Prion Surveillance Center Fighting For Life

Alzheimer’s, Creutzfeldt-Jakob, Mad Cow and Chronic Wasting All Caused By Prions

By John Fauber, Reporter, MedPage Today

When Tim Suroviak developed cognitive problems after coming home following heart surgery last year, one of the first questions doctors asked was whether he was a hunter. He was not, though his family did eat venison a few times year.

Suroviak, 63, deteriorated quickly from a rare brain condition known as Creutzfeldt-Jakob disease (CJD). The disease is in the same family as chronic wasting disease, which is endemic in Wisconsin’s deer herd. The Suroviaks live in Wausau, Wisconsin.

After his death, Suroviak’s brain was sent to a lab in Ohio, which led to some shocking news for the family: The type of CJD he had was especially rare, caused by a genetic defect.

That discovery allowed family members to be tested for the disease. So far, no one, including his two daughters, carries the mutation, said Suroviak’s wife, Monica.

But the search for rare prion diseases, whether genetic, sporadic, or potentially caused by eating meat from infected animals, could be curtailed beginning next year.

Prions and Alzheimer's disease

The country’s prion disease surveillance center that looks for new brain disorders would lose all of its federal funding and cease operations under President Trump’s proposed fiscal 2018 budget.

For 20 years, the brains of people who died of suspected prion diseases, such as Creutzfeldt-Jakob disease, have been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland.

The center also is the only licensed lab that can test spinal fluid from living people who have suspected Creutzfeldt-Jakob disease. Such tests can allow families to find out if someone has the disease and what type it is, and then plan how to spend the person’s remaining months. The disease is always fatal.

The loss in funding would come at a time of heightened concern over whether chronic wasting disease can infect people. Recent research showed that it could infect monkeys that were fed venison from infected deer.

At the same time, the number of Creutzfeldt-Jakob cases has jumped substantially nationally and especially in Wisconsin, where chronic wasting disease has now been identified in wild deer in 18 counties. For now, officials attribute the increase to better surveillance and an aging population.

chronic wasting disease

“I don’t know why we want to stop surveillance at a time like this,” said Debbie Yobs, president of the Creutzfeldt-Jakob Disease Foundation. “It doesn’t seem logical.”

The foundation is a nonprofit organization that supports families affected by prion diseases, as well as raising awareness and supporting medical research.

For the Suroviaks, the lab provided peace of mind. First, it let the family know that Tim had a genetic form of the disease, which occurs in between 5 percent and 15 percent of cases. Overall, Creutzfeldt-Jakob disease occurs in about 1 to 1.5 one million people a year worldwide with the vast majority being the sporadic type, meaning the cause is unknown.

While no family members have tested positive for the disease, it turns out that two first cousins of Suroviak died of the disease, Monica Suroviak said.

“If the testing had not been done, we would have never known,” she said.

In 2002, the year CWD was discovered in Wisconsin, six cases of Creutzfeldt-Jakob disease were recorded in the state, according to the Department of Health Services. In two of the last four years, 13 cases have been recorded in the state. That’s a 117 percent increase.

Nationally, there also has been an increase in Creutzfeldt-Jakob cases. In 2002, there were 260 cases, compared with 481 in 2015, an 85 percent increase, according to data from the CDC.

mad cow disease

Creutzfeldt-Jakob is closely related to the form of mad cow disease that infected people, primarily in Great Britain, in the late 1990s and early 2000s, after they ate beef from infected cows. Indeed, human mad cow disease is known as variant Creutzfeldt-Jakob. Both diseases attack the brain, and death usually occurs within a year.

Chronic wasting disease now is in 21 states, primarily in the Upper Midwest and Rocky Mountain states.

Mark Zabel, a prion researcher at Colorado State University, said chronic wasting disease may be mutating and someday could infect people, if it hasn’t already.

“If you don’t have a prion surveillance center that looks for it, you are going to miss it,” he said. “Then, it probably is going to be too late.”

About 400 brains are sent to the prion surveillance center each year. Since 1997 it has contracted with the CDC to provide prion disease surveillance. It has developed a vast library of tissue samples that help show how prion diseases manifest and mutate in people.

Prion diseases are not static; they evolve, Jiri Safar, MD, director of the prion surveillance center and a professor of pathology and neurology at Case Western, said.

“We do not have evidence of a jump to humans, but that is the concern among many in the public health and research community,” he said. (Editor’s Note: There also is no evidence of a reliable species barrier.)

The federal government spends about $6 million a year supporting prion disease surveillance.

Christine Pearson, a spokesperson for the CDC, would not say whether it supported the budget cut.

“The President’s budget is an important part of the federal appropriation cycle; however, Congress will determine final funding levels for all federal agencies,” she said in an email.

Thanks to the Milwaukee Journal Sentinel and MedPage Today for the great report.

Prion Disease News

Alzheimer's disease public relations firm

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Sewage Sludge On Land Spreading Brain Disease

Infectious Waste Spreading Brain Disease

Brain disease is attacking record numbers of people around the world. Microcephaly in infants is part of the same scourge. The global epidemic is being fueled by infectious waste that’s contaminating our food, water, air and more. This infectious waste (biosolids) contains deadly and unstoppable neurotoxins, but it’s being spread like fertilizer in virtually every country around the world.

biosolids land application contaminates food water

In 1972, world leaders realized that dumping millions of tons of sewage sludge into the oceans killed entire underwater ecosystems. Some nations stopped the dumping immediately. Others did not.

The U.S., for example, finally passed the Ocean Dumping Ban Act of 1988. It required dumping all municipal sewage sludge and industrial waste on land. That meant dumping it into landfills or dumping it openly on land, including farms, ranches, national forests, city parks, golf courses, playgrounds, sport fields and beyond. The Act went into effect in 1992 and it sparked a public health disaster. The practice is spreading tons of pathogens into the lives of people, livestock, wildlife and beyond every day.

Landfills designed to handle these toxins are expensive. So, the dumpers hired a public relations firm to convince innocent citizens that neurotoxins and carcinogens are fertilizer. The PR firm started calling this toxic waste biosolids. It’s even sold in bags at your local home and garden store as soil for your garden and potting plants. It’s death dirt.

infectious waste

Since then, millions of tons of infectious sewage sludge have been given to farmers as fertilizer and dumped into food and water supplies around the world every year. Those farmers and ranchers are being paid to dump deadly sewage sludge on their land and shut up. Sick livestock are sold or buried as quickly as possible. Those that make it to market are consumed by people and pets, while permanently contaminating everything that they touch.

The landowners are held harmless if the sewage sludge causes damage to people or property downwind, downstream or on the dinner table. Landowners are literally making a killing with government assistance. Unfortunately, the practice of dumping extreme quantities of sewage sludge on land has created an even bigger public health problem. It’s killing people, wildlife, livestock and sea mammals downstream.

biosolids land application

Prions are infectious proteins responsible for a group of diseases known as transmissible spongiform encephalopathy (TSE). The operative word is transmissible.

TSEs have a wide range of confusing names, which helps cloak this global disaster:

  • bovine spongiform encephalopathy (mad cow disease) in cattle;
  • scrapie in sheep;
  • Creutzfeldt-Jakob disease in humans; and
  • chronic wasting disease in deer, elk, moose and reindeer.
  • badgers, mink, cats, elephants, dolphins and many other mammalian species have died from TSE. The concept of a species barrier is a myth. A deadly prion is a deadly prion. They don’t discriminate.

According to Nobel Laureate Stanley Prusiner, Alzheimer’s disease, ALS and Huntington’s disease also are on the TSE spectrum. All are fatal, neurodegenerative brain diseases.

Infectious prions are in the bodily fluids of its victims, including blood, urine, mucus, saliva and feces. As such, these victims send prions to the municipal sewage treatment plant where they remain untouched.

“There is now real evidence of the potential transmissibility of Alzheimer’s,” says Thomas Wiesniewski M.D. a prion and Alzheimer’s researcher at New York University School of Medicine. “In fact, this ability to transmit an abnormal conformation is probably a universal property of amyloid-forming proteins.”

A new study published in the journal Nature renews concern about the transmissibility of Alzheimer’s disease between people. A second study by the same scientist in early 2016 adds to the stack of evidence.

Wastewater effluent and sewage sludge applied to land recycles prions into the environment. Once dumped on open land, prions remain infectious. Irrigation, precipitation and wind carry the prions into groundwater, streams, lakes, oceans and airways, including homes, offices and beyond.

wastewater treatment plant

Reckless wastewater treatment policies and practices are now fueling a global epidemic of neurodegenerative disease among people, wildlife and livestock. In fact, Europe just reported its first case of chronic wasting disease in a reindeer in Norway. There will be many more.

The risk assessments for the land application of sewage sludge (LASS) are based on fraud and outdated information. The risk assessments were developed back in the 1970s and 1980s–before we knew about prions and other killers in modern sewage streams, including many forms of infectious medical waste. These outdated risk assessments make the entire practice illegal today under bioterrorism laws. Common sense makes them immoral and a crime against humanity.

Because of these reckless practices, it’s time to reform many laws, practices and policies. For example, it’s vital to demand testing for mad cow disease in beef cattle and hope like hell that dairy producers aren’t spreading the disease in milk, cheese and meat. Wisconsin, dairy land U.S.A., has an epidemic among wild deer. It has dumped sewage sludge in virtually every county. There is no reason to believe that the cattle are immune from the prion epidemic that’s being fueled by sick soil in Wisconsin, Colorado and beyond.

mad cow disease

There is no reliable test for live animals, yet, which means that animal health is paramount for public health. There also is no testing of crops grown in sewage sludge, despite the science that has proven that crops for humans and livestock absorb the toxins and pathogens that they are grown in–including deadly prions.

Ironically, the United States passed homeland defense laws to protect our food and water supplies from potential terrorists. Many other nations followed suit. When the U.S. government enacted the Bioterrorism Preparedness and Response Act of 2002, it classified prions as select agents that pose an extreme risk to food, water and much more. With reckless policy, the U.S. transferred responsibility for the management of select agents to the Centers for Disease Control (CDC–a private corporation that serves as the Coverup Division). These defenders of public health quietly took prions off the list of select agents because the regulation criminalized entire industries and several reckless practices. They obviously chose to defend the bottom line of corporations and the wastewater treatment industry instead of public health.

Unfortunately, prions linger in the environment, homes, hospitals, nursing homes, dental offices and beyond infinitely. They migrate, mutate, multiply and kill with unparalleled efficiency. Prions defy all attempts at sterilization and inactivation.

Prions shed from humans are the most deadly. They demand more respect than radiation. They’re being ignored by regulators and industry alike. As such, food and water sources are being contaminated with the deadliest forms of prions. Municipal water systems can’t stop them from reaching water taps in millions of homes. Filtration doesn’t phase them.

As stated earlier, the risk assessments for biosolids, sewage sludge and reclaimed wastewater were questionable when they were developed and they are total failures now. Plus, these risk assessments do not account for the possibility of sewage sludge dumped on land going airborne via windstorms and tornadoes. These events now leave a trail of sickness and death in their wake. Airborne sewage is a killer. It dumps the toxins, pathogens and superbugs everywhere.

Valley Fever caused by land application of sewage sludge

Unfortunately, the U.S. exported these bad practices to other nations who proceeded to contaminate their food and water supplies with sewage. If hospitals can’t stop prions, neither can the brain surgeons at wastewater treatment plants.

The legislation banning ocean dumping was very explicit about the need to stop dumping potentially infectious medical waste into the oceans. Ironically, the current policy that promotes LASS ignores the risk of infectious medical waste and many other threats. It also ignores radionuclides, endocrine disruptors, birth control pills, antibiotics, flame-retardants and other toxins and superbugs. This toxic waste belongs in a lined landfill not our watersheds and food supplies. It’s time for immediate reforms.

The same sewage-borne toxins and pathogens are still contaminating our oceans. Now, they’re dumped in further upstream. Entire watersheds are now being infected—including the oceans. The body count among people, livestock and wildlife has been stacking up ever since ocean dumping began phasing out. The nightmare is worse than ever.

caregivers Alzheimer's disease

Biosolids and other forms of sewage mismanagement are now contributing to a global epidemic of neurological disease, including Alzheimer’s disease, Parkinson’s disease, autism, mad cow disease, chronic wasting disease, microcephaly and more. Industry and governments are scrambling to blame the global epidemic on anything but contaminated soil, water, food and air. They are playing dumb in the face of fraud and scientific suppression. Negligence is too kind of a word.

Sewage sludge and reclaimed wastewater also contaminate our food with listeria, e-coli, salmonella and other killers. In fact, scientists are forced to come up with deceptive new names for the growing list of sewage-related ailments, including Zika virus, West Nile virus, epizootic hemorrhagic fever, equine herpes, valley fever and others. Industrial disease. Taxpayer dollars at work.

As mentioned earlier, crops contaminated by sewage sludge can uptake prions and deliver them throughout the plant. Crops then deliver deadly prions to mammals that consume them. In fact, infected plants are spreading prion disease to several species, including humans. When hamsters consumed infected wheat grass, the animals were infected with prion disease. Researchers also found deadly prions in plants that just made surface contact with infected urine and feces.

“These findings demonstrate that plants can efficiently bind infectious prions and act as carriers of infectivity, suggesting a possible role of environmental prion contamination in the horizontal transmission of the disease,” said Claudio Soto, the lead investigator from the University of Texas at Houston.

Killer prions are impossible to stop. Prions are contributing to the death of millions of people now. Victims produce and spread prions daily because they’re in the bodily fluids of all victims. Millions of people with brain disease are contaminating their homes and communities, while exposing caregivers and family members to the contagion. The sewage from these victims is contaminating the local wastewater treatment plant and everything that enters or leaves these facilities, including reclaimed wastewater and sewage sludge. Once dumped on open land, these contagions remain infectious as they migrate, mutate and multiply forever.

Prions and Alzheimer's disease

Prions demand containment and isolation, not distribution and consumption through air, food and water. These toxins demand lined landfills not reckless dumping on our dinner tables. Since prions migrate, mutate and multiply, dilution is not a solution. Prions are a public health nightmare, not to mention the carnage taking place among other mammals.

The world has never done an effective job of managing its sewage. It’s an industry that drives by looking in the rear view mirror. It only swerves when the dead-end road is littered with body bags. After enough people get sick and die, new alternatives emerge. Today is no different.

We now have nearly eight billion people competing for food, water, open spaces and places to dump their sewage. As prion disease spreads and as industrial-scale agriculture becomes more intensive, sewage is becoming deadlier by the day. The stakes have never been higher.

The bodies are stacking up. The contamination grows stronger and spreads further every day. It’s time to stop dumping sewage sludge on land because of the prion risk and many others not accounted for in the antiquated and fraudulent risk assessments. It’s time for citizens to defend our land, water and air. Homelands around the world are under assault and ISIS has nothing to do with it. The terrorists are home-grown traitors. It’s treason.

Today, the land application of sewage sludge is killing mammals and more around the world. Pathogens in sludge are causing brain disease, cancer and death. Let’s take a meaningful stand for food safety. Just say no to sewage sludge in our watersheds and food supplies. Safer alternatives exist.

treat Alzheimer's disease

Take a free preview of our new eBook to learn everything that you need to know about the epidemic and the mismanagement. The rest of the book explains how to defend yourself with aversion and targeted nutrition. Eating organic foods is one way to minimize your exposure to sewage-borne toxins and pathogens. There are no silver bullets.

Please join our global coalition of Homeland Defenders. Join our campaign for truth and reform. Please write to Gary Chandler for more information gary@crossbow1.com

Read More At http://crossbowcommunications.com/land-application-of-sewage-sludge-spreading-brain-disease/

Do Prions Cause All Forms Of Neurodegenerative Disease

Deadly Proteins Link Creutzfeldt–Jakob Disease, Alzheimer’s, Parkinson’s

By Diana Kwon, Scientific American

In the human form of mad cow disease, called Creutzfeldt-Jakob, a person’s brain deteriorates—literally developing holes that cause rapidly progressing dementia. The condition is fatal within one year in 90 percent of cases. The culprits behind the disease are prions—misfolded proteins that can induce normal proteins around them to also misfold and accumulate.

Scientists have known that these self-propagating, pathological proteins cause some rare brain disorders, such as kuru in Papua New Guinea. But growing evidence suggests that prions are at play in many, if not all, neurodegenerative disorders, including Alzheimer’s, Huntington’s and Parkinson’s, also marked by aggregations of malformed proteins.

Prions and Alzheimer's disease

Until recently, there was no evidence that the abnormal proteins found in people who suffer from these well-known diseases could be transmitted directly from person to person. The tenor of that discussion suddenly changed this September when newly published research in the journalNature provided the first hint such human-to-human transmission may be possible. (Scientific American is part of Springer Nature.)

For the study, John Collinge, a neurologist at University College London, and his colleagues conducted autopsies on eight patients who died between the ages of 36 and 51 from Creutzfeldt-Jakob. All the subjects had acquired the disease after treatment with growth hormone later found to be contaminated with prions. The surprise came when the researchers discovered that six of the brains also bore telltale signs of Alzheimer’s—in the form of clumps of beta-amyloid proteins, diagnostic for the disease—even though the patients should have been too young to exhibit such symptoms.

Alzheimer's disease prevention and treatment

These observations suggest that the tainted hormone injections might have carried small amounts of beta-amyloid proteins that triggered the formation of more such proteins. Neither Alzheimer’s nor any known human prion diseases are contagious through direct contact. Yet human transmission of prion diseases has occurred through certain medical procedures and, in the case of kuru, cannibalism. The new study therefore raises the possibility that Alzheimer’s is a transmissible disease with an etiology akin to prion diseases.

The new finding is provocative, but experts advise caution in interpreting the results. For instance, neuroscientist John Trojanowski of the University of Pennsylvania points to the small size of the study and lack of direct evidence for transmission in support of causality. But if it is eventually shown that Alzheimer’s and other neurodegenerative diseases indeed share the same basic pathological pathway and mechanism, treatments could target one and all.

“Transmission may occur in only a small percentage of human cases,” says Claudio Soto, a professor of neurology at the University of Texas Health Science Center at Houston. “But the underlying principle is the most important thing that could lead to new opportunities for therapeutic interventions and diagnostics.” Investigators such as Soto and Collinge are working on ways to detect in body fluids the presence of small clumps of the transmissible proteins now thought to be involved in Alzheimer’s and other neurodegenerative diseases, which could represent a diagnostic advance.

Alzheimer's disease treatment

Such detection will likely be difficult. A study published online in September in the journal Nature Neuroscience by Mathias Jucker of the University of Tübingen in Germany and his colleagues required extremely sensitive methods to find minuscule clumps of beta-amyloid proteins, referred to as seeds, in mice brains. These seeds appear to be able to regain pathological properties even after six months of lying dormant. These possibly prion-like proteins might, therefore, exist in the brain long before symptoms develop, at levels too low to be found by routine tests.

One potentially prion-like protein may cause several diseases, according to a study published this summer by Nobel laureate Stanley Prusiner, who discovered prions in the 1980s. Prusiner and his colleagues found that a “strain” of alpha-synuclein—the misfolded protein involved in Parkinson’s—can cause a similar but rare neurodegenerative disease, called multiple-system atrophy. Understanding how variants of these disease-causing proteins differ in shape and how the particular configuration influences their pathogenic nature is destined to become a focus of future research.

“There’s evidence that both prions and beta-amyloid exist as different strains and have very different biological effects,” says Lary C. Walker of Emory University, who was involved in the Nature Neuroscience study. “I think understanding this will give us insight into what’s happening in disease.”

As the evidence increases, more scientists now suspect that prionlike processes probably underlie all neurodegenerative disorders. Prusiner expected the current change in thinking: in his 1997 Nobel Prize lecture, he predicted that the understanding of prion formation could “open new approaches to deciphering the causes of and to developing effective therapies for the more common neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (ALS).”

Alzheimer’s Disease News via http://www.scientificamerican.com/article/are-prions-behind-all-neurodegenerative-diseases/

Prion Science Putting Dementia In Perspective

Alzheimer’s, Creutzfeldt-Jakobs, Parkinson’s Disease All Part Of Prion Spectrum

Editor’s Note: On September 9, 2015 additional research adds to the evidence that suggests that Alzheimer’s disease is a transmissible disease. Scientists have shown that tissues can transmit symptoms of the disease between animals. A new study published in the journal Nature raises additional concern about the transmissibility of Alzheimer’s disease between people and between species.

A scientific truth triumphs not by convincing its opponents but because its opponents eventually die, said influential physicist Max Planck. For Nobel prize-winning neurologist Stanley Prusiner, the quotation is “so mean” that he doesn’t like to use it. “But it is absolutely true,” he says.

Prions and Alzheimer's disease

Prusiner won the Nobel prize in 1997 for his discovery of prions – infectious proteins that cause fatal neurodegenerative diseases in people and animals, the most famous of which is BSE or mad cow disease and its human form, variant CJD. But his claim to have found an entirely new type of disease-causing agent, which he first termed a prion in 1982, was treated as heretical by many of his peers and the media for years. Bacteria, viruses, fungi and parasites are the only known infectious agents – a mere protein, with its lack of genetic material, is not alive so can’t transmit disease – so an army of naysayers maintained. Even his Nobel prize in physiology or medicine, for which he was the sole winner, didn’t silence all the critics.

“I understood the skepticism,” says Prusiner. “When there is a really new idea in science, most of the time it’s wrong, so for scientists to be skeptical is perfectly reasonable…[But] it didn’t make it any easier.”

Madness and Memory by Stanley Prusiner

Prusiner tells the story of his discovery in a new autobiographical book, Madness and Memory. He wrote the book, he says, both to ensure that the story was recorded in his own words and the science was properly described.

Prusiner was born in Des Moines, Iowa, in 1942. As a youngster, he had no interest in science and was happy to get Bs in school with little effort. When, later, he wanted to take advanced chemistry, a subject he liked because he didn’t have to memorize anything, the school said he wouldn’t be able to comprehend the science. He took a lower course, but went on to major in the subject at the University of Pennsylvania, following it up with a medical doctorate received in 1968.

Alzheimer's disease transmission

It was during his chemistry degree that he got his first taste of research – a summer project to help boost his application to medical school. He found the project, studying hypothermia in rats, fascinating and by the end of medical school had become excited about the possibility of pursuing biomedical research as a career.

After completing a grueling internship in medicine that was required for the post, he took a research job at the US National Institutes of Health. He remained at the NIH for three years, studying enzymes in bacteria, before deciding it was time to move on and build his own laboratory.

Prusiner found his scientific destiny after an encounter with a patient with a rare brain disorder in San Francisco in 1972. He had recently begun a clinical residency in neurology at the University of California, San Francisco (UCSF), with the goal of identifying a big problem to investigate, when a patient dying of Creutzfeldt-Jakob disease (CJD) was placed under his care.

The disease was thought to be caused by a “slow virus” that took many months or years to produce symptoms and, intrigued, Prusiner began reading up. “The more I read, the more fascinated I became,” he says.

Kuru disease

Other seemingly related slow virus diseases included scrapie (which occurs in sheep) and kuru (found in the Fore people of Papua New Guinea and spread by cannibalism), all three causing a spongy degeneration of the brain and being transmissible to similar species via injection of infected brain tissue. Yet no actual viruses had ever been isolated and previous work by British scientists on the scrapie agent had even found it had some strange properties, including being resistant to killing by radiation.

They had raised the controversial prospect that it may not even contain DNA or RNA. Prusiner had his problem: he would isolate and characterize the elusive infectious agent responsible for scrapie, which could be studied with rodents, and in so doing shed light on these so-called slow virus diseases.

He began work in 1974 having accepted an academic position at UCSF and despite colleges’ warnings that the problem would be too difficult to crack. It was tough going. He lacked funds to pay for the upkeep of the thousands of laboratory animals he needed and the work was slow because the disease took so long to manifest (he found crucial private funding and sped up the work by moving from mice to hamsters and redesigning his measurement method). “I could now do in one year what would have taken me 80,” he says.

As experimental data began to accumulate, Prusiner grew puzzled. He had anticipated that the scrapie agent he was enriching and purifying from brain material would turn out to be a different and interesting virus. Yet his preparations lacked any genetic material that would indicate one. All he found was a protein. He summarized the work in a journal article in Science in 1982, introducing the term “prion” to denote such a particle.

“I just thought it was really counterproductive to keep calling it a virus when it wasn’t,” says Prusiner. “If you call it that and you believe it at some level, then you miss the next set of experiments.”

The word came from Prusiner’s pondering how “protein” and “infectious” might fit together. When “proin” didn’t sound quite right, he flipped two letters. “What else was I going to do?” he laughs. “I couldn’t come up with some clever Greek words because I don’t know any Greek.” (He pronounces it “pree-on“.)

The word and the concept elicited what he describes as a “firestorm” of criticism and skeptics began staking careers on hunting down the scrapie virus (it has never been found). One particularly low moment he recalls was a 1986 article in the science magazine Discover, which accused him of being more interested in fame than science. He adopted a policy, which he maintained for years, of not speaking to the press.

Prusiner’s answer to his scientific doubters was to keep producing data. Among his contributions, he characterized the scrapie prion and added CJD and other diseases to the list caused by prions. He also showed how prions replicate. They come in two forms, he found, with different shapes: a normal uninfectious form that all animals and people have that is particularly abundant in the brain (it is encoded by a prion protein gene) and a more stable disease-causing form. The disease-causing form can act like a template to guide the normal form to refold into the disease-causing one.

In the late 1980s, as scientific data converged, the tide began to turn on the acceptance of the work. He was elected to various professional bodies and began winning awards. Soon afterwards, so-called knockout mouse studies (which abolished the prion gene in mice making them resistant to prion infection) added further evidence.

mad cow disease

 

Then, in 1996, the first cases in Britain of the human form of mad cow disease were reported and prions were implicated. Variant CJD, it was suspected, had arisen from the consumption of beef infected with BSE, which had been identified as a prion disease using Prusiner’s methods after it was first reported in Britain a decade earlier. A year later, Prusiner won the Nobel prize.

Did the spotlight on prions influence the Nobel committee’s decision?

“It didn’t hurt,” he says.

At the press conference that followed, he faced incredulous journalists still insisting prions were an impossibility. “A Nobel prize doesn’t wipe the scepticism away for some people,” he says.

Prusiner attributes his tenacity in the face of years of doubt to the nature of the problem itself. He would have quit, he says, except there was no alternative that excited or captivated him more. But good scientist, he adds, also stay focused on the problem, going deeper and deeper trying to understand it. That is where the “real opportunity to discover something lies”, he says.

Prusiner is now looking for ways to stop prion diseases (which he believes includes Alzheimer’s and Parkinson’s – though the science of this is not yet settled). For despite all that has been revealed about the strange world of prions, they remain a death sentence to those infected. “We don’t have a single therapy,” he says.

http://www.theguardian.com/science/2014/may/25/stanley-prusiner-neurologist-nobel-doesnt-wipe-scepticism-away

Despite Low Alzheimer’s Disease Rates, CJD Surging In India

CJD Cases Diagnosed Every Month In Mumbai

Murlidhar Ahire lies lifeless in the bedroom of his Vikhroli apartment, while his younger son, Tushar tells us he was an active 69-year-old until two months ago. The retired Air India employee has been diagnosed with Creutzfeldt Jakob disease (CJD), a supposedly rare and deadly neurological brain disorder.

Until 10 days ago, Ahire was at Jaslok Hospital’s isolation ward. But with specialists able to do little except offer supportive treatment, now, one room of the flat has been turned into a nursing unit. Although global incidence is one in a million per year, doctors in Mumbai have noticed a rise in numbers, with one patient diagnosed with CJD every month.

Alzheimer's disease treatment

Research has been unsuccessful at isolating an organism in patients (called a prion actually). What they have learned is that it’s difficult to kill and lacking in genetic information in the form of nucleic acids (DNA or RNA). It is also characterized by an elongated incubation period, sometimes stretching several decades, before symptoms appear.

In keeping with the leading scientific theory, Dr. Annu Aggarwal, consultant neurologist and specialist in cognitive and behavioral neurology with Kokilaben Dhirubhai Ambani Hospital, says prion proteins are to blame. Prion proteins can be normal or infectious. Both contain the same sequence of amino acids, but the infectious form tends to take a folded shape. “Just like a plank of wood must be carved into furniture, proteins have to be folded to form a structure that’s useful to the human body.

Mad Cow Disease In Land Of Sacred Cows

Misfolded proteins known as prions cause many forms of prion disease, including mad cow, Alzheimer’s, chronic wasting, Hutchinson’s and possibly Parkinson’s disease. Prions accumulate in the nervous system and then throughout the body as they convert healthy proteins into deadly ones.” The chain reaction is what leads to brain damage and neuron loss. The disease is indistinguishable from Alzheimer’s disease and the diagnosis between one or the other is often just a coin flip. And the deterioration is often swift. Tushar says Ahire was unrecognizable in a matter of a week.

caregivers Alzheimer's disease

It started with a simple feeling of imbalance, which he addressed by using a walking stick. Soon after, during a visit to the bank in September, he found himself clutching onto walls for support before he could be seated. A battery of tests revealed nothing. Doctors advised little other than rest. Bed wetting was followed by dizziness, nausea and weakness.

When Tushar returned home one night, he found his father staring at a wall. “All he said was, ‘Something is happening to me but I don’t know what’,” remembers the self employed mechanical engineer, who is on a sabbatical to care for Ahire.

While subsequent tests continued to reveal little about his condition, he was developing symptoms that would raise the suspicion of any neurologist. “Once, my mother called me at work to say my father had been in the shower for two hours. When he walked out, he said he had spent the usual 10 minutes in there,” Tushar remembers.

He researched online for memory loss and decided to admit him to a hospital. Within the next week, Ahire’s left leg and arm were paralysed. His tonsils had swollen, leaving him unable to swallow or speak. “He spoke slowly in a measured way, as if he was taking time to churn out his thoughts,” says Tushar.

The bigger challenge for family and doctors battling CJD is that in about 85 percent cases, it occurs as a sporadic disease with no recognizable pattern of transmission. Diagnosis is equally baffling.

Ahire’s diagnosis involved an MRI scan to note characteristic patterns of brain degeneration, a CSF test (Cerebro Spinal Fluid) that measures chemicals in the fluid that surrounds the brain and spinal chord and an EEG (Electroencephalogram) to record the brain’s electrical pattern. “Generally, an MRI reflects classic signs that point to CJD, but sometimes, it doesn’t,” says Dr Om Srivastava, director of infectious diseases at Jaslok. Hopeful families insist on running multiple tests even after a diagnosis has been made, which Srivastava calls nothing but an academic exercise. Only a brain biopsy – removal of a piece of tissue from the patient’s brain for examination by a neuropathologist – can confirm the condition. But it’s often discouraged because it’s a complicated and dangerous procedure, and costs a fortune.

It’s often then that doctors use the rule of elimination. They weed out all other possible conditions or types of dementia before arriving at a CJD diagnosis.

The symptoms, often occurring in combination, are a patient’s best signal. While the initial neurological and psychological symptoms include difficulty walking, talking, despair, dizziness, vision problems, anxiety and trouble sleeping, they gradually advance into loss of bladder-bowel control, voluntary movement and memory.

Tushar agrees Ahire had trouble sleeping, and would often complain of being left alone when lights were switched off at bedtime. Unable to speak, chew or swallow, he is now fed every morning with vegetable and lentil puree, protein powder and medicines crushed in water through an abdominal tube.

biosolids land application contaminates food water

Researchers across the world are trying to identify factors that affect prion activity and conditions that make a patient susceptible to the disease. But in the absence of any real cure, doctors say they are reduced to attending to symptoms. “If a patient suffers from anxiety, we prescribe anti-anxiety pills. If he has problem walking, we provide gait therapy,” says Aggarwal.

Another essential part of supportive care is to ensure that hydration and nourishment is accurate so that secondary infections from broken skin otherwise don’t arise.

“When you’re lying in bed, you are predisposed to clots and aspiration in the lungs (which can cause pneumonia) due to secretion deposits,” says Srivastava. Blood thinners are prescribed, and physiotherapy through air mattresses to provide minute vibration and pressure to the lungs is what’s offered.

Families often shoulder the additional burden of misinformation and misdiagnosis. Hospitals, including a suburban one that Ahire was initially admitted to, don’t have isolation rooms.

Considering CJD as a severely contagious disease, they shun patients. It was only when he was moved to an ACU (Ambulatory Care Unit) at Jaslok that his family learned that the condition cannot be transmitted through casual contact (this is not true). It isn’t airborne either, which means isolation isn’t mandatory. It’s only exposure to spinal cord fluid and brain tissue of an infected individual that can lead to an infection. (Untrue. Prions are in all bodily fluids, including saliva. Sneezing and coughing can send prions into the air and onto other surfaces, where sterilization is impossible.)

Read More: http://garychandler.com/mumbai-doctors-see-rise-in-creutzfeld-jakob-disease/

UK May Have More People Incubating Mad Cow Disease

Creutzfeldt-Jakob, Mad Cow, Alzheimer’s All Deadly Prion Diseases

UK ministers might fund a renewed investigation of “hidden” mad cow disease infection in the British population. The government discussed the issue in response to a select committee report on Creutzfeldt-Jakob Disease (CJD), the human version of mad cow disease. Both are prion diseases (also known as transmissible spongiform encephalopathies–or TSEs). Prions also cause Alzheimer’s disease.

mad cow disease UK

Evidence suggests that the rogue prion proteins responsible for the disease in genetically susceptible people can have a very long incubation period. In some cases individuals can carry prions for decades, or even all their lives, without developing any symptoms. These carriers, however, are contagious long before presenting symptoms of CJD.

MPs on the Science and Technology Select Committee said they feared the vCJD “storm” that caused widespread alarm in the late 1990s may only have abated and could return. In their report, they call for a large scale study of vCJD prevalence to be conducted using a new diagnostic blood test developed by Medical Research Council (MRC) scientists.

Alzheimer's disease treatment

Outlining the Government’s response, public health minister Jane Ellison said: “We will explore the possibility of using the prototype test developed by the Medical Research Council Prion Unit to carry out a blood prevalence study, as the Committee recommended.”

But the Government pointed out that a number of scientific and technical hurdles had to be crossed before such a study could be undertaken. It also stressed that there were competing research funding priorities, and said starting the work within 12 months as suggested by the select committee was “ambitious.”

A total of 175 cases of vCJD were reported in the UK and 49 in other countries between October 1996 and March 2011. Thousands of cattle were destroyed, burned or buried to help contain the deadly prion disease.

The origin of the disease was supposedly traced to meat contaminated with brain and spinal cord tissue, especially cheap-cut burgers and pies consumed in the 1980s. As it turns out, the entire carcass and bodily fluids of infected animals are infectious.

land application sewage sludge and disease

Scientists discovered that variant CJD was effectively the same disease as bovine spongiform encephalopathy (BSE), which affects cattle. It is always fatal and there is no cure. CJD is closely related to Alzheimer’s disease–in fact doctors can’t tell the difference in most cases. Both diseases are part of a larger family called prion disease or transmissible spongiform encephalopathy (TSE). The only differences between the diseases may be a different mutation of the deadly prion.

Strict controls have been in place since 1996 to keep infectious prions out of the human food chain and the use of meat-and-bone mix has been outlawed.

Prions and Alzheimer's disease

It is also possible for vCJD to be transmitted through blood transfusions, surgeries and dental procedures and other pathways, including human sewage. Documented cases of vCJD peaked in 2000, when 28 people died from the disease in the UK. Many more cases were likely misdiagnosed as Alzheimer’s disease. The prion pathogen lurks in soil, water and other environmental pathways.

 

Source: https://uk.news.yahoo.com/probe-hidden-mad-cow-disease-170757618.html#JaTgkgZ

Can The Brain Reverse Damage From Prion Disease

Editor’s Note: Yet another scientific journal briefly mentions the prion connection between several neurological disorders. The other two that should be added to the conversation are mad cow disease and chronic wasting disease (wildlife). Prion disease is prion disease. The global epidemic is real and spreading really fast.

Cell Generation Might Neutralize Rogue Proteins

In Alzheimer’s disease, Parkinson’s, variant Creutzfeldt-Jakob and other brain-wasting prion diseases, cells in the brain gradually deteriorate, begin to function abnormally, and die. However, some parts of the brain have the capacity to self-repair and make new brain cells. A new study suggests there could be a way to harness this and perhaps preserve brain function in neurodegenerative diseases.

Alzheimer's disease treatment

Writing in the journal Brain, study leader Dr. Diego Gomez-Nicola and colleagues from the Centre for Biological Sciences at the University of Southampton in the UK describe how previous studies have already revealed that even in neurodegenerative diseases there is evidence that the brain carries on attempting to repair itself.

One area of the brain that shows evidence of self-repair or neurogenesis is the dentate gyrus, which forms part of the hippocampus, which controls learning and memory.

Studying the brains of mice with a prion disease, the team found evidence of increased self-repair in the dentate gyrus that partially compensated for the loss of brain cells caused by the disease. Their detailed investigation helped them identify how the new brain cell populations were generated over time, and how they integrated with existing brain circuits.

They found that as long as the self-repair occurred in the early or middle stages of the brain-wasting disease, new brain cells integrated into existing circuits in a way that preserved some brain function – but this failed when the disease was advanced.

The team says they also found evidence to suggest increased self-repair in postmortem brain samples of patients who had variant Creutzfeldt-Jakob disease and Alzheimer’s disease when they died.

treat Alzheimer's disease

The authors conclude that the brain has some ability to orchestrate self-repair and suggest there is a time-limited window of opportunity for potential treatments to boost this mechanism and preserve brain function in patients with neurodegenerative diseases.

Dr. Gomez-Nicola says the study opens new avenues “to identify what specific signals are used to promote this increased neurogenic response, with views focused in targeting neurogenesis as a therapeutic approach to promote the regeneration of lost neurons.”

As they strike primarily in mid- to late-life, neurodegenerative diseases become increasingly more common in aging populations. For instance, in the US, where estimates suggest by 2030 around 20% of the population will be over the age of 65, some 5 million Americans are living with Alzheimer’s disease and another 1 million have Parkinson’s. Finding treatments and cures for neurodegenerative diseases has never been more urgent.

Funds from the European Union Seventh Framework Programme and the Medical Research Council helped finance the study.

In October 2013, Medical News Today learned how researchers found a new target for drugs against brain cell death. Writing in Science Translational Medicine, they report how they blocked a major pathway leading to brain cell death in mice using an orally administered drug-like compound.

Source: http://www.medicalnewstoday.com/articles/278623.php

Another Man In Texas Dies Of Mad Cow Disease

Editor’s Note: The deadly prions behind mad cow disease and other neurological disorders in humans and animals is unstoppable. People with the disease are highly contagious long before they exhibit clinical signs of the disease. They infect everything that they touch and much, much more. Equally as troubling as the second death within days is the urgency to dismiss public concerns with misinformation. Pandora’s box is wide open. Read the lies for yourself as U.S. officials bash the food supplies of other nations to cover its own vulnerabilities.

CJD An Environmental Nightmare

A second death from the human version of mad cow disease (prion disease) has occurred in Texas; however, health care authorities say there is no cause for alarm. Medical personnel with the Centers for Disease Control and Prevention in Atlanta, Georgia, working with the Texas Department of State Health Services (DSHS), have confirmed a diagnosis of variant Creutzfeldt-Jakob Disease (vCJD) – a fatal brain disorder commonly known as mad cow disease – in a patient who recently died in Texas.

food and water contamination and disease

The diagnosis was confirmed when laboratory results from an autopsy of the patient’s brain tested positive for variant CJD, which is characterized by dementia and holes or a spongy appearance in the brain. First described in 1996 in the United Kingdom, vCJD is a rare, degenerative, fatal brain disorder in humans. Additionally, there is no cure or known treatment of vCJD; it is invariably fatal.

Although unconfirmed, the disease is believed to be caused by consumption of products from cows infected with bovine spongiform encephalopathy (BSE), or “mad cow” disease. However, in three cases from the United Kingdom, infection might have occurred via a blood transfusion from an asymptomatic, infected donor. (Actually, there are many sources of deadly prions.)

Prions and Alzheimer's disease

Worldwide, more than 220 vCJD patients have been reported, with a majority of them – 177 cases – in the United Kingdom and 27 cases in France. The case in Texas is the fourth to be reported in the United States and the second in this state. In each of the three previous cases, infection apparently took place outside the United States, including two cases from the UK and one from Saudi Arabia. The history of this fourth patient included extensive travel to Europe and the Middle East, supporting the theory that infection occurred outside the US.

The CDC and DSHS investigations will likely confirm further details of the latest patient’s history – including the potential source of infection. No information was available on the DSHS website indicated where the latest death due to vCJD had occurred in Texas.
The first patient, who succumbed to vCJD in the US, was born in the UK in the late 1970s and lived there until relocating to Florida in 1992. Symptoms began in November 2001 and the patient died in June 2004. The patient never donated or received blood, plasma or organs; never received human growth hormone; nor did the patient ever have major surgery other than having wisdom teeth extracted in 2001. Additionally, there was no family history of CJD.

The second patient, also a native of the UK, resided in Texas from 2001 to 2005. His symptoms began in early 2005 while living in Texas. He then returned to the UK, where his illness progressed. Variant CJD was diagnosed and confirmed by neuropathology at the time of his death.

While living in the United States, the patient had no history of hospitalization, of having invasive medical procedures or of donating or receiving blood or blood products. 
Physicians believe the patient contracted the disease in the UK while living there from 1980 to 1996, the defined “period of risk for human exposure” to the agent causing “mad cow disease.” His stay in the United States was too short for the incubation period for vCJD to have occurred here.

Although born and raised in Saudi Arabia, the third patient lived in the US since late 2005. In addition, the patient occasionally stayed in the United States for as long as three months at a time since 2001 and there was a shorter visit in 1989. The patient’s onset of symptoms occurred in spring 2006.

In late November 2006, a research team at the University of California San Francisco Memory and Aging Center confirmed this patient’s vCJD clinical diagnosis by pathologic study of brain biopsy tissue. The patient had no history of receiving blood, a past neurosurgical procedure or residing in or visiting European countries.

Due to the expected greater-than-seven-year incubation period for food-related vCJD, this patient was most likely infected from confirmed consumption of BSE-contaminated cattle products while living in Saudi Arabia as a child. The patient had no history of donating blood and a public health investigation has identified no known risk of transmission to US residents from this patient.

The latest confirmed death from vCJD occurred in Texas last month. The diagnosis was confirmed when laboratory results from an autopsy of the patient’s brain tested positive for vCJD. According to a CDC report, the patient in Texas had traveled “extensively” to Europe and the Middle East.

CJD transmissible

“There are no Texas public health concerns or threats associated with this case,” administrators with DSHS posted on the agency’s website. “Infection likely occurred outside the United States.”

A surveillance program by the US Department of Agriculture tests brain tissue extracted from approximately 40,000 dead cows annually for BSE, targeting animals considered most at risk. 

Another integral part of the US food safety net ensures that animal tissues that carry BSE, such as the brain and spinal cord, are removed from cattle before being processed for food. There is no evidence that people can get “mad cow disease” or vCJD from eating muscle meat – which is used for ground beef, roasts and steaks – or from consuming milk or milk products.

Source: http://www.bccourier.com/Archives/News_detail.php?contentId=17348

Whole Foods Recalls Beef Over Mad Cow Risk

Another Symptom Of Mismanaged System

More than 4,000 pounds of rib-eye and other fresh beef products have been recalled because they could contain contaminated materials linked to mad cow disease.

The meat in question was processed at Fruitland American Meat in Jackson, Missouri, and distributed to a Whole Foods distribution center in Connecticut, which services its New England stores, and a restaurant in New York City and another one in Kansas City, Missouri. The beef was produced and packaged between September 2013 and April 2014.

chronic wasting disease

The inspectors found no indication any of the animals slaughtered showed signs of bovine spongiform encephalopathy, the formal name of mad cow disease. In a statement issued by the USDA Food Safety and Inspection Service, a spokesperson said: “All of these animals received full inspection, both before and after slaughter, by FSIS personnel and showed no abnormal signs or symptoms associated with BSE.

“Out of an abundance of caution, FSIS issued a Class II recall (a remote risk) for product that does not have paperwork showing that nerve tissue was removed. FSIS and the company have received no reports of adverse reactions due to consumption of these products.”

The USDA requires brain and spinal tissue be removed from meat products from cattle 30 years and older because it can carry the protein that causes mad cow disease. People who consume meat tainted with mad cow disease could develop a rare, fatal disease in humans called variant Creutzfeldt-Jakob disease.

Mad cow disease and vCJD

The first cases of vCJD were first reported in 1996, and so far a total of 229 patients with this disease from 12 countries have been identified, according to the CDC.

Source: http://www.cnn.com/2014/06/12/health/beef-recall-mad-cow-disease/

U.S. Serves Misinformation After Texas Man Dies Of Mad Cow Disease

Admission Exposes More Mismanagement Of Prion Disease

The United States has confirmed it’s fourth case of a deadly and highly contagious brain disease. The revelation leaves many more questions than answers and gives us added information and ammunition to demand truth, accountability and reform on many levels.

The actual number of people dying from related diseases is much, much higher. In fact, prion diseases are killing more than 44 million people around the world right now. This epidemic will get much worse. It will never get any better.

food and water contamination and disease

Unfortunately, the confirmation in Texas reveals a bureaucracy and policies that are misguided, short-sighted and reckless at best. Those who are perpetuating these policies with myths and misinformation are either incompetent, negligent or criminal–none of which are acceptable. Secrecy serves no one, except those who are willing to profit from our misfortune. Collusion doesn’t make these merchants of death or their families immune from prion disease, which is already killing millions of people around the world.

Neurodegenerative diseases are on the rise around the world among people of all ages. Similar trends are being observed in wildlife and if we honestly tested more livestock around the world, we would likely see the exact same trend. The reason for the rise in these unstoppable diseases is a pesky pathogen called a prion (PREE-on). Prions are a deadly form of protein that acts like a cancer within our nervous system. Prions migrate, mutate and multiply, which is why we have a global epidemic on our hands now.

We know prion diseases as mad cow, Alzheimer’s, Creutzfeldt-Jakob (CJD), chronic wasting (CWD), scrapie, Huntington’s, Kuru and possibly Parkinson’s. These diseases, despite their cleverly disguised names, all are what we call Transmissible Spongiform Encephalopathies (TSE). These diseases spread in a variety of ways, which makes the Texas death and public statements even more troubling. 

Statements from the U.S. Center for Disease Control (CDC) and the Texas Department of Health Services are very troubling. These statements continue to reflect a government and policies that are quick to dismiss a disease, a pathogen and pathways that are unstoppable. To avoid scrutiny, both agencies are not disclosing the name, gender, age, location or time of death of the victim. If those details were released, we could expose the mismanagement and warn the public to take appropriate precautions. It begs the question, are these agencies here to protect the public or the status quo of a reckless system that treats people as consumers and collateral. Once again, we are funding public services and policies with tax dollars that do not serve the public. The same culture exists in every government around the world, which again speaks of the international collusion involved in the suppression of the truth. Therefore, we ask what type of governments lie to the people to protect themselves and their special friends.

Prions and Alzheimer's disease

If we don’t demand the truth and reform, we are putting our families at risk. Prion disease, which ever name you want to call it, will never go away. The risk will spread further every day, much like radiation spreads in the environment or similar to the way cancer spreads in the body. Victims are spreading their disease throughout their homes and communities. Sewage systems are spreading the problem even further.

If you prefer not to be a guinea pig in the largest open-lab science experiment in the history of the world, dig in and stand up. Ask these questions and more:

1. Where did this Texas victim live and travel over the past five years?

2. Where was the man hospitalized?

3. Did he have surgery or dental work in the past five years?

4. Did he donate blood?

5. Did he eat at any local restaurants?

6. What sewage districts did he live and work within and what do those sewage districts do with their waste (biosolids, water reclamation, river discharge, etc.)

7. What was his profession?

8. Why is the CDC so sure that there is no risk to others? That is impossible.

9. Why are we so quick to blame his disease on foreign food supplies, when the pathways to this disease are so numerous and so mismanaged?

10. Are you incompetent, negligent or criminal in your statements and policies?

If you really care about this issue and want to fight for the truth and reform, please contact us in the United States at 602-999-7204 or gary@crossbow1.com . We’re all fighting for our lives and the future of our children. You can make a difference in your personal life, the life of friends and family and in the future of the world. We are talking about a form of environmental contamination that threatens life as we now it. The risks in your life are very real. (For legal reasons, let me say that the above comments are my opinions.) 

crossbow logo white1public relations firm Alzheimer's disease

Crossbow Communications is a full-service advertising agency and public relations firm in Denver, Colorado and Phoenix, Arizona. The firm specializes in issue management and public affairs, with an emphasis on healthcare, natural resource conservation and human rights. Crossbow has helped influence public opinion and public policy around the world. It has won state and national awards, while setting state and national records for our clients. http://crossbowcommunications.com/public-affairs-firm-phoenix/