Transmissible Spongiform Encephalopathies
The U.S. government’s monitoring system for cases of Creutzfeldt-Jakob disease, a fatal human brain illness, could be missing tens of thousands of victims, scientists and consumer advocates said.
Creutzfeldt-Jakob disease or CJD can be caused by prion exposure, including eating beef contaminated with mad cow disease, but the critics assert without a better tracking system it might be impossible to determine whether any CJD cases are due to mad cow disease or obtain an accurate picture of the prevalence of the disorder in the United States.
In order to understand the threat, one must understand the dynamics of this neurological disease. Alzheimer’s disease, for example, is a member of an aggressive family of neurodegenerative diseases known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.”
TSEs are caused by a deadly protein called a prion (PREE-on). As such, TSEs also are referred to as prion disease. The critical factor is that prions are unstoppable. The pathogen spreads through the bodily fluids and cell tissue of its victims. Blood, saliva, mucus, milk, urine and feces carry deadly prions from victims. All tissue is infectious just because of the contact with the contaminated blood.
TSEs also include Creutzfeldt-Jakob disease, Parkinson’s, Huntington’s, mad cow disease and chronic wasting disease in the deer family. Few, if any, mammals are immune. There is no cure.
Dr. Stanley Prusiner, an American neuroscientist from the University of California at San Francisco, earned a Nobel Prize in 1997 for discovering and characterizing deadly prions and prion disease. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the importance of his research. According to Prusiner, TSEs all are on the same disease spectrum, which is more accurately described as prion disease. He claims that all TSEs are caused by prions.
Prions are unstoppable and the pathogen spreads through the bodily fluids and cell tissue of its victims. Prions shed from humans are the most deadly mutation. They demand more respect than radiation. Infected surgical instruments, for example, are impossible to sterilize and hospitals throw them away. Prions are in the skin, blood, saliva, urine, feces, mucus, and bodily tissue of its victims. Many factors are contributing to the epidemic. Prions are now the X factor. Industry and government are not accounting for them or regulating them. They are ignoring the threat completely, which violates the Bioterrorism Preparedness and Response Act of 2002 in the United States. Other nations also are ignoring laws developed to protect food, air and water.
“There is now real evidence of the potential transmissibility of Alzheimer’s disease,” 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 (prions).”
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.
The first symptoms of CJD typically include memory loss and difficulty keeping balance and walking. As the disease destroys the brain, patients rapidly progress in a matter of months to difficulty with movement, an inability to talk and swallow and, finally, death.
Clusters of CJD have been reported in various areas of the United States — Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in 1999-2000 and Texas in 1996. In addition, several people in New Jersey developed CJD in recent years, including a 56-year-old woman who died on May 31, 2003. Although in some instances, a mad cow link was suspected, all of the cases ultimately were classified as sporadic. The common thread is more likely sewage sludge, also known as biosolids, dumped on land.
People who develop CJD from eating prion-contaminated beef have been thought to develop a specific form of the disorder called variant CJD. But new research, released last December, indicates the mad cow pathogen can cause both sporadic CJD and the variant form. A deadly prion is a deadly prion and there are now hundreds, if not thousands, of mutations.
“Now people are beginning to realize that because something looks like sporadic CJD they can’t necessarily conclude that it’s not linked to mad cow disease,” said Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, who conducted a 1989 study that found 13 percent of Alzheimer’s patients actually had CJD.
Several studies, including the one by Manuelidis, have found autopsies reveal 3-percent-to-13-percent of patients diagnosed with Alzheimer’s or dementia actually suffered from CJD. Those numbers might sound low, but there are five million Alzheimer’s cases and hundreds of thousands of dementia cases in the United States. A small percentage of those cases could add up to 120,000 or more CJD victims going undetected and not included in official statistics.
Experiences in England and Switzerland — two countries that discovered mad cow disease in their cattle — have heightened concerns about the possibility some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since mad cow was first detected in British herds in 1986.
Switzerland discovered last year its CJD rate was twice that of any other country in the world. Switzerland had been seeing about eight to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.
The CDC says the annual rate of CJD in the United States is one case per million people, but the above studies suggest the true prevalence of CJD is not known, Manuelidis told UPI.
Diagnosing CJD or Alzheimer’s disease is difficult because no test exists that can identify either disease in a living patient with certainty. So physicians must rely on the patient’s symptoms to determine which illness might be present. Sometimes, however, the symptoms of one disease can appear similar to the other disorder. The only way to determine the disease conclusively is to perform an autopsy on the brain after death.
Unfortunately, although autopsies once were performed on approximately half of all corpses, the frequency has dropped to 15 percent or less in the United States. The National Center for Health Statistics — a branch of the CDC — stopped collecting autopsy data in 1995.
“If we don’t do autopsies and we don’t look at people’s brains, we have no idea about what is the general prevalence of these kinds of infections and (whether) it is changing,” Manuelidis said.
At the same time autopsies have been declining, the number of deaths attributed to Alzheimer’s disease has increased more than 50-fold since 1979, going from 857 deaths then to nearly 50,000 in 2000. Though it is unlikely the dramatic increase in Alzheimer’s is due entirely to misdiagnosed CJD cases, it “could explain some of the increase we’ve seen,” Manuelidis said.
“Neurodegenerative disease and Alzheimer’s disease have become a wastebasket” for mental illness in the elderly that is difficult to diagnose conclusively, she said. “In other words, what people call Alzheimer’s now is more broad than what people used to call it, and that has the possibility of encompassing more diseases — including CJD.”
The autopsy studies that found undiagnosed CJD cases raise the question of whether the United States “already has an undetected epidemic here,” Jeff Nelson, director of vegsource.com, a vegetarian advocacy Web site, told UPI.
“What’s the source of that?” Nelson asked. “Could it be the same source of encephalopathy we saw in mink?”
Nelson referred to an outbreak of a mad-cow-type disorder in mink in Wisconsin in the 1980s. The origin was traced back to the animals’ diet, which included parts of so-called downer cattle — sick cows that are unable to stand, which often indicates a neurological disease, including mad cow. The mink disease raised concerns about whether U.S. cattle were carrying a mad-cow-like pathogen even prior to the U.K. epidemic that began in 1986.
Andrew Monjan, chief of the neuropsychology of aging program at the National Institute of Aging — part of the National Institutes of Health in Bethesda, Md. — acknowledged there has been an increase in U.S. Alzheimer’s cases. However, he told UPI, this probably is due to the aging of the population — as people grow older, they develop a higher risk of developing Alzheimer’s (bullshit).
There’s been no change in the number of CJD cases in the country and there has been clearly a tracking of the unusual cases of CJD that could be due to mad cow disease, Monjan said. However, Terry Singletary, coordinator of CJD Watch — an organization founded to track CJD cases — says efforts to track the disease have been close to nonexistent. For example, only 12 states require such reports. Therefore, many cases might be going undetected, unreported or misdiagnosed.
If more states made CJD a reportable illness, there would be more clusters detected across the United States, said Singletary, who became involved with CJD advocacy after his mother died from a form of CJD known as Heidenhain variant. In the 18-year period between 1979 and 1996, he noted, the country saw a jump from one case of sporadic CJD in people under the age of 30 — a warning sign for a link to mad cow because nearly all of the U.K. victims were 30 years of age or younger — to five cases in five years between 1997 and 2001. “That represents a substantial blip,” he told UPI.
Singletary also said there have been increases in sporadic CJD in France, Germany and Italy, all of which have detected mad cow disease in their cattle.
So far, the CDC has refused to impose a national requirement that physicians and hospitals report cases of the disease. The agency has not chosen to make CJD a reportable disease because “making it reportable is not necessarily directly helpful in surveillance because in some states where it’s reportable you may not get the physician to report it,” said Dr. Ermias Belay, CDC’s medical epidemiologist working on CJD.
Instead, the agency relies on other methods, including death certificates and urging physicians to send suspicious cases to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, which is funded by the CDC. However, because autopsies generally are not done, if a CJD case is misdiagnosed as Alzheimer’s or dementia, a correct diagnosis might never be determined and therefore the cause of death listed on a death certificate might be inaccurate.
Belay told UPI he discounted this possibility. It is unlikely to happen, he said, because it is easy to distinguish CJD from Alzheimer’s — the two conditions display different symptoms.
Manuelidis disagreed. It can be quite difficult to determine accurately if a patient has CJD, as evidenced by her study, in which respected and competent neurologists and psychiatrists at Yale originally diagnosed patients with Alzheimer’s, yet were wrong at least 13 percent of the time. Another study conducted at the University of Pennsylvania, which found 6 percent of dementia patients actually were suffering from CJD, supports the difficulty in distinguishing the illnesses correctly.
The U. Penn. researchers concluded: “These results show that in patients with a clinical diagnosis of dementia, the etiology (cause) cannot be accurately predicted during life.”
In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC’s investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases.
Belay said the CDC follows up on all cases of CJD that occur in people under age 55, as these could be linked to variant — mad-cow-related — CJD. But so far, all have turned out to be sporadic forms of the disease. About 30 cases of the disorder occur each year in the United States in this age group, while the remaining 270 or so are older.
The case of Carrie Mahan — a Philadelphia woman who developed a brain disorder that appeared to be CJD and died from it in 2000 at the age of 29 — illustrates just how difficult it can be to diagnose the disease.
Mahan’s physician, Dr. Peter Crinos of the University of Pennsylvania Medical Center, ruled out other disorders and felt certain the young woman had died of CJD, a concern that raised the possibility of a link to mad cow disease because of her young age. When neuropathologist Nicholas Gonatas, who had seen CJD before, examined Mahan’s brain after her death, he, likewise, was confident he detected the microscopic, sponge-like holes caused by the disease. But when he sent brain samples to the NPDPSC, the results came back negative. Gonatas, convinced the surveillance center’s finding was erroneous, sent off two more samples, only to have them both come back negative.
Subsequent research, however, has shown the test used by the surveillance center cannot rule out CJD, said Crinos, an assistant professor of neurology.
“There’s no question that Carrie had a spongiform encephalopathy,” Crinos said, but added although it appeared to be CJD, it is difficult if not impossible to say if it was due to mad cow disease.
Crinos told UPI until the CDC implements a better tracking system, a lot of questions will remain about CJD and cases like Carrie Mahan’s. One central question: Why are cases of what is presumed to be a rare disease popping up in clusters in certain areas of the country? Crinos said the clustering suggests an environmental or food-borne cause, but so far, “No one knows the answer to that.”
The Second Part Of The Prion Equation
We can split hairs about the diagnoses, misdiagnoses and causes of the prion disease forever, but the problem with prions is that the victims are infectious themselves and serve as prion incubators and distributors. The bodily fluids of victims are loaded with infectious prions. Blood, urine, feces, saliva and mucus transport the disease throughout the victims’ environments. Downstream, wastewater treatment plants, for example, are spreading this infectious waste far and wide because they are incapable of stopping prions. All by-products and discharges from wastewater treatment plants are infectious waste, which are contributing to the global epidemic of neurodegenerative disease among humans, wildlife and livestock. Sewage treatment plants can’t detect or stop prions. Just ask the U.S. EPA and the industry trade organization—the Wastewater Effluent Federation. Sewage sludge (biosolids) and wastewater reclamation are causing widespread contamination.
Once unleashed on the environment, prions remain infectious. They migrate, mutate and multiply as they infect crops, water supplies and more.
Deer, elk, moose and reindeer are now contracting prion disease from humans. To help cloak the epidemic, it’s called chronic wasting disease (CWD). Deer with CWD are proverbial canaries in a coal mine. They are being killed by government sharpshooters to help cover up the problem. It’s insane.
Alzheimer’s Disease News via http://www.organicconsumers.org/madcow/CJD122903.cfm
Gary Chandler is a prion expert. He is the CEO of Crossbow Communications, founder of Sacred Seedlings and Earth News, and author of the Language and Travel Guide To Indonesia and several other books about health and environmental issues around the world. Chandler also is connecting the dots to the global surge in neurological disorders.