Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

National Prion Disease Pathology Surveillance Center Cases Examined1 (July 31, 2010)


Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD


1996 & earlier 51 33 28 5 0 0


1997 114 68 59 9 0 0


1998 88 52 44 7 1 0


1999 120 72 64 8 0 0


2000 146 103 89 14 0 0


2001 209 119 109 10 0 0


2002 248 149 125 22 2 0


2003 274 176 137 39 0 0


2004 325 186 164 21 0 1(3)


2005 344 194 157 36 1 0


2006 383 197 166 29 0 2(4)


2007 377 214 187 27 0 0


2008 394 231 204 25 0 0


2009 425 259 216 43 0 0


2010 204 124 85 20 0 0


TOTAL 3702(5) 2177(6) 1834 315 4 3



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 16 cases in which the diagnosis is pending, and 18 inconclusive cases;


6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.


http://www.cjdsurveillance.com/pdf/case-table.pdf



14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

see page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf



I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete.

Thanks for your interest.''

Best regards,

Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

P.4.23

Transmission of atypical BSE in humanized mouse models

Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA

Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.

Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.

Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.

The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.

Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice.

BSE-H is also transmissible in our humanized Tg mice.

The possibility of more than two atypical BSE strains will be discussed.

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf



Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



SEE FULL TEXT ;

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html



Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more Prionbaloney ?


http://prionunitusaupdate.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html



2010

Original Article

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein


>>> Because 8 out of 10 patients had a positive family history of dementia in the original study, a genetic cause was suspected. Although all cases were homozygous for valine at codon 129 of the PrP gene, NO mutations were detected. <<<


http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html



IN CONFIDENCE AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.


snip...


http://collections.europarchive.org/tna/20080102185948/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf



and ;


In Confidence Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells


3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. BSE was not reported in the USA.


http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf



Monday, October 19, 2009


Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009


http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



Sunday, September 6, 2009


MAD COW USA 1997 [SECRET VIDEO]


http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html


U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? [SEE VIDEO at bottom]


http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN [SEE VIDEO]

video has now been removed from internet...Nov. 2014...tss

http://maddeer.org/video/embedded/prusinerclip.html


here is some of the text ;


-------- Original Message --------

Subject: US SENATOR AND STAN THE MAN SLAM USDA ''DAMNING TESTIMONY''

Date: Wed, 3 Mar 2004 15:15:24 –0600

From: "Terry S. Singeltary Sr." flounder@wt.net

Reply-To: Bovine Spongiform Encephalopathy BSE-L@uni-karlsruhe.de

To: BSE-L@uni-karlsruhe.de

######## Bovine Spongiform Encephalopathy #########

Greetings List members,

damning testimony below. be sure to _first_ open up real player competely, then paste your url in there. this worked best for me.........TSS

US SENATOR AND STAN THE MAN SLAM USDA ''DAMNING TESTIMONY''

Senator Michael Machado from California

''USDA does not know what's going on''.

''USDA is protecting the industry''.

''SHOULD the state of California step in''

Stanley Prusiner

''nobody has ever ask us to comment''

''they don't want us to comment''

''they never ask''

i tried to see Venemon, after Candian cow was discovered with BSE. went to see lyle. after talking with him...

absolute ignorance...

then thought I should see Venemon...

it was clear his entire policy was to get cattle bonless beef prods across the border...

nothing else mattered...

his aids confirmed this...

5 times i tried to see Venemon, never worked...

eventually met with carl rove the political...

he is the one that arranged meeting with Venemon...

just trying to give you a sense of the distance...

healh public safety...

was never contacted...

yes i believe that prions are bad to eat and you can die from them...

END

PLEASE NOTE THESE VIDEOS HAVE BEEN REMOVED FROM THE INTERNET $$$

Dr. Stan bashing Ann Veneman - 3 minutes

http://maddeer.org/video/embedded/08snip.ram

Recall Authority and Mad Cow Disease: Is the Current System Good for Californians?

Tuesday, February 24, 2004

JOINT HEARING

AGRICULTURE AND WATER RESOURCES HEALTH AND HUMAN SERVICES AND SELECT COMMITTEE ON GOVERNMENT OVERSIGHT - MACHADO, ORTIZ, and SPEIER, Chairs

Choose a RealPlayer video --->

Selected excerpts:

Opening Statement by Senator Michael Machado

http://maddeer.org/video/embedded/machado.html

Elisa Odibashian - Consumers Union

http://maddeer.org/video/embedded/odibashian.html

Anthony Iton - Alemeda County Health

http://maddeer.org/video/embedded/iton.html

USDA's "memorandum of understanding"

http://maddeer.org/video/embedded/usda.html

Dave Louthan - Killed the Mad Cow

http://maddeer.org/video/embedded/louthan.html

Dennis Laycraft - Canadian Cattlemen's Association

http://maddeer.org/video/embedded/laycraft.html

Stanley Prusiner - Discoverer of Prions

http://maddeer.org/video/embedded/prusiner.html

Steven DeArmond - Professor of Neuropathology

http://maddeer.org/video/embedded/dearmond.html

Entire 5 hour hearing - The California Channel

(scroll down to "022404 Senate Info-Hearing")

http://www.calchannel.com/february2004.htm


PLEASE NOTE THIS HEARING IN CALIFORNIA, THE VIDEOS HAVE BEEN REMOVED FROM THE INTERNET IN BOTH THE OFFICIAL, AND UNOFFICIAL WEBSITES I.E. MADDEER.ORG AND CALIFORNIA SENATE SITE WHERE THEY ONCE WERE...I HAVE REQUESTED VIDEO FROM CALIFORNIA OFFICIALS, AND THEY STILL REFUSE TO GIVE THEM UP TO ME. wish jay maddeer.org (or somebody) would send me a copy...



Sunday, November 13, 2011

*** California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock

http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/california-bse-mad-cow-beef-recall-qfc.html

Tuesday, November 04, 2014

The pathological and molecular but not clinical phenotypes are maintained after second passage of experimental atypical bovine spongiform encephalopathy in cattle

 
Tuesday, November 04, 2014

Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011

http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html


Sunday, April 12, 2009


r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada


http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-problem-dont.html


Thursday, June 24, 2010


Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues Volume 16, Number 7–July 2010



http://bse-atypical.blogspot.com/2010/06/accumulation-of-l-type-bovine-prions-in.html



Saturday, June 12, 2010


PUBLICATION REQUEST AND FOIA REQUEST

Project Number: 3625-32000-086-05 Study of Atypical Bse


http://bse-atypical.blogspot.com/2010/06/publication-request-and-foia-request.html



Archive Number 20100405.1091 Published Date 05-APR-2010 Subject PRO/AH/EDR> Prion disease update 1010 (04)


snip...

[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"


http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101



> Up until about 6 years ago, the pt worked at Tyson foods where she


> worked on the assembly line, slaughtering cattle and preparing them for


> packaging. She was exposed to brain and spinal cord matter when she


> would euthanize the cattle.



http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8




CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html



Monday, April 5, 2010

UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://prionunitusaupdate.blogspot.com/2010/04/update-cjd-texas-38-year-old-female.html


Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010


http://cjdtexas.blogspot.com/2010/06/usa-cases-of-dpcjd-rising-with-24-cases.html



Sunday, July 11, 2010

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s


http://creutzfeldt-jakob-disease.blogspot.com/2010/07/cjd-2-cases-mclennan-county-texas.html



Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review


http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html



Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary

Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.


http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009


http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate.blogspot.com/2010/01/human-prion-diseases-in-united-states.html



my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd



HOW many of you recieved a written CJD Questionnaire asking real questions pertaining to route and source (and there are many here in North America) ?

IS every case getting a cjd questionnaire asking real questions ???

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION USA PRION UNIT


http://cjdquestionnaire.blogspot.com/




BSE MAD COW FIREWALL IN THE USA, THE MAD COW FEED BAN, WHAT BAN ?


10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm


Tuesday, March 2, 2010

Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA

http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html


Monday, March 1, 2010

ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010

http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html


Terry S. Singeltary Sr. (Submitted question): Monday, April 5, 2010

Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010

http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html


Thursday, March 19, 2009

MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA WITH ONGOING 12 YEARS OF DENIAL

http://madcowfeed.blogspot.com/2009/03/millions-and-millions-of-pounds-of-mad.html




TSS

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