cjdvoice 500+ strong, and cjd foundation forms partnership with R-CALF in this news release, without a word to cjd voice members ???
----- Original Message ----- From: TERRY SINGELTARY To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:CJD-L@LISTS.AEGEE.ORG ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:cjdvoice@yahoogroups.com ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:BLOODCJD@YAHOOGROUPS.COM ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:MADcow@lists.iatp.org Sent: Saturday, April 11, 2009 10:49 AM Subject: CJD FOUNDATION SIDES WITH R-CALFERS NO BSE OR HUMAN TSE THERE OF IN USA 'don't be fooled'
Greetings CJD Voice,
PLEASE be aware, R-CALFERS don't believe the USA has mad cow disease. R-CALFERS believe that the only problem in North America is in Canada, and that it's all Canada's fault. R-CALFERS ALSO believe that no human TSE in the USA is caused by eating beef. R-CALFERS only believe in the UKBSEnvCJD only theory. so in my humble opinion, they kinda mirror the CJD Foundations beliefs. dont' be fooled. fool me once, shame on you, fool me twice, shame on me. oh, it looks pretty, but please, i urge everyone here, do not be fooled. 569 members of CJDVOICE and only a few in a click communicates amongst themselves with the cjd foundation (the new one, not cele's old cjd foundation). why was this not posted on the cjd foundation web site (i may have missed it, but could not find it on homepage)? are they not proud that they have now sided with a cattle group that refuses to acknowledge the problem with mad cow disease in the USA, or a human TSE there of ? or maybe this is why it took over a decade or more to get a cjd questionnaire that would ask real questions pertaining to route and source of agent, as opposed to just how and by whom it was diagnosed?
i do not now, and will not ever support a partnership with R-CALF about any TSE, until they stand up and look at themselves in a mirror and admit that the USA is in the same boat as Canada, and that the USA also has a mad cow problem. the sham that took place in 2004, the so called extensive USA BSE surveillance program, was just that, a sham, and proved to be just that. the feed ban was just as much a sham, and proven to be so.
do not be fooled cjdvoice, please do not be fooled. ...terry
The CJD Foundation has issued this information in partnership with R-CALF (This is not a News Release)
Friday, April 10, 2009 FDA Food Ban
The CJD Foundation sent out an email to its members yesterday regarding a comment period on proposed delays to the feed ban set to go into effect later this month. I've pasted the email below in its entirety. Please help out by sending the attached letter in, or writing a personal one of your own. And thanks to the CJD Foundation for getting the word out about this!
The CJD Foundation has issued this information in partnership with R-CALF (This is not a News Release)
To: The CJD Foundation Family Members and Friends and R-CALF USA Members and Affiliates
From: Florence Kranitz, President, the CJD Foundation and Bill Bullard, R-CALF USA CEO
snip...end... SEE FULL TEXT CJD FOUNDATION PARTNERSHIP WITH R-CALF EMAIL AT BOTTOM
Fri Apr 10, 2009 2:58 pm
http://ranchers.net/forum/viewtopic.php?t=34018
Fri Apr 10, 2009 2:58 pm
old Bullard really cracks me up. he is either as corrupt as the day is long, or just plain stupid. Bullard and r-calf et al, should stop worrying about Canada, and worry about their own back yard. stop covering up mad cow disease and using the SSS policy. if they would have taken care of business back in 1997, we would not be discussing this. Until a BSE Inquiry is addressed here in the USA against the USDA/FDA et al, and a true enhanced BSE Surveillance and testing program be put forth, we still will never no how many mad cows the USA really have $$$ the last enhanced BSE surveillance program was put forth, even the top prion Scientist said it was terribly flawed, and still is.
i said it a long time ago, ALL CATTLE FOR HUMAN AND ANIMAL CONSUMPTION SHOULD BE TESTED FOR 5 YEARS IN A ROW, before we know to a true extent, just what kind of problem we have. and all the rules and regulations on the mad cow feed ban will not work, unless they are strongly enforced, with severe ramifications when the rules are broke. stupidity is not acceptable anymore. ...
terry
4/10/2009 10:11:00 AM
R-CALF: Producers, Consumers Urged To Do What’s Right To Protect Against BSE
Washington, D.C. – In a highly unusual move, the U.S. Food and Drug Administration (FDA) on Thursday published in the Federal Register a last-minute notice regarding the agency’s intent to postpone its April 27, 2009, implementation of the final rule (published one year ago) that would enhance the U.S. feed ban to better protect against the spread of bovine spongiform encephalopathy (BSE, or mad cow disease). The FDA is providing the public with only seven days – through April 16, 2009 – to submit comments on whether the enhanced feed ban should be delayed for 60 days.
The U.S. Department of Agriculture (USDA) increased the risk of BSE in the United States by allowing into the U.S. millions of Canadian cattle, particularly Canadian cattle over 30 months (OTM) of age, which are of higher-risk for the disease. USDA states that the prevalence of BSE in the Canadian cattle herd is between three cases per million to eight cases per million cattle. The U.S. Centers for Disease Control and Prevention (CDC) states that the level of BSE prevalence in the Canadian cattle herd is up to 48-fold higher than the prevalence estimated in the U.S. cattle herd.
Just in 2008, nearly 1.6 million Canadian cattle, including OTM cattle, were imported into the United States. A recent USDA risk assessment model that incorporated a BSE prevalence rate for Canada of fewer than four cases per million head of cattle predicted the U.S. would import more than 100 BSE-infected cattle from Canada over the next 20 years.
The current U.S. feed ban, implemented in 1997, is actually weaker than Canada’s initial feed ban because it does not ban the feeding of plate waste and poultry litter to cattle. Canada’s feed ban has proved ineffective in controlling the spread of BSE in Canadian cattle, and after Canada began detecting multiple cases of BSE in animals born years after the feed ban, it relented to the repeated urging of international scientists and then enhanced its feed ban. Canada’s enhanced feed ban, implemented in July 2007, now protects Canadian consumers against the spread of BSE from Canadian cattle by closing known transmission routes, including cross-contamination and inadvertent feeding of contaminated cattle parts. The FDA now plans to delay providing U.S. consumers with the same level of protection afforded Canadian consumers against these same Canadian cattle that are now being imported into the United States.
“This last-minute proposal to postpone the new FDA feed ban, needed to minimize the heighted BSE risk from Canadian cattle, is designed to position the U.S. cattle industry between a rock and a hard place, and we hope that U.S. producers and U.S. consumers will see through this manipulative tactic and force USDA and the FDA to do what’s right,” said R-CALF USA CEO Bill Bullard.
The fact is that USDA has purposely exposed U.S. consumers and the U.S. cattle herd to an unacceptable risk of BSE by allowing Canada’s OTM cattle to freely enter the U.S. food supply, feed supply and cattle herd. USDA should not have allowed these higher-risk cattle into the U.S. until after it determined whether it was feasible to assume the additional costs necessary to mitigate this increased risk – the cost of upgrading the FDA feed ban.
“Like the original Canadian feed ban, the current U.S. feed ban is insufficient to address the heightened BSE risk in Canadian cattle,” Bullard emphasized. “Either USDA must immediately eliminate the source of this heightened BSE risk by prohibiting the importation of OTM Canadian cattle, or FDA must immediately implement the 2008 BSE final rule to mitigate this heightened risk. There are no responsible alternatives.”
R-CALF USA encourages U.S. consumers and U.S. cattle producers to submit comments on the FDA’s proposal before midnight Eastern, April 16, 2009. For information on how to submit a public comment and to see a sample comment letter, go to www.r-calfusa.com and click on “Food Safety.”
http://www.cattlenetwork.com/Content.asp?ContentID=305985
BSE MRR TSS, R-CALF ON CANADA VS USA
Bill Rancher
Joined: 10 Feb 2005 Posts: 1418 Location: GWN Posted: Fri Jan 05, 2007 9:49 am Post subject:
Texan wrote:
Hey Terry, I'd like to get a little further clarification on something if/when you have time. I'm not sure if I'm reading you correctly....
flounder wrote:
This is what sank my battleship in regards to testifying for r-calf. they actually appoached me about it, but i told them i would be glad to testify, but i was not stopping at the Canadian border, my testimony was to come south as well if given the opportunity. and that ended that, but i did supply them with a load of data, for whatever that was worth.
I highlighted the parts that confuse me. This almost makes it seem as if R-CALF was asking you to testify for them, but changed their mind when they found out that you were going to tell the WHOLE truth, instead of just the truth as regards Canadian imports.
I thought that R-CALF was only interested in the WHOLE truth - not just the selected parts of the truth that fit their protectionist agenda? After reading your post, it makes a person wonder. Maybe I read it wrong...
Am I reading this correctly, Terry? That can't be right, can it? Thanks.
I was wondering exactly the same thing Texan.
_________________
Canadian Beef....A cut above the rest!
my answer to big muddy from canada ;
hello there Texan,
yep, you read it right. don't know what ya'll gonna do without me. you know i plan on retiring from this mess soon. the pay is simply too excessive ;-( i fed them all i had at the time, and they shot the teacher. then hired old stanley i heard, go figure, must have been all those PhDs i had ;-)
as with the fuji-tv, when they came here and interviewed me for a BSE show, that i don't know what happened too, or the madcowboy documentary i was asked to proofread, and did, assured i would get some credit for, to never hear from again, to the speech in south Korea i was to make Nov. 23, but was shipwrecked somehow there too, and that might have been a good thing considering all the riots, and they did get the information anyway, to the TSS documentary, that too fell apart for good reasons i suppose, to helping creekstone, and finally to the NIH attempted destruction of an historical bank of donated tissue from CJD victims, and that one i think i did manage to stop, and that thanks to a Republican John Cornyn, i simply think it's time to let you fellars and gals clear this mess up. i have wasted enough time. it will be a decade next Christmas. i just would hate to keep kicking the same old mad cow. i know what happened for the most part, and the ones that don't get it now, never will.
now there Texan, as far as your question, and confusion ;-) i bet you thought i was not going to answer it, or, maybe hoping i would ;
flounder wrote:
This is what sank my battleship in regards to testifying for r-calf. they actually appoached me about it, but i told them i would be glad to testify, but i was not stopping at the Canadian border, my testimony was to come south as well if given the opportunity. and that ended that, but i did supply them with a load of data, for whatever that was worth.
I highlighted the parts that confuse me. This almost makes it seem as if R-CALF was asking you to testify for them, but changed their mind when they found out that you were going to tell the WHOLE truth, instead of just the truth as regards Canadian imports.
I thought that R-CALF was only interested in the WHOLE truth - not just the selected parts of the truth that fit their protectionist agenda? After reading your post, it makes a person wonder. Maybe I read it wrong...
Am I reading this correctly, Terry? That can't be right, can it? Thanks.
=========================================================
hello again there Texan,
i don't guess it matters anymore, i don't think ill be testifying for anyone, unless it is my own execution.
i was willing to participate in good faith, and sound science, that is why i think i was never sent to testify,
because in my opinion, R-Calf only wanted to cherry-pick the science, to use to there advantage, to try and
claim that Canada had a worse BSE problem than the USA, and i could not conceed to that. the science did
not confirm this. all one has to do is read the BSE GBR risk assessments, and that is why GW/OIE et al revised
there own risk assessments ;-) the BSE MRR policy.
i don't know, maybe i misinterpreted it all, maybe not, you can be the judge ;
oh what tangled webs we weave, when all we do is practice to deceive. ...TSS
SNIP...END... SEE FULL TEXT ;
*** http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=12
http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=24
http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=36
http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=48
THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ...
Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ...
Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm
O.I.E. .......... ??? GOD HELP US!
sample survey via oie for bse is about 400 test via 100 million cattle, if i am not mistaken. MOST countries that went
by these OIE guidelines all eventually went down with BSE. ...TSS
http://www.oie.int/downld/SC/2005/bse_2005.pdf
THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE.
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. ...
Page 95 of 98
8/3/2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3412&disposition=attachment&contentType=crtext
The CJD Foundation has issued this information in partnership with R-CALF (This is not a News Release)
Friday, April 10, 2009 FDA Food Ban
The CJD Foundation sent out an email to its members yesterday regarding a comment period on proposed delays to the feed ban set to go into effect later this month. I've pasted the email below in its entirety. Please help out by sending the attached letter in, or writing a personal one of your own. And thanks to the CJD Foundation for getting the word out about this!
The CJD Foundation has issued this information in partnership with R-CALF (This is not a News Release)
To: The CJD Foundation Family Members and Friends and R-CALF USA Members and Affiliates
From: Florence Kranitz, President, the CJD Foundation and Bill Bullard, R-CALF USA CEO
Date: April 9, 2009
Subject: FDA Comment Period on Feed Ban Starts Today – Ends April 16 Please consider commenting on FDA’s plan to delay the scheduled April 27, 2009, implementation of the BSE feed ban needed to address the heightened risk of BSE we already have assumed by allowing high-risk Canadian cattle into the United States. Below is a short background, instructions on how to submit comments, and a sample letter you can use to submit your comments. We need thousands of comments on this issue! Background: Our April 7, Member Alert provided more background on this issue but today the U.S. Food and Drug Administration (FDA) has opened a 7-day public comment period to solicit public comments on whether the FDA should delay implementation of its upgraded bovine spongiform encephalopathy (BSE) feed ban. The U.S. Department of Agriculture (USDA) and the FDA have purposely positioned consumers and the cattle industry between a rock and a hard place. There is no doubt that cattle producers who rely on rendering services to pick up deadstock will experience a hardship if the FDA’s new BSE feed ban rule takes effect. The government is banking on cattle producers to support this last minute delay for this very reason. But, the government has purposely backed our cattle industry into a corner and we should not quietly allow this kind of manipulation. The government, through the USDA, has exposed U.S. consumers and the U.S. cattle herd to an unacceptable risk of BSE by allowing Canada’s highest-risk cattle – cattle over 30 months (OTM) of age – to freely enter the U.S. food supply, feed supply, and cattle herd. USDA should not have allowed these higher-risk cattle into the U.S. until after it determined if it was feasible to assume the additional costs necessary to mitigate this increased risk – the cost of upgrading the U.S. feed ban. But this is exactly what has happened and this is what we are now faced with. We have to demand that our government do what is right by acting immediately to protect the health and safety of all U.S consumers and cattle herds. They must eliminate the source of the higher BSE risk by closing the border to OTM Canadian cattle and by immediately implementing the upgraded feed ban needed to minimize this higher risk. There are no responsible alternatives.
Below is a sample letter you can use to submit your comments to the FDA. We need thousands of comments submitted within the next 7 days, so please circulate this Alert as widely as possible and encourage as many people as possible to submit comments as well.
How to submit your written comments (Deadline April 16, 2009): 1. Go to http://www.regulations.gov/ 2. Type in FDA-2002-N-0031 3. Click on box that states: “Select to find documents accepting comments or submissions.” 4. Click on: Go>> 5. Click on the balloon shaped icon that states “Send a Comment or Submission.” 6. Follow the instructions on the “Public Comment and Submission Form.”
Sample Comment Letter for Your Use (You are free to use all or part of this letter):
April 9, 2009 Division of Dockets Management (HFA-305) Food and Drug Administration 5630 Fishers Lane, Rm. 1061Rockville, MD 20852
Re: Docket Number: FDA-2002-N-0031 (formerly Docket No. 2002N-0273)
Dear Administrator,
As the family member of a loved one who has died of a Creutzfeldt-Jakob Disease (CJD), one form of which can be acquired by ingesting BSE contaminated beef, I want to express my outrage at the recent announcement of the U.S. Food and Drug Administration’s (FDA) delay of its April 17, 2009 scheduled implementation of the final rule titled “Substances Prohibited from Use in Animal Food or Feed,” commonly referred to as the 2008 BSE final rule. They have made this announcement with full knowledge that the U.S. Department of Agriculture (USDA) continues to subject U.S. consumers and the U.S. cattle herd to a heighted risk of bovine spongiform encephalopathy (BSE) from imports of live Canadian cattle, particularly imports of Canadian cattle over 30 months (OTM) of age.
In Canada the disease occurrence is between three cases per million to eight cases per million cattle. The U.S. Centers for Disease Control and Prevention (CDC) states the level of BSE prevalence in the Canadian cattle herd is 18-fold to 48-fold higher than the prevalence estimated in the U.S. cattle herd. Just in 2008, nearly 1.6 million Canadian cattle were imported into the United States. By delaying the implementation of the Feed Ban the FDA risks yet another break in an already severely compromised food safety firewall.
When USDA reopened the U.S. border in 2007 to Canada’s highest-risk cattle population – OTM cattle – its risk modeling based on a Canadian BSE prevalence of fewer than 4 cases per million predicted that the U.S. would import over 100 head of BSE-infected cattle from Canada over the next 20 years. In addition, the risk modeling showed that human exposure to BSE would increase. However, as the CDC explained, the BSE prevalence in Canada could well be 8 cases per million, meaning that USDA likely has grossly underestimated the risk of introducing BSE-infected cattle into the U.S. as a result of allowing OTM Canadian cattle imports. Canada already has detected 16 native cases of BSE in its OTM cattle herd, 10 of which were born after the 1997 feed ban. The most recent of these cases was detected just last November. Nine of Canada’s BSE-infected cattle met USDA’s age requirements to be exported to the United States, as they were born after March 1, 1999, the date after which USDA erroneously claims BSE-infectivity was no longer circulating in Canada.
The current U.S. feed ban implemented in 1997 is comparable to the initial Canadian feed ban also implemented in 1997. Canada’s feed ban proved ineffective at preventing the spread of BSE in Canada. Despite the repeated urging of international scientists, Canada resisted any upgrades to its feed ban until after it detected multiple BSE cases in cattle born years after its 1997 feed ban. Canada’s July 2007 upgraded feed ban now protects Canadian consumers against the spread of BSE from Canadian cattle by closing known transmission routes, including cross-contamination and inadvertent feeding of contaminated cattle parts. It is unthinkable that the FDA would not afford U.S. consumers the same level of protection against these same Canadian cattle that are imported into the United States.
The FDA cannot legitimately argue that its current feed ban implemented in 1997, which is nearly identical to Canada’s original feed ban also implemented in 1997, is any more effective at mitigating Canada’s heightened BSE risk within U.S. borders than it was in mitigating Canada’s heightened BSE risk in Canada. Nor can FDA ignore the scientific evidence that overwhelmingly shows that the current U.S. feed ban is insufficient to mitigate the heightened BSE risk associated with OTM cattle imported from Canada. These higher-risk OTM Canadian cattle are entering the U.S. at the rate of several thousand per week, are being commingled in the U.S. cattle herd where some would be expected to die, and are entering both the U.S. food system as well as the U.S. animal feed system. The U.S. already is accepting Canada’s higher BSE risk without the protections necessary to mitigate that higher risk.
The FDA cannot bury its head in the sand and pretend the upgraded feed ban contained in the 2008 BSE final rule is not urgently needed to mitigate the increased BSE risk associated with the importation of millions of Canadian cattle. In fact, the FDA already has failed to timely implement an upgraded feed ban, which should have been implemented before USDA began to expose U.S. consumers and the U.S. cattle herd to Canada’s heightened BSE risk.
The FDA has an absolute responsibility to protect the health and safety of U.S. consumers and the U.S. cattle herd against this foreign animal disease which is always 100% fatal, and has been known to cross the species barrier infecting humans with variant Creutzfeldt-Jakob Disease (vCJD), the human form of BSE. We need only look to the United Kingdom’s recent tragic experience when it was discovered that BSE had crossed the species barrier to humans. Thus far this preventable disease has caused the deaths of 168 young adults. The long incubation period (which can be up to 40 years), means that tragically, there could be many more cases in the future. The FDA must break away from the manipulative actions by corporate-controlled, self-serving trade associations that have caused both FDA and USDA to endanger the health and safety of U.S. consumers and the U.S. cattle herd by exposing them to an unnecessary and avoidable risk of BSE.
The USDA must immediately eliminate the source of this heightened BSE risk by prohibiting the importation of OTM Canadian cattle, and the FDA must immediately implement the 2008 BSE final rule to mitigate this heightened risk. There are no responsible alternatives.
http://cjdadvocacy.blogspot.com/2009/04/fda-food-ban.html
http://www.cjdfoundation.org/
Saturday, January 24, 2009
Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock
2008 Annual Report
http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html
Thursday, April 9, 2009
Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed
http://madcowfeed.blogspot.com/2009/04/docket-no-fda2002n0031-formerly-docket.html
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
see full text ;
http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
snip...
*** NOTE ***
please include venison/sheep/lamb and the bovine to any of the above questions.
example=brain tanning deer/elk hide or any other topics that pertain to transmission of TSEs
_________________________________________________
example=antler velvet nutritional supplements
_________________________________________________
_any_ nutritional supplements??? name/ingredients
_________________________________________________
example=elk/deer brains ie/scrambled, sandwich or otherwise
_________________________________________________
snip...
http://cjdquestionnaire.blogspot.com/
CJDF Questionnaire Update The CJD Foundation’s questionnaire efforts continue to be successful. Since September 2008, we have received 305 completed questionnaires! All of the collected information has been entered into our database and is currently being analyzed by our epidemiologist consultant, Steven Korzeniewski. He is busy preparing an overview of the data which he will be presenting at the 2009 Conference in July. Our questionnaire is used to obtain an overview of case histories. We are looking for possible trends or similarities in patient backgrounds. The questionnaire offers each family, who is willing to share their story, a safe and meaningful way to do so. At the present time, we are the only repository for anecdotal CJD patient information in the United States. If you have not yet filled out a questionnaire and are willing to do so, please contact us at 1-800-659-1991 or mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000416/!x-usc:mailto:help@cjdfoundation.org. Thank you to all of the families who have taken the time to fill out a questionnaire, your information is extremely valuable and greatly appreciated!
http://www.cjdfoundation.org/content/newsletters/March2009.pdf
Thursday, March 19, 2009 Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)
http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html
rare atypical strain of sporadic cjd ??? seems these rare strains are increasing ???
Wednesday, February 04, 2009
Creutzfeldt-Jacob disease presenting as severe depression: a case report
http://creutzfeldt-jakob-disease.blogspot.com/2009/02/creutzfeldt-jacob-disease-presenting-as.html
A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004
http://creutzfeldt-jakob-disease.blogspot.com/2009/02/case-control-study-of-sporadic.html
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
snip...
DOES ANYONE BESIDES ME SEE A PATTERN YET ???
Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.
SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.
Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.
snip...
Sporadic creutzfeldt-jakob disease in two adolescents
http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1
see full text sporadic CJD the big lie;
snip...
IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???
lets look at the full circle, to date ;
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
Sunday, August 10, 2008 A New Prionopathy OR more of the same old BSe and sporadic CJD
http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
snip...
Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
snip...
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html
Elsevier Editorial System(tm) for The Lancet Infectious Diseases 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. snip...
see full text 31 pages ;
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
snip...
i am reminded of a few things deep throat told me years ago;
=================================================2001
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people......... Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie
Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!
As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
================================================
greetings again voice,
then i remind everyone to read this;
'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.'
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
DO NOT BE FOOLED !
http://cjdquestionnaire.blogspot.com/
Saturday, April 04, 2009 An unusually presenting case of sCJD-The VV1 subtype
http://creutzfeldt-jakob-disease.blogspot.com/2009/04/unusually-presenting-case-of-scjdthe.html
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
Saturday, April 11, 2009
Sunday, April 20, 2008
Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
Dear Member:
Once again we are writing to thank you for your continued support in enhancing surveillance of prion diseases in the United States and to bring you up to date on the National Prion Disease Pathology Surveillance Center (NPDPSC).
In large part because of your support, the number of cases examined by biopsy, autopsy and 14-3-3 protein determination has increased significantly over the years (see Tables 1 and 2). We are now able to establish a definitive diagnosis of prion disease in an estimated 60–70% of the cases in the United States, a percentage which exceeds that in even some major surveillance centers. In addition, we receive from you cerebrospinal fluid (CSF) for 14-3-3 determination, a surrogate protein which is helpful in the diagnosis of prion disease, probably in most if not all cases of suspected Creutzfeldt-Jakob disease (CJD). We are making constant efforts to reach our goal of at least 80% definitively diagnosed cases.
The major obstacle to our further increasing the autopsy rate remains the inadequate reporting of suspected cases of CJD to the NPDPSC or to the State Health Department, which in turn would notify us. Since you are the one likely to request the 14-3-3 test on these cases, please include in your request the information needed to contact you, which we will do if the test proves positive. If your institution uses a referral laboratory to send us the CSF, please provide your name, phone, and fax numbers to the lab, which will in turn submit it to us along with the sample. If this information is missing in the request accompanying the CSF sample (as it happens in about 30% of the cases), we will be unable to contact the caregiving physician. Having your contact information would also allow us to send results directly to you, thus reducing turnaround times.
If you suspect CJD in a case, but do not plan to request the 14-3-3 test, please nevertheless notify us of this suspected case; because we try to contact the caregiving physician in all cases of suspected prion disease in order to offer support in discussions with the families regarding autopsy, whenever such discussions are deemed appropriate. According to our data, families refuse autopsy in fewer than 10% of cases. In fact, they generally wish to have the autopsy carried out but may be unaware that it is free of charge. Therefore, if you notify us of every case of suspected prion disease, we believe that our 80% goal can be achieved.
Most of you may be aware that the usefulness of the 14-3-3 test is limited because of the large number of tests whose results are ambiguous. Accordingly, we are working to supplement or replace the 14-3-3 test with the CSF tau protein test, which will eliminate or very much reduce the number of ambiguous test results while increasing the overall accuracy of diagnosis.
The importance to public health in the U.S. of timely diagnosis and monitoring of human prion diseases is unquestionable. Here are some compelling reasons for this:
Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S. There has been increased occurrence and recognition in the U.S. of chronic wasting disease (CWD), an endemic prion disease affecting elk and deer, as well as uncertainties concerning CWD's transmissibility to humans. Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD. These facts make it very clear that careful monitoring of human prion diseases is crucial, because currently it is the only means of rigorous prion surveillance which can promptly detect human exposure to BSE and CWD in the U.S. should it occur. The mission of the NPDPSC is precisely to facilitate and expedite this surveillance.
However, in order to accomplish its mission, the NPDPSC must examine as many cases as possible by analysis of frozen and fixed tissue, which is the only way to accurately identify and classify prion diseases. This requires that an autopsy be performed. Our free-of-charge autopsy coordination service provides assistance in arranging and paying for autopsies, body transportation, and shipment of brain tissues. Results are reported to the senders (see Table 3 for turnaround times). It is also important that when requested, the patient's clinical history be sent to the NPDPSC along with autopsy and biopsy tissues so that each case can be better diagnosed and reported to CDC.
Again, we thank you very much for your continued help in reporting to our Center all cases of possible prion disease. If you have any concerns or questions, please call the NPDPSC at 216-368-0587 or e-mail at cjdsurv@case.edu. The website is www.cjdsurveillance.com.
Sincerely, Pierluigi Gambetti, MD Director, National Prion Disease Pathology Surveillance Center
Stephen M. Sergay, MB, BCh President, American Academy of Neurology
Table 1 Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD 1996 or earlier 42 32 25 4 0 0 1997 115 68 56 9 0 0 1998 93 53 45 7 1 0 1999 114 69 61 8 0 0 2000 151 103 89 14 0 0 2001 209 117 108 9 0 0 2002 258 146 119 22 2 0 2003 274 176 132 41 0 0 2004 335 184 155 21 0 13 2005 349 194 147 39 1 0 2006 385 193 151 31 0 14 2007 357 200 144 21 0 0 Totals 26825 15356 1232 226 4 2
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 acquired in Saudi Arabia; 5 Includes 11 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 26 cases with type determination pending (2 from 2006, 24 from 2007), and 45 cases with adequate tissue to establish the diagnosis of prion disease, but not the type; in all these cases, vCJD could be excluded.
Table 2
Table 3 Test on biopsy tissue Turnaround times Western Blot (WB) of the prion protein (PrP). (Establishes presence and type of scrapie PrP; frozen tissue required) 3–5 business days Histology and PrP immunohistochemistry (IHC). (Establish presence and distribution of scrapie PrP on fixed tissue) 4 business days 14-3-3 determination in CSF 5 business days Test on autopsy tissue Turnaround times WB of PrP 14 business days Histology and IHC of PrP 21 business days PrP gene sequencing. (Identifies mutations and codon 129 genotype needed for precise prion disease type diagnosis) 4–8 weeks (biopsies and autopsies) Summary report with prion disease type diagnosis based on all above tests 6–10 weeks (biopsies and autopsies)
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
P03.135] CSF Findings in a Large United States Sporadic CJD Cohort
Michael Geschwind, Aissa Haman, Charles Torres-Chae, Benjamin J. Raudabaugh, Gillian Devereux, Bruce Miller, San Francisco, CA
OBJECTIVE: Determine the CSF profile and the diagnostic sensitivity and specificity of various CSF proteins in a US sCJD cohort (n=196). BACKGROUND: The diagnostic utility of various biomarkers for CJD is controversial. We examined the sensitivity and specificity for various CSF proteins in 800 potential prion cases referred to our center over the past five years. DESIGN/METHODS: Medical records were reviewed and/or patients were evaluated at our center. Data was stored in a secure clinical relational database that was queried for CSF findings, including cell count, protein, IgG index, oligoclonal bands (OCBs), 14-3-3, neuron specific enolase (NSE), Total Tau (T-Tau) in patients with probable or definite sporadic CJD and non-prion rapidly progressive dementias (RPD), most of whom were referred as suspected CJD cases. T-Tau positive if >1300 pg/ml; NSE positive if >35 ng/ml. Probable sCJD diagnosis was based on modifications to WHO 1998 criteria, allowing other focal cortical signs (e.g., aphasia, apraxia, neglect) AND a positive MRI or EEG (14-3-3 not used for diagnosis). RESULTS: 14-3-3 protein (n=131) sensitivity was only 50% (47% for definite; 52% for probable sCJD). NSE (n=34) sensitivity was 59% (65% for definite; 50% for probable sCJD). T-Tau (n=18) was the most sensitive at 72% (78% for definite; 67% for probable sCJD). The specificity of these biomarkers among our CJD and RPD controls (n=68) was 70% for 14-3-3 (n=33), 80% for NSE (n=15), and 100% for T-Tau (n=4). The 14-3-3 had the lowest sensitivity and specificity. CONCLUSIONS/RELEVANCE: Our data is contrary to other published data suggesting high sensitivity and specificity of these proteins for sCJD. DWI MRI has better sensitivity and specificity and should be used in diagnostic criteria. We question the utility of these CSF proteins for CJD diagnosis. A prion-specific test is needed. Supported by: John Douglas French Foundation for Alzheimers Research, the McBean Foundation, NIA K23AG021989-01, NIH-NINDS contract N01-NS-0-2328. Category - Aging and Dementia SubCategory - Clinical
Tuesday, May 1, 2007 4:00 PM
Poster Sessions: HIV and Prion Diseases (4:00 PM-7:30 PM)
The embargo for all abstracts to be presented at the 59th Annual Meeting is in effect until the date and time of the presentation unless otherwise noted on the abstract and/or press release. If there are questions, please contact the AAN media and public relations team.
===============================
http://www.abstracts2view.com/aan2007boston/sessionindex.php?day=2007-05-01&session=PO02-L
Debate Continues as New Studies Support MRI, Not CSF Markers, to Diagnose CJD
Neurology Today 5 June 2007; Volume 7(11); pp 6,10-11 Hurley, Dan
Outline article in brief study protocols experts comment mri as a diagnostic tool references Links View Article PDF
ARTICLE IN BRIEF
1. Two new studies aim to improve diagnosis of Creutzfeldt-Jakob Disease (CJD): one found a low sensitivity and specificity for CSF biomarkers for sporadic CJD; another study found MRI an effective diagnostic tool in a small number of patients with familial CJD.
BOSTON-A new, larger study by a San Francisco neurologist who previously challenged the use of the 14-3-3 protein in CSF for diagnosing sporadic Creutzfeldt-Jakob Disease (CJD) has again found a disappointingly low sensitivity and specificity for the biomarker.
Although the specificity and sensitivity of two other biomarkers, neuron specific enolase (NSE) and Total Tau (T-Tau), proved better than those of 14-3-3, neither were good enough to make them useful in diagnosing CJD, concluded Michael D. Geschwind, MD, PhD, assistant professor of neurology at the Memory and Aging Center of the University of California-San Francisco (UCSF). The findings were presented in a poster session here at the AAN annual meeting in May.
MRI appears to be a more reliable diagnostic tool, Dr. Geschwind said, and that view was supported by another poster at the meeting reporting high sensitivity and specificity for familial CJD with diffusion tensor imaging (DTI).
STUDY PROTOCOLS
Dr. Geschwind and colleagues examined the sensitivity and specificity for CSF in potential prion cases referred to the UCSF center in the past five years. Among 142 patients tested for 14-3-3, the sensitivity was only 49 percent overall - 47 percent for those with a diagnosis of sporadic CJD, and 50 percent for those with probable CJD.
Among the 40 tested for NSE, sensitivity was 63 percent (64 percent for definite CJD; 60 percent for probable CJD). Among the 20 tested for T-Tau, sensitivity was 70 percent (67 percent for definite CJD; 75 percent for probable CJD).
The specificity of the three biomarkers was 66 percent among the 44 controls tested for 14-3-3, 83 percent among the 18 controls tested for NSE, and 100 percent for the four controls tested for T-Tau.
Our data are contrary to other published results suggesting high sensitivity and specificity of these proteins for sporadic CJD, according to Dr. Geschwind and colleagues. We question the utility of these CSF proteins for CJD diagnosis.
EXPERTS COMMENT
But two neurologists who have led other studies with more positive findings said their views remain unchanged - that the biomarkers are a useful, though imperfect, tool for diagnosing CJD.
The data have limitations but also strengths, said Allen J. Aksamit Jr., MD, associate professor of neurology at the Mayo Clinic College of Medicine in Rochester, MN. If you have a select patient group who fit the clinical criteria for CJD and exclude every other possible diagnosis, it's a fairly sensitive and specific test. You use it as a weight to put on one side for or against the diagnosis.
In 2003, Dr. Aksamit espoused a similar view in an Archives of Neurology editorial (60:803-804) accompanying Dr. Geschwind's first paper challenging the clinical value of the 14-3-3 protein for diagnosis of sporadic CJD. In that study, Dr. Geschwind reviewed 32 pathologically confirmed cases on which 14-3-3 testing had been performed; only 17 cases had a positive result, a sensitivity of only 53 percent (Arch Neurol 2003;60:813-816).
Figure. Dr. Allen J. Aksamit Jr.: The data have limitations but also strengths. If you have a select patient group who fit the clinical criteria for CJD and exclude every other possible diagnosis, it's a fairly sensitive and specific test. You use it as a weight to put on one side for or against the diagnosis.
[ Click here to enlarge ]
The largest data set published so far on biomarkers in CJD patients comes from a European consortium led by Inga Zerr, MD, a neurologist at Georg-August University in Götingen, Germany. She and colleagues reported last year on 1,859 patients with sporadic, genetic, iatrogenic, and variant CJD and 1,179 controls, finding a sensitivity of 85 percent for 14-3-3, 86 percent for T-Tau, and 93 percent when the results of both tests were combined with S100b and NSE (Neurology 2006;67:637-643).
I am aware that the sensitivity of the tests in Dr. Geschwind's laboratory is lower than we reported and I have discussed this with him on several occasions, Dr. Zerr said in an e-mail. I have no idea why the data are so different.
One possible explanation, she and Dr. Aksamit suggested, is that at UCSF - a referral institution where many patients go for a second opinion - Dr. Geschwind is seeing more patients with slowly progressing disease than in the European group. Dr. Zerr's data showed, as have previous studies, that the sensitivity of the biomarkers is highest in patients with the shortest disease duration.
Another possible reason the two studies have reached such different conclusions is that Dr. Zerr included patients with genetic, iatrogenic, and variant CJD, whereas Dr. Geschwind's group includes only sporadic cases. Different case definitions and different testing methods might also contribute to the different findings.
Figure. Dr. Isak Prohovnik: MRI does seem to have better sensitivity and specificity than the biomarkers. But neither our group nor his [Dr. Geschwind's] have sufficient numbers yet.
[ Click here to enlarge ]
In an interview with Neurology Today, Dr. Geschwind said of the European group's findings, I think they may have a selection bias. Every time they publish a study, their sensitivity goes lower and lower. Eventually it will hit ours.
He added in an e-mail, I believe there are occasional cases in which these biomarkers, such as the 14-3-3, total-tau or NSE, may be helpful in diagnosing CJD. The problem is that because these CSF proteins clearly can be elevated in other diseases that clinically mimic CJD, one must be very thorough in ruling out other conditions. Some of these conditions include cancers, neurologic autoimmune (paraneoplastic and non-paraneoplastic) dementias, sarcoid, Hashimoto encephalopathy, and even atypically rapid presentations of other neurodegenerative diseases.
After speaking with Dr. Geschwind in front of his poster, Dr. Aksamit Jr. told Neurology Today: It's all in the selection of the patient population being reviewed, and how high you set the bar for these laboratory tests. If you set the bar high, you'll get higher specificity but lower sensitivity. Our recommendation at Mayo is to set the bar high, to enhance specificity at the expense of sensitivity.
Dr. Aksamit noted, however, that 14-3-3 is no longer being tested at Mayo, because the reagents for a quantitative version of the test, which the institution had preferred over the standard Western blot, are no longer available. Instead, Mayo is testing NSE.
Despite the questions raised by Dr. Geschwind's paper, Dr. Zerr said: I completely agree with Dr. Aksamit that biomarkers in CSF are useful, when tested in the right populations. This is what we always stress when we are talking with physicians: Use CSF in the right differential diagnosis; that is, in dementia.
MRI AS A DIAGNOSTIC TOOL
The one point on which all three neurologists agreed is that MRI seems to be a powerful diagnostic tool. Although MRI is not part of the clinical criteria for CJD yet, said Dr. Zerr, we strongly recommend that neurologists have the MRI done and consider it as an additional test.
Another poster presented at the meeting shared the results of DTI testing on a small group of patients with familial CJD. Isak Prohovnik, PhD, professor of psychiatry and radiology at Mount Sinai School of Medicine in New York City, reported that DTI was abnormal in the basal ganglia of ten of 11 patients, eight of 11 in different cortical areas, and six of 11 in the thalami. No involvement was seen in the brain stem or cerebellum.
When correlated with the clinical findings, DTI was positive for the frontal cortex in eight of nine patients, and for the motor cortex in seven of nine patients. In the basal ganglia, the DTI was positive in 10 patients, although clinical findings were present in only eight. Clinical cerebellar signs were seen in 11 patients and brain-stem signs in seven of the 11, although no positive findings showed up in those regions on either DTI or other MRI sequences.
MRI does seem to have better sensitivity and specificity than the biomarkers, Dr. Prohovnik said, noting that Dr. Geschwind has also used MRI. But neither our group nor his have sufficient numbers yet.
In 2005, Dr. Geschwind co-authored a paper on diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) in CJD, finding them to be 91 percent sensitive, 95 percent specific, and 94 percent accurate (Am J Neuroradiol 26:1551-1562). In 2005, Dr. Zerr was the senior author on a paper published in Brain (128:2026-2033), which concluded that FLAIR and DWI improved the clinical diagnosis and should be incorporated in the World Health Organization's diagnostic criteria for sporadic CJD.
REFERENCES
• Aksamit AJ. Cerebrospinal fluid 14-3-3 protein: Variability of sporadic Creutzfeldt-Jakob disease, laboratory standards, and quantitation.
• Geschwind MD, Martindale BS, Miller BL, et al. Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease.
• Sanchez-Juan P, Green A, Zerr I, et al. CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease.
• Young GS, Geschwind MD, Dillon WP, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: High sensitivity for diagnosis.
• Tschampa HJ, Kellenberg K, Zerr I, et al. MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: A study on inter-observer agreement.
http://www.aan.com/elibrary/neurologytoday/?event=home.showArticle&id=ovid.com%3a%2fbib%2fovftdb%2f00132985-200706050-00006
CJD USA
*Acquired in UK ** Acquired in Saudi Arabia *** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007. **** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was adequate to establish the presence but not the type; in all cases, vCJD could be excluded.
-- 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]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
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
CJD USA RISING
The statistical incidence of CJD cases in the United States has been revised to reflect that there is ONE CASE per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. 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
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003. Volume 3, Issue 8, August 2003, Page 463
"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
Vol. 285 No. 6, February 14, 2001 JAMA
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
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Sunday, 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
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
NEW LONG TERM CASE STUDY (HUGE) CJD IN KIDS USA
March 28, 2008, 12:10AM USDA CERTIFIED DEAD STOCK DOWNER COW SCHOOL LUNCH PROGRAM LIST OF SCHOOLS AFFECTED STATE BY STATE (dead stock downers i.e. non-ambulatory, the most high risk for mad cow disease)
http://downercattle.blogspot.com/2008/03/usda-certified-dead-stock-downer-cow.html
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE
http://downercattle.blogspot.com/
DOWNER COW BLUES SENATORS WANT CRACKDOWN
http://downercattle.blogspot.com/2008/03/downer-cow-blues-senators-want.html
FDA lists school districts that got recalled meat Lawmakers had demanded info be released (see schools listed state by state)
http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of BovineSpongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
TSS
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
Dear Member:
Once again we are writing to thank you for your continued support in enhancing surveillance of prion diseases in the United States and to bring you up to date on the National Prion Disease Pathology Surveillance Center (NPDPSC).
In large part because of your support, the number of cases examined by biopsy, autopsy and 14-3-3 protein determination has increased significantly over the years (see Tables 1 and 2). We are now able to establish a definitive diagnosis of prion disease in an estimated 60–70% of the cases in the United States, a percentage which exceeds that in even some major surveillance centers. In addition, we receive from you cerebrospinal fluid (CSF) for 14-3-3 determination, a surrogate protein which is helpful in the diagnosis of prion disease, probably in most if not all cases of suspected Creutzfeldt-Jakob disease (CJD). We are making constant efforts to reach our goal of at least 80% definitively diagnosed cases.
The major obstacle to our further increasing the autopsy rate remains the inadequate reporting of suspected cases of CJD to the NPDPSC or to the State Health Department, which in turn would notify us. Since you are the one likely to request the 14-3-3 test on these cases, please include in your request the information needed to contact you, which we will do if the test proves positive. If your institution uses a referral laboratory to send us the CSF, please provide your name, phone, and fax numbers to the lab, which will in turn submit it to us along with the sample. If this information is missing in the request accompanying the CSF sample (as it happens in about 30% of the cases), we will be unable to contact the caregiving physician. Having your contact information would also allow us to send results directly to you, thus reducing turnaround times.
If you suspect CJD in a case, but do not plan to request the 14-3-3 test, please nevertheless notify us of this suspected case; because we try to contact the caregiving physician in all cases of suspected prion disease in order to offer support in discussions with the families regarding autopsy, whenever such discussions are deemed appropriate. According to our data, families refuse autopsy in fewer than 10% of cases. In fact, they generally wish to have the autopsy carried out but may be unaware that it is free of charge. Therefore, if you notify us of every case of suspected prion disease, we believe that our 80% goal can be achieved.
Most of you may be aware that the usefulness of the 14-3-3 test is limited because of the large number of tests whose results are ambiguous. Accordingly, we are working to supplement or replace the 14-3-3 test with the CSF tau protein test, which will eliminate or very much reduce the number of ambiguous test results while increasing the overall accuracy of diagnosis.
The importance to public health in the U.S. of timely diagnosis and monitoring of human prion diseases is unquestionable. Here are some compelling reasons for this:
Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S. There has been increased occurrence and recognition in the U.S. of chronic wasting disease (CWD), an endemic prion disease affecting elk and deer, as well as uncertainties concerning CWD's transmissibility to humans. Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD. These facts make it very clear that careful monitoring of human prion diseases is crucial, because currently it is the only means of rigorous prion surveillance which can promptly detect human exposure to BSE and CWD in the U.S. should it occur. The mission of the NPDPSC is precisely to facilitate and expedite this surveillance.
However, in order to accomplish its mission, the NPDPSC must examine as many cases as possible by analysis of frozen and fixed tissue, which is the only way to accurately identify and classify prion diseases. This requires that an autopsy be performed. Our free-of-charge autopsy coordination service provides assistance in arranging and paying for autopsies, body transportation, and shipment of brain tissues. Results are reported to the senders (see Table 3 for turnaround times). It is also important that when requested, the patient's clinical history be sent to the NPDPSC along with autopsy and biopsy tissues so that each case can be better diagnosed and reported to CDC.
Again, we thank you very much for your continued help in reporting to our Center all cases of possible prion disease. If you have any concerns or questions, please call the NPDPSC at 216-368-0587 or e-mail at cjdsurv@case.edu. The website is www.cjdsurveillance.com.
Sincerely, Pierluigi Gambetti, MD Director, National Prion Disease Pathology Surveillance Center
Stephen M. Sergay, MB, BCh President, American Academy of Neurology
Table 1 Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD 1996 or earlier 42 32 25 4 0 0 1997 115 68 56 9 0 0 1998 93 53 45 7 1 0 1999 114 69 61 8 0 0 2000 151 103 89 14 0 0 2001 209 117 108 9 0 0 2002 258 146 119 22 2 0 2003 274 176 132 41 0 0 2004 335 184 155 21 0 13 2005 349 194 147 39 1 0 2006 385 193 151 31 0 14 2007 357 200 144 21 0 0 Totals 26825 15356 1232 226 4 2
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 acquired in Saudi Arabia; 5 Includes 11 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 26 cases with type determination pending (2 from 2006, 24 from 2007), and 45 cases with adequate tissue to establish the diagnosis of prion disease, but not the type; in all these cases, vCJD could be excluded.
Table 2
Table 3 Test on biopsy tissue Turnaround times Western Blot (WB) of the prion protein (PrP). (Establishes presence and type of scrapie PrP; frozen tissue required) 3–5 business days Histology and PrP immunohistochemistry (IHC). (Establish presence and distribution of scrapie PrP on fixed tissue) 4 business days 14-3-3 determination in CSF 5 business days Test on autopsy tissue Turnaround times WB of PrP 14 business days Histology and IHC of PrP 21 business days PrP gene sequencing. (Identifies mutations and codon 129 genotype needed for precise prion disease type diagnosis) 4–8 weeks (biopsies and autopsies) Summary report with prion disease type diagnosis based on all above tests 6–10 weeks (biopsies and autopsies)
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
P03.135] CSF Findings in a Large United States Sporadic CJD Cohort
Michael Geschwind, Aissa Haman, Charles Torres-Chae, Benjamin J. Raudabaugh, Gillian Devereux, Bruce Miller, San Francisco, CA
OBJECTIVE: Determine the CSF profile and the diagnostic sensitivity and specificity of various CSF proteins in a US sCJD cohort (n=196). BACKGROUND: The diagnostic utility of various biomarkers for CJD is controversial. We examined the sensitivity and specificity for various CSF proteins in 800 potential prion cases referred to our center over the past five years. DESIGN/METHODS: Medical records were reviewed and/or patients were evaluated at our center. Data was stored in a secure clinical relational database that was queried for CSF findings, including cell count, protein, IgG index, oligoclonal bands (OCBs), 14-3-3, neuron specific enolase (NSE), Total Tau (T-Tau) in patients with probable or definite sporadic CJD and non-prion rapidly progressive dementias (RPD), most of whom were referred as suspected CJD cases. T-Tau positive if >1300 pg/ml; NSE positive if >35 ng/ml. Probable sCJD diagnosis was based on modifications to WHO 1998 criteria, allowing other focal cortical signs (e.g., aphasia, apraxia, neglect) AND a positive MRI or EEG (14-3-3 not used for diagnosis). RESULTS: 14-3-3 protein (n=131) sensitivity was only 50% (47% for definite; 52% for probable sCJD). NSE (n=34) sensitivity was 59% (65% for definite; 50% for probable sCJD). T-Tau (n=18) was the most sensitive at 72% (78% for definite; 67% for probable sCJD). The specificity of these biomarkers among our CJD and RPD controls (n=68) was 70% for 14-3-3 (n=33), 80% for NSE (n=15), and 100% for T-Tau (n=4). The 14-3-3 had the lowest sensitivity and specificity. CONCLUSIONS/RELEVANCE: Our data is contrary to other published data suggesting high sensitivity and specificity of these proteins for sCJD. DWI MRI has better sensitivity and specificity and should be used in diagnostic criteria. We question the utility of these CSF proteins for CJD diagnosis. A prion-specific test is needed. Supported by: John Douglas French Foundation for Alzheimers Research, the McBean Foundation, NIA K23AG021989-01, NIH-NINDS contract N01-NS-0-2328. Category - Aging and Dementia SubCategory - Clinical
Tuesday, May 1, 2007 4:00 PM
Poster Sessions: HIV and Prion Diseases (4:00 PM-7:30 PM)
The embargo for all abstracts to be presented at the 59th Annual Meeting is in effect until the date and time of the presentation unless otherwise noted on the abstract and/or press release. If there are questions, please contact the AAN media and public relations team.
===============================
http://www.abstracts2view.com/aan2007boston/sessionindex.php?day=2007-05-01&session=PO02-L
Debate Continues as New Studies Support MRI, Not CSF Markers, to Diagnose CJD
Neurology Today 5 June 2007; Volume 7(11); pp 6,10-11 Hurley, Dan
Outline article in brief study protocols experts comment mri as a diagnostic tool references Links View Article PDF
ARTICLE IN BRIEF
1. Two new studies aim to improve diagnosis of Creutzfeldt-Jakob Disease (CJD): one found a low sensitivity and specificity for CSF biomarkers for sporadic CJD; another study found MRI an effective diagnostic tool in a small number of patients with familial CJD.
BOSTON-A new, larger study by a San Francisco neurologist who previously challenged the use of the 14-3-3 protein in CSF for diagnosing sporadic Creutzfeldt-Jakob Disease (CJD) has again found a disappointingly low sensitivity and specificity for the biomarker.
Although the specificity and sensitivity of two other biomarkers, neuron specific enolase (NSE) and Total Tau (T-Tau), proved better than those of 14-3-3, neither were good enough to make them useful in diagnosing CJD, concluded Michael D. Geschwind, MD, PhD, assistant professor of neurology at the Memory and Aging Center of the University of California-San Francisco (UCSF). The findings were presented in a poster session here at the AAN annual meeting in May.
MRI appears to be a more reliable diagnostic tool, Dr. Geschwind said, and that view was supported by another poster at the meeting reporting high sensitivity and specificity for familial CJD with diffusion tensor imaging (DTI).
STUDY PROTOCOLS
Dr. Geschwind and colleagues examined the sensitivity and specificity for CSF in potential prion cases referred to the UCSF center in the past five years. Among 142 patients tested for 14-3-3, the sensitivity was only 49 percent overall - 47 percent for those with a diagnosis of sporadic CJD, and 50 percent for those with probable CJD.
Among the 40 tested for NSE, sensitivity was 63 percent (64 percent for definite CJD; 60 percent for probable CJD). Among the 20 tested for T-Tau, sensitivity was 70 percent (67 percent for definite CJD; 75 percent for probable CJD).
The specificity of the three biomarkers was 66 percent among the 44 controls tested for 14-3-3, 83 percent among the 18 controls tested for NSE, and 100 percent for the four controls tested for T-Tau.
Our data are contrary to other published results suggesting high sensitivity and specificity of these proteins for sporadic CJD, according to Dr. Geschwind and colleagues. We question the utility of these CSF proteins for CJD diagnosis.
EXPERTS COMMENT
But two neurologists who have led other studies with more positive findings said their views remain unchanged - that the biomarkers are a useful, though imperfect, tool for diagnosing CJD.
The data have limitations but also strengths, said Allen J. Aksamit Jr., MD, associate professor of neurology at the Mayo Clinic College of Medicine in Rochester, MN. If you have a select patient group who fit the clinical criteria for CJD and exclude every other possible diagnosis, it's a fairly sensitive and specific test. You use it as a weight to put on one side for or against the diagnosis.
In 2003, Dr. Aksamit espoused a similar view in an Archives of Neurology editorial (60:803-804) accompanying Dr. Geschwind's first paper challenging the clinical value of the 14-3-3 protein for diagnosis of sporadic CJD. In that study, Dr. Geschwind reviewed 32 pathologically confirmed cases on which 14-3-3 testing had been performed; only 17 cases had a positive result, a sensitivity of only 53 percent (Arch Neurol 2003;60:813-816).
Figure. Dr. Allen J. Aksamit Jr.: The data have limitations but also strengths. If you have a select patient group who fit the clinical criteria for CJD and exclude every other possible diagnosis, it's a fairly sensitive and specific test. You use it as a weight to put on one side for or against the diagnosis.
[ Click here to enlarge ]
The largest data set published so far on biomarkers in CJD patients comes from a European consortium led by Inga Zerr, MD, a neurologist at Georg-August University in Götingen, Germany. She and colleagues reported last year on 1,859 patients with sporadic, genetic, iatrogenic, and variant CJD and 1,179 controls, finding a sensitivity of 85 percent for 14-3-3, 86 percent for T-Tau, and 93 percent when the results of both tests were combined with S100b and NSE (Neurology 2006;67:637-643).
I am aware that the sensitivity of the tests in Dr. Geschwind's laboratory is lower than we reported and I have discussed this with him on several occasions, Dr. Zerr said in an e-mail. I have no idea why the data are so different.
One possible explanation, she and Dr. Aksamit suggested, is that at UCSF - a referral institution where many patients go for a second opinion - Dr. Geschwind is seeing more patients with slowly progressing disease than in the European group. Dr. Zerr's data showed, as have previous studies, that the sensitivity of the biomarkers is highest in patients with the shortest disease duration.
Another possible reason the two studies have reached such different conclusions is that Dr. Zerr included patients with genetic, iatrogenic, and variant CJD, whereas Dr. Geschwind's group includes only sporadic cases. Different case definitions and different testing methods might also contribute to the different findings.
Figure. Dr. Isak Prohovnik: MRI does seem to have better sensitivity and specificity than the biomarkers. But neither our group nor his [Dr. Geschwind's] have sufficient numbers yet.
[ Click here to enlarge ]
In an interview with Neurology Today, Dr. Geschwind said of the European group's findings, I think they may have a selection bias. Every time they publish a study, their sensitivity goes lower and lower. Eventually it will hit ours.
He added in an e-mail, I believe there are occasional cases in which these biomarkers, such as the 14-3-3, total-tau or NSE, may be helpful in diagnosing CJD. The problem is that because these CSF proteins clearly can be elevated in other diseases that clinically mimic CJD, one must be very thorough in ruling out other conditions. Some of these conditions include cancers, neurologic autoimmune (paraneoplastic and non-paraneoplastic) dementias, sarcoid, Hashimoto encephalopathy, and even atypically rapid presentations of other neurodegenerative diseases.
After speaking with Dr. Geschwind in front of his poster, Dr. Aksamit Jr. told Neurology Today: It's all in the selection of the patient population being reviewed, and how high you set the bar for these laboratory tests. If you set the bar high, you'll get higher specificity but lower sensitivity. Our recommendation at Mayo is to set the bar high, to enhance specificity at the expense of sensitivity.
Dr. Aksamit noted, however, that 14-3-3 is no longer being tested at Mayo, because the reagents for a quantitative version of the test, which the institution had preferred over the standard Western blot, are no longer available. Instead, Mayo is testing NSE.
Despite the questions raised by Dr. Geschwind's paper, Dr. Zerr said: I completely agree with Dr. Aksamit that biomarkers in CSF are useful, when tested in the right populations. This is what we always stress when we are talking with physicians: Use CSF in the right differential diagnosis; that is, in dementia.
MRI AS A DIAGNOSTIC TOOL
The one point on which all three neurologists agreed is that MRI seems to be a powerful diagnostic tool. Although MRI is not part of the clinical criteria for CJD yet, said Dr. Zerr, we strongly recommend that neurologists have the MRI done and consider it as an additional test.
Another poster presented at the meeting shared the results of DTI testing on a small group of patients with familial CJD. Isak Prohovnik, PhD, professor of psychiatry and radiology at Mount Sinai School of Medicine in New York City, reported that DTI was abnormal in the basal ganglia of ten of 11 patients, eight of 11 in different cortical areas, and six of 11 in the thalami. No involvement was seen in the brain stem or cerebellum.
When correlated with the clinical findings, DTI was positive for the frontal cortex in eight of nine patients, and for the motor cortex in seven of nine patients. In the basal ganglia, the DTI was positive in 10 patients, although clinical findings were present in only eight. Clinical cerebellar signs were seen in 11 patients and brain-stem signs in seven of the 11, although no positive findings showed up in those regions on either DTI or other MRI sequences.
MRI does seem to have better sensitivity and specificity than the biomarkers, Dr. Prohovnik said, noting that Dr. Geschwind has also used MRI. But neither our group nor his have sufficient numbers yet.
In 2005, Dr. Geschwind co-authored a paper on diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) in CJD, finding them to be 91 percent sensitive, 95 percent specific, and 94 percent accurate (Am J Neuroradiol 26:1551-1562). In 2005, Dr. Zerr was the senior author on a paper published in Brain (128:2026-2033), which concluded that FLAIR and DWI improved the clinical diagnosis and should be incorporated in the World Health Organization's diagnostic criteria for sporadic CJD.
REFERENCES
• Aksamit AJ. Cerebrospinal fluid 14-3-3 protein: Variability of sporadic Creutzfeldt-Jakob disease, laboratory standards, and quantitation.
• Geschwind MD, Martindale BS, Miller BL, et al. Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease.
• Sanchez-Juan P, Green A, Zerr I, et al. CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease.
• Young GS, Geschwind MD, Dillon WP, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: High sensitivity for diagnosis.
• Tschampa HJ, Kellenberg K, Zerr I, et al. MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: A study on inter-observer agreement.
http://www.aan.com/elibrary/neurologytoday/?event=home.showArticle&id=ovid.com%3a%2fbib%2fovftdb%2f00132985-200706050-00006
CJD USA
*Acquired in UK ** Acquired in Saudi Arabia *** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007. **** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was adequate to establish the presence but not the type; in all cases, vCJD could be excluded.
-- 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]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
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
CJD USA RISING
The statistical incidence of CJD cases in the United States has been revised to reflect that there is ONE CASE per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. 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
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003. Volume 3, Issue 8, August 2003, Page 463
"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
Vol. 285 No. 6, February 14, 2001 JAMA
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
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Sunday, 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
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
NEW LONG TERM CASE STUDY (HUGE) CJD IN KIDS USA
March 28, 2008, 12:10AM USDA CERTIFIED DEAD STOCK DOWNER COW SCHOOL LUNCH PROGRAM LIST OF SCHOOLS AFFECTED STATE BY STATE (dead stock downers i.e. non-ambulatory, the most high risk for mad cow disease)
http://downercattle.blogspot.com/2008/03/usda-certified-dead-stock-downer-cow.html
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE
http://downercattle.blogspot.com/
DOWNER COW BLUES SENATORS WANT CRACKDOWN
http://downercattle.blogspot.com/2008/03/downer-cow-blues-senators-want.html
FDA lists school districts that got recalled meat Lawmakers had demanded info be released (see schools listed state by state)
http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of BovineSpongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
TSS
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