# First Ever Study To Investigate Impact Of Cwd On Humans



## terry (Sep 13, 2002)

From: TSS ()
Subject: First ever study to investigate impact of chronic wasting disease on humans
Date: November 26, 2005 at 6:06 pm PST

First ever study to investigate impact of chronic wasting disease on humans

By Gail Glover


Researchers at Binghamton University have a first-ever opportunity to determine if Chronic Wasting Disease (CWD) in deer can be spread to humans who ingest infected meat.

Ralph M. Garruto, professor of biomedical anthropology at Binghamton University, State University of New York is heading up a study to monitor the health implications of a group of people who are known to have consumed venison infected with CWD. Recently discovered in both wild and captive deer herds in New York, CWD is similar to mad cow disease in that it concentrates in the spinal cord and brain, and is caused by a virtually indestructible mutated protein called a prion.

We dont know if CWD can be transmitted to humans, said Garruto. So this group, some of whom we know for sure ate infected meat, offers us a unique opportunity. Im hoping the study will allow us to determine conclusively if this disease can affect humans in the same way mad cow disease has been shown to cause neurological disease in those who consume infected beef.

The study focuses on a group of people who attended a sportmens feast in Verona, NY, earlier this year. It is known that at least some of the attendees, all of whom were offered a variety of entree choices, consumed venison from a deer infected with CWD. Upon hearing of the dinner, Garruto approached the Oneida County Health Department (OCHD) to determine if they would assist in a scientific examination of the people who ate the meat.

Although not everyone involved is particularly concerned or fearful, it is important for us to protect the health of all county residents, said Ken Fanelli, OCHD representative. Professor Garrutos study is a proactive response to determining what, if any, will be the long-term health effects, which is one of our most important responsibilities.

Over 50 participants have already signed up to be part of the study that will involve an initial interview and completion of a questionnaire to help assess risk, including the role played by individuals at the dinner, what they ate, their place of residence, occupation, medical history and other activities. The study will monitor the health of the participants over a period of six years. No invasive testing will be performed and identities will be kept strictly confidential.

The people who take part in this project can be assured that every measure will be taken to ensure their privacy, said Garruto. Their contribution is vital to the success of this first of its kind research that may hold world-wide significance in the study of CWD and similar prion diseases.

CWD was first discovered in Colorado in 1967 and has since been documented in several Rocky Mountain and Midwest states. This year, New York State became the first state west of the Mississippi to report CWD in both privately owned and wild deer herds found in parts of Oneida County. Most recently in September, West Virginia reported its first cases of the disease. How the disease is spread from deer-to-deer and how it may impact the environment in which infected animals graze is unclear.

Were looking at an issue that could have multiple impacts, said Garruto. Human health and keeping the food supply safe is of primary concern. But we also have to monitor how to keep this epidemic from spreading among deer and across species, from deer to cattle, both of which could have huge economic as well as health implications.

Garruto notes that although a prion disease appears to be transmitted through direct animal-to-animal contact and/or indirect exposure, including contaminated water, soil and brouze by saliva, urine, and feces, it is still unclear as to how its transmitted.

CWD demands a lot more attention than its been getting, says Garruto. Too little research has been done so far to be sure humans cant contract the disease and we do not know if transmission from deer to cattle who share the same grazing land is possible. This is an important missing link as cow to human cross-species transmission does take place, evidenced by the mad cow and variant Creutzfeldt Jacob Disease epidemics in Europe. This study will give us some solid conclusions and allow us determine how to manage the risks.


Researchers at Binghamton University have a first-ever opportunity to determine if Chronic Wasting Disease (CWD) in deer can be spread to humans who eat "infected" meat.

Ralph M. Garruto, a professor of biomedical anthropology, is heading up a study to monitor the health implications of a group of people who are known to have consumed venison infected with CWD. Recently discovered in both wild and captive deer herds in New York, CWD is similar to mad cow disease in that it concentrates in the spinal cord and brain, and is caused by a virtually indestructible mutated protein called a prion. "We don't know if CWD can be transmitted to humans," Garruto said. "So having a group that we know for sure ate infected meat offers us a unique opportunity. I'm hoping the study will allow us to determine conclusively if this disease can affect humans in the same way mad cow disease has been shown to cause neurological disease in those who consume infected beef."

The study focuses on a group of people who attended a feast in Verona earlier this year and who might have consumed venison from a deer infected with CWD. Upon hearing of the dinner, Garruto approached the Oneida County Health Department (OCHD) to determine if officials there would assist in a scientific examination of the people who ate the meat. "Although not everyone involved is particularly concerned or fearful, it is important for us to protect the health of all county residents," said Ken Fanelli, an OCHD representative. "Professor Garruto's study is a proactive response to determining what, if any, will be the long-term health effects, which is one of our most important responsibilities." More than 50 people have signed up to be part of the study, which will involve an interview and questionnaire to help assess risk, including the role played by individuals at the dinner, what they ate, their place of residence, occupation, medical history and other activities. The study will monitor the health of the participants over six years. No invasive testing will be performed and identities will be kept confidential. "The people who take part in this project can be assured that every measure will be taken to ensure their privacy," Garruto said. "Their contribution is vital to the success of this 'first of its kind' research that may hold world-wide significance in the study of CWD and similar prion diseases."

CWD was first discovered in Colorado in 1967 and has since been documented in several Rocky Mountain and Midwest states. This year, New York became the first state west of the Mississippi to report CWD in both privately owned and wild deer herds found in parts of Oneida County. How the disease is spread from deer to deer and how it may impact the environment in which infected animals graze is unclear. "We're looking at an issue that could have multiple impacts," Garruto said. "Human health and keeping the food supply safe is of primary concern. But we also have to monitor how to keep this epidemic from spreading among deer and across species, from deer to cattle, both of which could have huge economic as well as health implications."


http://research.binghamton.edu/discovere/discovere files/TopStories/ChronicWaste.htm


> The study will monitor the health of the

> participants over a period of six years


this is not long enough due to the incubation period.

it would be interesting to know more about this study, i.e. consumption, dose etc. just what the feast consisted of, just meat, or did any have organs/srms, the gutting and cleaning of this deer, where this took place, by whom, and what else was cut up there and where did that go. any cuts and abrasions etc. if any of these participants in this feast went on later to have medical procedures, gave blood, dental, etc. how they plan to follow this? i would like to know more about the pathology of the deer they feasted on too, that might be interesting as well?


TSS


Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi

Summary

Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of

electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD

classifications.


snip...


The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species?


http://neurology.thelancet.com Published online October 31, 2005


Neurology 2003;60:176-181
© 2003 American Academy of Neurology


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


VIEWS & REVIEWS:
Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, and Lawrence B. Schonberger

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Neurology 2003; 60: 176-181 [Abstract] [Full text] [PDF]


Correspondence published:

Reply to Singletary
Ryan A. Maddox, MPH, Ermias D. Belay, MD, Lawrence B. Schonberger, MD (26 March 2003)

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary (26 March 2003)

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

Send Post-Publication Peer Review to journal:


Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States


Email Terry S. Singeltary:


[email protected]


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


http://www.neurology.org/cgi/eletters/60/2/176#535


http://www.neurology.org/cgi/eletters/60/2/176#535


LANCET INFECTIOUS DISEASE JOURNAL


Volume 3, Number 8 01 August 2003


Newsdesk


Tracking spongiform encephalopathies in North America


Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost

my mom to hvCJD (Heidenhain variant CJD) and have been searching for

answers ever since. What I have found is that we have not been told the

truth. CWD in deer and elk is a small portion of a much bigger problem.


49-year-old Singeltary is one of a number of people who have remained

largely unsatisfied after being told that a close relative died from a

rapidly progressive dementia compatible with spontaneous

Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

encephalopathy (BSE), Americans might get a similar disorder from

chronic wasting disease (CWD) the relative of mad cow disease seen among

deer and elk in the USA. Although his feverish search did not lead him

to the smoking gun linking CWD to a similar disease in North American

people, it did uncover a largely disappointing situation.


Singeltary was greatly demoralised at the few attempts to monitor the

occurrence of CJD and CWD in the USA. Only a few states have made CJD

reportable. Human and animal TSEs should be reportable nationwide and

internationally, he complained in a letter to the Journal of the

American Medical Association (JAMA 2003; 285: 733). I hope that the CDC

does not continue to expect us to still believe that the 85% plus of all

CJD cases which are sporadic are all spontaneous, without route or source.


Until recently, CWD was thought to be confined to the wild in a small

region in Colorado. But since early 2002, it has been reported in other

areas, including Wisconsin, South Dakota, and the Canadian province of

Saskatchewan. Indeed, the occurrence of CWD in states that were not

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.


To date, experimental studies have proven that the CWD agent can be

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

lymphoid tissue before invasion of the central nervous system. Yet the

plausibility of CWD spreading to people has remained elusive.


Part of the problem seems to stem from the US surveillance system. CJD

is only reported in those areas known to be endemic foci of CWD.

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.


Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

prohibiting material from CWD-positive animals from being used as an

ingredient in feed for any animal species, epidemiological control and

research in the USA has been quite different from the situation in the

UK and Europe regarding BSE.


Getting data on TSEs in the USA from the government is like pulling

teeth, Singeltary argues. You get it when they want you to have it,

and only what they want you to have.


Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.


Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunters two of whom were friends who died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.


CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very

limited.


http://infection.thelancet.com/journal/journal.isa


TSS


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