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

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

ALL is well in the USA, no mad cows, and no human mad cow there from. it's all the Canadians fault, the U.K.'s fault, and or Saudi Arabia's fault, even though Saudi Arabia has never documented mad cow disease in their cows. and if you ask the USDA, they too will tell you it was all Canada's fault, Saudi Arabia's and or the UK's, and or a spontaneous event of genetic differences, but no mad cows and or human mad cow there from. RIGHT $$$

I propose that this third case of nvCJD documented in the USA has as much, or even more of a chance of being an indigenous home grown nvCJD case in the USA, as that of being exposed the short time this person was in Saudi Arabia, a country that has never had a case of mad cow disease documented. THE USA import of highly infectious materials from documented BSE countries, including the U.K. was phenomenal, let alone it's own TME exposed MBM from right here in the USA. Strange there is no case history of this supposedly Saudi Arabia source case and history of time in the USA compared to time in Saudi Arabia, food habits, and such? It would be nice to compare and try and figure up incubation time periods with this third case, age, symptoms, beginning clinical signs to death, etc, a good case study in other words, where is it ???

let's look at a few factors ;

Incubation period: The incubation period in vCJD is difficult to establish, but is estimated to be around 10 years. The incubation period in transfusion transmitted vCJD has been between 6.5 and 8 years.


http://ecdc.europa.eu/en/healthtopics/Documents/2018_ECDC_Variant_Creutzfeldt_FACTSHEET.pdf


The exact incubation period for foodborne vCJD is unknown. However, a range of possible incubation periods was estimated for 4 vCJD patients who likely acquired the disease during their residence in the United Kingdom and for 5 vCJD patients reported as part of a cluster in Leicestershire, England (Table). The median of the estimated range of incubation periods for these 9 vCJD patients was 13 years. ...

In June 2005 the US Department of Agriculture confirmed BSE in an = 12-year-old cow born and raised in Texas. This is the first time an indigenous BSE case was detected in the United States. A previous BSE-positive cow identified in Washinton State was imported from Canada. ...

http://www.mhlw.go.jp/shingi/2006/01/dl/s0126-10c45.pdf


ACTUALLY, another highly suspect mad cow was detected in Texas, and was covered-up. nobody likes to speak of this mad cow too much ;

Statement on Texas cow with central nervous system symptoms

Date: 05 May 2004 - 0:00 PDT

The Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

Source: FDA http://www.fda.gov/bbs/topics/news/2004/NEW01061.html


Actually, it's been one blunder after another blunder with the BSE surveillance and BSE feed ban of August 4, 1997. you can it documented here ;

http://madcowusda.blogspot.com/ and http://madcowtesting.blogspot.com/


ONE must consider, North America has documented c-BSE, atypical h-BSE, and atypical l-BSE (BASE). Also documented, two strains of CWD in deer and elk, i.e. typical CWD and the Wisconsin strain, to date. Also documented, many many typical strains of Scrapie in sheep and goats, with the atypical Nor-98 scrapie also being documented, to date. Also, two strains of TME in mink, the hyper and the drowsy TME. Who knows about feline and canine TSE, but over the years, decades, at some point, all of these species have been rendered and fed back to food producing animals for humans and animals. This is fact.

WE ALSO SEE atypical sporadic CJD showing up in young and old people in the USA, some of long duration. incubation and symptoms different in some cases, some pathology difference, SO WHY is it impossible to believe that a sub-type of the sporadic CJDs could be from consumption of these atypical animal TSE cases and or iCJD there from, and just not look like the typical UK c-BSE human TSE i.e. vCJD aka nvCJD ??? friendly fire i.e. pass it forward i.e. iatrogenic Creutzfeldt Jakob Disease there from, could play a bigger role in the transmission in consumption itself, and be simply hiding itself as a sporadic CJD. ...

CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

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


let's look at the three case reports of nvCJD in the USA, and lets see what the incubation periods were ;


Variant Creutzfeldt-Jakob Disease Death, United States: 1st Case Report


The only variant Creutzfeldt-Jakob disease (vCJD) patient identified in the United States died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. The patient likely acquired the disease while growing up in Great Britain before immigrating to the United States in 1992. Additional vCJD patients continue to be identified outside the United Kingdom, including 2 more patients in Ireland, and 1 patient each in Japan, Portugal, Saudi Arabia, Spain and the Netherlands. The reports of bloodborne transmission of vCJD in 2 patients, 1 of whom was heterozygous for methionine and valine at polymorphic codon 129, add to the uncertainty about the future of the vCJD outbreak.

snip...

see case history on 1st nvCJD case in USA here ;

http://www.medscape.com/viewarticle/512024_2


A 22-year-old Florida resident became the first person in the USA to be diagnosed with probable variant Creutzfeldt-Jakob disease (vCJD), according to the US Centers for Disease Control and Prevention (CDC). Because the young woman was raised in the UK when the BSE outbreak was at its peak, officials believe that she contracted the disease there. The case report, which is published in Morbidity and Mortality Weekly Report (2002; 51: 927—29;

http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(02)00474-7/fulltext


Probable Variant Creutzfeldt-Jakob Disease in a US Resident—Florida, 2002

JAMA. 2002;288:2965-2967.

MMWR. 2002;51:927-929

On April 18, 2002, the Florida Department of Health and CDC announced the occurrence of a likely case of variant Creutzfeldt-Jakob disease (vCJD) in a Florida resident aged 22 years. This report documents the investigation of this case and underscores the importance of physicians increasing their suspicion for vCJD in patients presenting with clinical features described in this report who have spent time in areas in which bovine spongiform encephalopathy (BSE) is endemic.

In early November 2001, the patient sought medical care for depression and memory loss that adversely affected the patient's work performance. The primary-care physician referred the patient to a psychologist. In early December 2001, the patient received a traffic ticket for failing to yield the right of way. In mid-December 2001, the patient had involuntary muscular movements, gait changes, difficulty dressing, and incontinence. In January 2002, the patient was evaluated in a local emergency department for these symptoms. A computerized tomography scan of the head revealed no abnormalities; a panic attack was diagnosed, and the patient was treated with an anti-anxiety medication.

In late January 2002, the patient's mother, a resident of the United Kingdom, took the patient to England, where medical evaluations were conducted during the next 3 months. During this period, the patient's memory loss and other neurologic symptoms worsened. The patient experienced falls with minor injuries, had difficulty taking a shower and dressing, and was unable to remember a home telephone number or to make accurate mathematical calculations. The patient subsequently became confused, hallucinated, and had speech abnormalities with lack of content, bradykinesia, and spasticity. The patient was referred to a neurologist, who suspected vCJD and subsequently referred the patient to the National Prion Clinic in the United Kingdom.

Medical evaluations at the National Prion Clinic included an electroencephalogram (EEG), which revealed a normal alpharhythm, and magnetic resonance imaging (MRI) studies, which revealed signal abnormalities in the pulvinar and metathalamus region that were suggestive of vCJD. The patient had a tonsil biopsy, and a Western blot analysis of the biopsy tissue demonstrated the presence of protease-resistant prion protein (PrP-res) with the characteristic pattern of vCJD; an immunohistochemical test for PrP-res also supported a diagnosis of vCJD. Analysis of the prion protein gene detected no mutation and showed methionine homozygosity at codon 129, consistent with all 105 vCJD patients tested in the United Kingdom (R. Will, Western General Hospital, Edinburgh, Scotland, personal communication, 2002).

The patient received experimental treatment with quinacrine for 3 months. As of late September 2002, the patient had become bedridden, experienced considerable weight loss requiring surgical insertion of a feeding tube, and was no longer communicating with family members. On the basis of a case definition developed in the United Kingdom, the patient's illness met criteria for a probable case of vCJD.1

The patient was born in the United Kingdom in 1979 and moved to Florida in 1992. The patient never had donated or received blood, plasma, or organs and never had received human growth hormone. There was no family history of CJD. In October 2001, before the onset of the illness, the patient's wisdom teeth were extracted, but there was no history of major surgery.

Reported by:

S Wiersma, MD, State Epidemiologist, Florida Dept of Health. S Cooper, MRCP, R Knight, FRCP, National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, Scotland; AM Kennedy, MD, National Prion Clinic, Dept of Neurology, St. Mary's Hospital, London; S Joiner, MSc, Medical Research Council Prion Unit, Dept of Neurodegenerative Disease, Institute of Neurology, London, United Kingdom. E Belay, MD, LB Schonberger, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

CDC Editorial Note:

snip...full text ;


http://jama.ama-assn.org/cgi/content/full/288/23/2965


http://www2a.cdc.gov/han/ArchiveSys/ViewMsgV.asp?AlertNum=00085


http://www.cdc.gov/ncidod/EID/vol11no09/pdfs/05-0371.pdf


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1919



Variant Creutzfeldt-Jakob Disease Death, United States: 2nd Case Report


vCJD (Variant Creutzfeldt-Jakob Disease)

Update: Variant Creutzfeldt-Jakob Disease in a U.K. Citizen Who Had Temporarily Resided in Texas, 2001-2005

In November 2005, the U.K. National Creutzfeldt-Jakob Disease (CJD) Surveillance Unit in Edinburgh, Scotland notified the Centers for Disease Control and Prevention (CDC) about a probable variant CJD diagnosis in a 30-year-old man who resided in Texas during 2001-2005. The patient had onset of symptoms in early 2005 while in Texas. He then returned to the United Kingdom, where his illness progressed, and a diagnosis of variant CJD was made. This diagnosis was confirmed neuropathologically after the patient's death.

The variant CJD diagnosis was initially based on typical clinical manifestations of the disease and demonstration of the characteristic “pulvinar sign” on magnetic resonance imaging of the brain. No biopsy tissues are available for pathologic confirmation of the diagnosis. While living in the United States, the patient had no history of hospitalization, of having invasive medical procedures, or of donation or receipt of blood and blood products.

The patient almost certainly acquired the disease in the United Kingdom. He was born in the United Kingdom and lived there throughout the defined period of risk (1980-1996) for human exposure to the agent of bovine spongiform encephalopathy (BSE, commonly known as “mad cow” disease). His stay in the United States was too brief relative to what is known about the incubation period for variant CJD. For additional information about the incubation period for variant CJD, see Belay ED, Sejvar JJ, Shieh WJ, et al. “Variant Creutzfeldt-Jakob Disease Death, United States,” Emerg Infect Dis 2005; available at

http://www.cdc.gov/ncidod/EID/vol11no09/05-0371.htm.


By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. Since variant CJD was first reported in 1996, a total of 195 patients with this disease from 11 countries have been identified. As of August 11, 2006, variant CJD cases have been reported from the following countries: 162 from the United Kingdom, 20 from France, 4 from Ireland, 2 from the United States (including the current case), and one each from Canada, Italy, Japan, Netherlands, Portugal, Saudi Arabia, and Spain. Similar to the two U.S. cases, two of the four cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom.One of the 20 French cases may also have been infected in the United Kingdom. Strong scientific evidence indicates that variant CJD results from the transmission to humans of the agent that causes BSE in cattle. The BSE outbreak in cattle that was first detected in the 1980s in the United Kingdom has spread to many other European countries, and cases in cattle have been identified outside of Europe, in Canada, Israel, Japan, and the United States.

Date: August 14, 2006 Content source: National Center for Infectious Diseases

http://www.cdc.gov/ncidod/dvrd/vcjd/other/probablevcjd_texas2001_2005_111805.htm



Variant Creutzfeldt-Jakob Disease Death, United States: 3rd Case Report


NO DETAILED case report I could find on this very unusual and assumed case of nvCJD in the USA, but here is cdc short report ;

vCJD (Variant Creutzfeldt-Jakob Disease)

Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East

The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a vCJD case in a U.S. resident. This is the third vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The two previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scientific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).

Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.

The current case-patient has no history of receipt of blood, a past neurosurgical procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.

As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).

As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1).

1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354.

Date: November 29, 2006

http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm


The third US resident with vCJD was born and raised in Saudi Arabia and beginning in 2001 he occasionally stayed in the United States for periods of up to 3 months duration [30], [31]. The patient relocated to the United States in 2005 where onset of vCJD symptoms was experienced in the spring of 2006. The diagnosis of vCJD was confirmed based on pathological study of adenoid and brain biopsy tissues in November 2006. The patient died later in 2006. The patient had no past history of neurosurgical procedures or visits to European countries. A previous case of vCJD attributed to consumption of BSE-contaminated cattle products had been reported in a Saudi Arabian resident [13].


http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0008521?utm_source=feedburner&utm_medium=feed&utm_campaign=Feed%3A+plosone%2FPLoSONE+%28PLoS+ONE+Alerts%3A+New+Articles%29


re-Human Prion Diseases in the United States Posted by flounder on 01 Jan 2010 at 18:11 GMT

I kindly disagree with your synopsis for the following reasons ;


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


MORE BSe here from cdc about this 3rd case of vCJD in the USA ;


The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a case of variant Creutzfeldt-Jakob disease (vCJD) in a Virginia resident. There is no evidence to suggest that this case of vCJD was caused by anything the patient was exposed to while residing in the U.S. or that this situation represents a public health threat to any U.S. resident.

For more information on vCJD, visit CDC’s Web site at http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm.


Virginia CJD Epidemiology CJD is currently a reportable condition in Virginia only if the ill person is less than 55 years of age; therefore data on this condition are very limited.

snip...

One limitation to our understanding of CJD in Virginia and the US is that surveillance mainly uses death certificate data. However, it is very likely that some cases do not get recorded. CJD is an uncommon disease and physicians who are not familiar with the signs and symptoms may mistake it for other conditions. Evidence of the under-reporting of cases comes from some European countries, where enhanced surveillance has found higher than expected rates due to increased awareness and better diagnosis. 11 In addition, one small study reported that as many as 13% of patients diagnosed with Alzheimer’s disease were found upon autopsy to have actually had CJD.12 Therefore, it seems likely that the current data underestimate the true incidence of CJD in Virginia and in the US.

http://www.vdh.state.va.us/Epidemiology/Surveillance/Bulletin/pdf/vebjan05.pdf


Creutzfeldt-Jakob Disease (CJD) In 2006, a case of Creutzfeldt- Jakob Disease (CJD) in an individual less than 55 years of age was reported. Sporadic CJD occurs in Virginia; the age criteria is intended to screen for potential cases of new variant CJD (nvCJD). On further investigation, this individual was found to have new variant CJD. Epidemiologic investigation suggested that it was extremely unlikely that this individual acquired the infection in the U.S. However, this counted as only the third case of nvCJD ever diagnosed in the U.S. A high index of suspicion and early testing is important for diagnosing CJD, since arranging for brain biopsy or autopsy to confirm the diagnosis may be important for families and healthcare professionals.

http://www.vdh.virginia.gov/Epidemiology/Surveillance/Bulletin/pdf/vebjul07.pdf


From: TSS
Subject: Third case of vCJD reported in the United States
Date: December 7, 2006 at 11:08 am PST


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


Third case of vCJD reported in the United States Editorial Team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office

A clinical diagnosis of variant Creutzfeldt Jakob Disease (vCJD) was confirmed after brain biopsy investigations in a United States (US) resident and reported in November [1]. The patient is a young man who grew up in Saudi Arabia and lived in the US since late 2005. Before that he visited the US once in 1989 and several times after 2001. He has never visited any country in Europe or received a blood transfusion nor has he undergone any neurosurgical procedure. This vCJD case is the third in a US resident. The previous two patients both grew up in the United Kingdom (UK), and this is where they were believed to have been infected [2].

In Saudi Arabia, the first and only previous case of vCJD was reported in 2005. This was suspected to be related to consumption of meat contaminated with the prion agent which causes bovine spongiform encephalitis in cattle (BSE). The European Food Safety Authority (http://www.efsa.org) has not published a geographical BSE risk assessment for Saudi Arabia [3] and there have been no cases of BSE in cattle reported by Saudi Arabia to the World Organisation for Animal Health (http://www.oie.int). Although the UK is not the only potential beef exporter to have had a BSE epidemic, it remains plausible, subject to Saudi Arabia's import policy, that contaminated beef was inadvertently imported from the UK to Saudi Arabia in the period before 1996 (when the EU banned the export of UK beef and cattle).

Based on this patient's history, the occurrence of a previously reported case of vCJD in Saudi Arabia, and the expected length of the incubation period for food-related vCJD, the most likely source of infection is thought to be contaminated meat products the patient consumed as a child when living in Saudi Arabia. The patient has no known history of donating blood, and investigations have identified no risk of onwards transmission within the US.

Variant Creutzfeldt-Jakob disease was first identified in the United Kingdom in the mid-1990s. As of November 2006, worldwide there have been 200 vCJD cases: 164 patients in the United Kingdom, 21 in France, four in Ireland, three in the US (including the present case), two in the Netherlands and one each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain [4]. All patients, except 10 (including the present case) had lived either in the United Kingdom (170 cases) or in France (20 cases). Evidence so far indicates that the most probable source of infection in most cases was consumption of meat products contaminated with the prion agent causing BSE.

References: Centers for Disease Control and Prevention.

Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East.

(http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm)


Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354. European Food Safety Authority . Geographical BSE Risk (GBR) assessments covering 2000-2006. List of countries and their GBR level of risk as assessed by the Scientific Steering Committee and the (EFSA). 1 August 2006.

(http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/summary_list_countries.Par.0001.File.dat/GBR_assessments_table_Overview_assessed_countries_2002-2006.pdf)



The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf


Variant Creuzfeldt-Jakob disease. Current data – December 2006.

(http://www.cjd.ed.ac.uk/vcjdworld.htm)


http://www.eurosurveillance.org/ew/2006/061207.asp#2


>>>The patient is a young man who grew up in Saudi Arabia and lived in the US since late 2005. Before that he visited the US once in 1989 and several times after 2001. He has never visited any country in Europe or received a blood transfusion nor has he undergone any neurosurgical procedure.<<<


Heaven forbid anyone suggest that this unlucky Soul was contaminated in the USA from vCJD. This would just be preposterous, wouldn't it $$$ i am reminded of a few things deep throat told me years ago;


============================================================



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 a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork 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 a great 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.)


================================================================


12/07/06


HOWEVER, if you ONLY consider an OUTSIDE source of mbm from the UK, you will see that indeed the UK did dump a great deal of mad cow poison on the middle east, compared to the amount they dumped on USA. comparing from my records from the U.K., the USA imported about 44 tons of UK mbm or greaves, Canada got 83 tons in 3 years, 1993, 1994, and 1995. compared to about 6,985 tons exported from the UK to Saudi Arabia over a period of about 20 years, with Saudi importing mbm from UK as late as 1995. ISRAEL ALSO IMPORTED A GREAT DEAL OF THIS POISON, 30,006 TONS of MBM FROM UK. A great deal was also imported to Asian Countries as well. HOWEVER, we cannot state that this is indeed a case of vCJD exported to the USA from Saudi with certainty. now we all know that the USDA will paint this pig with lipstick and take it to the dance, to the prom and anywhere else they can take it, but the fact still remains, they cannot state this with scientific proof. IF you look at the USA and it's TSE problem, the rendering industry, and the fact that the USA shipped the technology of continous rendering to the UK, only to start using 5 years later, then look at the 100s, if not thousands of tons of potentially and most likely TSE tainted feed used in the USA for the last 2 decades, the likelyhood of this being a USA source of vCJD, in my opinion, is possible as well. In 2006 alone, the amount of ruminant protein still being fed to USA cattle is not only phenominal, but also very very disturbing. ...TSS



----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Monday, December 04, 2006 10:55 AM
Subject: Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East



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



Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East



The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a vCJD case in a U.S. resident. This is the third vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The two previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scientific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).



Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.



The current case-patient has no history of receipt of blood, a past neurosurgical procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.



As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).



As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1). 1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354. Date: November 29, 2006 Content source: National Center for Infectious Diseases




http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm


The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a case of variant Creutzfeldt-Jakob disease (vCJD) in a Virginia resident. There is no evidence to suggest that this case of vCJD was caused by anything the patient was exposed to while residing in the U.S. or that this situation represents a public health threat to any U.S. resident. For more information on vCJD, visit CDC’s Web site at

http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm.


http://www.vdh.virginia.gov/news/Alerts/vCJD.htm


TSS


#################### https://lists.aegee.org/bse-l.html ####################


http://cjdtexas.blogspot.com/



[There is inconsistency in the number of vCJD cases attributed to the United States. The Virginia Department of Health/CDC report cited in the above list 3 cases -- 2 cases probably contracted in the U.K. and the young adult described above who was born and raised in Saudi Arabia. The EUROCJD data for October 2006 in part (2) above also lists 2 U.S. cases, both assumed to have been contracted in the U.K. The U.S. National Prion Disease Pathology Surveillance Center Data published on 8 Nov 2006 (part 3 above), on the other hand, list only a single U.S. case believed to have been contracted in the U.K. - Mod.CP]



http://www.promedmail.org/pls/otn/f?p=2400:1001:19224::::F2400_P1001_BACK_PAGE,F2400_P1001_ARCHIVE_NUMBER,F2400_P1001_USE_ARCHIVE:1001,20061205.3431,Y



http://vcjd.blogspot.com/



Thursday, February 4, 2010

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009


http://seac992007.blogspot.com/2010/02/spongiform-encephalopathy-advisory.html


Monday, February 01, 2010


Import Alert 17-04 BSE CJD HIGH RISK TISSUES, Nutritional Supplements and Cosmetics


http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-17-04-bse-cjd-high-risk.html


Monday, February 01, 2010


Import Alert 57-20 and 84-03 Human Dura Mater and risk factors there from due to Creutzfeldt Jakob Disease (CJD)


http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-57-20-and-84-03-human-dura.html


Wednesday, February 3, 2010


Import Alert 62-07 Sygen Injectable (Bovine-Extracted GMI Monosialoganglioside) manufactured from bovine brain starting material


http://bseusa.blogspot.com/2010/02/import-alert-62-07-sygen-injectable.html


Wednesday, February 3, 2010


Import Alert 71-02 Detention Without Physical Examination Of Animal Feeds And Feed Ingredients That May Contain Ingredients Of Animal Origin Import Alert 71-02


http://madcowfeed.blogspot.com/2010/02/import-alert-71-02-detention-without.html


Wednesday, February 3, 2010


Import Alert 99-25 Detention Without Physical Examination of Animal Feed...BSE...and Not the Subject of a Valid USDA Import Permit Import Alert 99-25


http://madcowfeed.blogspot.com/2010/02/import-alert-99-25-detention-without.html


BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein


Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: j.collinge@prion.ucl.ac.ukReceived August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002.


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed



USA sporadic CJD cases rising ; 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 RISING



SWITZERLAND CJD is a predominantly sporadic disorder but can also occur as a dominantly inherited or infective condition. Only one of the 26 most recent confirmed cases was identified as carrying a disease related mutation of the PRNP gene, none had identifiable iatrogenic exposure, and none resembled variant CJD. Thus 25 of the 26 cases appear to be sporadic cases. Sporadic CJD is distributed worldwide with a reported incidence of about one in a million per year. Raised awareness of the disease in recent years could account for an increase in reported cases of CJD, although neither an increase in the average age of patients nor more frequent recognition of CJD amongst residents of nursing homes (where dementing illness is prevalent and misdiagnosis might be expected) were seen in the Swiss cases. Moreover, improved ascertainment as an explanation for the observed increase would imply levels of under-reporting in countries other than Switzerland, which appear implausible. The authors of the Lancet report suggest that the rise in cases might be due to some form of unidentified iatrogenic transmission or to exposure to a zoonotic source of infection, though cases do not resemble variant Creutzfeldt-Jakob disease (vCJD). The ongoing surveillance of CJD in Switzerland and the rest of Europe is essential to monitor the situation to see if this rise is sustained in Switzerland, and if a similar rise occurs in other countries (see http://www.eurocjd.ed.ac.uk/).


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1921



Prion data suggest BSE link to sporadic CJD Declan Butler Predicting the number of cases of Creutzfeldt–Jakob disease (CJD) in people as a result of transmission of bovine spongiform encephalopathy (BSE) has just got more difficult.Whereas it was thought that BSE only caused a new form of the disease called variant CJD (vCJD), a study in mice from a team led by John Collinge at University College London suggests that it may also cause a disease indistinguishable from the commonest form of classical, or 'sporadic', CJD (E.


http://www.nature.com/nature/journal/v420/n6915/full/420450a.html



IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ; However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52). IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76


http://www.eurocjd.ed.ac.uk/sporadic.htm


Switzerland sporadic CJD ; Swiss rise in CJD raises concerns over possible BSE link [LONDON] THE LANCET Plaque attack: Swiss patients have spongiform patterns in the brain typical of sporadic CJD. The number of people dying from Creutzfeldt-Jakob disease (CJD) has risen sharply in Switzerland -- sparking fears of a possible link with bovine spongiform encephalopathy (BSE). BSE is thought to be the cause of a distinctive form of the brain-wasting disease known as variant CJD. The Swiss cases, in contrast, are standard 'sporadic' CJD. Each year between 1997 and 2000, no more than 11 Swiss people developed CJD. But 19 cases were reported in 2001, and seven were recorded in the first quarter of this year. This is some four times higher than the incidence elsewhere, reports a team led by Adriano Aguzzi of the University Hospital Zurich (M. Glatzel et al. Lancet 360, 139-141; 2002). The increase could be a mere statistical blip, or it may be due to increased awareness of the disease leading to more diagnoses. More disturbing is the possibility that the cases are linked to the consumption of BSE-infected meat products -- which would mean that the BSE agent can cause two distinct forms of CJD. Possible links between the Swiss CJD cases and BSE will now be explored by strain-typing experiments in which the disease is transmitted to mice. These tests will take at least a year to complete. "It's the best way to establish or exclude any suspected link," says Moira Bruce of the UK Institute for Animal Health's Neuropathogenesis Unit in Edinburgh.


======================================


Experiences in England and Switzerland -- two countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since mad cow was first detected in British herds in 1986. Switzerland discovered last year its CJD rate was twice that of any other country in the world. Switzerland had been seeing about eight to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.


http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r


Mouse model sheds new light on human prion disease

snip...

Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.


snip...


http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm


Monday, May 19, 2008

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS


http://bseinquiry.blogspot.com/


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


Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl

Journal European Journal of Epidemiology Publisher Springer Netherlands ISSN 0393-2990 (Print) 1573-7284 (Online) Issue Volume 1, Number 1 / March, 1985 P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron (1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France


Abstract


A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron (1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology


http://www.springerlink.com/content/j344470112792q50/


http://www.springerlink.com/content/j344470112792q50/fulltext.pdf

?page=1 2.



Sporadic CJD normally occurs in people in their 50s and 60s although it can occur more rarely in younger age groups. Until this year the youngest case of sporadic CJD in the UK had been in a 34 year old. Other countries, howver, have reported sporadic CJD in teenagers. Those we know about are;

* in the USA, a 16 year old in 1978;

* in France, a 19 year old in 1982;

* in Canada, a 14 year old of UK origin in 1988;

* in Poland cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

* Creutzfeldt's first patient in 1920 was aged 23. full text ;


http://collections.europarchive.org/tna/20081106132604/http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.PDF


J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom * To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk. Accepted 15 April 2007


Abstract Background:


Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional. Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent. Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD. Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1


http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html


http://stanford.wellsphere.com/cjd-article/a-novel-human-disease-with-abnormal-prion-protein-sensitive-to-protease-prionopathy/641206


WHERE IS IT WRITTEN, that scrapie and or atypical scrapie, CANNOT OR WILL NOT, infect humans ???


or rather, where has it been proven of this myth ???


fact is, there is more scientific evidence to support transmission of scrapie to humans, than there is, showing that it will not. ...


Monday, December 14, 2009


Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html


Thursday, January 07, 2010


Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 and FISCAL YEAR 2008


http://scrapie-usa.blogspot.com/2010/01/scrapie-and-nor-98-scrapie-november.html


TRANSMISSION STUDIES DO NOT LIE, only the politicians and the industry that put these politicians in office do ;


1: J Infect Dis 1980 Aug;142(2):205-8


Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.


Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.


Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. snip... The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: 6997404


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract



12/10/76



AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE


Office Note CHAIRMAN: PROFESSOR PETER WILDY


snip...


A The Present Position with respect to Scrapie A] The Problem


Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.


The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.


It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.


Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"


Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.


snip...


76/10.12/4.6


http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf



Nature. 1972 Mar 10;236(5341):73-4.


Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).


Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland


SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).


http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html



Epidemiology of Scrapie in the United States 1977


http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf


http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf


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


snip...


http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf


http://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf



2005 DEFRA Department for Environment, Food & Rural Affairs

Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk


GTN: FAX:


Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518


21 November 2001


Dear Mr Singeltary


TSE IN HOUNDS


Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.


As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.


Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.


Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less critical. For more details see-


http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf


http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.


Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK


You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.


I hope this is helpful

Yours sincerely 4


HUGH MCDONAGH BSE CORRESPONDENCE SECTION


IN CONFIDENCE


CONCEPT NOT FOR FURTHER STUDY OF MATERIAL OBTAINED IN A SURVEY OF HOUNDS FOR EVIDENCE OF A SCRAPIE-LIKE SPONGIFORM ENCEPHALOPATHY (SE)


snip...


b) Fibrillar material closely similar to SAF, found in BSE/Scrapie, was observed in 19 (4.3%) cases, all of which were hounds > 7 years of age. 14/19 of these suspected SAF results correlated with cases in the unresolveable histopathological category.


snip...

The following proposals address the hypothesis that the hound survey observations represent a PrP related or scrapie-like disease of dogs in which the pathological response, and possible the spread of infectivity, is neuroanatomically localized. By inference this could also mean that the disorder is clinically silent and non-progressive.


http://www.bseinquiry.gov.uk/files/yb/1995/02/09001001.pdf



http://web.archive.org/web/20030907001733/www.bseinquiry.gov.uk/files/yb/1995/02/09001001.pdf



http://www.humanitarian.net/law/biodefense/bse_12004.html



http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html



2008 - 2010

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



CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

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



Saturday, January 2, 2010

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


http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html



my comments to PLosone here ;


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




14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.



http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml


From: xxxx To: Terry Singeltary Sent: Saturday, December 05, 2009 9:09 AM Subject: 14th ICID - abstract accepted for 'International Scientific Exchange'

Your preliminary abstract number: 670

Dear Mr. Singeltary,

On behalf of the Scientific Committee, I am pleased to inform you that your abstract

'Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009'

WAS accepted for inclusion in the INTERNATIONAL SCIENTIFIC EXCHANGE (ISE) section of the 14th International Congress on Infectious Diseases. Accordingly, your abstract will be included in the "Intl. Scientific Exchange abstract CD-rom" of the Congress which will be distributed to all participants.

Abstracts accepted for INTERNATIONAL SCIENTIFIC EXCHANGE are NOT PRESENTED in the oral OR poster sessions.

Your abstract below was accepted for: INTERNATIONAL SCIENTIFIC EXCHANGE

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Author: T. Singeltary; Bacliff, TX/US

Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange

This abstract has been ACCEPTED.

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Authors: T. Singeltary; Bacliff, TX/US

Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Body: Background

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

Methods

12 years independent research of available data

Results

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

Conclusion

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries.

I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion


http://www.isid.org/14th_icid/


http://www.isid.org/publications/ICID_Archive.shtml


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


Monday, October 19, 2009

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

snip...

I ask Professor Kong ;

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

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

Professor Kong reply ;

.....snip

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

Thanks for your interest.''

Best regards,

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

END...TSS

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS

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


for those interested, please see full text ;


Friday, January 29, 2010 14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)



http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html



Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION


http://cjdquestionnaire.blogspot.com/



Saturday, June 13, 2009

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


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



Sunday, January 17, 2010

BSE USA feed inspection violations 01/01/2009 to 01/17/2010 FDA BSE/Ruminant Feed Inspections Firms Inventory Report


http://madcowfeed.blogspot.com/2010/01/bse-usa-feed-inspection-violations.html



Tuesday, January 19, 2010

CVM's OR Develops New PCR-Based Method for Testing Animal Feed


http://madcowfeed.blogspot.com/2010/01/cvms-or-develops-new-pcr-based-method.html



Tuesday, January 26, 2010

Establishing a Fully Integrated National Food Safety System with Strengthened Inspection, Laboratory and Response Capacity Draft 09/24/09


http://fdafailedus.blogspot.com/2010/01/establishing-fully-integrated-national.html




WHO WILL WATCH THE CHILDREN FOR CJD OVER THE NEXT 5 + DECADES ???


For 4+ years, dead stock downer cows, the most high risk cattle for mad cow type disease, and other deadly pathogens, were fed to our kids, via the NSLP and the USDA all across our Nation.

Do you actually believe that the USDA et al jumped in on the law suit against Westland/Hallmark, at the time the largest beef recall in USA history, just because a few animals were abused on a video, or to cover their ass, for letting our children, from school district to school district, from state to state, be fed dead stock downer cows.


>>>In the papers, the government alleges the meatpacking plant slaughtered and processed downer cows for nearly four years — from January 2004 to September 2007 — at the average rate of one every six weeks... <<<



http://downercattle.blogspot.com/2009/09/suit-meatpacker-used-downer-cows-for-4.html



Do you actually believe all these schools recalled this meat because of a few cattle being abused, see list ; FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008


http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf


IF url does not work above, go to this link to find out if any of your children and their school were part of this recall ; go to this site ;


http://www.fns.usda.gov/fns/


left hand corner search ; Hallmark/Westland Meat Packing Co. Beef Recall your should get this ;


http://65.216.150.153/texis/search?pr=FNS


1 through 1 of 1 matching documents, best matches first. sort by date 1: Hallmark - Westland SFA Reporting by State - 3-24-2008.xls Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008 The U.S. Department of Agriculture ...


http://www.fns.usda.gov/...ety/Hallmark-Westland_byState.pdf



PLEASE SEE ALSO ;


Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.


http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf



some history on dead stock downers in the USA ; Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf



TME


http://transmissible-mink-encephalopathy.blogspot.com/2009/07/transmissible-mink-encephalopathy.html



http://transmissible-mink-encephalopathy.blogspot.com/2007/12/phenotypic-similarity-of-transmissible.html



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


CWD


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




Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518