Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006

Sunday, December 16, 2007

Creutzfeldt-Jakob Disease Surveillance in Texas 2000–2006

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease caused by prions. The disease is usually fatal within a year and there is currently no known treatment or cure. CJD has been a reportable condition in Texas since 1998. There are currently four known types: sporadic, familial, variant, and iatrogenic. Diagnosis is complex and direct examination of brain tissue is required for case confirmation and identification of the type of CJD. During 2000–2006, there were 181 cases of confirmed, probable, or possible CJD diagnosed among Texas residents, including 88 cases of sporadic CJD, 5 cases of familial CJD, and 1 case of variant CJD. Most case-patients were male (55%) and 55 years of age or older (78%). The Texas Department of State Health Services is available to assist health care professionals with arrangement of free diagnostic testing and to provide resources for family support.

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Texas Surveillance

CJD has been a reportable condition in Texas since 1998. The majority of CJD cases are reported to the Texas Department of State Health Services (DSHS) by test or autopsy reports received from the Prion Center. A few cases are discovered by routine death certificate review and some cases are reported by a local or regional health department, health care provider, or family member. Copies of results from all CJD-related testing performed by the Prion Center are sent to DSHS as a part of routine surveillance procedures. DSHS reviews test results and investigates all cases with positive test results including those with elevated CSF 14-3-3 protein levels. Table 1 summarizes the number of cases for all types of CJD in Texas (2000–2006). Each year in Texas, the total number of confirmed, probable, or possible sporadic CJD cases has remained somewhat constant (mean=13; range= 6–15). In addition, on average, one case of familial CJD was diagnosed each year in Texas during 2000–2006, all from separate families. Only 1 case of variant CJD has ever been diagnosed in Texas. The patient was a former resident of the United Kingdom, where the exposure was likely to have occurred. Texas has a population of 23 million, and since the national rate of sporadic CJD is about 1 per million, it is expected that approximately 23 cases of CJD would occur each year in the state. Therefore, it is believed that CJD is currently underreported in Texas. Table 2 describes sporadic and familial CJD cases in Texas according to gender and age. Of the patients diagnosed with CJD during 2000–2006, 58% were male and 79% were 55 years of age or older. Figure 1 depicts the number of CJD cases per county for the years 2000–2006. Note that higher numbers of cases are located in counties with higher populations.

CJD should be suspected and reported to DSHS in individuals who meet the following criteria:

1) Dementia of early onset (younger than 55 years of age) or 2) Rapidly progressive dementia and one of more of the following: • Movement disorder • Painful sensory symptoms • Visual disturbances or 3) Diagnosed by a physician as having CJD

Case Classification

The World Health Organization (WHO) established recommended standards for all types of CJD surveillance in 1997 (Table 3). The United States, including Texas, has adopted these surveillance standards to determine case classification for sCJD, fCJD and iCJD. For vCJD, CDC has developed diagnostic case criteria for use in the United States (Table 3). Direct examination of brain tissue through either biopsy or autopsy is highly recommended for all types of CJD and is required for confirmation of sporadic CJD and variant CJD. A sCJD case is determined to be ‘probable’ if there is enough diagnostic testing to suggest CJD but no autopsy has been performed. Patients considered for

probable sCJD must exhibit progressive dementia and have a typical EEG and/or elevated CSF 14-3-3 protein. In addition they must display at least 2 of 4 clinical features: myoclonus, visual or cerebellar disturbance, akinetic mutism, and/or pyramidal/extrapyramidal dysfunction. For a case to be listed as ‘possible’, the patient must have progressive dementia and at least 2 of the listed clinical features, disease duration of less than 2 years and either no EEG testing or EEG atypical for CJD. Iatrogenic CJD must have a known risk factor, e.g., high risk surgery or cadaver-derived pituitary hormone. Familial CJD is considered confirmed in a patient with a neuropsychiatric disorder if a first degree relative had confirmed or probable CJD or a disease-specific prion protein gene mutation has been identified. Table 3 describes the case classification for each type of CJD.

Conclusions

CJD is an emerging disease that may be misdiagnosed and appears to be underreported in Texas. As with other emerging diseases, the partnership of the medical community and public health epidemiologists provides a framework to share information and expertise among local, state, federal and international colleagues and to detect and respond to

diseases that are rapidly fatal, difficult to diagnose, and rare. Enhanced surveillance will provide a better understanding of the epidemiology of this disease and its impact and opportunities for mitigation. Recognition of possible CJD and reporting by health professionals is an integral part of CJD surveillance in Texas. CJD is a reportable disease in Texas. All suspected cases should be reported within one week to the Texas Department of State Health Services, Infectious Disease Control Unit. Confirmation and typing of the disease requires neuropathological confirmation by direct examination of brain tissue, usually postmortem, by the Prion Center. It is important to establish the precise type of CJD to help monitor disease occurrence especially for variant CJD. Physicians should strongly consider arranging for autopsies of suspected or clinically-diagnosed CJD patients. The Texas Department of State Health Services can assist health professionals by arranging free diagnostic testing and providing resources for family support.

References...snip...end...TSS

Table 1. Cases of Creutzfeldt-Jakob disease in Texas*§

Type 2000 2001 2002 2003 2004 2005 2006 Total

Sporadic

Confirmed 9 8 4 6 7 8 5 47

Probable 2 4 1 5 5 6 3 26

Possible 3 1 1 4 1 0 0 10

Subtotal 14 13 6 15 13 14 8 83

Familial 0 1 1 1 0 1 1 5

Iatrogenic 0 0 0 0 0 0 0 0

Variant 0 0 0 0 0 0 1† 1

Total 14 14 7 16 13 15 10 89

*Based on data as of June 15, 2007.

† Confirmed in United Kingdom and reported to Texas Department of State Health Services through Centers for Disease Control and Prevention.

§ Based on year of death.

Table 2. Creutzfeldt-Jakob disease cases* in Texas by gender and age 2000–2006†§

Characteristics 2000 2001 2002 2003 2004 2005 2006 Total

Gender

Male 10 8 3 6 10 7 7 51

Female 4 6 4 10 3 8 2 37

Age (years)

<55>55 11 14 4 12 10 11 7 68

* Based on data as of June 15, 2007.

† Includes cases of possible, probable and confirmed sporadic CJD and confirmed familial CJD.

§ Based on year of death.

Volume 64/Number 8/ November 5, 2007

http://www.dshs.state.tx.us/idcu/epilink/volume_64/issue_8/docs/640804.pdf


please notice Texas 2006 ;

† Confirmed in United Kingdom and reported to Texas Department of State Health Services through Centers for Disease Control and Prevention.

Only 1 case of variant CJD has ever been diagnosed in Texas. The patient was a former resident of the United Kingdom, where the exposure was likely to have occurred. Texas has a population of 23 million, and since the national rate of sporadic CJD is about 1 per million, it is expected that approximately 23 cases of CJD would occur each year in the state. Therefore, it is believed that CJD is currently underreported in Texas. ...END...TSS

also see ;

http://www.dshs.state.tx.us/idcu/disease/creutzfeldt-jakob/data/


Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A. Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of Health, 1Austin and 2Tyler, Texas

Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform encephalopathy, is caused by prions composed of proteinaceous material devoid of nucleic acid. CJD occurs sporadically (generally 1 case/1,000,000 population per year) in older patients (average age of 65) and is characterized by rapidly progressive dementia, accompanied by severe muscle spasms and incoordination. Death usually occurs within 3 to 12 months (average 7 months). CJD activity in Texas, which has a population of nearly 19 million, appeared to be typical. The statewide death rate for 1995 and 1996 was just under 1/1,000,000. In April of 1997, the Texas Department of Health became aware of an increased number of possible CJD cases in a 23-county area of NE Texas with a population of just over one million. After review of medical and pathology records, four patients were identified with definite classic CJD and three were identified with probable CJD. Dates of death for the eight patients were from April, 1996 through mid-July 1997. The patients were from 46 through 65 years of age; four were male and three were female. A case-control study to identify risks for CJD in NE Texas has been initiated.

http://www.jifsan.umd.edu/tse/Rawlings.htm


North American Equity Research

New York

13 January 2004

BSE (Mad Cow) Update:

Do Reports of sCJD Clusters Matter?

· There have been seven cases of human sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 2.1 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. · There is no proven link between sCJD and BSE, and hence it is considered a different disease from vCJD (which has been linked to BSE). However, the existence of clusters raises the question of “contamination" or “infection”, and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. · Clusters are not spontaneous, they normally have a source. Moreover, some cases of sCJD may have been improperly diagnosed as Alzheimer's.· We continue to believe that as long as no further cases of BSE-positive cows are found in North America and the industry has respected the 1997 ban on animal feed for live cattle, beef consumption in the US will not suffer. · Moreover, due to political pressure we expect key overseas markets (Japan, South Korea, and Mexico) to open up to US beef in the next six months – the recent 20% drop in cattle prices can be attributed mainly to these import bans. · However, two concerns linger and should be kept in mind by investors, 1) Has the 1997 ban on animal feed for live cattle been honored by the beef industry? 2) Can clusters of cases of sporadic CJD (or sCJD) really be a variant of CJD and indeed be linked to BSE? In this note we focus on the issue of sCJD clusters, and the potential impact that the growing debate on clusters could have on beef consumption in the US. United States Foods Pablo E. Zuanic(1-212) 622-6744pablo.zuanic@jpmorgan.comChristopher M. Bledsoe(1-212) 622-6386christopher.m.bledsoe@jpmorgan.comDaniel Ogbonna(212) 622-6382daniel.c.ogbonna@jpmorgan.com

State of Our Views Regarding BSE in the US

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Existence of Clusters of sCJD May Imply They Are Really Cases of vCJD There have been seven sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 1.2 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. The existence of clusters raises the question of “contamination" or “infection”, and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. Clusters are not spontaneous, they normally have a source.

A cluster consists of two statistical improbabilities: 1) multiple cases occurring in a relatively limited geographic area, and 2) multiple cases occurring within the same time period. The most recent cluster was found in Cherry Hill, New Jersey. The others have been found in Lehigh, Pennsylvania (1986-90), Allentown, Pennsylvania (1989-92), Tampa, Florida (1996-97), Oregon (2001-02), and Nassau County, New York (1999-2000). Given that sCJD occurs randomly in one out of one million cases, it is a statistical rarity to find an sCJD cluster – let alone six. The following tables highlight known clusters in the US.

Table 1:

Clustered sCJD Deaths

Local sCJD Deaths

Time Span State Local Area Pop. (MM) Period (mo.) Total Ann'lized

1986-1990 PA Lehigh Valley 0.5 48 18 4.5

1989-1992 PA Allentown 2.5 36 15 5.0

1996-1997 FL Tampa 2.2 18 13 8.7

1996-1999 TX Denton .01 38 4 1.3

1999-2000 NY Nassau County 1.3 12 7 7.0

2001-2002 OR Entire State 3.4 24 14 7.0

2000-2003* NJ Cherry Hill Area 1.7 36 12 4.0

Source: JPMorgan.

The second table, below, shows what portion of the state's total expected sCJD cases (as based on a one per million occurrence) were found in the local cluster, comparing the local cluster's portion of cases with the local area's portion of the state's total population. The greater the factor between the former and the latter suggests a higher statistical improbability that the cluster is spontaneous (sCJD).

Table 2: Clustered sCJD Deaths vs. Expected State Cases

Annual Statewide Local Area (% of Time Span State Local Area sCJD Deaths* exp. state cases state pop.

1986-1990 PA Lehigh Valley 11.9 37.8% 4.5%

1989-1992 PA Allentown 12.0 41.7% 20.8%

1996-1997 FL Tampa 14.1 61.5% 15.7%

1996-1999 TX Denton 20.9 6.1% .02%

1999-2000 NY Nassau County 18.1 38.7% 7.4%

2001-2002 OR Entire State 3.4 205.9% 100.0%

2000-2003* NJ Cherry Hill Area 8.0 50.0% 21.6%

* *State cases are extrapolated based on state population and the 1 per million national average. Source: JPMorgan.

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Copyright 2003 J.P. Morgan Chase & Co.—All rights reserved.

THIS MATERIAL IS ISSUED AND DISTRIBUTED IN MALAYSIA BY J.P. MORGAN MALAYSIA SDN. BHD. (18146-X).


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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


US Concern CJD Screening May Miss Thousands Of Cases

By Steve Mitchell Zwire.co, United Press International (via COMTEX) 7-23-3

The UK Department of Health website maintains the only comprehensive source of comparative data on the incidence of the various forms of CJD in any population. The UK Department of Health monthly reports are reproduced in ProMED-mail close to the beginning of each month (see references below). - Mod.CP]

The federal government's monitoring system for cases of Creutzfeldt-Jakob disease (CJD), a fatal human brain illness, could be missing tens of thousands of victims, scientists and consumer advocates have told United Press International.

Variant CJD [abbreviated as CJD (new var.) or vCJD in ProMED-mail] can be [contracted] by eating beef [from cattle] with mad cow disease (bovine spongiform encephalopathy - abbreviated as BSE), but the critics assert that, without a better tracking system, it might be impossible to determine whether any [cases of sporadic CJD] are [cases of vCJD] or to obtain an accurate picture of the prevalence of the disorder in the United States.

Beginning in the late 1990s, more than 100 people contracted vCJD in the United Kingdom and several European countries after eating beef infected with BSE. [The mode of transmission of the BSE agent to humans has not been established conclusively, but is presumed on circumstantial grounds to be a consequence of consumption of contaminated meat. - Mod.CP]

No case of [BSE] has ever been detected in U.S. cattle, and the monitoring system of the Centers for Disease Control and Prevention (CDC) has never detected a case of vCJD . Nevertheless, critics say, the CDC's system [may] miss many cases of the disease, which currently is not treatable and is always fatal.

The first symptoms of CJD typically include memory loss and difficulty keeping balance and walking. As the disease destroys the brain, patients rapidly progress in a matter of months to difficulty with movement, an inability to talk and swallow and, finally, death. Spontaneously-occurring or sporadic CJD is a rare disorder. Only about 300 cases appear nationwide each year, but several studies have suggested that the disorder might be more common than thought and that as many as tens of thousands of cases might be going unrecognized. Clusters of [sporadic] CJD have been reported in various areas of the United States -- Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in 1999-2000, and Texas in 1996. In addition, several people in New Jersey developed CJD in recent years, including a 56-year-old woman who died on 31 May 2003. Although in some instances, a [BSE] link was suspected, all of the cases ultimately were classified as sporadic CJD.

People who develop CJD [presumably as a result of] eating BSE-contaminated beef have been thought to develop the specific form of the disorder called variant CJD. But new research, released in December 2002 [see ProMED-mail post archived as: CJD (new var.) - UK: update 2003 (03) 20030204.0299], [suggests] that the [BSE] pathogen [may] cause both sporadic CJD and the variant form. "Now people are beginning to realize that because something looks like sporadic CJD they can't necessarily conclude that it is not linked to [BSE]," said Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, who conducted a 1989 study that found 13 percent of Alzheimer's patients actually had CJD.

Several studies, including the Manuelidis study, have found that autopsies reveal 3 to 13 percent of patients diagnosed with Alzheimer's or dementia actually suffered from CJD. Those numbers might sound low, but there are 4 million Alzheimer's cases and hundreds of thousands of dementia cases in the United States. A small percentage of those cases could add up to 120 000 or more CJD victims going undetected and not included in official statistics.

Experiences in [the UK] and Switzerland -- both countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility that some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since BSE was first detected in British herds in 1986. Switzerland discovered in 2002 that its CJD rate was twice that of any other country in the world. Switzerland had been seeing 8 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.

The CDC says the annual rate of CJD in the United States is one case per million people, but the above studies suggest the true prevalence of CJD is not known, Manuelidis told UPI. Diagnosing CJD or Alzheimer's is difficult because no test exists that can identify either disease in a living patient with certainty. So physicians must rely on the patient's symptoms to determine which illness might be present. Sometimes, however, the symptoms of one disease can appear similar to the other. The only way to determine the disease conclusively is to perform an autopsy on the brain after death. Unfortunately, although autopsies once were performed on approximately half of all corpses, the frequency has dropped to 15 percent or less in the United States. The National Center for Health Statistics (NCHS) -- a branch of the CDC -- stopped collecting autopsy data in 1995. "If we don't do autopsies and we don't look at people's brains ... we have no idea about the general prevalence of these kinds of infections and (whether) it is changing," Manuelidis said.

While autopsies have been declining, the number of deaths attributed to Alzheimer's has increased more than 50-fold since 1979, going from 857 deaths then to nearly 50 000 in 2000. Though it is unlikely the dramatic increase in Alzheimer's is due entirely to misdiagnosed CJD cases, it "could explain some of the increase we've seen," Manuelidis said.

"Neurodegenerative disease and Alzheimer's disease have become a waste-basket" for mental illness in the elderly that is difficult to diagnose conclusively, she said. "In other words, what people call Alzheimer's now is [broader] than what people used to call it, and that has the possibility of encompassing more diseases -- including CJD." The autopsy studies that found undiagnosed CJD cases raise the question of whether the United States "already has an undetected epidemic here," Jeff Nelson, director of Vegsource.com, a vegetarian advocacy web-site, told UPI. "What's the source of that?" Nelson asked. "Could it be the same source of encephalopathy we saw in minks?"

Nelson referred to an outbreak of a [transmissible spongiform encephalopathy] in minks in Wisconsin in the 1980s. The origin was traced back to the animals' diet, which included parts of so-called downer cattle -- sick cows that are unable to stand, which often indicates a neurological disease, including mad cow. The mink disease raised concerns about whether U.S. cattle were carrying a mad-cow-like pathogen even prior to the U.K. epidemic that began in 1986.

Andrew Monjan, chief of the neuropsychology of aging program at the National Institute of Aging (part of the National Institutes of Health in Bethesda, Maryland), acknowledged there has been an increase in U.S. Alzheimer's cases. However, he told UPI, this probably is due to the aging of the population -- as people grow older, they develop a higher risk of developing Alzheimer's. "There's been no change in the number of CJD cases in the country, and there has been clearly a tracking of the unusual cases of CJD" that could be due to mad cow disease, Monjan said.

However, Terry Singletary, coordinator of CJD Watch -- an organization founded to track CJD cases -- says efforts to track the disease have been close to nonexistent. For example, only 12 states require such reports. Therefore, many cases might be going undetected, unreported, or misdiagnosed. If more states made CJD a reportable illness, there would be more clusters detected across the United States, said Singletary, who became involved with CJD advocacy after his mother died from a form of CJD known as Heidenhain variant.

In the 18-year period between 1979 and 1996, he noted, the country saw a jump from one case of sporadic CJD in people under the age of 30 -- a warning sign for a link to [BSE], because nearly all of the U.K. victims were 30 years of age or younger, to 5 cases in 5 years between 1997 and 2001. "That represents a substantial blip," he told UPI. Singletary also said there have been increases in sporadic CJD in France, Germany, and Italy, all of which have detected mad cow disease in their cattle.

So far, the CDC has refused to impose a national requirement that physicians and hospitals report cases of the disease. The agency has not chosen to make CJD a reportable disease because "making it reportable is not necessarily directly helpful in surveillance, because in some states where it's reportable you may not get the physician to report it," said Dr. Ermias Belay, CDC's medical epidemiologist working on CJD. Instead, the agency relies on other methods, including death certificates and urging physicians to send suspicious cases to the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University in Cleveland, which is funded by the CDC.

However, because autopsies generally are not done, if a CJD case is misdiagnosed as Alzheimer's or dementia, a correct diagnosis might never be made, and therefore the cause of death listed on a death certificate might be inaccurate. Belay told UPI he discounted this possibility. It is unlikely to happen, he said, because it is easy to distinguish CJD from Alzheimer's -- the 2 conditions display different symptoms.

Manuelidis disagreed. It can be quite difficult to determine accurately whether a patient has CJD, as evidenced by her study, in which respected and competent neurologists and psychiatrists at Yale originally diagnosed patients with Alzheimer's, yet were wrong at least 13 percent of the time. Another study conducted at the University of Pennsylvania, which found 6 percent of dementia patients actually were suffering from CJD, supports the difficulty in distinguishing the illnesses correctly. The U. Penn. researchers concluded that: "These results show that in patients with a clinical diagnosis of dementia, the etiology cannot be accurately predicted during life."

In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC's investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases. Belay said that the CDC follows up on all cases of CJD that occur in people under age 55, as these could be linked to variant (BSE-related) CJD. But so far, all have turned out to be sporadic forms of the disease. About 30 cases of the disorder occur each year in the United States in this age group, while the remaining 270 or so are older.

The case of a Philadelphia woman who developed a brain disorder that appeared to be CJD and died from it in 2000 at the age of 29 -- illustrates just how difficult it can be to diagnose the disease. Her physician, Dr. Peter Crinos of the University of Pennsylvania Medical Center, ruled out other disorders and felt certain the young woman had died of CJD, a concern that raised the possibility of a link to mad cow disease because of her young age. When neuropathologist Nicholas Gonatas, who had seen CJD before, examined the woman's brain after her death, he, likewise, was confident he detected the microscopic, sponge-like holes caused by the disease. But when he sent brain samples to the NPDPSC, the results came back negative. Gonatas, convinced the surveillance center's finding was erroneous, sent off 2 more samples, only to have them both come back negative. Subsequent research, however, has shown the test used by the surveillance center cannot rule out CJD, said Crinos, an assistant professor of neurology. "There's no question that the young woman had a spongiform encephalopathy," Crinos said, but added although it appeared to be CJD, it is difficult if not impossible to say whether it was due to mad cow disease.

Crinos told UPI until the CDC implements a better tracking system, a lot of questions will remain about CJD and cases like the young Philadelphia woman's. One central question: Why are cases of what is presumed to be a rare disease popping up in clusters in certain areas of the country? Crinos said the clustering suggests an environmental or food-borne cause, but so far, "No one knows the answer to that."

http://www.zwire.com/site/news.cfm?newsid=9882702&BRD=1713&PAG=740&dept_id=226965&rfi=6


ProMED Mail promed@promedmail.org

[The above article raises some significant issues related to CJD surveillance in the USA, and factors that may be contributing to a significant under-reporting of CJD cases. First and foremost, CJD is not a nationally reportable disease. Even with nationally reportable diseases, one sees significant under-reporting, probably related to the health care delivery system in the USA, which is heavily weighted towards private-sector providers. Unfortunately the private sector is less consistent with disease reporting to state and federal levels. In the absence of a national mandate for reporting of a disease, the incentive becomes negligible, so the traditional "tip of the iceberg" seen in disease reporting shrinks even further.

The study suggesting that up to 13 percent of cases diagnosed with Alzheimer's and other dementias may be due to CJD points out another weak link in CJD surveillance. The diagnosis does necessitate an autopsy, and autopsy rates have declined markedly in the USA, especially in the elderly. Hence the "Catch 22" -- what is needed for the diagnosis is an autopsy, but the autopsy isn't performed (as has been the case in many of the CJD "cluster" reports). The reverse of "seek and ye shall find" is "don't look and the problem isn't there." - Mod.MPP]


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

http://cjdusa.blogspot.com/


CJD QUESTIONNAIRE

http://cjdquestionnaire.blogspot.com/


SCRAPIE USA

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


NOR-98 ATYPICAL SCRAPIE CASES USA

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


CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA

http://cjdmadcowbaseoct2007.blogspot.com/


Transmissible Mink Encephalopathy TME

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


CHRONIC WASTING DISEASE

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


TSEAC MEETING


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

From: Terry S. Singeltary Sr.

To: FREAS@CBER.FDA.GOVCc: william.freas@fda.hhs.gov ; rosanna.harvey@fda.hhs.gov

Sent: Wednesday, November 29, 2006 1:24 PM

Subject: TSE advisory committee for the meeting December 15, 2006 [TSSSUBMISSION]

November 29, 2006

Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al,

a kind and warm Holiday Greetings to you all. i kindly wish to submit the following to the TSE advisory committee for the meeting December 15, 2006, about the assessment for potential exposure to vCJD in human plasma-derived antihemophilic factor (FVIII) products manufactured from U.S. plasma donors and related communication material ;

http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-20251.htm


i see the media picked up on this as a 'low risk', from what the gov. agency perceived to be to them;

http://www.newsday.com/news/health/ats-ap_health14nov27,0,7955259.story?coll=ny-leadhealthnews-headlines


however, i seem to disagree. from my primitive ciphering, i see it anotherway. this is a huge catastrophic risk. 3 in 160 is 1.9%. so call that 2% which is 1 in 50 or twenty per thousand or 20,000 per million. also, what about the mixed genotypes/mixed susceptibility? what about the silent carriers that donated tainted blood? what about the sporadic CJDs of UNKNOWN strain or phenotype? this risk assessment is just more BSe to me. just another in a long line of ...

Greetings again Dr. Freas et al at FDA,

THIS was like closing the barn door after the mad cows got loose. not only the red cross, but the FDA has failed the public in protecting them from the TSE aka madcow agent. TSE agent i.e. bse, base, cwd, scrapie, tme, and any sub strains thereof. we do not know if these strains will or have transmitted to humans as subclinical TSE or clinical disease, and we do not know if they have or will transmit second, third, forth passage via friendly fire i.e. multiple potential routes via medical, surgical, pharmaceutical etc. IF you remember correctly Dr. Freas et al, i called this long ago, almost 6 years ago ;

PDF]Freas, William TSS SUBMISSION Terry S. Singeltary Sr. [flounder@wt.net] Monday, January 08, 200l 3:03 PM freas ...Freas, William From: Terry S. Singeltary Sr. [flounder@wt.net] Sent: Monday, January 08,200l 3:03 PM To: freas@CBS5055530.CBER.FDA.GOV

Subject: CJD/BSE (aka madcow) Human/Animal TSE?s--U.S.--Submission

To Scientific Advisors and Consultants Staff January 2001 Meeting (short version)

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version). I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products.

snip...

I am beginning to think that the endless attempt to track down and ban, potential victims from known BSE Countries from giving blood will be futile. You would have to ban everyone on the Globe eventually? AS well, I think we MUST ACT SWIFTLY to find blood test for TSE's, whether it be blood test, urine test, eyelid test, anything at whatever cost, we need a test FAST. DO NOT let the incubation time period of these TSEs fool you. To think of Scrapie as the prime agent to compare CJD, but yet overlook the Louping-ill vaccine event in 1930's of which 1000's of sheep where infected by scrapie from a vaccine made of scrapie infected sheep brains, would be foolish. I acquired this full text version of the event which was recorded in the Annual Congress of 1946 National Vet. Med. Ass. of Great Britain and Ireland. From the BVA and the URL is posted in my (long version). ...

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf


see full text ;

http://tseac.blogspot.com/


vCJD case study highlights blood transfusion risk

http://vcjdblood.blogspot.com/


Wednesday, October 24, 2007

MADCOW USDA the untold story

http://madcowusda.blogspot.com/


FOIA MAD SHEEP MAD RIVER VALLEY

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

http://foiamadsheepmadrivervalley.blogspot.com/


[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


[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


SEAC 99 DECEMBER 14, 2007

Conclusions

14. Preliminary research findings suggest that the potential risk of transmission of vCJD via dental procedures may be greater than previously anticipated. Although this research is incomplete, uses an animal model exposed to relatively high doses of infectivity, and there are no data from infectivity studies on human oral tissues, these findings suggest an increased possibility that vCJD may be relatively efficiently transmitted via a range of dental procedures. Ongoing infectivity studies using human oral tissues and the other studies suggested here will enable more precise assessment of the risks of vCJD transmission through dental procedures.

15. Guidance was issued to dentists earlier this year recommending that endodontic files and reamers be treated as single use which, provided it is adhered to, will remove any risk of a self-sustaining epidemic arising from re-use of these instruments. To minimise risk it is critical that appropriate management and audit is in place, both for NHS and private dentistry.

16. It is also critical that a detailed and comprehensive assessment of the risks of all dental procedures be conducted as a matter of urgency. While taking into account the continuing scientific uncertainties, this will allow a more thorough consideration of the possible public health implications of vCJD transmission via dentistry and the identification of possible additional precautionary risk reduction measures. The assessment will require continued updating as more evidence becomes available on the transmissibility of vCJD by dental routes, and on the prevalence of infection within the population. A DH proposal to convene an expert group that includes dental professionals to expedite such an assessment is welcomed. Given the potential for transmission via dentistry, consideration should be given to the urgent assessment of new decontamination technologies which, if proved robust and effective, could significantly reduce transmission risks.

SEAC June 2007

27 SEAC Epidemiology Subgroup (2006) position statement of the vCJD epidemic.

http://www.seac.gov.uk/statements/state260106subgroup.htm



28 DH (2007) Precautionary advice given to dentists on re-use of instruments

http://www.gnn.gov.uk/environment/fullDetail.asp?ReleaseID=279256&NewsAreaID=2&NavigatedFromDepartment=False


see full text 17 pages ;

http://www.seac.gov.uk/papers/99-7.pdf


SEAC 99th meeting on Friday 14th December 2007

DECEMBER 14, 2007, 10 year Anniversary of my Moms death 'confirmed' from Heidenhain Variant Creutzfeldt Jakob Disease

Greetings,

AS one of them _lay_ folks, one must only ponder ;

"WITH the Nor-98 now documented in five different states so far in the USA in 2007, and with the TWO atypical BSE H-BASE cases in Texas and Alabama, with both scrapie and CWD running rampant in the USA, IS there any concern from SEAC with the rise of sporadic CJD in the USA from ''UNKNOWN PHENOTYPE'', and what concerns if any, in relations to blood donations, surgery, optical, and dental, do you have with these unknown atypical phenotypes in both humans and animals in the USA ???"

"Does it concern SEAC, or is it of no concern to SEAC?"

"Should it concern USA animal and human health officials?"

snip...

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

From: xxxxxxxxxx
To: flounder9@verizon.net
Sent: Thursday, November 22, 2007 5:39 AM
Subject: QUESTION FOR SEAC

Mr Terry S Singeltary Sr., Bacliff, Texas 77518 USA.

Dear Mr Singeltary,

"Thank you for your e-mail of yesterday with the question for SEAC. I can confirm that this will be asked at the meeting on your behalf and the question and answer will appear in the minutes of the meeting which will be published on the SEAC Internet site."

snip...end...TSS

Archive Number 20071105.3602 Published Date 05-NOV-2007 Subject PRO/AH/EDR> Prion disease update 2007 (07)

PRION DISEASE UPDATE 2007 (07)

****************************** A ProMED-mail post

snip...

[2] USA: National Prion Disease Pathology Surveillance Center Date: June 2007 Source: National Prion Disease Pathology Surveillance Center (USA) [edited]

CJD Cases examined ---------------------- Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD

1996 / 42 / 32 / 26 / 4 / 0 / 0

1997 / 115 / 68 / 57 / 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 / 208 / 116 / 106 / 9 / 0 / 0

2002 / 255 / 143 / 118 / 23 / 2 / 0

2003 / 272 / 174 / 132 / 41 / 0 / 0

2004 / 334 / 183 / 157 / 21 / 0 / 1*

2005 / 352 / 195 / 152 / 37 / 1 / 0

2006 / 372 / 186 / 143 / 30 / 0 / 1**

2007 / 120 / 68 / 35 / 7 / 0 / 0

TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2

*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://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


snip...full text ;

http://seac992007.blogspot.com/


Sunday, December 16, 2007

Risk factors for sporadic Creutzfeldt-Jakob disease

Published Online: 11 Dec 2007

Copyright © 2007 American Neurological Association

Original Article

Risk factors for sporadic Creutzfeldt-Jakob disease

Hester J. T. Ward, FFPH 1 *, Dawn Everington, MSc 1, Simon N. Cousens, MA 2, Blaire Smith-Bathgate, RGN 1, Michelle Gillies, MRCP 1, Katy Murray, MRCP 1, Richard S. G. Knight, FRCPE 1, Peter G. Smith, DSc 2, Robert G. Will, FRCP 1 1National Creutzfeldt-Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom 2Department of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, London, United Kingdom

email: Hester J. T. Ward (h.ward@ed.ac.uk)

*Correspondence to Hester J. T. Ward, National CJD Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, United Kingdom

Funded by: Department of Health; Grant Number: 121/7400 Scottish Executive Health Department; Grant Number: R39924

Abstract

Objective

Although surgical transmission of Creutzfeldt-Jakob disease (CJD) has been demonstrated, these iatrogenic cases account for only a small proportion of all CJD cases. The majority are sporadic CJD (sCJD) cases of unknown cause. This study investigated whether some cases classified as sCJD might have an unrecognized iatrogenic basis through surgical or other medical procedures

Methods

This study compared medical risk factors from 431 sCJD cases referred 1998 to 2006 with 454 population control subjects. Possible geographic and temporal links between neurological and gynecological operations in 857 sCJD cases referred from 1990 to 2006 were investigated

Results

A reported history of ever having undergone surgery was associated with increased risk for sCJD (odds ratio, 2.0; 95% confidence interval, 1.3-2.1; p = 0.003). Increased risk was not associated with surgical categories chosen a priori but was confined to the residual category other surgery, in which the increase in risk appeared most marked for three subcategories: skin stitches, nose/throat operations, and removal of growths/cysts/moles. No convincing evidence was found of links (same hospital, within 2 years) between cases undergoing neurosurgery or gynecological surgery

Interpretation

It is unlikely that a high proportion of UK sCJD cases are the result of transmission during surgery, but we cannot exclude the possibility that such transmission occurs occasionally. A study based on accurate surgical histories obtained from medical records is required to determine whether the increased risk associated with reported surgical history reflects a causal association or recall bias. Ann Neurol 2007

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

Received: 24 May 2007; Revised: 5 September 2007; Accepted: 1 October 2007 Digital Object Identifier (DOI)

10.1002/ana.21294 About DOI

Additional Material

http://www3.interscience.wiley.com/cgi-bin/abstract/117861913/ABSTRACT?CRETRY=1&SRETRY=0


***

which the increase in risk appeared most marked for three subcategories:

skin stitches, nose/throat operations, and removal of growths/cysts/moles.

10 January 1990

Other US BSE risks: the imported products picture

24 Jul 00 Trade Statistics: UK to US

Compiled by Terry S. Singeltary Sr of Bacliff, Texas

[Opinion (webmaster): The US has focused for years on tracing, containing, and eradicating live animal imports from the UK or other countries with acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep. This strategy does not acknowledge imports of rendered bovine products from England during the BSE period nor secondary products such as surgical catgut, which is to say surgical cowgut, or dairy cattle embryos, vaccines for veterinarian and human medicines. What has become of these?

Mr. Singeltary, who lost his mother to CJD of unexplained origin a few years back and went on to became a well-known TSE activist, has tracked down voluminous pertinent import data through correspondence with UK officials and searches of government web sites. Imports of such products are frequently cited by Europeans in rating BSE risks in the US and in shutting out US exports.

Many people's eyes glaze over when reviewing reams of sometimes older trade statistics. There is no proof that any of the imported products was contaminated with BSE nor if so, any evidence that any BSE product lead to infection in US livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr. Singeltary establish that an appalling variety and tonnage of products that were imported by the US from the UK and othr BSE-affected countries during the peak of the BSE epidemic years.]

* which the increase in risk appeared most marked for three subcategories:

* skin stitches, nose/throat operations, and removal of growths/cysts/moles.

10 January 1990

Other US BSE risks: the imported products picture

24 Jul 00 Trade Statistics: UK to US

Compiled by Terry S. Singeltary Sr of Bacliff, Texas

[Opinion (webmaster): The US has focused for years on tracing, containing, and eradicating live animal imports from the UK or other countries with acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep. This strategy does not acknowledge imports of rendered bovine products from England during the BSE period nor secondary products such as surgical catgut, which is to say surgical cowgut, or dairy cattle embryos, vaccines for veterinarian and human medicines. What has become of these?

Mr. Singeltary, who lost his mother to CJD of unexplained origin a few years back and went on to became a well-known TSE activist, has tracked down voluminous pertinent import data through correspondence with UK officials and searches of government web sites. Imports of such products are frequently cited by Europeans in rating BSE risks in the US and in shutting out US exports.

Many people's eyes glaze over when reviewing reams of sometimes older trade statistics. There is no proof that any of the imported products was contaminated with BSE nor if so, any evidence that any BSE product lead to infection in US livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr. Singeltary establish that an appalling variety and tonnage of products that were imported by the US from the UK and othr BSE-affected countries during the peak of the BSE epidemic years.]

10 January 1990

NOT FOR PUBLICATION

COMMITTEE ON SAFETY OF MEDICINES

WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY

SURGICAL CATGUT SUTURES

2.1 At the first meeting of the Working Party on Bovine Spongiform Encephalopathy on 6 September 1989, detailed consideration was given to XXXXX Surgical Catgut. This arose from the Company's response to the Letter to License Holders, indicating that the bovine small intestine source material was derived from UK cattle, unlike 8 other licensed catgut sutures. In contrast XXXXX Surgical Catgut was stated to hold over 90% share of the market for catgut sutures, and to constitute approximately 83% of all sutures used in U.K.

IMPORTS OF SUTURES FROM THE KNOWN BSE COUNTRY;

snip... please see full text ;

http://creutzfeldt-jakob-disease.blogspot.com/


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