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From: TSS ()
Subject: Health officials still waiting for CJD results on cluster of suspect CJD victims
Date: August 27, 2005 at 8:38 am PST

Health officials still waiting for CJD results
Surveys have been completed
By Sandy Miller
Times-News writer

TWIN FALLS -- Idaho health officials on Friday were still waiting for the final test results on the brain tissue of one of five women who've died since January after being diagnosed with Creutzfeldt-Jakob disease, a rare progressive neurodegenerative disorder that's always fatal.

"As of now, we haven't heard anything," said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare on Friday afternoon. "We are hoping to get the results soon."

Health officials received the preliminary results on the woman, which revealed that she did indeed die of a prion disease. Shanahan said the department hopes to get the final results from the National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University early next week.

A sixth person, a man from Elmore County diagnosed with CJD, is still alive, Shanahan said. Of the five women, four were from Twin Falls County and one woman was from Minidoka County. All six were over the age of 60.

The only way to confirm a case of CJD and its type is by examining brain tissue. Autopsies were performed on three of the women and their brain tissue was sent to the National Prion Disease Pathology Surveillance Center to be studied. Two of the women died before their CJD was reported by their physicians and autopsies were not performed, Shanahan said.

All six people were between the ages of 60 and 83 and given their ages, Idaho health officials suspect the women had sporadic CJD and not the variant form of CJD that people get when they eat meat from a cow with bovine spongiform encephalopathy - commonly known as mad cow disease. The average age of people who've died from sporadic CJD is 68. The average age of people who've died from the variant form of CJD is 28, according to the Centers for Disease Control and Prevention.

CJD is a fatal disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

The unusually high number of cases has drawn attention from state health officials as well as the CDC. Normally, the disease infects just one person per 1 million people worldwide a year. In the United States, fewer than 300 cases of CJD are reported each year, according to the CDC.

In Idaho, there were an average of 1.1 cases a year between 1984 and 2004, Shanahan said.

"The most cases we've had in a single year was three until now," he said.

State epidemiologist Dr. Christine Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, examined medical records and Becker conducted surveys with all the victims' families. The Department of Health and Welfare is now entering the information into a computer program to determine if the six people had anything in common.

"The state is compiling the data and finalizing the information," Becker said. "We're going back and evaluating the data we have. We're conversing with the CDC about all the information."

Two clusters of sporadic CJD deaths in recent years occurred in New Jersey, involving 12 deaths; and in Oregon, involving 14 deaths. Those clusters, however, unfolded over a period of 36 months and 24 months, and in population areas of 1.7 million and 3.4 million, respectively.

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at

Story published at on Saturday, August 27, 2005

>>>Two clusters of sporadic CJD deaths in recent years occurred in New Jersey, involving 12 deaths; and in Oregon, involving 14 deaths. <<<

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.

what i considered a cluster of CJD victims in southeast Texas,
never got the attention of the TDH or CDC. it was just all
sporadic CJDs of no importance...tss

1: Ann Clin Lab Sci 2001 Apr;31(2):211-2

A cluster of Creutzfeldt-Jacob disease patients from Nassau County,
New York, USA.

Adikari D, Farmer P.

A Cluster of Creutzfeldt-Jacob Disease Patients from Nassau County, New
York, USA

February, 2001 Annals of Clinical and Laboratory Science Vol. 31
no. 2 p. 211-2

Creutzfeldt-Jacob Disease (CJD) is a rare and transmissible neurological
disorder, which has been increasing in frequency in the United States
over the past two decades. [...]

We report 7 cases of CJD from North Shore University Hospital, a
community-based teaching institution that serves Nassau County on Long
Island, NY. These cases were diagnosed and died during the 12-mo period
between mid-June 1999 to midJune 2000... These data suggest a CJD death
rate in Nassau County... approximately 6 times the national rate. During
the previous 1-yr period, no case of CJD was diagnosed in our laboratory

The authors are concerned that the number of CJD cases in our catchment
area appears to have increased dramatically during the 12-mo period.


Farmer PM, Kane WC, Hollenberg-Sher J.

Incidence of Creutzfeldt-Jakob disease in Brooklyn and Staten Island.

N Engl J Med. 1978 Feb 2;298(5):283-4. No abstract available.
PMID: 339092

2: Nosal R, Kapoor A, Shanin R.

Cluster of cases of Creutzfeldt-Jakob disease--Ontario.
Can Dis Wkly Rep. 1991 Jan 19;17(3):12. No abstract available.
PMID: 2001596

There is one report of a possible cluster of CJD cases in Canada;
between April 1989 and October 1990, six cases were reported in the
province of Ontario, from a population of 9.5 million (1986 census
figure). Two of the patients had come from areas of Czechoslovakia with
a high incidence of familial-type disease, but no other risk factors
were associated with these cases (7).

An Evaluation of a Suspected Cluster

of Creutzfeldt-Jakob Disease (CJD) in

New Jersey

May 2004

Division of Epidemiology, Environmental and

Occupational Health



5. Evaluation of suspected cluster of CJD

The evidence does not support the existence of an outbreak of CJD among attendees at

the GSRT, nor does it suggest that case-patients with CJD were exposed to BSEcontaminated

beef in the period 1988 to 1992 or at any other time at the Garden State

Racetrack in New Jersey.

a. The largest and earliest cattle outbreak of BSE in the world occurred in the UK and to

date the number of infected cattle has been many orders of magnitudes greater than the

number of reported probable and definite vCJD cases in the UK. Thus, an outbreak of

either variant or sporadic CJD in the US without more evidence of BSE in this country is

extremely unlikely.

b. All persons throughout the world with BSE-related vCJD tested as of January 2004

have had a specific genetic make-up characteristic of about 40% of the general

population (Will et al, 2004). None of the three pathologically confirmed CJD casepatients

who were tested for this marker were documented to have it.

c. The UK’s National CJD Surveillance Unit reports no evidence that sporadic CJD has

been caused by BSE. There was an increase in sporadic CJD in the UK from 1990-1996

due to improved case ascertainment (SEAC, 2004). This is to be expected for any disease


following the institution of a new and active surveillance system. However, there has

been no unusually high incidence of sporadic forms of CJD from 1997-2003 in the UK,

despite the much heavier exposure of the UK population to the BSE agent between 1980

and 1996 compared to the US population (UK Department of Health).

d. Ongoing comparisons of exposures of sporadic CJD case-patients with controls in

Europe, as conducted and interpreted by experts in the UK, have been reported to provide

no convincing evidence of an increased risk of sporadic forms of CJD through dietary

habits (Will et al., 2004).

e. As illustrated in Figure 1, all but two of the presumed food-related deaths due to the

BSE agent in the UK occurred in person younger than 55 years of age. In contrast, based

on the information available at NJDHSS through April 2004, 10 of the 11 decedents with

definite or probable CJD between 1995-2003 about whom JS has raised concerns were

>55 at the time of death.

f. None of the six patients in this report who had brain tissue submitted for examination at

the NPDPSC were documented to have vCJD, the form associated with BSE exposure.

Rather, they each had either a non-prion disease or form of sporadic CJD that differed

from each other. These laboratory data provide scientific evidence against a theory that

any of these illnesses were related to each other or to BSE.

h. There is no evidence of an increased number of CJD case-patients in the region closest

to the GSRT, in adjacent Philadelphia County, or in New York City.

F. Future CJD surveillance plans in New Jersey

1. General

The key to investigating potential causes of CJD in the US is to encourage brain

autopsies of all patients who die of suspected CJD. New Jersey is among the states that

require reporting by medical providers of suspected case-patients with CJD. Because

physicians nationally and in NJ generally do an inadequate job of reporting all reportable

diseases, states use additional methods to ensure as complete ascertainment of cases of

interest as possible. The NJDHSS currently also reviews NJ hospital billing data and

death certificate data to detect case-patients with CJD that were not reported by

physicians, making it more likely to have complete ascertainment of case-patients than

other states. The CDC also reviews national mortality data from the National Center for

Health Statistics. When a CJD case-patient under 55 years of age is reported, the CDC

contacts the state health department to review the details of this case-patient.

The NJDHSS periodically sends out to all physicians in the state, and to every newly

licensed physician, information on reporting requirements for all diseases and conditions

listed in the Administrative Code regulations (N.J.A.C. 8:57), including CJD. The

NJDHSS plans to notify physicians in the next few months about the recently adopted

revised regulations pertaining to all reportable diseases, including materials such as

pocket cards and magnets to remind physicians and other health care providers about

their reporting responsibilities.

In addition, the NJDHSS will be sending a specific letter prepared by the NPDPSC to

neurologists and pathologists, informing them of the need to consider CJD in the

differential diagnosis of a patient with a rapidly progressive neurological disorder with

dementia, and the free services provided by the NPDPSC. Furthermore, the NPDPSC is

prepared to make arrangements and provide for the costs of the autopsy.

The NJDHSS will continue to review all suspected cases-patients with CJD detected

through passive reporting by health providers, and by reviewing hospital billing data and

death certificate data. It will encourage physicians to obtain permission from family

members for autopsies of suspected case-patients at the time of death and submit brain

tissue and other laboratory data to the NPDPSC for expert analysis.

The CJD program at the CDC also has notified the NJDHSS that it intends to provide

limited funds, as it does to several other states, to enhance CJD surveillance activities in

the state. The NJDHSS plans to enhance education on prion disease, targeted to

neurologists and pathologists, the physicians most likely to be in a position to diagnose a

potential case of CJD. Unfortunately, autopsies in general are now only rarely done and

this is especially true for suspected CJD case-patients where pathologists are reluctant to

perform autopsies (Louie at al, 2004). However, as a result of the effort made by the

autopsy program of the NPDPSC, the number of autopsies performed in CJD casepatients

is currently close to 60% of the number of expected cases.

2. Continued evaluation of the suspected CJD cluster at the GSRT

The NJDHSS will update its report on the suspected cluster of CJD case-patients at the

GSRT once it receives the information from the pending case-patients. If there are

additional reported case-patients that need to be assessed, the NJDHSS will evaluate

those that were NJ residents, and will refer the non-NJ resident case-patients to other

states for evaluation, where appropriate.

Given the epidemiologic and pathologic evidence described above, the NJDHSS believes

that conducting a case-control study by interviewing next-of-kin or friends of the definite

and probable cases is unwarranted at this point for assessing the proposed hypothesis that

BSE-contaminated beef in the US caused an increased incidence or cluster of CJD cases

in New Jersey. The proposed hypothesis is not supported by the weight of the evidence

presented in this report and the CDC report (CDC, 2004a) to justify conducting such a

research project that is very unlikely to yield meaningful information.


May 7, 2004 / 53(Early Release);1-4

Creutzfeldt-Jakob Disease Not Related to a Common Venue --- New Jersey, 1995--2004

Beginning in June 2003, the New Jersey Department of Health and Senior Services (NJDHSS) and CDC were notified of a suspected cluster of deaths caused by Creutzfeldt-Jakob disease (CJD) in persons reportedly linked to Garden State Racetrack in Cherry Hill, New Jersey. Concerns were raised that these deaths might have resulted from consumption of meat contaminated with the agent causing bovine spongiform encephalopathy (BSE, commonly called "mad cow disease") served at racetrack restaurants during 1988--1992. Consumption of BSE-contaminated cattle products has been linked to a new variant form of CJD (vCJD) in humans. This report summarizes the results of an investigation that determined the deaths were not linked causally to a common source of infection. The findings underscore the need for physicians to arrange for brain autopsies of all patients with clinically suspected or diagnosed CJD.

Available clinical and neuropathologic findings were reviewed for 17 suspected CJD deaths referred to NJDHSS and CDC. To investigate the deaths of these 17 persons, all of whom were reportedly linked to Garden State Racetrack, health-care providers were contacted and relevant medical records obtained by NJDHSS, other state health departments, and CDC. Providers were asked to submit available brain autopsy tissue to the National Prion Disease Pathology Surveillance Center (NPDPSC), a national prion disease diagnostic referral laboratory established by CDC and the American Association of Neuropathologists.

Sufficient demographic and clinical information was available to classify 11 of the 17 deaths as resulting from a definite or probable case of a classic form of CJD*, on the basis of World Health Organization criteria (1). Of the remaining six decedents, neuropathologic analyses documented that three deaths resulted from causes unrelated to either vCJD or classic CJD (Table 1). Three deaths reported as resulting from CJD remain under investigation. Excluding the three deaths for which CJD was ruled out, the 14 remaining deaths occurred over a period of approximately 9.25 years (1995--2004); the average number of cases per complete year (i.e., excluding 2004) was 1.44 (range: zero to three cases). Eleven of the 14 decedents were male; median age was 69.5 years (range: 50--83 years). Six of the decedents resided in New Jersey, four in Pennsylvania, and one each in Connecticut, Delaware, Maryland, and Virginia.

Neuropathologic analysis in the five definite cases with available brain tissue specimens was diagnostic of classic CJD; none had the characteristic pathologic findings of vCJD. A genotype at codon 129 of the prion protein gene (a genetic marker associated with specific subtypes of CJD) was determined for three of the five CJD deaths confirmed pathologically (Table 1). None of the decedents had the methionine homozyogosity or the characteristic Western blot pattern present for persons with vCJD. In addition, the reported CJD subtypes differed from one another. For the six deaths without tissue diagnosis, available clinical and diagnostic evidence, including illness duration, electroencephalographic patterns, and presence of protein 14-3-3 (a marker for classic CJD) in cerebrospinal fluid was consistent with a probable diagnosis of classic CJD (Tables 1 and 2). None of the decedents had a diagnosis of vCJD.

For 1995--2002, using CDC's national multiple cause-of-death file (2002 data are preliminary) compiled annually by the National Center for Health Statistics, the annual death rate from CJD in the United States has been stable at approximately one case per 1 million persons per year (Figure 1). The CJD death rate for New Jersey during the same period was similar.

In 2001, Garden State Racetrack was closed permanently. The number and ages of all persons visiting or dining at the racetrack is unknown. However, according to New Jersey Racing Commission records, attendance at the racetrack during 1988--1992 was approximately 4.1 million. Based on an annual CJD rate of 3.4 cases per 1 million persons (CDC, unpublished data, 2004) and an overall death rate from all causes of 2.9% for persons aged >50 years, the occurrence over approximately 9.25 years (1995--2004) of at least 14 CJD-related deaths among as few as 300,000 persons aged >50 years would not be unusual. This number is within the estimated range of the number of persons attending and dining at the racetrack, given the known attendance.

Reported by: P Gambetti, MD, National Prion Disease Pathology Surveillance Center, Case Western Reserve Univ, Cleveland, Ohio. J Hadler, MD, Connecticut Dept of Public Health. A Hathcock, PhD, M Drees, MD, Delaware Health and Social Svcs. D Blythe, MD, Maryland Dept of Health and Mental Hygiene. E Bresnitz, MD, M Gerwel, MD, New Jersey Dept of Health and Senior Svcs. M Hawkins, MD, Philadelphia Dept of Public Health; A Weltman, MD, Pennsylvania Dept of Health. J Marr, MD, A Buckler, MD, C Novak, MD, Virginia Health Dept. C Rothwell, MS, K Kochanek, MA, R Anderson, PhD, Div of Vital Statistics, National Center for Health Statistics; J Sejvar, MD, E Belay, MD, R Maddox, MPH, A Curns, MPH, R Holman, MS, L Schonberger, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

Editorial Note:

CJD is a neurodegenerative disease characterized by rapidly progressive dementia associated with brain pathology marked by diffuse spongiform degeneration; the disease is invariably fatal (2). According to the leading hypothesis, CJD is caused by an unconventional transmissible agent, an abnormal protein (i.e., prion) that is able to induce abnormal folding of normal cellular proteins, leading to neuronal death. Prions are believed to cause transmissible spongiform encephalopathies (TSEs) that include scrapie in sheep, BSE in cattle, chronic wasting disease (CWD) in deer and elk, and CJD in humans.

Two major forms of CJD have been recognized, classic and variant (3). Classic CJD has been recognized since the early 1920s and is characterized by certain distinct clinical and diagnostic features (Table 2). The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual (4). Risk increases with age, and in persons aged >50 years, the annual rate is approximately 3.4 cases per million.

Variant CJD was first described in 1996 in the United Kingdom and has different clinical characteristics than classic CJD (Table 2) (2,3). The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD (Figure 2). In addition, all vCJD cases have neuropathologic findings distinctly different from those of classic CJD (5), and all have had a particular genetic profile (i.e., homozygosity for methionine) at codon 129 of the prion protein gene (4). Thus, cases of vCJD can be distinguished from classic CJD on the basis of clinical and pathologic data. Epidemiologic and laboratory evidence indicate that the agent causing BSE in cattle can be transmitted to humans via consumption of BSE-contaminated cattle products, causing vCJD (2,3). However, this evidence also suggests that the risk is low for having vCJD, even after consumption of contaminated product. In 1996, because of the emergence of vCJD in the United Kingdom, CDC enhanced its surveillance for CJD in the United States (6).

No evidence has indicated that any of the 17 reported deaths resulted from vCJD. The CJD subtypes were determined in four decedents, and the subtype in each differed from the others; this heterogeneity provides scientific evidence against a common etiology for these cases. Although one study reported that BSE-infected mice expressing methionine homozygosity at codon 129 produced prions with a molecular phenotype consistent with a subtype of classic CJD (7), these animal data cannot be reliably extrapolated to humans in the absence of other supporting evidence. In 2003, the Spongiform Encephalopathy Advisory Committee of the United Kingdom concluded that these data did "not provide strong evidence to support" the hypothesis that exposure to BSE can produce a sporadic CJD-like phenotype in humans (8). In the United Kingdom, where the largest epidemic of BSE has occurred and an unusually large proportion of the population has been exposed to the BSE agent, the absence of an unusually high incidence of classic CJD patients or an elevated proportion of CJD patients with methionine homozygosity at codon 129 (9) supports the lack of association between BSE and sporadic CJD. In the United Kingdom, prion disease experts have looked specifically for evidence of BSE-related disease other than vCJD among classic CJD cases. No evidence of a new phenotype has been uncovered (R.G. Will, M.D., National CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland, personal communication, 2004).

Neuropathologic evaluation, particularly by immunohistochemistry or Western blot, is the most definitive method to 1) diagnose human prion diseases, 2) monitor for vCJD and various subtypes of CJD, and 3) detect the possible emergence of new prion diseases in the United States. Although not all decedents in this investigation had pathologic specimens available for review, demonstration of the absence of a classic CJD or vCJD diagnosis in certain patients and diagnosis of classic CJD in others indicated these patients did not die from BSE-related disease. This investigation underscores the need for physicians to pursue autopsies of all decedents with clinically suspected and diagnosed CJD and to use the TSE diagnostic services provided free of charge by NPDPSC. Information regarding this surveillance center is available at or by telephone, 404-639-3091.

CDC will continue to work with and support state health officials in New Jersey and nationally to conduct surveillance for CJD. Better defining the normal occurrence of subtypes of sporadic CJD and other TSEs will facilitate earlier recognition of vCJD or any other human prion disease that might emerge in the United States.

* Those types of CJD that differ from vCJD and usually indicate sporadic CJD.


World Health Organization. Global surveillance, diagnosis, and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation, 1998. WHO/EMC/ZDI/98.9. Available at http://www.
Belay E, Schonberger L. Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Clin Lab Med 2002;22:849--62.
Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerg Infect Dis 2001;7:6--16.
Will RG, Alpers MP, Dormont D, Schonberger LB. Infectious and sporadic prion diseases. In: Prusiner SB, ed. Prion Biology and Diseases. New York, New York: Cold Spring Harbor Laboratory Press, 2004:629--71.
Ironside JW. Neuropathologic findings in new variant CJD and experimental transmission of BSE. FEMS Immunol Med Microbiol 1998; 21:91--5.
Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003;60:176--81.
Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propogate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002;23:6358--66.
European Spongiform Encephalopathy Advisory Committee. Final minutes of the 77th annual meeting, February 11, 2003. Available at
Maddox RA, Belay ED, Schonberger LB. Reply to Singletary. Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States (Letter). 2003. Available at

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The Queniborough CJD cluster

The Times - front page
Alert on village's cluster of CJD deaths
AN URGENT government investigation was underway last night into a
cluster of cases of the human form of "mad cow" disease in
Leicestershire, where four people have died.
The investigation follows advice that the four deaths and another
probable case - out of only 75 nationwide - were unlikely to have
occurred by chance. And experts are pointing to the village of
Queniborough as a possible link between them.
Glenn Day, 35, lived there, Stacey Robinson, 18, was a former resident,
and Pamela Beyless, 24, often visited. All three died in 1998
A 19-year-old man who died in May and a 24-year-old who is thought to
have CJD also come from the same part of Leicestershire. It was the
fifth diagnosis in the area that triggered the investigation.


and how far does the Queniborough CJD cluster really reach ?

I was just looking at this email you sent me. Thurcaston
is about 6 miles from Queniborough - near enough. Is there
any way of contacting this family?


I've just been talking to professor bob will who runs the CJD
surveillance unit. He is very keen to learn more about the US case you
mentioned to me - the woman who lived in Thurmaston. So am I.

Could you contact them asap and ask if they will speak to us?


Sadly, everything points to Mrs Soukup being a victim of sporadic CJD.
In particular, she died aged 74 - whereas the oldest victim to date has
been 54. Also she and her husband were in britain in the 1970s - before
any cattle had come down with BSE. IN fact before any of the cattle
destined to get BSE had even been born. Despite this Professor Bob Will
does want to speak to the Soukups to find out more about the case.


personal email tss

since then, we have a documented case of nv/v CJD in 74 year old.

1: Cent Eur J Public Health 2003 Mar;11(1):19-22

Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases
in Orava and Liptov regions (northern Slovak focus) 1983-2000.

Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T.

Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius
University, Sklabinska 26, Martin, 037 53 Slovakia.

While familial cases of Creutzfeldt-Jakob disease are extremely rare
all over the world, 3 familial clusters were observed between
1983-2000 in a relatively small area situated in the North of
Slovakia. Prevalence of CJD in this area exceeded the overall
prevalence in Slovakia more than 8 times. The majority of CJD
patients admitted consuming sheep brain. Most patients lived in
small secluded villages with rather common familial intermarriage.
CJD affected both sexes equally. All patients were prior to the
disease mentally normal individuals. Shortly after the onset of CJD
their mental status deteriorated remarkably with an average survival
rate of 3.6 months.

PMID: 12690798


1: Eur J Epidemiol 1991 Sep;7(5):520-3

"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.

Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.

Institute of Preventive and Clinical Medicine, Bratislava.

Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in
1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a
coincidence of genetic and environmental risks in clustering patients.
Since Spongiform Encephalopathies might be transmitted orally, (Bovine
Spongiform Encephalopathy), the possibility of zoonotic source of CJD
cases in Orava was also considered. A deficient knowledge about the
occurrence of scrapie in Slovakia stimulated an examination of sheep
with signs of CNS disorders in two flocks of Valasky breed in Orava. In
one flock, neurohistopathological examination revealed in sheep brains
lesions characteristic for scrapie. Frozen brain tissue of these animals
were used for the detection of scrapie associated fibrils. They were
found in 2 animals from the same flock. This is the first laboratory
confirmation of scrapie in Czecho-Slovakia. The possible epidemiological
and economical implications are emphasized.

Morbidity and Mortality Weekly Report

Weekly February 21, 2003 / Vol. 52 / No. 7

Fatal Degenerative Neurologic Illnesses in Men Who Participated

in Wild Game Feasts — Wisconsin, 2002

depar department tment of health and human ser services vices

Centers for Disease Control and Prevention


128 Atrial Fibrillation as a Contributing Cause of Death and

Medicare Hospitalization — United States, 1999

131 Potential Exposures to Airborne and Settled Surface Dust

in Residential Areas of Lower Manhattan Following the

Collapse of the World Trade Center — New York City,

November 4–December 11, 2001

136 Smallpox Vaccine Adverse Events Among Civilians —

United States, January 24–February 18, 2003

136 Notice to Readers

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder

in humans. CJD is one of a group of conditions known as

transmissible spongiform encephalopathies (TSEs), or prion

diseases, that are believed to be caused by abnormally configured,

host-encoded prion proteins that accumulate in the central

nervous tissue (1). CJD has an annual incidence of

approximately 1 case per million population in the United

States (1) and occurs in three forms: sporadic, genetically

determined, and acquired by infection. In the latter form, the

incubation period is measured typically in years. Recent evidence

that prion infection can cross the species barrier

between humans and cattle has raised increasing public health

concerns about the possible transmission to humans of a TSE

among deer and elk known as chronic wasting disease (CWD)

(2). During 1993–1999, three men who participated in wild

game feasts in northern Wisconsin died of degenerative neurologic

illnesses. This report documents the investigation of

these deaths, which was initiated in August 2002 and which

confirmed the death of only one person from CJD. Although

no association between CWD and CJD was found, continued

surveillance of both diseases remains important to assess

the possible risk for CWD transmission to humans.

Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years

with a history of seizures since 1969 sought treatment for

recurring seizures, increasing forgetfulness, and worsening

hand tremors. Electroencephalographic (EEG) examination

demonstrated focal epileptiform activity and nonspecific diffuse

abnormalities, but no specific diagnosis was made. In

February 1993, he was hospitalized for increasing confusion,

ataxia, and movement tremors of his extremities. A magnetic

resonance image (MRI) demonstrated mild, nonspecific

enhancement along the inferior parasagittal occipital lobe.

A repeat EEG showed bifrontal intermittent, short-interval,

periodic sharp waves, suggesting a progressive encephalopathy;

a diagnosis of CJD was suspected. The man died later

that month; neuropathologic examination of brain tissue during

autopsy indicated subacute spongiform encephalopathy,

compatible with CJD.

The man was a lifelong hunter who ate venison frequently.

He hunted primarily in northern Wisconsin but also at least

once in Montana. He hosted wild game feasts at his cabin in

northern Wisconsin from 1976 until shortly before his death.

Fixed brain tissue obtained during the autopsy was sent for

analysis to the National Prion Disease Pathology Surveillance

Center (NPDPSC) and reexamined at the institution where

the autopsy was conducted. Histopathologic examination did

not substantiate the diagnosis of prion disease. In addition,

27 brain tissue sections were negative for prions by

immunostaining despite positive antibody reactions against

other proteins (controls), which indicated that other epitopes

in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with

no previous history of a neurologic disease sought evaluation

and treatment following a 3-month history of progressive difficulty

in writing and unsteadiness of gait. A computerized

126 MMWR February 21, 2003


Centers for Disease Control and Prevention. [Article

Title]. MMWR 2003;52:[inclusive page numbers].

Centers for Disease Control and Prevention

Julie L. Gerberding, M.D., M.P.H.


David W. Fleming, M.D.

Deputy Director for Public Health Science

Dixie E. Snider, Jr., M.D., M.P.H.

Associate Director for Science

Epidemiology Program Office

Stephen B. Thacker, M.D., M.Sc.


Office of Scientific and Health Communications

John W. Ward, M.D.


Editor, MMWR Series

Suzanne M. Hewitt, M.P.A.

Managing Editor, MMWR Series

David C. Johnson

(Acting) Lead Technical Writer/Editor

Jude C. Rutledge

Teresa F. Rutledge

Jeffrey D. Sokolow, M.A.


Lynda G. Cupell

Malbea A. Heilman

Visual Information Specialists

Quang M. Doan

Erica R. Shaver

Information Technology Specialists

Division of Public Health Surveillance

and Informatics

Notifiable Disease Morbidity and 122 Cities Mortality Data

Robert F. Fagan

Deborah A. Adams

Felicia J. Connor

Lateka Dammond

Patsy A. Hall

Pearl C. Sharp

The MMWR series of publications is published by the

Epidemiology Program Office, Centers for Disease Control

and Prevention (CDC), U.S. Department of Health and

Human Services, Atlanta, GA 30333.

tomography (CT) scan and MRI examination of his head did

not indicate any abnormality. In June 1999, he was hospitalized

following onset of dementia, speech abnormalities, and

myoclonic jerking. An EEG indicated left-hemispheric periodic

sharp waves and moderate generalized background slowing;

CJD was diagnosed clinically. In July 1999, following

worsening symptoms and development of right upper extremity

dystonia, the patient died. Neuropathologic evaluation of

brain tissue during autopsy demonstrated widespread subcortical

spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison.

He made an estimated 12 visits to the cabin where the

wild game feasts were held, but he participated in only one

feast during the mid-1980s. Sections of fixed and frozen brain

tissue obtained during autopsy were analyzed at NPDPSC,

and prion disease was confirmed by immunohistochemical

and Western blot testing. The Western blot characteristics and

prion disease phenotype in this patient were consistent with

the most common form of sporadic CJD, classified as M/M

(M/V) 1 (3). Subsequent genetic typing confirmed the presence

of methionine homozygosity (M/M) at codon 129 of

the patient’s prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought

treatment for progressive slowing of speech, worsening

memory, and personality changes. By January 1993, his speech

was reduced to one-word utterances. Neurologic examination

showed a flat affect, decreased reflexes, and apraxia. A CT

head scan showed mild atrophy, and an EEG was normal.

Pick’s disease was diagnosed. By May, he was unable to perform

any daily living activities; he died in August 1993. Neuropathologic

evaluation of brain tissue during autopsy showed

symmetrical frontal lobe cerebral cortical atrophy and mild

temporal lobe atrophy. No Pick’s bodies or spongiform

lesions were observed.

The man had a history of eating venison and participated

regularly in wild game feasts held at the cabin owned by

patient 1. He was a lifelong hunter and hunted mostly in

Wisconsin but also in Wyoming and British Columbia. No

game was brought to the wild game feasts from his hunting

trips outside of Wisconsin. Examination of fixed brain tissue

sent to NPDPSC demonstrated no lesions indicative of CJD,

and immunohistochemical testing with antibody to the prion

protein did not demonstrate the granular deposits seen in prion


Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other

game that occurred at a cabin in northern Wisconsin owned

by patient 1 began in 1976 and continued through 2002.

Vol. 52 / No. 7 MMWR 127

These feasts typically involved 10–15 participants and usually

occurred on weekends before or during hunting seasons

in the fall and occasionally in the spring. Wild game brought

to these feasts usually were harvested in Wisconsin, but three

men who attended these feasts reported hunting in the western

United States and bringing game back to Wisconsin. These

activities took place in Colorado (near the towns of Cortez,

Trinidad, Collbran, Durango, and Meeker), Wyoming (near

the towns of Gilette and Cody), and Montana (near the town

of Malta). CWD was not known to be endemic in these areas

at the time that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who

were identified as possibly participating in the wild game feasts;

all were male. Information was obtained by direct interview

or from family members of decedents. Of the 45 persons, for

whom information was obtained, 34 were reported to have

attended wild game feasts. Seven of the 34 feast attendees

were deceased, including the three patients. None of the four

other decedents had a cause of death attributed to or associated

with a degenerative neurologic disorder. None of the living

participants had any signs or symptoms consistent with a

degenerative neurologic disorder.

Reported by: JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD,

R Wierzba, Div of Public Health, State of Wisconsin Dept of Health

and Family Svcs. P Gambetti, National Prion Disease Pathology

Surveillance Center, Case Western Reserve University, Cleveland, Ohio.

L Schonberger, MD, R Maddox, MPH, E Belay, MD, Div of Viral and

Rickettsial Diseases, National Center for Infectious Diseases; V Hsu,

MD, EIS Officer, CDC.

Editorial Note: CWD was first described in the United States

in the 1960s and classified as a TSE in 1978. Previously localized

to a contiguous endemic area in northeastern Colorado

and southeast Wyoming, since 2000, CWD has been found

in free-ranging deer or elk in Illinois, Nebraska, New Mexico,

South Dakota, Wisconsin, and outside the previously known

endemic areas of Colorado and Wyoming. CWD has been

identified also in captive deer or elk in Colorado, Kansas,

Minnesota, Montana, Nebraska, Oklahoma, South Dakota,

and Wisconsin (4). Because a variant form of CJD, with specific

neuropathologic and molecular characteristics that distinguish

it from sporadic CJD, has been associated with eating

cattle products infected with a prion that causes bovine

spongiform encephalopathy (5), concern has been raised about

the possibility that the prion associated with CWD might be

transmitted to humans in a similar way.

In this investigation, because only one of the three cases in

Wisconsin had neuropathologic confirmation of a prion disease,

no association could be made between case participation

in the wild game feasts and the development of CJD.

Although patient 2 had confirmed CJD, he was unlikely to

have eaten CWD-infected venison at these feasts because venison

and other game from outside Wisconsin that was served

at these feasts did not originate from known CWD-endemic

areas, and the man participated in the feasts only once. In

addition, the prion disease in this case was consistent with

the most common form of sporadic CJD, without apparent

unusual neuropathologic or molecular characteristics that

might occur if the prion related to CWD had been responsible

for the disease.

The findings in this report are subject to at least two limitations.

First, not all members participating in wild game feasts

could be identified, and not all persons listed as participating

could be contacted for interviews. Second, interviews that were

conducted required recall of events that occurred up to 25

years ago, limiting the detail or accuracy of events. However,

the similar responses obtained from different sources support

the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients

in whom the transmission of CWD was suspected also did

not provide convincing evidence for a causal relationship

between CWD and CJD (2). However, limited epidemiologic

investigations cannot rule out the possibility that CWD

might play a role in causing human illness. Ongoing surveillance

of CJD, particularly in states with CWD, is important

to assess the risk, if any, for CWD transmission to humans.

Because the confirmation of CJD and the detection of a new

prion disease require neuropathologic study of brain tissue,

physicians are encouraged to contact NPDPSC (http://; telephone, 216-368-0587) to confirm

diagnoses of CJD and to distinguish its various subtypes.

Because of the known severity of TSEs in humans and the

possibility that the CWD prion can affect humans, animals

with evidence of CWD should be excluded from the human

food or animal feed chains. Hunters and wild venison consumers

should follow precautionary guidelines available from

the Wisconsin Department of Agriculture, Trade, and Consumer

Protection (

to prevent potential exposures to the CWD agent.


1. Belay E. Transmissible spongiform encephalopathies in humans. Annu

Rev Microbiol 1999;53:283–314.

2. Belay E, Gambetti P, Schonberger L, et al. Creutzfeldt-Jakob disease in

unusually young patients who consumed venison. Arch Neurol


3. Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt-

Jakob disease based on molecular and phenotypic analysis of 300 subjects.

Ann Neurol 1999;46:224–33.

4. U.S. Department of Agriculture. Positive CWD cases: cumulative

through Dec 2002 (including farm herds already depopulated). Available



5.Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-

Jakob disease in the UK. Lancet 1996;347:921–5.


Chronic Wasting Disease and Potential Transmission to Humans
Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from:


Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.


Transmission to Other Animals
Concerns have been raised about the possible transmission of the CWD agent to domestic animals, such as cattle and sheep, which may come in contact with infected deer and elk or CWD-contaminated environments. If such transmissions were to occur, they would potentially increase the extent and frequency of human exposure to the CWD agent. In addition, passage of the agent through a secondary host could alter its infectious properties, increasing its potential for becoming more pathogenic to humans. This phenomenon may have occurred with BSE when a strain of scrapie, a possible original source of the BSE outbreak, changed its pathogenic properties for humans after infecting cattle. However, the exact origin of BSE remains unknown.

Although CWD does not appear to occur naturally outside the cervid family, it has been transmitted experimentally by intracerebral injection to a number of animals, including laboratory mice, ferrets, mink, squirrel monkeys, and goats (1,26). In an experimental study, the CWD agent was transmitted to 3 of 13 intracerebrally injected cattle after an incubation period of 22 to 27 months (27). The susceptibility of cattle intracerebrally challenged with the agent of this disease was substantially less than that observed after intracerebral scrapie challenge: nine of nine cattle succumbed to scrapie challenge after intracerebral injection (28). In ongoing experimental studies, after >6 years of observation, no prion disease has developed in 11 cattle orally challenged with the CWD agent or 24 cattle living with infected deer herds (E.S. Williams and M.W. Miller, unpub. data) (1). In addition, domestic cattle, sheep, and goat residing in research facilities in close contact with infected cervids did not develop a prion disease.

Analysis by immunohistochemical studies of the tissue distribution of prions in CWD-infected cervids identified the agent in the brain, spinal cord, eyes, peripheral nerves, and lymphoreticular tissues (Table 1) (29,30). Distribution of the CWD agent outside of the brain seems to be less widespread in elk than in deer (2). Involvement of the tonsils and peripheral nerves early in the course of experimental and natural prion infection suggests the possible involvement of the lymphoreticular and peripheral nervous systems in the pathogenesis and transmission of the disease (2,12,30,31).

Risk for Transmission to Humans
Epidemiologic Studies
The increasing detection of CWD in a wider geographic area and the presumed foodborne transmission of BSE to humans, resulting in cases of vCJD, have raised concerns about the possible zoonotic transmission of CWD (32). In the late 1990s, such concerns were heightened by the occurrence of CJD among three patients 30 years of age who were deer hunters or ate deer and elk meat harvested by family members (Table 2). However, epidemiologic and laboratory investigations of these case-patients indicated no strong evidence for a causal link between CWD and their CJD illness (33). None of the patients were reported to have hunted deer or eaten deer meat harvested in the CWD-endemic areas of Colorado and Wyoming. Such a history in unusually young CJD patients, if present, would have supported a causal link with CWD. Moreover, the testing of brain tissues from >1,000 deer and elk harvested from areas where the patients hunted or their venison originated did not show any evidence of CWD (33). In addition, the lack of homogeneity in the clinicopathologic manifestation and codon 129 of the prion protein gene among the three patients suggested that their illnesses could not be explained by exposure to the same prion strain. In vCJD, homogeneity of the genotype at codon 129 and the clinical and pathologic phenotype were attributed to the patients' exposure to the same prion strain, the agent of BSE.

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ≈22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.

In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient's disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36).

The second patient died from an illness lasting <16 months. The patient's illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient's death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient's parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin.

Recently, rare neurologic disorders resulting in the deaths of three men who participated in "wild game feasts" in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick's disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming.

In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35).

To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient's CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient's illness was consistent with the MM1 CJD phenotype.

Despite the decades-long endemicity of CWD in Colorado and Wyoming, the incidence of CJD and the age distribution of CJD case-patients in these two states are similar to those seen in other parts of the United States. From 1979 to 2000, 67 CJD cases from Colorado and 7 from Wyoming were reported to the national multiple cause-of-death database. The average annual age-adjusted CJD death rate was 1.2 per million persons in Colorado and 0.8 in Wyoming. The proportion of CJD patients who died before age 55 in Colorado (13.4%) was similar to that of the national (10.2%). The only CJD case-patient <30 years of age in Colorado had iatrogenic CJD linked to receipt of human growth hormone injections. CJD was not reported in persons <55 years of age in Wyoming during the 22-year surveillance period. ...snip...end


Title: Experimental Transmission of Chronic Wasting Disease (Cwd Mule Deer) Agent to Cattle by Intracerebral Route


Hamir, Amirali
Kunkle, Robert - bob
Cutlip, Randall - ARS RETIRED
Miller, Janice - ARS RETIRED
O'Rourke, Katherine
Williams, Elizabeth - UNIVERSITY OF WYOMING
Chaplin, Melanie - VET SERVICES AGENCY, UK
Richt, Juergen

Submitted to: Journal Of Veterinary Diagnostic Investigation
Publication Acceptance Date: January 3, 2005
Publication Date: May 1, 2005
Citation: Hamir, A.N., Kunkle, R.A., Cutlip, R.C., Miller, J.M., Orourke, K.I., Williams, E.S., Miller, M.W., Stack, M.J., Chaplin, M.J., Richt, J. 2005. Experimental Transmission Of Chronic Wasting Disease Agent To Cattle By Intracerebral Route. Journal Of Veterinary Diagnostic Investigation. 17:276-281.

Interpretive Summary: This communication reports final observations on experimental transmission of chronic wasting disease (CWD) from mule deer to cattle. Thirteen calves were inoculated into the brain with brain suspension from mule deer naturally affected with CWD. Three other calves were kept as uninoculated controls. The experiment was terminated 6 years post inoculation (PI). During that time, abnormal prion protein was demonstrated in the brain and spinal cord of 5 cattle by laboratory tests. However, consistent clinical signs and microscopic changes were not seen in any of these cattle. Age related changes were seen in both inoculated and control cattle. Findings of this study show that only 38% of the inoculated cattle were positive for CWD agent. Although inoculation directly into the brain is an unnatural route of exposure, and is the most severe challenge possible, this experiment shows that CWD transmission in cattle could have long incubation periods (up to 5 years). This finding suggests that oral exposure of cattle to CWD agent, a more natural potential route of exposure, would require not only a much larger dose of inoculum, but also, may not result in amplification of CWD agent within brain and spinal cord tissues during the normal lifespan of cattle. It is possible that a second bovine passage of material (cattle brain infected with CWD) from this study may result in a larger incidence of affected cattle with a shortened incubation time, and may produce different clinical and pathological findings. Such a study is now in progress. Also, experimental inoculations of cattle with CWD isolates from white-tailed deer and elk are needed to compare findings with the present study and these studies will be initiated in the near future. Impact: Results of this study show that although cattle inoculated directly into the brain with CWD succumb to the disease, the attack rate was rather small (38%) with this unnatural route of transmission. It is speculated that the oral route of infection may not result in replication of the agent during normal lifespan of cattle.
Technical Abstract: This communication reports final observations on experimental transmission of chronic wasting disease (CWD) from mule deer to cattle by the intracerebral route. Thirteen calves were inoculated intracerebrally with brain suspension from mule deer naturally affected with CWD. Three other calves were kept as uninoculated controls. The experiment was terminated 6 years post inoculation (PI). During that time, abnormal prion protein (PrPres) was demonstrated in the central nervous system (CNS) of 5 cattle by both immunohistochemistry (IHC) and Western blot (WB). However, microscopic lesions suggestive of spongiform encephalopathy in the brains of these PrPres positive animals were subtle in 3 cases and absent in 2 cases. The 3 uninoculated control cattle and 8 other inoculated animals euthanized during this time did not have PrPres in their CNS. Degenerative changes indicative of neuroaxonal dystrophy (NAD) were seen in dorsal medulla oblongata and appeared to be related to advancing age in both inoculated and control cattle. Analysis of the gene encoding bovine PRNP revealed homozygosity for alleles encoding 6 octapeptide repeats, serine (S) at codon 46 and S at codon 146 in all samples. Findings of this study show that although PrPres amplification occurred following direct inoculation into the brain, none of the affected animals had classic histopathologic lesions of spongiform encephalopathy. Furthermore, only 38% of the inoculated cattle demonstrated amplification of PrPres. Although intracerebral inoculation is an unnatural route of exposure, and is the most severe challenge possible, this experiment shows that CWD transmission in cattle could have long incubation periods (up to 5 years). This finding suggests that oral exposure of cattle to CWD agent, a more natural potential route of exposure, would require not only a much larger dose of inoculum, but also, may not result in amplification of PrPres within CNS tissues during the normal lifespan of cattle.

Page Modified: 08/26/2005

North American Equity ResearchNew York13 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 StatesFoods
Pablo E. Zuanic(1-212) 622-6744pablo.zuanic@jpmorgan.comChristopher M.
Bledsoe(1-212) 622-6386christopher.m.bledsoe@jpmorgan.comDaniel

State of Our Views Regarding BSE in the US
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? The government's General Accounting Office says it has not; 2)
Can clusters of cases of sporadic CJD (or sCJD as it is commonly known)
really be a variant of CJD and indeed be linked to BSE (vCJD is the
scientific term for the disease linked to mad cow)?
In this note we focus on the issue of sCJD clusters.
Do sCJD Clusters Matter?
The media focus (and as a result, the consumer at large) since December
23, thus far, has been on the potential of new BSE-positive cows being
found, and on the various initiatives the authorities are taking to
prevent an outbreak of BSE. However, the apparent existence of sCJD
clusters in the US has received little publicity. If sporadic (or
spontaneous) CJD is really spontaneous, it should not be found in
population clusters. The fact that it indeed has been found in clusters
raises concerns.
Understanding the "Difference" Between sCJD and vCJD
Prior to 1996 there was only one known type of CJD, and it was called
“sporadic” or “spontaneous” because it was unclear where it came from,
or how it was generated. In 1996 scientists in England "discovered" a
"variant" of CJD (vCJD), which they indicated could be linked to the
animal form of the disease (BSE or Mad Cow). Experts kept vCJD separate
from sCJD because unlike the new vCJD the original sCJD could not be
directly linked to BSE. However, not enough is known to be fully certain
that sporadic CJD is truly spontaneous and has no external catalyst. The
other notable difference between vCJD and sCJD is the incubation period.
Whereas sCJD has an average incubation period of 40 years and is
exceedingly rare in young people, vCJD can affect people of all ages and
has a much shorter incubation period of just two to five years. An even
more relevant difference is that sCJD is found in 1 out of 1 million
people per annum, or 5,000 cases per year on a global basis, while only
180 human cases of vCJD (the type of CJD linked to BSE) have ever been
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.

The CDC Is Currently Investigating the New Jersey Cluster
The US Center for Disease Control (CDC) has opened an investigation into
a cluster of deaths in an area surrounding Cherry Hill, New Jersey.
Specifically, after dismissing the case when it was first brought to
their attention earlier in 2003, the agency has since reversed course
and on December 31, 2003 sought out information from Janet Skarbek.
Skarbek, a Cinnaminson, New Jersey CPA believes she has uncovered a
common link between seven deaths in the local area and a restaurant at
the now-closed Garden State Race Track. All of the deaths had first been
identified as the randomly occurring (one out of one million) cases of
sporadic Creutzfeldt-Jakob Disease (sCJD), and six of the deaths
occurred between 2000 and 2003.
Science Expanding its Knowledge of CJDs
Have Cases of sCJD Been Overlooked?
Dr. Omar Bagasra believes that a 29 year old that died of presumably
sCJD in the New Jersey cluster may have died from a new, mutated form of
CJD since sCJD has a typical incubation period of 40 years and is
limited to elderly patients in almost all cases. Moreover, he suspects
that the link between the seven local deaths (clustered geographically
and chronologically) indicates that the new form of the disease is
caused by some external catalyst, unlike the randomly occurring sporadic
CJD (sCJD). He adds, though, that there may actually have been other
unreported CJD-related deaths in the area since the disease is often
misdiagnosed as Alzheimer’s.
Diagnoses of Alzheimer’s Might Have Been Cases of CJD
Lawrence Schonberger, the CDC epidemiologist who contacted Janet Skarbek
on December 31, is quoted separately as saying that sCJD is
underreported on death certificates, and that about 14 percent of cases
are missed. In fact, due to similarities between sCJD and Alzheimer’s
disease, a 1998 Yale study found that as many as 13 percent of
Alzheimer’s deaths are actually sCJD, but conservative estimates place
this number closer to 1 percent. If we extrapolate this finding to the
50,000 Alzheimer’s deaths each year in the US, the number of actual sCJD
deaths per year is somewhere between 500 and 6500. But, for us this
raises additional questions, since at a rate of one per million, the US
should not experience much more than 300 sCJD deaths in a single year.
Furthermore, Alzheimer cases have grown 50-fold in the last 25 years
from 857 cases in 1979 to 50,000 cases in today (albeit part of the
increase could very well be attributed to improvements in reporting).
Can sCJD Be Caused by External Agents?
A recent study out of Imperial College in London has led some to believe
that the same prions that cause the BSE-related vCJD may also cause a
disease that manifests itself in a way that more closely resembles
sporadic CJD. John Collinge, the scientist that conducted the
experiment, is basing this assertion on findings in the study’s mice,
which were injected with BSE prions. As expected, some of the mice
developed symptoms from vCJD, but unexpectedly, others suffered from
symptoms that more closely resembled sCJD. If true, the implications are
significant, as it will force scientists to consider whether cases of
sporadic CJD may actually have been caused by consumption of
contaminated beef.
Does All This Matter?
For now we await the results of the CDC investigation of the New Jersey
cluster. Previous investigations have found clusters to be just
coincidences. While this may be the case, we believe that the media may
start focusing more on the issue of clusters, and that the debate could
raise consumers’ concerns about beef.
But even in the worst case scenario that these clusters were indeed
linked to BSE, one could still make the argument that these cases were
generated before the 1997 ban on animal feed for cattle was imposed, and
that hence chances of contamination since then are unlikely. Still, the
question lingers, has the 1997 ban been respected? Will consumers
concerns increase as the discussion of CJD clusters hits the national media?
Bottom Line: If no new cases of BSE-positive cows are found and the
issue of CJD clusters is disregarded by consumers, then the effect on
beef consumption will be negligible. On the other hand, new cases of
infected cows and/or a wider debate of CJD clusters could indeed have an
effect on beef sales.

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THE most disturbing factor of this topic are the new atypical TSEs
showing up in not only cattle, but also sheep, and no one knows yet
about how many different strains of cwdTSE in deer/elk. WITH evidence of
sporadic CJD being very similar to these atypical TSEs in cattle and sheep,
and the findings from Asante, Collinge et al that BSE prions propagate
as either nvCJD or sporadic CJD, the ramifications of these findings are
very very worrisome and should not go ignored any further. WITH
the fact that there are over 20 documented strains of scrapie, and the
most logical hypothesis is scrapie to BSE, why would one believe in
only one phenotype of TSE in the bovine. IN fact, we have a new study
of two distinct prion strains derived from BSE in mice. YOU must not
continue to ignore these studies;

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

THE new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic Creutzfeldt-Jakob disease

Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice

Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt-Jakob disease: Implications for
human health

In an experimental study of the transmissibility of BSE to the pig,
seven of 10 pigs, infected at 1-2 weeks of age by multiple-route
parenteral inoculation with a homogenate of bovine brain from
natural BSE cases developed lesions typical of spongiform

THE recent discoveries of previously unidentified strains of
Scrapie such as 221C44 and the Nor9845;


UK Strategy for Research and
Development on Human and Animal
Health Aspects of Transmissible
Spongiform Encephalopathies


WHEN in fact, the findings from Marsh and the findings at
MISSION, TEXAS support even further evidence that there
are further strains of TSE in the USA besides that one
accidentally documented BSE case in Washington on
Dec. 23, 2003;

In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - Report of a visit to the USA - April-May 1989 - G A H
Wells [head of England's main veterinary lab]

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

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

Gerald Wells: Report of the Visit to USA, April-May 1989


The general opinion of those present was that BSE, as an
overt disease phenomenon, _could exist in the USA, but if it did,
it was very rare. The need for improved and specific surveillance
methods to detect it as recognised...


It is clear that USDA have little information and _no_ regulatory
responsibility for rendering plants in the US...


3. Prof. A. Robertson gave a brief account of BSE. The US approach
was to accord it a _very low profile indeed_. Dr. A Thiermann showed
the picture in the ''Independent'' with cattle being incinerated and thought
this was a fanatical incident to be _avoided_ in the US _at all costs_...


To be published in the Proceedings of the
Fourth International Scientific Congress in
Fur Animal Production. Toronto, Canada,
August 21-28, 1988

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

R.F. Marsh* and G.R. Hartsough

•Department of Veterinary Science, University of Wisconsin-Madison, Madison,
Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092

Epidemiologic investigation of a new incidence of
transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
suggests that the disease may have resulted from feeding infected
cattle to mink. This observation is supported by the transmission of
a TME-like disease to experimentally inoculated cattle, and by the
recent report of a new bovine spongiform encephalopathy in


Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough
and Burger who demonstrated that the disease was transmissible with a long incubation
period, and that affected mink had a spongiform encephalopathy similar to that found in
scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965).
Because of the similarity between TME and scrapie, and the subsequent finding that the
two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was
concluded that TME most likely resulted from feeding mink scrapie-infecied sheep.
The experimental transmission of sheep scrapie to mink (Hanson et al., 1971)
confirmed the close association of TME and scrapie, but at the same time provided
evidence that they may be different. Epidemiologic studies on previous incidences of
TME indicated that the incubation periods in field cases were between six months and
one year in length (Harxsough and Burger, 1965). Experimentally, scrapie could not be
transmitted to mink in less than one year.
To investigate the possibility that TME may be caused by a (particular strain of
scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie
agent, including their sheep or goat sources, were inoculated into a total of 61 mink.
Only one mink developed a progressive neurologic disease after an incubation period of
22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused
by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent
from an unidentified source.


A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin
reported that many of his mink were "acting funny", and some had died. At this time, we
visited the farm and found that approximately 10% of all adult mink were showing
typical signs of TME: insidious onset characterized by subtle behavioral changes, loss of
normal habits of cleanliness, deposition of droppings throughout the pen rather than in a
single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over
their _backs like squirrels. These signs were followed by progressive deterioration of
neurologic function beginning with locomoior incoordination, long periods of somnolence
in which the affected mink would stand motionless with its head in the corner of the
cage, complete debilitation, and death. Over the next 8-10 weeks, approximately 40% of
all the adult mink on the farm died from TME.
Since previous incidences of TME were associated with common or shared feeding
practices, we obtained a careful history of feed ingredients used over the past 12-18
months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy
cattle and a few horses. Sheep had never been fed.

Experimental Transmission. The clinical diagnosis of TME was confirmed by
histopaihologic examination and by experimental transmission to mink after incubation
periods of four months. To investigate the possible involvement of cattle in this disease
cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally
with a brain suspension from affected mink. Each developed a fatal spongiform
encephalopathy after incubation periods of 18 and 19 months.

These findings suggest that TME may result from feeding mink infected cattle and
we have alerted bovine practitioners that there may exist an as yet unrecognized
scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). A new
bovine spongiform encephalopathy has recently been reported in England (Wells et al.,
1987), and investigators are presently studying its transmissibility and possible
relationship to scrapie. Because this new bovine disease in England is characterized by
behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be
confused with rabies in the United Stales and not be diagnosed. Presently, brains from
cattle in the United States which are suspected of rabies infection are only tested with
anti-rabies virus antibody and are not examined histopathologically for lesions of
spongiform encephalopathy.
We are presently pursuing additional studies to further examine the possible
involvement of cattle in the epidemiology of TME. One of these is the backpassage of
our experimental bovine encephalopathy to mink. Because (here are as yet no agent-
specific proteins or nucleic acids identified for these transmissible neuropathogens, one
means of distinguishing them is by animal passage and selection of the biotype which
grows best in a particular host. This procedure has been used to separate hamster-
adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The intracerebral
backpassage of the experimental bovine agent resulted in incubations of only four months
indicating no de-adaptation of the Stetsonville agent for mink after bovine passage.
Mink fed infected bovine brain remain normal after six months. It will be essential to
demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic
association is to be confirmed.

These studies were supported by the College of Agricultural and Life Sciences,
University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United
States Department of Agriculture. The authors also wish to acknowledge the help and
encouragement of Robert Hanson who died during the course of these investigations.

Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and
natural transmission. J. Infec. Dis. 115:393-399.
Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson,
D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861.
Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and
clinical observations. 3. Infec. Dis. 115:387-392.
Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the
transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible
diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460.
Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle?
Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary
Medicine. University of Arizona, pp 20.
Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M.,
Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy
in cattle. Vet. Rec. 121:419-420.


Office Note


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

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

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



The BSE Inquiry / Statement No 61D

Professor Robert Will

Issued 16/12/1999 (Scheduled to give oral evidence 16 December 1999)



54. In February 1996, Dr M Zeidler drew up a list of cases of CJD aged less than 30

years identified from the world literature (provided with my first statement

[WS061]). I contacted colleagues in other countries to ask for specific

information on four of these cases. In early March I received telephone calls

from colleagues confirming that:

· Plaques had not been identified on neuropathological examination in three

cases of CJD aged 14,16 and 20 years from the USA and Canada.

· That the single case of sporadic CJD that we had identified in the world

literature aged less than 30 years with plaque deposition was probably a

hereditary form of human prion disease.



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.


76 pages on hound study;

Department for Environment,
Food & Rural Affairs

Area 307, London, SW1P 4PQ
Telephone: 0207 904 6000
Direct line: 0207 904 6287


Mr T S Singeltary
P.O. Box 42
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 peer 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, .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


Subject: Sheep
Date: Sat, 12 Jun 2004 14:26:04 EDT

Mr. Singeltary.

I hope this finds you well. As you are aware I left the USDA last year. I can only update you on the sheep before that time. Contact was established with the UK on doing the bioassay studies. They agreed. However, we were prioritized after their own needs, hence the delay. I am aware that there are now additional labs in Europe running the mouse bioassay strain typing. You will have to contact USDA for further word.

Linda Detwiler


-------- Original Message -------- Subject: re-85th Meeting of SEAC - 30.11.04
Date: Tue, 21 Dec 2004 16:56:55 -0000
From: "Barlow, Tom (SEAC)"
To: "''"

Dear Mr SingeltaryThank you for you enquiry to the SEAC secretariat about mouse bioassayscommissioned by the USDA to investigate TSE cases in imported sheep. After making a number of enquiries, it appears that Defra were not involvedwith this work. However, it is possible that a UK research laboratory wascontacted by the USDA about such tests but I have been unable to find outany further information. You may wish to make further enquiries with theUSDA.

Yours sincerely

Tom Barlow
Dr Tom Barlow
Spongiform Encephalopathy Advisory Committee (SEAC) Secretariat
Area 108, 1A Page Street, London SW1P 4PQTel: 0207 904 6267

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

From: Terry S. Singeltary Sr. []
Sent: 02 December 2004 20:19
To: Dale, Tabitha J (SEAC)
Subject: re-85th Meeting of SEAC - 30.11.04

Hello Tabitha, A kind greetings from Texas. ...snip...end


USDA 2003

We have to be careful that we don't get so set in the way we do things that
we forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.
Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.


Dr. Detwiler: It seems a good idea, but I'm not aware of it.
Another important thing to get across to the public is that the negatives
do not guarantee absence of infectivity. The animal could be early in the
disease and the incubation period. Even sample collection is so important.
If you're not collecting the right area of the brain in sheep, or if
collecting lymphoreticular tissue, and you don't get a good biopsy, you
could miss the area with the PRP in it and come up with a negative test.
There's a new, unusual form of Scrapie that's been detected in Norway. We
have to be careful that we don't get so set in the way we do things that we
forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.



Completely Edited Version

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado



Daily Update

On August 17, 2005, no inconclusive test results were reported.

National Veterinary Services Laboratory (NVSL) Immunohistochemistry (IHC)
Testing Summary

The BSE enhanced surveillance program involves the use of a rapid screening
test, followed by confirmatory testing for any samples that come back
"inconclusive." The weekly summary below captures all rapid tests conducted
as part of the enhanced surveillance effort.

It should be noted that since
the enhanced surveillance program began, USDA has also conducted
approximately 9,200 routine IHC tests on samples that did not first undergo
rapid testing.

This was done to ensure that samples inappropriate for the
rapid screen test were still tested, and also to monitor and improve upon
IHC testing protocols. Of those 9,200 routine tests, one test returned a
non-definitive result on July 27, 2005. That sample underwent additional
testing at NVSL, as well as at the Veterinary Laboratories Agency in
Weybridge, England, and results were negative.
To view the IHC testing numbers from 1990 through 2004, click on the
following link:

Aug. 15, 2005, 11:39PM

1,000 citations issued on mad cow regulations
Meatpackers didn't follow rules on tissue removal
Bloomberg News

U.S. government inspectors cited meatpackers more than 1,000 times over a
17-month period for violating rules concerning the removal of tissue
associated with mad cow disease, the U.S. Department of Agriculture said

Some 1,036 "noncompliance" reports covering the January 2004-May 2005 period
were released, the USDA said. The reports document instances of meatpackers
failing to properly remove "specified risk materials" or SRMs — brains,
spinal cord tissue and other tissues that scientists say harbor the disease.

"No specified risk materials got into the food supply" as a result of any of
the violations, said Lisa Wallenda Picard, a spokeswoman for the USDA's Food
Safety and Inspection Service. In all cases, corrective actions were taken
or unsafe practices were changed, Picard said.

The release of the noncompliance reports comes as Japan, normally the
biggest overseas customer for U.S. beef, debates whether to ease the ban on
U.S. beef it imposed in December 2003 after the U.S. found its first case of
mad cow disease.

Many of the violations cited by the USDA were related to paperwork mistakes,
according to the American Meat Institute, which represents Tyson Foods,
Smithfield Foods and other large U.S. meatpackers. The USDA released the
noncompliance reports to news organizations that had sought the information
in freedom of information requests, the Washington-based trade group said in
a prepared statement.

Consumer group Public Citizen said it was still reviewing all the documents
and would need several days to summarize the noncompliance reports. "I think
there still has to be a concern about meat from an infected animal making it
into the food supply," said Tony Corbo, legislative representative for
Public Citizen. "It is not a fail-safe system."

The meat industry disagreed.

"Some groups will no doubt attempt to use this information as evidence of
possible operational problems and even a food safety concern, when nothing
is further from the truth," said Jim Hodges, president of the AMI

The USDA ordered that the risk materials be removed from slaughtered cattle
Dec. 30, 2003, a week after the first U.S. mad cow case was confirmed.

The second case was confirmed in June, at an undisclosed location in Texas.

The noncompliance citations generally broke down into five categories: not
having sufficient plans to address SRM removal, inadequate SRM removal,
cross-contamination, poor record-keeping and inadequate age determination,
Tyson Foods spokesman Gary Mickelson said.

Reuters News contributed to this report.

and the beat goes on $$$


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