CDC - HUNTERS AND OTHER YOUNG PRION DISEASE VICTIMS – CDC SAYS NO LINK TO CWD
http://wwwnc.cdc.gov/eid/article/10/6/03-1082_article.htm
Volume 10, Number 6—June 2004
Perspective
Chronic Wasting Disease and Potential Transmission to Humans
Article Contents
Geographic Distribution of Chronic Wasting DiseaseChronic Wasting Disease in Free-ranging
Deer and ElkChronic Wasting Disease in Captive Deer and ElkTransmission to Other
AnimalsRisk for Transmission to HumansLaboratory
StudiesConclusionsAcknowledgmentReferencesFigureTable 1Table 2Suggested Citation
Ermias D. Belay* , Ryan A. Maddox*, Elizabeth S. Williams†, Michael W. Miller‡, Pierluigi
Gambetti§, and Lawrence B. Schonberger*
Author affiliations: *Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
†University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort
Collins, Colorado, USA; §Case Western Reserve University, Cleveland, Ohio, USA
Excerpts:
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 CWDendemic area. The case-patient’s illness began with a seizure and progressed to fatigue,
poor concentration, and depression. Memory loss, ataxia, speech abnormalities,
1
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
2
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-yearold man who was reported to have eaten venison from two deer harvested in a CWDendemic 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 casepatient <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.
*******************************************************************************************
Since 2004, when the CDC report was issued, there has been new research which indicates
humans may in fact be at risk of contracting the human strain of Chronic Wasting Disease.
http://www.jbc.org/content/early/2011/01/04/jbc.M110.198465.full.pdf
http://www.jbc.org/cgi/doi/10.1074/jbc.M110.198465 The latest version is at
JBC Papers in Press. Published on January 4, 2011 as Manuscript M110.198465
Generation of a new form of human PrPSc in vitro by inter-speciestransmission from
cervids prions
3
Marcelo A. Barria1 , Glenn C. Telling2 , Pierluigi Gambetti3 , James A. Mastrianni4
and Claudio Soto1,*
http://www.jbc.org/cgi/doi/10.1074/jbc.M110.198465 The latest version is at
“PAGES 6 AND 7
"Our findings demonstrate that cervid PrPSc, upon strain adaptation by serial passages in
vitro or in cervid transgenic mice, is capable of converting human PrPC to produce PrPSc
with unique biochemical properties, likely representing a new human prion strain. The
newly generated CWD-huPrPSc material has been inoculated into transgenic mice
expressing human PrP to study infectivity and disease phenotype and this data will be
published elsewhere"
Besides the importance of our results for public health in relation to the putative
transmissibility of CWD to humans, our data also illustrate a very important and novel
scientific concept related to the mechanism of prion transmission across species barriers.
Today the view is that species barrier is mostly controlled by the degree of similarity on
the sequence of the prion protein between the host and the infectious material (4). In our
study we show that the strain and moreover the stabilization of the strain plays a major
role in the inter-species transmission. In our system there is no change on the protein
sequence, but yet strain adaptation results in a complete change on prion transmissibility
with potentially dramatic consequences. Therefore, our findings lead to a new view of the
species barrier that should not be seen as a static process, but rather a dynamic biological
phenomenon that can change over time when prion strains mature and evolve. It remains
to be investigated if other species barriers also change upon progressive strain adaptation
of other prion forms (e.g. the sheep/human barrier).
Downloaded from www.jbc.org by guest, on January 11, 2011
7
“Our results have far-reaching implications for human health, since they indicate that cervid
PrPSc can trigger the conversion of human PrPC into PrPSc, suggesting that CWD might be
infectious to humans. Interestingly our findings suggest that unstable strains from CWD
affected animals might not be a problem for humans, but upon strain stabilization by
successive passages in the wild, this disease might become progressively more
transmissible to man.”
4
“"Here we report that a human prion strain that had adopted the cervid
prion protein (PrP) sequence through passage in cervidized transgenic mice
efficiently infected transgenic mice expressing human PrP, indicating that
the species barrier from cervid to humans is prion strain-dependent and
humans can be vulnerable to novel cervid prion strains"
JUNE 22, 2012 – Nobel Laureate Stanley Prusiner, UCSF, and colleagues, CONFIRM–
ALZHEIMER’S IS A PRION DISEASE – {“AB” = AMYLOID BETA – THE INFECTIOUS PROTEIN,
ALONG WITH TAU, IN ALZHEIMER’S DISEASE}
“Now he concludes, they (prions) are actually capable of multiplying in what he terms "alternative"
shapes, with each shape responsible for a different type of dementia. ”
“The brain diseases caused by prions include Alzheimer's, Parkinson's and Huntington's, amyotrophic
lateral sclerosis, also known as Lou Gehrig's disease, and other varied disorders known collectively
as the frontotemporal dementias, Prusiner said “
Victims of Chronic Wasting Disease may present with Creutzfeldt Jakob Disease or any one of a
number of other strains of neurodegenerative diseases.
********************************************************************************************************
The following are some excerpts from my files – there may be some duplicates with CDC
cases . . . and include newspaper reports of people who died from “ Creutzfeldt Jakob
Disease”.
***************************************************************
http://www.planetark.com/dailynewsstory.cfm/newsid/23244/story.htm
WASHINGTON STATE PETER PUTNAM AGE 35
US Mad Cow Link Questioned in Creutzfeldt-Jakob Cases
Mail this story to a friend | Printer friendly version
USA: December 29, 2003
NEW YORK/SAN FRANCISCO - Family and friends of American victims of Creutzfeldt-Jakob Disease ,
the fatal brain disorder sometimes linked to mad cow disease, on Friday questioned whether the
wasting illness that killed their loved ones was actually due to eating contaminated U.S. beef.
5
After federal authorities said on Tuesday that a cow in Washington state was found to have mad cow disease,
public health experts have been calling for a review of the U.S. Agriculture Department's screening procedures
for cattle.
But some victim's families have gone further, saying that the human form of the disease may have already hit
the United States and that the government has been lax in its testing possible links and enforcing safety
standards.
"The most frustrating part of this disease is that there are no answers," said Chris Turnley, whose
brother Peter Putnam, who grew up in Washington state, died of the disease last October at age 35.
"They need to figure out the cause but also start figuring out treatments."
So far, none of the roughly 300 cases of Creutzfeldt-Jakob Disease diagnosed in the United States each year
has been linked to U.S.-produced beef, said Pierluigi Gambetti, director of the National Prion Disease
Pathology Surveillance Center at Case Western University.
But Dr. Michael Greger, a doctor in Scarsdale, New York, and coordinator for Organic Consumers
Association, said it would be wrong to take comfort from that statistic. The disease has a long incubation
period and few dementia-related deaths in the United States are investigated.
"There have been no confirmed cases, but just as there weren't any confirmed cases of mad cow
disease, it is a function of how hard one looks for it," Greger said.
*******************************************************************************************************************************
NEW JERSEY CARRIE MAHAN AGE 29 AND SIX OTHERS ?????
http://www.alertnet.org/thenews/newsdesk/N26151750.htm
26 Dec 2003 21:34:55 GMT
US mad cow link raised in Creutzfeldt-Jakob cases
By Jed Seltzer
NEW YORK, Dec 26 (Reuters) - Family and friends of victims of Creutzfeldt-Jakob Disease, the fatal brain
disorder sometimes linked to mad cow disease, on Friday questioned whether the victims contracted the
condition from contaminated U.S. beef.
Janet Skarbek, an attorney and accountant from Cinnaminson, New Jersey, three years ago began
investigating the possibility that mad cow disease has afflicted and killed several people in or near
southern New Jersey.
Skarbek's suspicions center on the now-defunct Cherry Hill horse racetrack, where her mother worked. A
colleague there, Carrie Mahan, died at age 29 of Creutzfeldt-Jakob Disease.
Through obituary reports, Skarbek has since tracked down six other deaths over the past three years in
the southern New Jersey and Philadelphia area that were likely due to CJD. She says she contacted the
family and friends of all the victims, and found they all had eaten at the racetrack in the late 1980s or in
the 1990s.
"If I can find seven CJD people who ate at this racetrack, think of how much the government could do
with all the information they have," she said.
6
Skarbek said she was denied a request for information from the Centers for Disease Control to find out
what the agency knows. She sent an appeal on Thursday.
*************************************************************************
Writing in the BOSTON GLOBE, Terry J. Allen reported in late 1999 that, since 1996, Creutzfeld-Jakob
disease has been identified in 3 Americans younger than age 30.[3] All three are known to have hunted
extensively or eaten venison. There is no evidence that CWD disease has jumped from deer or elk to
humans, but the appearance of this extremely-rare disease in young people was the first evidence of a
problem in England, so health authorities in the U.S. say they are aggressively investigating all the
possibilities.
*******************************************************
Last summer in an Ann Arbor, MICHIGAN, hospital TWO YOUNG MEN, one 26
and the other 28, were both dying at the same time from classic or sporadic
CJD of unknown cause. They had lived their entire short lives in Michigan.
http://www.rockymountainnews.com/drmn/state/article/0,1299,DRMN_21_1121130,00.html
In a scientific abstract presented at a neurology convention in Denver, Dr. Norman Foster said two men,
ages 26 and 28, came to the University of Michigan Hospital simultaneously suffering a human TSE
called Creutzfeldt-Jakob disease (CJD).
CJD typically strikes about one in a million people in their 60s and 70s. It happens in those under 30 at a rate of
one case per 270 million people per year. The odds of two cases happening simultaneously in the same place
would approach one in 270 million squared.
Foster, a neurologist at the University of Michigan-Ann Arbor, said it is possible that the odds might be
much lower than previously thought for CJD in the young, and that many cases may be misdiagnosed.
But he said the two men did not have some of the typical symptoms of CJD. Their brain waves were different
and they lacked a diagnostic protein called 14-3-3 in spinal fluid. One was originally thought to have epilepsy.
It's possible that many cases diagnosed as viral encephalitis might be CJD, Foster suggested.
*****************************************************************
NEVADA – JIM KOEPKE
http://news.bbc.co.uk/1/hi/world/americas/1306563.stm MAY 1, 2001
A farm in the mountains of NEVADA may hold a key to the puzzle of Mad Deer disease.
The farm's owner, a cowboy called JIM KOEPKE, died suddenly from Creutzfeldt Jakob Disease (CJD),
an illness very similar to both BSE and the Mad Deer variant.
Brenda, Jim's widow, admits that he loved to hunt. It is possible that he may have died from eating infected deer
meat.
"I couldn't comprehend that a cowboy in the middle of farming Nevada could get such a horrendous disease,"
she said. "So I dragged the poor man to many other doctors looking for something that made an ounce of
sense."
7
Just coincidence?
Jim Koepke was the third deer hunter to die in the last two years from CJD. So far the evidence suggests
that it is just coincidence.
“in Colorado, where as many as one in five deer and elk now has the disease, the authorities are urging caution
... What is eerie is how similar the comments of American experts are to those of British government officials
during the early years of the Mad Cow crisis."
**********************************************************************
UTAH DOUG MCEWEN – age 28
Indeed, of the 3 cases detected in the U.S. since 1996, one nearly went undetected. Last year in UTAH,
Doug McEwen, 28, began to show an array of mysterious symptoms: loss of memory, loss of motor control,
mood swings, and disorientation. His wife, Tracey, says his doctors conducted hundreds of tests but could not
diagnose his disease. She happened to see a TV program on mad cow disease and she insisted that Doug's
doctors must test for CJD. A brain biopsy confirmed the diagnosis.
Doug McEwen was an avid hunter.
“It seems that "more could have been done" says Kay McEwen, his mother,
> from her home in Salt Lake City. "I don't think they knew what they were
> doing with him," she says of the neurologists and disease detectives who
> have studied slivers of her son's brain since his death in 1999. Part of
> their aim was to determine whether McEwen consumed venison infected with
> chronic wasting disease, a neurologic prion disorder in deer and elk,
> similar to mad cow, that has spread in some parts of Colorado, Wyoming,
> Michigan and Wisconsin.
>
> "Now I think they are trying to ignore it," as if it didn't exist and
> isn't any problem, McEwen says, her voice cracking. "I'm sorry but it's
> gotten so I can't talk about it."
“All those pleas that blood banks make when supplies dwindle spurred Doug
> McEwen to roll up his sleeves not once, not twice
> but more than a dozen times to donate blood.”
note:
Decemberf 2003 - "In December 2003, a single case of transfusion-acquired variant CJD
was reported from the United Kingdom. Since the time of the transfusion, which took place in
early 1996, a number of measures have been instituted to protect the blood supply, including
depletion of white blood cells, which are thought to be a potential source of infection.
Procedures for blood transfusions in the U.K. are currently under review."
http://www.mdtravelhealth.com/infectious/mad_cow_disease.html
PRIONS HAVE BEEN FOUND IN BLOOD
8
BRITISH CHILDREN WILL RECEIVE US BLOOD TO AVOID RISK OF VCJD
August 16, 2002 Agence France Presse English
LONDON - The UK health ministry was cited as saying Friday that Britain is to import frozen plasma from the
US for use in treating babies and young children to reduce the risk of them contracting the human form of "mad
cow" disease. The story says that earlier this month it emerged that the risk of the fatal human form of the
illness -- variant Creutzfeldt-Jakob Disease (vCJD) -- being transmitted through blood transfusions might be
higher than previously thought.
British tests revealed that one in six animals given blood from sheep infected with a similar disease went on to
develop the illness, suggesting that there was an "appreciable" risk to humans, according to press reports.
The US plasma is intended for children born after 1995 as they are young enough not to have been exposed to
the infection in any other way, such as eating meat contaminated with bovine spongiform encephalopathy
(BSE), which has been linked to vCJD in humans.
******************************************************************
Wayne WATERHOUSE, Roger MARTEN and James BOTTS all ate venison and wild game at
Waterhouse's farm in WISCONSIN. Botts and Waterhouse died of Creutzfeldt-Jacob disease (CJD).
Marten succumbed to Pick's disease, but scientists now say that might have be a misdiagnosed case of
Creutzfeldt-Jakob, a disease that normally affects only one in 1 million people. (NOTE: CDC claims
Marten died of Pick’s Disease . . . but CDC has not yet released test results regarding Waterhouse and Botts.)
The researchers, some from the U.S. Centers for Disease Control and Prevention, concluded that although circumstances suggested a
connection with chronic wasting disease, they could find no "causal link." . . . . .
Now, the cases of Botts, Marten and Waterhouse raise some of the same questions.
Fred Bannister, a small-town doctor who knew all three men and took part in the wild game feeds, said a thorough investigation of the
case is needed.
"I'm just a little guy out here in the woods who has suspicions," said the semiretired Chetek family physician. "There is something
unusual."
Wayne WATERHOUSE, Roger MARTEN and James BOTTS all ate venison and wild game at Waterhouse's farm in
WISCONSIN. Botts and Waterhouse died of Creutzfeldt-Jacob disease (CJD). Marten succumbed to Pick's disease, but
scientists now say that might have be a misdiagnosed case of Creutzfeldt-Jakob, a disease that normally affects only one
in 1 million people. (NOTE: It has been confirmed that Marten died of Pick’s Disease . . . but CDC has not yet released
test results regarding Waterhouse and Botts.)] . . . . . . .
***********************************************************************************
9
***********************************************************************
KEVIN BOSS, AGE 39
The death of D. Kevin BOSS former Minneapolis resident from Creutzfeldt-Jakob disease
(CJD) otherwise might be unremarkable except for this: BOSS got the disease
at an unusually young age, 39, and he regularly consumed venison, including
deer meat from WISCONSIN.
*************************************************************************
-- In 1984, neurologists at Baylor College of Medicine investigated FOUR SUSPICIOUS DEATHS involving
unrelated patients, all with a history of eating the brains of wild animals. All the patients eventually died, and
Creutzfeldt-Jakob was the suspected cause.
****************************************************************************
-- A 1997 study in the journal Lancet linked FIVE UNRELATED CJD PATIENTS from different towns in
rural KENTUCKY to a history of eating squirrel brains.
Kentucky Doctors Warn Against Regional Dish: Squirrels' Brains
By SANDRA BLAKESLEE
August 29, 1997
NY Times
Doctors in Kentucky have issued a warning that people should not eat squirrel brains, a regional delicacy,
because squirrels may carry a variant of mad cow disease that can be transmitted to humans and is fatal.
Although no squirrels have been tested for mad squirrel disease, there is reason to believe that they could be
infected, said Dr. Joseph Berger, chairman of the neurology department at the University of Kentucky in
Lexington. Elk, deer, mink, rodents and other wild animals are known to develop variants of mad cow disease
that collectively are called transmissible spongiform encephalopathies.
In the last four years, 11 cases of a human form of transmissible spongiform encephalopathy, called CreutzfeldtJakob disease, have been diagnosed in rural western Kentucky, said Dr. Erick Weisman, clinical director of the
Neurobehavioral Institute in Hartford, Ky., where the patients were treated. "All of them were squirrel-brain
eaters," Weisman said. Of the 11 patients, at least six have died.
Within the small population of western Kentucky, the natural incidence of this disease should be one person
getting it every 10 years or so, Weisman said. The appearance of this rare brain disease in so many people in
just four years has taken scientists by surprise.
http://www.mad-cow.org/~tom/victim23.html#Kentucky
**************************************************************************
In October 2001, a study in the journal Archives of Neurology reported on the cases of three people who
contracted CJD and who had been deer hunters or had regularly eaten venison.
10
The three individuals, who were from MAINE, OKLAHOMA AND UTAH , all were under the age of 30
and came down with the disease between 1997 and 2000. The vast majority of CJD patients get the
disease in their 60s and 70s.
"One of the three young CJD victims had eaten deer shot near Rangely, MAINE, so last November
federal officials took samples of brains from 299 deer shot in western Maine. Authorities said at the time
they were quite sure Maine deer are not harboring CWD. So far, test results have not been released. “
*****************************************************************************
Meanwhile, a 68-year-old INDIANA man with a fondness for beef-brain sandwiches died of CJD last
summer. Beef-brain sandwiches are a local delicacy in Indiana, introduced years ago by German
immigrants. The EVANSVILLE (INDIANA) COURIER reported that John Hiedingsfelder, a forensic
pathologist in Evansville, said he had seen three cases of CJD in the past year. No connection to mad cow
disease has been established in the Indiana cases. Roberta Heiman, a staff writer for the EVANSVILLE
(INDIANA) COURIER reportedly received a warning from a cattleman's association not to publish any
further articles about this subject.
****************************************************************************
CALIFORNIA GARY PADGHAM AGE 50
September 2002
http://www.sfgate.com/cgi-bin/article.cgi?f=/c/a/2002/09/07/BA33712.DTL
Doctors study brain tissue of elk hunter
Montana man had symptoms similar to mad cow disease
David Perlman, Chronicle Science Editor
Researchers at UC San Francisco are analyzing the brain tissue of a Montana elk hunter who
died last month with symptoms of a rare human disorder similar to mad cow disease in cattle
and the chronic wasting disease that has felled elk and deer in the Midwest and Rocky
mountains.
Last October, 50-year-old Gary Padgham of Bozeman first showed the stumbling gait, the
dementia and the impaired vision and memory loss that are typical of Creutzfeldt-Jacob
disease, known also as CJD. He was taken to a hospital in Seattle where doctors first
diagnosed his illness as Huntington's disease, and then as CJD.
Padgham was later moved to Monterey where his family lives, and after he died in August his
body was taken to UCSF for a specialized autopsy.
The institution is the nation's primary center for research in the varied brain diseases of
humans and animals that are all believed to be caused and transmitted by malformed brain
proteins called prions.
CJD is known to be one of the prion diseases, and researchers at UCSF study as many as 20
cases of suspected prion brain diseases a year in an effort to learn more about the invariably
fatal malady.
11
Specialists at the California state health department said the chances are "slim to none" that
Padgham could have contracted his disease from eating the meat of the elk he hunted in
Montana.
According to the Federal Centers for Disease Control and Prevention, chronic wasting disease
has hit captive deer and elk herds in several Midwestern and mountain states, and some wild
deer in Wisconsin -- a development that has caused widespread fears among hunters.
In California, the Department of Fish and Game has asked hunters to stop bringing deer and
elk parts back from states where the wasting disease has been found, and will seek a
regulation banning the import of all high-risk parts, such as brains.
The CDC and other state agencies are also investigating the deaths of Padgham and three
other hunters in Montana and Wisconsin. Two of the three died of Creutzfeldt-Jacob disease
and one of a different brain disorder called Pick's Disease, where prions are not known to be
involved.
E-mail David Perlman at dperlman@sfchronicle.com.
AND MONTANA elk hunter Gary PADGHAM died of the brain-wasting Creutzfeldt-Jakob disease in
Monterey, California, in August 2002, setting off fearful speculation that bad meat may have been the
cause.
Gary Padgham didn't hunt.
But as a fishing guide, the 50-year-old had many friends who shot elk and deer and would often give him meat,
said Lynda Jandt, Padgham's long-time significant other and mother of his child.
He typically served elk meat to his clients on fishing trips to give them a true Montana experience. Joseph
Stevens, who grew up with Padgham in Monterey, Calif., went on a five-day fishing trip on the Smith River
with him two years ago. Elk was served for dinner on that trip.
South Korea banned imported elk meat from Canada after CWD was discovered in some herds
there. (September 2002)
*********************************************************************
COLORADO OTTO BERNS AGE 63
Man who hunted in CWD area dies of brain disease
By Lou Kilzer, Rocky Mountain News
July 11, 2002
A 63-year-old Thornton man who hunted elk and deer in a COLORADO area beset with chronic wasting disease died early
Wednesday from a similar human brain disease.
12
OTTO BERNS first noticed signs of memory loss in early May. Doctors struggled to find the correct diagnosis, first telling his family
that he had suffered a stroke.
After his condition worsened, Berns' daughter, Nicki, told doctors her father was an avid venison eater who hunted north of Fort
Collins. She said she suspected he was suffering some form of CJD, or Creutzfeldt-Jacob disease. In June, a biopsy confirmed her
suspicions.
“Otto
Berns was an avid hunter whose annual fall hunts in northern Colorado produced a freezer full of venison,
which always was replenished by the following year's hunt.
"I love the taste of venison," she said. Hunting "was our source of meat. It would last us the whole year."
Ironically, it was the venison that killed her father, Nicki Berns said.
"I'm 100 percent certain," she said. "I have no doubts in my mind. There are too many hunters getting
this disease."
*****************************************************************************************
COLORADO GARY HOPKINS AGE 53
Man vs. nature
Mad cow disease has researchers taking a close look at deer and elk
By Debra Melani, News Staff Writer
January 23, 2001
Sic months before his death, GARY HOPKINS was a vibrant, seemingly healthy 52-year-old man, a
Colorado native and lover of the outdoors. At the end of those six months, the Aurora man was little
more than a sack of bones, curled listlessly in a fetal position. He died Oct. 4, two days after his 53rd
birthday.
Pat Hopkins recalls his father's rapid decline as "unreal." He says he wouldn't take photos of him in those last
months, because "I just wanted to remember him the way he was before."
Today, Hopkins says, he wonders whether his father, who hunted avidly in the Fort Collins area, died because
he ate the meat of deer infected with chronic wasting disease, CWD.
***************************************************************************
PUB MED
1: Arch Neurol 2001 Oct;58(10):1673-8
Creutzfeldt-Jakob disease in unusually young patients who consumed venison.
Belay ED, Gambetti P, Schonberger LB, Parchi P, Lyon DR, Capellari S, McQuiston JH, Bradley K,
Dowdle G, Crutcher JM, Nichols CR.
Division of Viral and Rickettsial Diseases, Centers for Disease Control and Prevention, Mailstop A-39,
1600 Clifton Rd, Atlanta, GA 30333, USA.
BACKGROUND: Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer
and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly
consumed deer or elk meat created concern about the possible zoonotic transmission of CWD.
OBJECTIVE: To examine the possible transmission of CWD to humans. PATIENTS: Three unusually
young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000. METHODS:
13
We reviewed medical records and interviewed family members and state wildlife and agriculture officials.
Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein
gene analyses. MAIN OUTCOME MEASURES: Presence or absence of established CJD risk factors, deer
and elk hunting in CWD-endemic areas, and comparison of the evidence for the 3 patients with that of a
zoonotic link between new variant CJD and bovine spongiform encephalopathy. RESULTS: None of the
patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals
and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique
neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion
protein gene, or prion characteristics different from those of classic variants. CONCLUSIONS: Although
the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible
relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing
CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to
humans.
PMID: 11594928 [PubMed - indexed for MEDLINE]
[NOTE: “new-variant” CJD from CWD may manifest itself differently from the prion disease vCJD,
which is the human form of Mad Cow Disease (Bovine Spongiform Encephalopathy) -- which has
infected at least 128 people in Europe and killed 115 in the UK.]
http://www.bse.org.uk/files/yb/1991/01/04004001.pdf
“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 . . . “
Is it possible the “experts” in the USA are bending over backwards to avoid admitting any CJD or
TSE -- particularly in young people -- is related to BSE or CWD -- in order to protect the cattle and
hunting industries ?
************************************************************************
Below is Rob Zaleski's column in the Madison Capital Times April 2, 2001, 11
months before it was announced that CWD was in Wisconsin. He reports then
on how Kazmierczak's colleague Mary Proctor tried to manage press coverage
of the highly suspicious CJD death of Mary Riley, a 43 year old mom and venison eater
in the Waupaca area.
Is the Centers for Disease Control now investigating Riley's death? No!
It turns out that the ONLY suspicious deaths that CDC is currently
investigating in Wisconsin are the 2 CJD deaths of northern hunters that the
Milwaukee Journal Sentinel reported this summer.
All other deaths are the domain of the state, which as we can see from these
articles and quotes is engaged in a PR campaign to deny risks.
Zaleski's column is reprinted below and shows that even before CWD appeared,
state officials were belittling concerns of CJD deaths in the state.
Bottom line: Do not trust state health officials on CWD risks, they are
engaged in PR rather than the protection of public health
John Stauber, co-author, Mad Cow USA <>
DEATH LEADS TO FEARS AND ANGER
WISCONSIN 43 year old MARY RILEY
14
Capital Times, The (Madison, WI)
Published on April 2, 2001
Byline: Rob Zaleski
It will be months - probably years - before Glenn Riley recovers from the
recent grisly death of his 43-year-old wife, Mary.
He knows it and accepts it - and, frankly, would prefer not to talk about
it. His wife was diagnosed with sporadic Creutzfeldt-Jakob disease - a rare
illness similar to the human form of mad cow disease known as variant CJD at the Marshfield Clinic last fall and she essentially went mad, losing all
control of her physical and mental functions, before her death Jan. 6.
I wrote about her harrowing demise a few weeks later but did not identify
her by name, because Glenn Riley and his two teenage sons were worn out from
the ordeal and didn't want any more publicity.
But Riley, who runs a tavern near Shawano and happens to be my wife's
stepbrother - as I noted in the column - now feels compelled to speak out.
For two reasons.
Since his wife's obituary and a short story on her death appeared in The
Post-Crescent, Appleton, he's heard from three other families in Wisconsin
who have lost loved ones to the disease - including a man whose uncle in
Oshkosh died Dec. 2.
So while Riley can appreciate why government officials are going to great
lengths to assure U.S. residents that there hasn't been a single death in
this country linked to the human form of mad cow - as opposed to the United
Kingdom, where 90 people have died from it - he wonders if they aren't
understating the possibility that it might already be here.
Or if sporadic CJD - which researchers say strikes about 250 people a year
in the United States - might be becoming more widespread than experts
believe.
And the second reason Riley's decided to speak out?
He's miffed.
Shortly after his wife's death, he heard from friends that news director
Jack Barry of WDUX radio in Waupaca had interviewed Mary Proctor, chief of
the state Department of Health and Family Services' communicable disease
epidemiology section, regarding Mary Riley's death. Those friends - and
Barry - say Proctor seemed skeptical that Mary Riley had actually died of sporadic CJD, since the disease is so
rare.
Why, Glenn Riley wants to know, would Proctor take such a stance particularly since he received a copy of the autopsy confirming that his
wife died of the illness?
Proctor, contacted last week, denied that she'd been skeptical and said that
because CJD is not monitored on the state level, she has no idea what caused
Mary Riley's death.
She also pointed out that people can write anything they want on a death
certificate. "You could say a person died of heart failure or an in-grown
toenail."
15
When I suggested it certainly sounded like she was skeptical, she denied
that was the case and snapped, "This conversation is over. And if you quote
me I'll sue you."
Judd Aiken, a CJD specialist at the University of Wisconsin-Madison, seemed
taken aback when told of Proctor's response. In fact, Aiken says, he himself
has received a "fair number" of e-mails from Wisconsin residents who have
lost family members to sporadic CJD, which leads him to wonder if perhaps
the state should start tracking the disease.
(The federal Centers for Disease Control and Prevention in Atlanta says its
most recent data on sporadic CJD shows that 240 people died of the disease
in the United States in 1998, including six in Wisconsin.)
Aiken emphasizes that he and other researchers don't want to come off as
alarmists, for obvious reasons. At the same time, he says, the ongoing
tragedy in the United Kingdom makes it critical that researchers come up
with a cure.
He also says his heart goes out to those who have watched a loved one
succumb to this truly horrible disease.
"And then to find out that the scientific and medical communities can't tell
you what the cause was - it's gotta be tough," he says. "I mean, I'm sure
they're all looking for some sort of closure.
"And right now, we can't give them that."
(End of Column.)
****************************************************************************
-- In 1986, a study involving 26 CJD patients by researchers at Temple University and the National Institutes of Health
found that exposure to deer through a hobby such as hunting resulted in up to a NINEFOLD INCREASED RISK FOR CJD.
****************************************************************************
A new kind of killer
In tracking a lethal disease that threatens humans and animals, scientists have become the hunters
By JOHN FAUBER and MARK JOHNSON
jfauber@journalsentinel.com
Last Updated: Oct. 26, 2002
The requirements for monitoring Creutzfeldt-Jakob disease aren't adequate, according to Gambetti, who
directs the National Prion Disease Pathology Surveillance Center at Case Western Reserve. Last year, his
lab received brain tissue samples from only half the Creutzfeldt-Jakob cases in the U.S., even though it is
supposed to receive samples from all of them.
"It's a big problem," Gambetti said. "I could miss a variant CJD (mad cow) case."
Even with the incomplete monitoring, Gambetti said, a couple of disturbing trends might be developing.
"What I'm seeing is more younger cases, which was unheard of before," he said, referring to people in
their 20s and 30s.
16
******************************************************************************************
http://southwestfarmpress.com/management/texas-becomes-18th-stateconfirmed-cwd-deer
July 12, 2012
Texas becomes 18th state with confirmed CWD in deer
CWD confirmed in Texas mule deer.
Wildlife checkpoints throughout hunting season to monitor CWD.
CWD has also documented in deer in 19 states and 2 Canadian
provinces.
***************************************************************
Vignette V/5 Thu Aug 08 02:26:56 2002
AUGUST 8 2002
Medical experts at the conference were also discussing a recently
discovered case in Wisconsin, in which three hunters in their 50s and 60s
who often engaged in so-called "venison feasts" all died in the 1990s of
neurological diseases, two of them from CJD.
http://www.rockymountainnews.com/drmn/state/article/0,1299,DRMN_21_1312942
,00.html
Greg:
CAN CWD BE TRANSMITTED TO CATTLE AND HUMANS?
Extensive research
(http://www.madcow.com) indicates it may be possible.
IF you allow a
plant rendering 1 million pounds of deer carcasses to discharge wastes
into a local sewage treatment plant - can you be sure the sludge will
contain no prions?
Chronic wasting disease
CWD carriers among us?
Expert: Study needed to learn if humans can harbor disease
By Todd Hartman and Lou Kilzer, Rocky Mountain News
August 8, 2002
A National Institutes of Health scientist called Wednesday for further
research to determine if deer, elk, cattle - or even humans - might be
silent carriers of chronic wasting disease, harboring the illness until it
can infect others.
17
Richard Race, a leading-edge researcher on CWD and similar diseases, told
450 fellow scientists, government officials and wildlife advocates that
"One might wonder if there are people who are (carriers)."
Race's experiments on mice and hamsters show that some maladies in the
family of diseases known as transmissible spongiform encephalopathies
(TSEs) can lie undetected in one set of test animals, then spring to life
with fatal consequences when transmitted to others.
"If these people are subclinical carriers, do they represent a threat to
other people?" Race asked.
Already, human cases of a related brain disease - Creutzfeldt-Jakob
Disease, or CJD - have been transmitted during surgical processes
involving contaminated body tissues and medical instruments.
While there are no known cases of CWD - a degenerative neurological
disease of deer and elk - infecting humans, the tenor of debate on the
issue has changed.
A year ago, some biologists scoffed at the notion that CWD was any risk to
humans. Wednesday, a parade of experts ventured to the podium to discuss
their research into what has become a major area of study.
"The (possibility of) transmissibility of CWD to humans remains unclear,"
said Gregory Raymond, a colleague of Race's at the NIH's Rocky Mountain
Laboratories in Montana.
Race discussed his research into a molecular species barrier that may - or
may not - stop CWD from infecting humans, cattle or other species.
University of Wyoming veterinarian Beth Williams updated the audience on
another critical issue: Could roaming deer and elk infected with CWD pass
it to cattle, therefore jeopardizing the beef supply?
Ongoing experiments show three cattle with CWD injected into their brains
have become infected. But other cattle exposed to the disease orally and
simply by sharing pasture space with infected deer and elk over the past
five years have not become sick.
"These experiments are not complete, and we know the incubation period
(for the disease) can be a long time," Williams said. "But so far I'd have
to say the news is good as far as CWD transmissibility to cattle."
But Race said Williams' study, and another like it in Iowa, need to be
extended. His suggestion: Once the experiment is complete, take brain
matter from surviving cattle and inject it back into uninfected cattle and
mule deer to see if the next generation of animals contracts CWD.
This is similar to the kind of experiments Race had overseen involving
18
passing the disease between hamsters and mice, and goes to the question of
whether the disease can be carried without causing disease in its current
host.
Race believes the infectious agents may be able to evolve and adapt as
they pass through generations of species, then at some point cause active
disease in a species other than that of its original host.
Amir Hamir, a scientist working at a USDA laboratory in Iowa, said he
would welcome talking with Race about the experiment with cattle and deer.
"That can be done," he said. "But it is very, very expensive" because of
the cost of caring for large mammals over years.
Medical experts at the conference were also discussing a recently
discovered case in Wisconsin, in which three hunters in their 50s and 60s
who often engaged in so-called "venison feasts" all died in the 1990s of
neurological diseases, two of them from CJD.
The cases have only now have come to the attention of the Centers for
Disease Control. The CDC's interest was fueled by the February discovery
by Wisconsin wildlife officials of CWD in the state's white-tailed deer.
"We are investigating as we speak," said Ermias Belay, prion disease
expert with the CDC.
In an interview, he said his agency wants to examine the deceased men's
brain tissue and confirm diagnoses made when they died. Investigators are
also gathering reams of information from family members. Still, Belay
said, it may be hard to be definitive about the cases.
"You can speculate, but there is no hard data to tell you if they had
exposure to (CWD-contaminated venison) in Wisconsin," he said. "One of
these (men) died in 1993. If he was exposed in Wisconsin, you have to look
back 10 to 20 years" at the meat he consumed. The meat - if meat even had
anything to do with it - could have come from many other places, Belay
said.
The greatest concern was expressed by Western Slope veterinarian Dick
Steele. He fretted that CWD is a human health threat that isn't being
taken seriously enough by wildlife officials.
He said if the disease does adapt to cross into humans, and proves as
infectious as CWD is among elk and deer, there would be "some pretty
serious consequences." He also noted that, compared with British exposure
to mad cow disease - where more than 120 people have died out of a
population 60 million - exposure to CWD is far lower, perhaps too low for
any human cases to be detected yet.
"Human exposure to CWD has been so limited that statistically we are
unlikely to have seen one single case at this time," Steele wrote in a
19
summary of his presentation. "To say there is no evidence that CWD cannot
transmit to people is quite premature."
*******************************************************************
Subject: [CJDVoice] Lab tests planned in case of Grand Forks man who died
Saturday
September 29,2004 - NORTH DAKOTA
Michael
Jose, 48 year old hunter dies of CJD
Associated Press
FARGO, N.D. - Lab tests are planned to confirm whether a Grand Forks
man died of Creutzfeldt-Jakob Disease, a rare neurological condition with
symptoms similar to mad cow disease, health officials say.
Michael Jose, 48, of Grand Forks, died Saturday. The cause of death was
listed as classic CJD.
Scientists say classic CJD, though rare, is not unheard of in North
Dakota or in the United States while mad cow disease, known as variant
CJD,
has not been found in this country.
Brain tissue will be sent to the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University in Cleveland to
provide more information, said Larry Shireley, the state epidemiologist.
"You get two concerns," Shireley said. "One is chronic wasting disease.
There's no evidence that can be transmitted to humans.
"The new variant CJD has been associated with mad cow. We have had no
cases in the United States," Shireley said.
"Even though this individual is certainly younger than what we would
expect, right now there's nothing to indicate this is anything else but
the regular CJD," Shireley said. "Obviously, we won't know that until we
have more tests."
Scientists say classic CJD usually affects people in their 60s and 70s,
and about 250 people in this country are diagnosed with it each year.
Symptoms include trouble standing, walking or talking, and 90 percent of
its victims die within a year.
"I've worked on at least one or two cases," Shireley said. "It is
extremely rare - about one in a million, but it does occur."
Variant CJD is known as a young person's disease, with half its victims
20
dying by age 28, experts say. Early symptoms tend to include
depression, pain or numbness.
Chronic wasting disease is known to affect deer and elk. Like scrapie
in sheep and CJD and the variant CJD in people, it is linked to the
presence of prions, or misshapen proteins that destroy the brain.
Health officials know little about Jose, Shireley said. They did learn
Tuesday that he was a hunter, as are many other North Dakotans, and they
had no indication that he had traveled to other countries, Shireley said.
Lab results from Cleveland are not expected for several weeks, he said.
NEWS UPDATE Jun, 20, 2008 BELLINGHAM, WASHINGTON
Local pastor Tom Hunter dies
Mayor had recognized musician for his contributions
BELLINGHAM HERALD FILE PHOTO
Tom Hunter of Bellingham, who died Friday morning, hosted an annual annual Christmas Eve
celebration at the Mt. Baker Theatre Pastor Hunter was 61.
UPDATED AT 9:37 A.M.
BELLINGHAM — Tom Hunter, a local musician and pastor who shared his music with children
in Whatcom County public schools for decades, died early this morning after a short battle
with Creutzfeldt-Jakob disease.
News of Hunter's death came from a blog that Hunter's wife, Gwen Hunter, and children had
been updating for the public as the disease progressed (visit the Hunters' blog, here.)
Creutzfeldt-Jakob disease is an extremely rare and incurable degenerative brain disorder. In
general, it is rapidly fatal.
Hunter had recently been hired as pastor of First Congregational Church, United Church of
Christ in Bellingham before his diagnosis.
Hunter was appreciated by many for Christmas concerts at the Mount Baker Theatre and other
activities, and Bellingham Mayor Dan Pike had declared June 5 “Tom Hunter Day” after
learning of the pastor’s diagnosis.
A public vigil is scheduled for 7 p.m. today at the church, 2401 Cornwall Ave. For more
information, call the church at (360) 734-3720.
http://tomhunterblog.blogspot.com/
TOM'S HEALTH
21
Tom Hunter was recently diagnosed with Creutzfeldt-Jacob Disease which is rapidly destroying his
beautiful brain. CJD is not contagious. All we've been told at this time is that he does not have the
familial type. There is a lot of mis-information on the web. The most accurate and up-to-date site for
learning about this disease and supporting efforts to find a cure is www.cjdfoundation.org
*******************************************************************************
http://www.tehrantimes.com/Description.asp?Da=12/10/02&Cat=7&Num=3
DECEMBER 2002
Fears Rising of Another Variant of Mad-Cow Disease in Humans
LONDON -- The number of people with a human form of mad-cow disease could be much higher
than originally thought, according to a new study.
Since 1990, there have been 117 confirmed deaths in Britain from the variant CJD, which until now
was assumed to be the only disease linked to eating BSE-infected beef, DPA reported.
But scientists at the Medical Research Council's Prion Unit in London believe they have identified
links between BSE and a second type of the human brain disease -- sporadic CJD.
The government's latest figures show that from 1990 to November this year, 588 people died from
sporadic CJD, including 28 in 1990, 63 in 1998, and 53 last year.
The scientists, led by Professor John Collinge, cast further doubt on the safety of sheep meat by
suggesting that more animals -- including humans -- could carry and transmit the diseases than
previously thought.
The researchers wrote: "It remains of considerable concern whether BSE has transmitted to, and is
being maintained in, European sheep flocks".
They said that given the widespread infection of sheep breeds with scrapie, it was possible some had
contracted BSE but that this infection had been hidden by the other disease.
A full study is needed of all the tonsils surgically removed over a 12-month period -- around 80,000 -to map the extent of CJD infection in the population, the Medical Research Council argued.
The British Department of Health is thought to be considering such a plan. The research team used a
series of experiments on mice that had been genetically altered so they would display the human
effects of a prion - an infectious protein. The "transgenic" mice were then exposed to BSE-infected
material and the changes in the prion protein were monitored.
22
As expected, some developed VCJD, but the researchers wrote that, surprisingly, other mice showed
effects of sporadic CJD. "These data suggest that more than one BSE-derived prion strain might
infect humans; It is therefore possible that some patients with ... sporadic CJD may have a disease
arising from BSE exposure," they wrote.
The researchers said their findings were important when considering the present sporadic CJD
outbreak in Switzerland, which had the highest incidence of cattle BSE in Europe over the past 12
years.
There was a two-fold increase in sporadic CJD in the last 18 months in Switzerland, while cases of
VCJD remain low, a spokesman for the MRC said.
There are many different strains of human and animal prion diseases.
From Hilary Clarke in Rome
An article about an Italian family with a rare brain disease whose victims die from lack of sleep struck a
chord in this anxious nation last week.
The story published on the internet site of the left-of-centre newspaper La Repubblica beat reports of
anarchist letter bombs and erupting volcanoes as the best-read story, due to the horrific and swift deaths
of the sufferers.
Fatal Family Insomnia is estimated to affect one in 33 million people, but one in four of the family in
question from Treviso in northern Italy, with 15 family members having died of the condition over the
past 30 years.
'In the summer of 1973, I saw a 49-year-old suffering with symptoms apparently linked to anxiety and
depression. She had changed jobs and couldn't adapt,' explained the doctor. 'She said she felt sick all the
time and couldn't walk properly.'
'The woman seemed to sleep, but like a sleepwalker, physically acted out her dreams. However, unlike
sleepwalkers, when called she became immediately lucid and could recount blow by blow what she was
doing.'
Within six months the woman was dead, weighing just five stone. The family's neurologist Ignazio Roiter
decided to give a journalist from La Repubblica access to the family, whose identity remains a secret, to
gain publicity for a society to be formed in Treviso on January 7 to promote research into rare brain
conditions linked to abnormal proteins called prions. These include the disease suffered by the Treviso
family, called Fatal Family Insomnia and Creutzfeld-Jakob disease, the human version of mad cow
disease.
According to the La Repubblica article, five years later the woman's 53-year-old sister began to develop
the same symptoms and also died within the year. Dr Roiter took the woman's brain to a specialist of rare
neurological pathologies in Switzerland where they detected the disappearance of certain neurons in a
small part of the brain.
23
Back in Treviso, Dr Roiter began some genealogical detective work and using parish records dating from
the 1800's discovered around 50 unexplainable deaths in the family put down to insanity and alcoholism.
In 1983 a third member of the family, the women's brother, died of the disease. 'He used to be a very
energetic man but suddenly he started sweating profusely, his pupils became as small as pin heads,
mucus came out of his nose, he had an irrepressible twitch and above all a tiredness that brought him to
his knees,' explained a family member.
Sleeping pills and other sedatives have no effect because the neurons required for sleep are missing,
because the prions kill off the neurons that are required to bring about slumber.
In 1986 an article about the Treviso family appeared in the Journal of Medicine and doctors came
forward to reveal dozens of other cases in Europe and Japan.
Then in the early 1990s the scandal surrounding mad cow disease provoked a new interest in prions.
Meanwhile, doctors in Italy began to test the DNA of other members of the family for the disease. A total
of 50 of the 304 possible carriers have tested positive, but not surprisingly nobody wanted to know the
results.
******************************************************************************
http://www.agenziaitalia.it/english/news.pl?doc=200212271634-0150-RT1-CRO-0NF82&page=0&id=agionline-eng.italyonline
MAD COW: BSE, 86TH CASE CONFIRMED
December 2002
(AGI) - Rome, Italy Dec. 27 - The Health Minister informed that the Experimental Zooprofilactic
Institute of Turin confirmed the positive results of a Bse test done on a female bovine.
The case comes from a cattle-breading in the province of Brescia and is the 86th case of Bse in Italy
resulting from one million tests performed up to now to verify the presence of the so-called "mad cow
disease." (AGI)
Alzheimer’s and Creutzfeldt Jakob are sister diseases . . .
http://www.hc-sc.gc.ca/pphb-dgspsp/hcai-iamss/cjd-mcj/cjdss-ssmcj/0500phys_e.html
CJD Surveillance System
The CJD-Surveillance System (CJD-SS) was launched in April 1998 when an information
package concerning our project was mailed out to all physicians whom we thought may be
involved in the care of persons diagnosed with CJD. As of 1 October 1999, we have had 85
referrals to the CJD-SS from physicians who encounter these cases in their practice (Figure
1). Of these 85 referrals we have had gene sequencing done on 30 and autopsies done on 55.
The majority of the reports have been received from Ontario, British Columbia and Quebec;
this representation is as expected as this reflects the provinces with the highest concentration
of the Canadian population. Of these referrals we have had postive confirmation of CJD by
pathology on 39 cases; the remaining were either not definitively confirmed by autopsy (11),
definitively diagnosed as other than CJD (four) or still awaiting confirmation (31). We have had
three iatrogenic (dura mater-derived) cases as well as three cases of Gerstmann-Sträussler24
Scheinker Syndrome (GSSS) reported to the system. No cases of vCJD have been identified in
Canada.
*******************************************************************************************************************
************
THREE CASES OF CJD IN MANITOBA . . .
http://www.gov.mb.ca/health/publichealth/cdc/protocol/cjd.pdf
all cases "classical" . . .
***************************************************
http://www.zarcrom.com/users/alzheimers/odem/cjd1.html
Creutzfeldt-Jakob Disease Fact Sheet
By CJD Voice
Creutzfeldt-Jakob Disease[CJD] may have greater public health consequences than the
suspected number of confirmed cases might indicate. CJD is not reportable in most states
and is often misdiagnosed or omitted from death certificates. CJD is thought to be caused by
prions. While the infectivity can be reduced, it is extremely difficult to kill these infectious
agents. Normal sterilization procedures do not eliminate contamination.
********************************************************************************
CJD and AD = the same ?
http://www.freeessays.cc/db/40/rfh143.shtml
To track the disease the Center for Disease Control has initiated a four-state study of death certificates
(Altman), but since death certificates are not always accurate Davanpour) the survey may not provide an
accurate assessment. This leaves the true prevalence in the United States and other countries remaining a
mystery.
Compounding the uncertainty, autopsies are rarely performed on atypical dementia patients (Harrison)
because medical professionals fear infection (Altman). The officially reported rate of Creutzfeldt-Jakob
Disease incidence is less than one case per million people per year (World). An informal survey of
neuropathologists, however, registered a theoretical range of 2 - 21% of all dementia as actually
Creutzfeldt-Jakob Disease (Harrison) and hundreds of thousands of Americans suffer from severe
dementia every year (Brayne; United).
25
Two other studies average about a 3% Creutzfeldt-Jakob Disease rate among dementia patients
(Mahendra; Wade). A preliminary 1989 University of Pennsylvania study showed that 5% of patients
diagnosed with dementia were actually dying from Creutzfeldt-Jakob Disease (Boller). It would seem that
Creutzfeldt-Jakob Disease is seriously underdiagnosed.
The most common misdiagnosis of Creutzfeldt-Jakob Disease is Alzheimer's disease (Harrison).
Creutzfeldt-Jakob Disease was even described as “Alzheimer’s in fast forward (Wlalzek).” The
symptoms and pathology of both diseases overlap. There can be spongy changes in the brain (a classic
effect of Creutzfeldt-Jakob Disease) in Alzheimer's disease, for example, and senile plaques deposited on
the brain (a classic effect of Alzheimer's disease) in Creutzfeldt-Jakob Disease (Brown).
The causes may overlap as well; epidemiological evidence suggests that people eating meat more than
four times a week for a prolonged period have a three times higher chance of suffering dementia than
long-time vegetarians (Giem), although this result may be confounded by vascular factors (Van Duijn).
Paul Brown, medical director for the U.S. Public Health Service (Gruzen), said that the brains of the
young people who died from the new variant Creutzfeldt-Jakob disease in Britain even look like the
brains of Alzheimer's patients (Hager).
Stanley Prusiner, the scientist who coined the term prion, speculates that Alzheimer's may in fact turn
out to be a prion disease as well (Prusiner). In younger victims the disease could look like multiple
sclerosis or a severe viral infection, according to Alzheimer's expert Gareth Roberts (Brain).
Twenty percent or more of people clinically diagnosed with Alzheimer's disease are found at autopsy to
not have had Alzheimer's at all (McKhann). At Yale, out of 46 patients clinically diagnosed with
Alzheimer's, 6 were proven to have actually had Creutzfeldt-Jakob Disease at autopsy (Manuelidis).
In another post-mortem study 3 out of 12 Alzheimer patients actually died from a spongiform
encephalopathy which is the class of diseases that Creutzfeldt-Jakob Disease belongs to (Teixeira). These
spongiform encephalopathies appear to be diseases that enter the brain and cause holes to appear which
can then alter an animal’s functioning and abilities. Eventually death occurs and the brains of these
animals strongly resemble sponges.
******************************************************************************************
*****
http://neuro-www.mgh.harvard.edu/forum/NursingF/9.13.986.49PMCreutzfeldt-Jakob
THE YALE STUDY - In one study conducted by Yale University researchers 13% of clinically-diagnosed
Alzheimer patients were found upon autopsy to have CJD. (Manuelidis, Elias E. and Laura Manuelidis
Alzheimer Disease and Associated Disorders_ 3 (1989): 100-109 Suggested Links between Different Types
of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and Retroviral CNS Infections.)
**************************************************************************************
VICTIMS OF PRIONS DISEASES . . from news accounts . . .
26
NEW YORK 71 YEAR OLD CAROLINE CONFORTI
http://www.poughkeepsiejournal.com/today/frontpage/stories/fr010204s2.shtml
Friday, January 2, 2004
Illness strikes brain, devastates lives
Human form of mad cow is fatal
By Dan Shapley
Poughkeepsie Journal
It looked like old age had caught up to her, but all too fast.
It was the summer of 2002, and Hyde Park resident Caroline Conforti stumbled
occasionally when walking. She was getting more forgetful.
By Labor Day, her hands trembled and she fell frequently. She had trouble speaking
and seeing clearly. Sometimes an arm would jerk straight up in the air and stiffen so
stubbornly that it took both her husband and daughter to coax it back down. She was so
forgetful, she sometimes forgot to brush her teeth.
Darryl Bautista
Caroline Conforti's
husband, Michael, and
daughter, Linda, hold a
picture of Caroline, who
died in February from
Creutzfeldt-Jacob disease at
age 71.
Local neurologists started treating her for a pinched nerve. But she lapsed into a coma, around Halloween,
before she was finally diagnosed at Lenox Hill Hospital in Manhattan. By February she was dead at age 71.
75 YEAR OLD MARY MCKINLEY –Georgia
http://www.gwinnettdailyonline.com/GDP/archive/article2F265C5F7F394A87A4B44ADCD9483847.asp
Some claim human link to mad cow
By Douglas Sams
doug.sams@gwinnettdailypost.com
BUFORD — The speed of Mary McKinley’s decline shocked everyone.
She was always known for her sharp memory, but began to forget what day it was.
She was treasurer of her Sunday school class at Sugar Hill Baptist, but suddenly struggled to balance her own
checkbook.
Once as level-headed as they come, she suffered what appeared to be panic attacks during the middle of the
night in which she labored to breathe, felt like throwing up and shook violently.
It was late May 2000 when the symptoms began. Within three months, the 75-year-old McKinley was dead.
A biopsy of her brain tissue showed the cause of her death was Creutzfeldt-Jakob Disease, an extremely
rare, fatal disorder whose victims often die within a year. Medical experts insist there is no known link
between classic CJD and mad cow disease.
McKinley’s daughters, Sharron Potts and her sister Debbie Creel-Martin, are convinced the experts are
wrong.
“The scientists don’t really know,” Potts, 56, said. “They don’t want to talk about it. They are trying to
put as little information out there as they can.”
Florence Kranitz, executive director of the CJD foundation, said lately she gets up to 10 calls a day from
people who lost their loved ones to the illness and mistakenly believe it may have been caused by eating
beef from cattle with mad cow disease.
27
The calls have increased in the wake of the mad cow scare that started Dec. 23, when the U.S.
Department of Agriculture said it found the disease in a cow in Washington.
LONG ISLAND NEW YORK 61 YEAR OLD MAXINE POSTAL
http://www.newsday.com/news/local/longisland/ny-lipost033609231jan03,0,4425477.story?coll=ny-linews-headlines
Suffolk Lawmaker Maxine Postal Dies of Brain Disorder
By Katie Thomas
Staff Writer
January 3, 2004
When Maxine Postal first ran for the Suffolk County Legislature in 1987, she ran.
In the weeks leading up to the election, Postal would don a T-shirt that read "Elect Maxine Postal" and jog
through North Amityville during rush hour with her husband tagging alongside in a van.
Postal won, despite being in a district with more registered Republicans than Democrats, and she continued to
win for eight more elections.MAXCThe former presiding officer died New Year's Day at a hospice in West
Palm Beach, Fla., just weeks after she was diagnosed with Creutzfeldt-Jakob Disease, a rare and
degenerative brain disorder. She was 61.
28
29