorphism at codon 129 of the PRNP
gene and the proteinase K (PK) digested abnormal prion protein (PrPres)
identified on Western blotting (type 1 or type 2). These biochemically
distinct PrPres types have been considered to represent potential distinct
prion strains. However, since cases of CJD show co-occurrence of type 1 and
type 2 PrPres in the brain, the basis of this classification system and its
relationship to agent strain are under discussion. Different brain areas
from 41 sCJD and 12 iatrogenic CJD (iCJD) cases were investigated, using
Western blotting for PrPres and two other biochemical assays reflecting the
behaviour of the disease-associated form of the prion protein (PrPSc) under
variable PK digestion conditions. In 30% of cases, both type 1 and type 2
PrPres were identified. Despite this, the other two biochemical assays found
that PrPSc from an individual patient demonstrated uniform biochemical
properties. Moreover, in sCJD, four distinct biochemical PrPSc subgroups
were identified that correlated with the current sCJD clinico-pathological
classification. In iCJD, four similar biochemical clusters were observed,
but these did not correlate to any particular PRNP 129 polymorphism or
western blot PrPres pattern. The identification of four different PrPSc
biochemical subgroups in sCJD and iCJD, irrespective of the PRNP
polymorphism at codon 129 and the PrPres isoform provides an alternative
biochemical definition of PrPSc diversity and new insight in the perception
of Human TSE agents variability.


snip...


Prion Strains and PrPSc Phenotype
Although prion strains can only be identified definitively by bioassay,
molecular in vitro tools to characterize PrPSc are more and more widely used
for the rapid identification of particular agents, such as BSE in cattle,
sheep, rodent and humans (vCJD) [20],[21]. This has come to be termed
“molecular strain typing” and although widely employed, the exact
relationship between PrPSc biochemistry and the biological properties of the
agents responsible remain to be determined. In sCJD, the presence of four
distinct PrPSc biochemical forms apparently correlated to
clinico-pathological phenotypes as defined by Parchi et al. [2] could be an
indication of the involvement of different TSE agents.

iCJD cases are a consequence of accidental human to human TSE transmission,
most likely representing transmission of sCJD. The identification in iCJD
cases of the four PrPSc signatures identified in sCJD is consistent with the
existence of distinct prions associated with these biochemical forms.

Three examples of human-to-human transmission of variant CJD through blood
transfusion have now been identified. While all blood donors were MM at
codon 129 PRNP, the recipients had either a MM (n = 2) or a MV genotype (n =
1). Despite this genotype difference there appears to have been conservation
of the disease phenotype and PrPres type in all “secondary” vCJD cases
[22]–[25]. These observations could suggest that in case of inter-human
transmission, difference in donor/recipient genotype could result in
un-altered abnormal PrP signature.

Our identification of MM GH iCJD cases harbouring similar PrPSc signature as
a VV1 sCJD case or of a VV dura mater iCJD case similar to MM2 sCJD might
indicate preservation of a specific PrPSc biochemical signature after human
to human transmission between individuals of different codon 129 genotypes.

Treatment with extracts of GH contaminated by CJD has lead to a high number
of iCJD cases in France and the UK. The codon 129 genotypes of the affected
individuals in the two countries differ, with the French cohort
predominantly MM and MV and the British cohort MV and VV [26]. In the
absence of any clear explanation for this finding, it was suggested that it
might be due to contamination of different batches of GH with different
prion strains from individuals of differing PRNP codon 129 genotypes. Our
identification of different biochemical forms of PrPSc in GH French patients
and in UK patients is consistent with this hypothesis. The variability
observed within the French GH cases could signify involvement of different
prion strains, consistent with multiple contaminated GH batches in the
French epidemic.

Conclusion
The identification in this study of different PrPSc species in CJD patients
with the same PRNP polymorphism at codon 129 and WB PrPres profile offers a
new perspective on our understanding of the relationship between PrP
biochemistry, prion disease phenotype and agent strain. We highlight two
novel approaches to analysing PrPSc in sCJD and iCJD and offer evidence that
these analyses provide potentially-strain associated information, which
appears to be lacking from the conventional WB assay.


snip...


SEE FULL TEXT ;


http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000029


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Since this article does not have an abstract, we have provided the first 150
words of the full text and any section headings.


To the Editor:

In their Research Letter, Dr Gibbons and colleagues1 reported that the
annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable
since 1985. These estimates, however, are based only on reported cases, and
do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number
of persons with a diagnosis of Alzheimer disease in fact may have CJD,
although only a small number of these patients receive the postmortem
examination necessary to make this diagnosis. Furthermore, only a few states
have made CJD reportable. Human and animal transmissible spongiform
encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr
Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL
TEXT


http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

should be drastic measures to safeguard the medical and surgical arena

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

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


TSS




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