Published: October 14, 2004 (NY Times)

tate health officials are investigating an unusual cluster of deaths in an
upstate New York county that have all been caused by the same rare brain
disease, they said yesterday.

In the past three months, four people in Ulster County have died from
Creutzfeldt-Jakob disease, a neurological disorder that afflicts only one in
a million people a year worldwide.

There is no cure for the illness and it is invariably fatal, eating away at
the brain and causing symptoms that include behavioral changes, visual
disturbances, involuntary movements, blindness, weakness of the extremities
and a coma.

The disease gained widespread public attention in recent years because it
can, in some cases, be caused by exposure to mad cow disease.

However, state health officials cautioned that there was no reason to believe
that any of the people who died had become ill from contaminated meat.

"We are looking into the suspected cases in Ulster County, and at this point
we don't see anything that would be a concern for public health," said Claire
Pospisil, a spokeswoman for the New York State Department of Health.

The most common form is sporadic Creutzfeldt-Jakob disease, which remains
mysterious to doctors. It strikes people who are otherwise healthy and have
no known risk factors, according to the Centers for Disease Control and
Prevention.

There are 250 to 300 cases of this type of Creutzfeldt-Jakob disease annually
in the United States. New York health officials said they average 20 to 30
cases, largely of this type, a year. According to the C.D.C., the disease
cannot be transmitted through the air, or through touching or most other
forms of casual contact.

Another form of the disease is called variant Creutzfeldt-Jakob, which is
caused by some outside factor, like eating beef from cattle that died of mad
cow disease.

In the United States, only one person, a woman in Florida, has died of
variant Creutzfeldt-Jakob disease related to mad cow disease. The woman, who
died in 2002, had lived in England, and probably contracted the disease
there, health officials said.

Still, because of the unusual number of cases in Ulster County, which is in
the Catskills and has a population of roughly 177,000 people, officials said
they were trying to determine whether there was any connection between the
deaths.

One victim, Coleen Staccio, 46, who lived in Kingston, N.Y., was in decent
health when she started to experience intense headaches in July, her
daughter, Melissa Mentessi, said. Ms. Mentessi, 26, said her mother had a
seizure at home and was taken to Benedictine Hospital in Kingston but was
released several days later, with doctors mystified as to what was causing
the headaches.

However, in a few weeks, Ms. Staccio's headaches worsened, and she returned
to the hospital.

"The next day, when I came to see her, she didn't know dates, she didn't know
how to eat," Ms. Mentessi said.

Ms. Staccio was transferred to Albany Medical Center, where doctors first
mentioned Creutzfeldt-Jakob disease, Ms. Mentessi said. Ms. Staccio died on
Aug. 28.

About the time she died, Robert Tobey, 59, who also lived in Kingston, was
taken to Benedictine Hospital, on Aug. 27, because he was showing signs that
his family took for a stroke, said his daughter Stacey Tobey, 36.

Doctors at first thought his condition might have been caused by a severe
form of sleep apnea, which plagued Mr. Tobey, his daughter said. But "he
progressed to where he couldn't eat, couldn't swallow, couldn't speak," she
said.

She said he was transferred to Mount Sinai Medical Center in Manhattan and
died on Saturday night. His daughter said a biopsy confirmed that he had
Creutzfeldt-Jakob disease.

Health officials would not say whether further tests have shown what type of
Creutzfeldt-Jakob disease Mr. Tobey or Ms. Staccio died from, and they would
not reveal the names or ages of the other two victims.

Mr. Tobey's and Ms. Staccio's families have been in touch in recent days and
have tried to find anything that they might have had in common. All they have
found, so far, was that both had back surgery at Kingston Hospital - Mr.
Tobey in 1995 and Ms. Staccio in 1998.

*makes mental note not to kiss any local cattle*

There is an enlightening article in the October issue of Vanity Fair magazine, pg 240

Intitled "Order The Fish" by Eric Schlosser

It discusses the deadly E.Coli 0157:H7 strain varient, and *Mad Cow*

Basicly this author had reported that the above mentioned scenerio( in the posting by Oxygenbabe) was exactly what was likely to happen--if the gov't did not step up to bat quickly and do something about the haphazard way the beef industry has been testing the cattle

If you remember......Oprah brought this factoid up on her show....and was promptly sued by the Nat'l Cattlemens' Beef Association

this virus can live in your body up to 10years, and then start to show symtoms and even kill like in these cases...this jsut happened in a ct. hospital..same type incident..the hospital had to call people who had had surgery, as they hadn't properly sterilized the instraments after a person had brain surgery was found to have mad cow disease. they had to warn them to watch for symtoms over the next 10 years, that they possibly were infected..

talk about scary....

I definately dont want anything to do with gel caps if you can get sick with them!

But the big question is...if 2 out of 4 had back surgery (the other 2 who died aren't mentioned) years apart at the same hospital, why did those 2 die the same summer...all of a sudden...and who are the other 2?

This would be a major coincidence for a disease that supposedly has a long latency...4 dying the same summer

There are no good answers. Your story is scary too.

We had a dog die this way a few years ago and the vets had let me believe that is was probably cancer..He lost his appetite and slowly died..We did what we could to keep him comfortable and I even hand fed him to no avail..Now some of the vets are more knowledgeable and could have helped him.

I'm pasting in an article below from WrongDiagnosis.com and one of the theories has to do with manganese/copper imbalance. Sheep have a very low tolerance for copper and will go into a catatonic state if they get too much in their diet. This happened to our herd one time and I realized that the inability to tolerate copper probably makes them much more susceptible to prion disease.

In Colorado I've seen many elk and deer with chronic wasting disease. They're now finding that overcrowding has diminished their food supply and they're eating things like pine needles which are high in manganese.

We keep eating meat but we also take copper to help keep down the manganese levels (they compete). I saw a "This Old House" episode that was in New England. The owner had a stains in her dishwasher and was told it was from high manganese in the water.

Here's the article - it's quite informative:

Prion Diseases

Prion diseases are a newly discovered type of disease caused by strange infectious proteins. The best known disease is "mad cow disease" but there are in fact several diseases that appear to be caused by prions. Almost all of these diseases affect the brain.

Prions are infective proteins. They act somewhat like viruses but do not possess any DNA or RNA material, but appear to be only a protein. This idea is somewhat controversial still, but seems to be gaining acceptance.

The most media attention to prions has been due to the "mad cow disease" in animals, and the subsequent "variant CJD" that occurs in humans. However, there are numerous diseases believed to be related. The main diseases believed to be in the class of prion diseases include:

Animal prion diseases:

Bovine spongiform encephalopathy (BSE): the proper name for mad cow disease, causing brain damage.

Scrapies: in sheep (and other animals), caused by brain damage, leading to the sheep itching and scraping off their coats, hence the name.

Feline spongiform encephalopathy - in cats

Others: transmissible mink encephalopathy (TME), chronic wasting disease (CWD) of mule deer and elk

Human prion diseases:

Creutzfeldt-Jakob Disease (CJD): a rare 1-in-a-million disease that occurs spontaneously, with 10-15% inheritance, and unrelated to animal diseases. It usually leads to dementia.

variant CJD: a variant of CJD that may be related to BSE

Kuru: a brain damaging "laughing disease" in Papua New Guinea, where till recently, tribes ate brains of the dead.

Gerstmann Str�ussler Syndrome (GSS): also called Gerstmann-Str�ussler-Scheinker disease; inherited, mainly in midlife.
Fatal familial insomnia (FFI): a strange inherited disease where people actually die from being unable to fall asleep, usually occurring late in life. Unclear: may be caused by prions, or FFI may be an "opposite" to other prion diseases, possibly caused by a deficiency of good prions, rather than an overload of bad prions.

Alpers Syndrome: prion diseases in infants.
Contagion of Prion Diseases

Prions are not highly contagious. They are infective via injection of contaminated products, but not by air or touch. In other words, they are transmitted only via body fluids that contain the protein from within the blood or more commonly fluids from the infected brain.

For example, CJD has also been transmitted by mistakes in surgery and health procedures, especially brain surgery Prusiner 1 mentions that human CJD has been transmitted by "corneal transplantation, implantation of dura mater or electrodes in the brain, use of contaminated surgical instruments, and injection of growth hormone derived from human pituitaries (before recombinant growth hormone became available)".

Genetics of Prion Diseases
Prion diseases are inherited in some percentage of cases, but not all cases. Hence, they are clearly not pure genetic diseases.

Theories of Prion Infection
There is a lot of research about prions and their related diseases. Some of the possible theories include:

Protein-based infection by conformation: where a "bad" prion converts existing normal good proteins in the body into prions, so they increase by conformation or conversion of other proteins, not by replication.

Viruses rather than prions: some researchers still dispute the idea of prions as infective proteins, and claim that some kind of virus is actually involved. This seems doubtful given the difficulty in finding DNA or RNA material in infected samples, but is not yet out of the question.

Copper-Manganese theory: manganese overload, copper deficiency. A controversial theory about BSE (mad cow disease) that was popularized by farmer-amateur-chemist Mark Purdey. Prions are "metalloglycoprotiens" that should bind copper, but may bind manganese instead if copper is deficient and managanese is excessive. Prions have been shown in laboratories to become dangerous when exposed to high manganese and low copper. There are also numerous amounts of circumstantial evidence of the role of manganese and copper. The UK farmers were treating cows with an insecticide containing organophosphates which deplete copper, and were also feeding cows with supplementary chicken manure which is high in manganese due to chicken feed supplementation. There is also evidence of high manganese levels in areas of both variant-CJD cases (BSE-related) and areas of high CJD rates (non-BSE related). Manganese is also less easily absorbed by pigs and chickens, which do not seem to get BSE. Furthermore, manganese miners in times past used to suffer a "manganese madness" disease that seems similar to CJD. There are various conspiracy theories stating that a coverup has started to prevent lawsuits against the insecticide makers, and perhaps even against the makers of other organophosphate-containing products for humans, such as some head lice and scabies medications. In the worse case, such medications might be related to later neurodegeneration and even Alzheimer's disease.

Other trace element theories: with the popularization of the copper-manganese theory, various researchers are examining other trace elements. Possibly implicated in the diseases BSE and CJD are not only copper and manganese, but also iron, selenium, and zinc.

Why Do Prions Kill?

Why do these prion diseases kill animals and people? What causes the damage from the prion infection? The brains of victims are clearly riddled with "encepalopathy", spongy swellings within the brain, but is this the actual cause of death, a side effect itself, or even unrelated? As always there are several theories and possibilities:

Prion clumps cause brain cell damage: perhaps too many bad prions building into spongy deposits create stress within cells, notably brain cells, and eventually damage and kill these brain cells. The idea is that prion clumps become like tumors and damage good cells.

Jan

Ulster County has three federally recognized Superfund sites: the Hertel Landfill, Mohonk Road Industrial Plant, and Ellenville Scrap Iron and Metal. The 80-acre Hertel Landfill in the town of Plattekill was shut down in 1977 for repeatedly accepting waste it didn't have a permit to receive.

At least 50 pollutant-containing metal drums have been unearthed on the site. Ground and surface water are contaminated with iron and manganese and the soil is steeped in arsenic and chromium. Toxins have seeped into nearby wetlands, some of which have been bulldozed into the landfill mound, which has since been capped and fenced off.

The Mohonk Road Industrial Plant Superfund site in High Falls sprang from a septic tank, which for years collected the organic solvent waste of various industrial activities in a 43,000 square foot building. epa says there is ``the potential for significant human exposure through inhalation and ingestion of vocs [volatile organic compounds] in contaminated groundwater'' and that ``soils below ground surface may pose a threat to construction workers if disturbed.''

Over 70 homes and businesses that used to draw from the aquifer are using water filters, while millions of gallons of groundwater is extracted and cleaned, and a new water supply system is designed. In addition, thousands of tons of contaminated soil have been moved off to some other unfortunate piece of the planet.

here's one i found on msn.com..i'll be back with more...

Lisa

Posted 10/15/2004 6:24 AM

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FDA advisers: Safeguards for blood in U.S. are sufficient
WASHINGTON (AP) -- The United States doesn't plan to toughen safeguards on blood donations, despite two British cases in which recipients most likely were contaminated with mad cow disease after receiving transfusions from seemingly healthy donors.
Food and Drug Administration advisers agreed unanimously Thursday that current U.S. safeguards are sufficient.

"The United States is not Great Britain," said Dr. Stephen DeArmond, a member of the FDA panel and a University of California-San Francisco pathology professor.

DeArmond pointed to the lack of evidence that so-far symptomless mad cow infections suspected in thousands of British residents are an issue in the United States.

The United States has had only one confirmed case of mad cow disease, involving a Canadian-born cow. No humans are known to have been infected through exposure in the United States with variant Creutzfeldt-Jakob disease, the human equivalent of mad cow disease.

The FDA already bars blood donations from people who lived more than three months in Britain or who received transfusions there after 1979, among other safeguards.

The advisory committee agreed those restrictions are still warranted. Testimony suggested they reduced risks by 91% to Americans using donated blood products.

In both confirmed British infections, recipients got blood from donors who were young and apparently healthy at the time of donation. The chances were remote that the two confirmed were caused by eating mad-cow tainted beef -- at least 1 in 80,000 to 1 in 1 billion, the panel was told.

The newer case involves a woman in her 80s who died from an unrelated medical condition early this year, according to Dr. Robert Will, a neurologist who founded the British mad cow surveillance team.

Because the woman had received a blood transfusion in 1999 from a donor who later died from variant Creutzfeldt-Jakob disease, the woman's doctor did a more thorough analysis after her death.

The woman had no outward signs of neurological disease and her brain, spinal cord, tonsils and appendix were normal, Will told the panel. In her spleen and cervical lymph nodes, however, the woman harbored malformed, ineffective prion proteins.

Mad cow disease occurs when normal prion proteins found in organs and tissues, including the brain, change shape and coax healthy prions nearby to do the same. When enough of the proteins are altered, they deposit a plaque on the brain and surround the mark with spongy holes, killing the victim.

Will told the panel he didn't know if the woman would have ever developed symptoms of the neurological disorder.

Dr. James Sejvar, another federal adviser, said the new case raised questions whether some people exposed to mad cow might ultimately prove resistant to the devastating ailment or be unable to pass the infection to others.

"It's intriguing," said Sejvar, a neuroepidemiologist at the Centers for Disease Control and Prevention.

By testing 14,964 appendices of normal surgical patients in England, researchers learned that three were tainted with the ineffective proteins. That implies 237 people per million British residents -- or 3,808 people age 10 to 30 -- could silently incubate mad cow, Will said.

G. Michael Fitzpatrick, chief policy officer at America's Blood Centers, acknowledged worries about a potential second wave of mad cow cases in England.

Still, current safeguards appear to be working in the United States, Fitzpatrick said. Already, the nation's major blood suppliers, ABC and the American Red Cross, estimate current deferrals block 400,000 previous blood donors.

Instead of additional donor restrictions, the FDA should consider an "exit strategy" that would set thresholds for ending the blood supply sapping measures, Fitzpatrick said.

The federal advisers soundly reject that notion, agreeing unanimously that the current donor deferral strategies are still warranted.

Mad-cow disease spread in surgery?
Study suggests disease may be passed in muscle tissueNov. 6 - Swiss researchers said Thursday they found infectious proteins in the muscle tissue of patients who died of Creutzfeldt-Jakob disease and said the finding may suggest the rare and fatal brain disease could be passed on during standard surgery.

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The study, published in this week's issue of the New England Journal of Medicine, also raises the question of whether mad cow disease -- bovine spongiform encephalopathy -- might be passed on in muscle tissue and not simply in brain, lymph and spleen tissue.

Dr. Adriano Aguzzi, of the University Hospital of Zurich, led the study, in which the researchers examined the tissues of patients who died of CJD, Alzheimer's disease and other causes.

BSE, CJD and related diseases, including a new form of CJD caused by eating infected beef products, are caused by an infectious version of a protein called a prion.

There is no cure for any of the diseases and they are always fatal. BSE decimated Britain's cattle population and has spread to several countries, including Switzerland.

CJD occurs naturally in about one in a million people and its cause is unknown. The new variant of CJD, called vCJD, has been diagnosed in 139 people, according to the World Health Organization.

Most cases of CJD have no known cause -- they are sporadic. Up to 10 percent can be caused by a gene mutation and about 10 percent are caused by the use of infected brain, eye or nerve tissue in surgical operations.

The only way to confirm CJD is a brain autopsy, but experts are trying to develop a blood test. Animals can be tested for BSE using samples from the tonsils.

Aguzzi's team examined 36 patients with what was believed to be sporadic CJD who died between 1996 and 2002.

They found prions in all brain tissue and in 10 of 28 spleen specimens. They also found prions in eight of 32 muscle samples.

``Our findings arouse concern about the possibility of iatrogenic (caused by medical treatment) transmission of sporadic Creutzfeldt-Jakob disease,'' they wrote.

They stressed that they had not shown the muscle tissue was infectious and said perhaps the Swiss samples were unique in some way.

But they noted that patients who had undergone surgery of any kind had a slightly higher risk of CJD.

``Some allegedly sporadic cases may in reality have an iatrogenic origin,'' they wrote.

lisa

--------------------------------------------------------------------------------
Rense.com
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Louisiana Man Exposed
To Mad Cow/CJD During
Surgery Sues Hospital http://www.insidechina.com/news.php3?id=225603
11-30-00


BATON ROUGE, Louisiana (Reuters) -- A Louisiana man who may have been exposed to a rare, fatal brain-wasting disease during surgery is suing the university hospital where his operation was performed, his attorney said on Wednesday.

Michael Rebert, 28, of New Iberia, Louisiana, was told by his neurosurgeon at Tulane University Medical Center in New Orleans last month that he might have been exposed to Creutzfeldt-Jakob disease (CJD) during an operation in April due to contaminated surgical instruments, said lawyer Alicia Hoover of Baton Rouge.

The disease, the human variant of "mad cow disease," causes rapid deterioration of the brain, with progressive dementia and loss of physical functions, medical authorities said. Death usually occurs within a year after the onset of symptoms.

The suit, filed in state civil district court in New Orleans this week, seeks unspecified financial damages.

Hoover said Rebert underwent surgery in April to remove a small portion of his brain that caused him to have multiple and increasingly severe seizures in a form of epilepsy.

"The irony was that there was a big family debate over whether to have the surgery," she said. "His father, who died just three weeks before the operation, had made him promise to take care of his mother and a younger sister who has cerebral palsy."

Hoover added, "That's why he had the surgery, to be able to hold down a job and care for them," Hoover said.

Although CJD can take years to develop, she said, "More than 50 percent of those exposed to the disease through contamination from infected brain tissue show symptoms of the disease within 0.6 to 2.2 years."

Last month, Tulane confirmed that eight neurosurgery patients may have been exposed to CJD through surgical instruments that may have been contaminated by use on an earlier brain-surgery patient who had the disease.

The hospital said the instruments received routine washing and sterilization after being used on the earlier patient, but admitted that the risk of spreading the disease may not have been eliminated.

CJD was confirmed during an autopsy of the earlier patient, according to a written statement issued last month by Dr. Alan Miller, vice president for clinical affairs at the hospital. The disease can only be determined after an autopsy, he said.

The university on Wednesday refused to release any information not contained in the October statement, which said counseling and follow-up medical care was being offered to the eight patients who may have been exposed.

CJD is believed to be caused by a prion, an unconventional pathogen thought to transform normal protein molecules into deadly ones by altering their shape, authorities said.

Prions are resistant to normal sterilization procedures, Centers for Disease Control and Prevention spokesman Tom Skinner said. Instruments must be treated for an hour in an autoclave, a device used for sterilization employing superheated steam under pressure, at 270 degrees Fahrenheit (132 degrees Celsius) followed by submersion in sodium hydroxide for successful decontamination, Skinner said.

Creutzfeldt-Jakob disease is related to bovine spongiform encephalopathy, better known as mad cow disease, which is thought to be transmitted to humans through the eating of infected beef. At least 70 people in Great Britain have died from the bovine-related illness.

Skinner said the disease is so rare in the United States that it is found in only one in a million people.

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