CJD The Most Severe Form Of Prion Disease
Neurodegenerative disease is the fastest-growing cause of death in the world. It’s already a public health disaster and it will get worse.
Alzheimer’s disease and Creutzfeldt-Jakob disease (CJD) are essentially the same disease at different points on the same spectrum. Misdiagnosis, suppression of diagnoses, misinformation and mismanagement are contributing to the epidemic. Alzheimer’s and CJD are part of a spectrum disease called transmissible spongiform encephalopathy (TSE). The operative word is “transmissible.”
A variety of factors can trigger neurodegenerative disease, including genetics, head trauma and neurotoxins. Alzheimer’s disease alone is taking the lives of 50-100 million people around the world now. The epidemics are more severe in some countries than others. As millions die, even more will be diagnosed. Millions more are suffering in silence with a misdiagnosis or no diagnosis. We’re facing a public health disaster. Misinformation and mismanagement are fanning the flames.
Despite millions of deaths, experts suggest that the prevalence of the disease will quadruple by 2050, if not sooner. Unfortunately, there is a growing stack of evidence that Alzheimer’s disease, Parkinson’s disease and other brain diseases are transmissible. They also are being misdiagnosed and undiagnosed at an alarming rate. Deadly, self-replicating proteins appear to be one of the common threads.
Prions (PREE-ons) are a deadly and unstoppable form of protein that migrates, mutates, multiplies and kills with unparalleled efficiency. Prions cause fatal neurodegenerative disease in humans and animals by converting the cellular version of prion protein into a toxic form that erodes the brain and body. Prion disease often is described as a wasting disease that causes a loss of body mass and brain mass. Dr. Stanley Prusiner, an American neuroscientist from the University of California at San Francisco, earned a Nobel Prize in 1997 for discovering and characterizing prions and prion disease. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the importance of his research.
Prion disease also is known as transmissible spongiform encephalopathy (TSE). The operative word is “transmissible.” Prusiner claims that all forms of TSE are caused by infectious prions.
“I learned that scrapie, Creutzfeldt-Jakob disease and kuru were transmissible by injecting extracts of diseased brains into the brains of healthy animals,” Prusiner said. “Whether prions are responsible for common neurodegenerative diseases, such as Alzheimer’s disease is a possibility that should not be ignored.”
TSE is a spectrum disease that varies in severity and symptoms. It depends on which region of the brain is impacted first and by what prion mutation. Few cases are identical in terms of symptoms and diagnoses. When the presenting symptom is memory loss, the diagnoses flow along the following chart.
In humans, the prion spectrum includes Alzheimer’s disease, Parkinson’s disease and an extremely aggressive version known as Creutzfeldt-Jakob disease. The difference between these diseases is very slight and often indistinguishable to neurologists. For example, millions of people have the severe form of Alzheimer’s disease, which is known as Creutzfeldt-Jakob disease (CJD). CJD is the most severe form of prion disease in humans. CJD often kills within weeks or months of diagnosis (a definitive confirmation requires an autopsy).
According to neuroscientists Dr. Laura Manuelidis, at least 25 percent of Alzheimer’s diagnoses are actually CJD, which is further up the prion spectrum.
CJD, without dispute, is extremely infectious to caregivers and loved ones, but it has not been declared a reportable disease.
Millions of cases of deadly CJD are being misdiagnosed as Alzheimer’s disease. Millions of patients and caregivers are being misinformed, misguided and exposed to an aggressive prion disease.
“It is well known that CJD is transmissible via surgical or medical procedures involving prion-infected brain tissue. Our finding of infectious prions in skin is important since it not only raises concerns about the potential for disease transmission via common surgeries not involving the brain, but also suggests that skin biopsies and autopsies may enhance pre-mortem and post-mortem CJD diagnosis,” said Wenquan Zou, Associate Professor of Pathology and Neurology and Associate Director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve School of Medicine.
“The level of prion infectivity detected in CJD skin was surprisingly significant, but still much lower than that in CJD brains. Further investigation is necessary to determine whether extra precautions should be taken during non-neurosurgeries of CJD patients, especially when surgical instruments will be reused.”
“Infectious prions are detectable in skin samples of patients with sporadic Creutzfeldt-Jakob disease,” Zou said. “The prion-induced diseases are significant because they are infectious diseases that can be transmitted interspecies and intraspecies. Prions generated in the brain are detectable in skin tissue far earlier than the prion-caused brain damage.”
Prion infectivity is highly concentrated in brain tissue, but it’s also in all bodily fluids and tissue. Inter-personal CJD transmission has occurred after patients were exposed to surgical tools previously contaminated by CJD brain tissues. It’s happening due to many other pathways. Caregivers are at extreme risk.
Unfortunately, Prusiner’s science is being ignored and we all are facing a public health disaster because of the negligence and reckless disregard for public health. Misinformed caregivers, family members, healthcare workers and others are caught in the crossfire of a deadly form of protein.
Neurologists prefer not to touch or even be in the same room as a patient with CJD. The CJD Foundation and other advocacy organizations also remain mum on the risk of transmission. The CDC remains silent. Is this cone of silence incompetence, negligence or criminal misconduct?
Abnormal proteins also are associated with autism. In fact, it appears that age is the biggest difference between the neurodegenerative disease spectrum and autism spectrum disorders. Both spectrums share common environmental causes and pathologies. Plus, CJD is taking the lives of more and more young adults and teenagers.
Current diagnostic tools for CJD rely on brain tissue samples collected at either biopsy or autopsy, or through a spinal tap.
“Creutzfeldt-Jakob disease behaves like Alzheimer’s disease on steroids,” said Dr. Jennifer Majersik, an associate professor of neurology at the University of Utah.
Since prion disease is a spectrum disease, doctors can’t tell the difference between Alzheimer’s disease and CJD without a spinal tap and it’s still a guess. According to neuroscientists Dr. Laura Manuelidis, at least 25 percent of Alzheimer’s diagnoses are not Alzheimer’s disease. These misdiagnoses are actually CJD, which is further up the prion spectrum. CJD, without dispute, is extremely infectious to caregivers and loved ones. Millions of cases of deadly CJD are being misdiagnosed as Alzheimer’s disease. Millions of patients and caregivers are being misinformed, misguided and exposed to an aggressive disease. Misdiagnosis and misinformation regarding prion disease is a matter of life and death. The mismanagement doesn’t end here.
Spouses of those with Alzheimer’s disease or CJD are 600 percent more likely to contract the disease.
Surgical instruments infected with prions, for example, are impossible to sterilize and hospitals throw them away. Meanwhile, caregivers and family members are not warned. CJD victims are not quarantined. They are sent home to die in many cases. Reckless.
Many factors are contributing to the epidemic. Prions are now the X factor. Industry and government are not accounting for prions 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.
Unfortunately, there are so many variations of prions, that even a spinal tap can only detect prion disease. It can’t distinguish between all of the various mutations because they aren’t even in the science books, yet. Dr. Prusiner just announced the discovery of a new form of prion. The first such characterization in 40 years. Just a few thousand more to go. Unfortunately, it’s time to focus on containment not characterization.
The only definitive diagnosis of prion disease requires an autopsy, which rarely happens with neurological disease due to concerns about deadly contamination and exposure. All doctors are guessing with each Alzheimer’s diagnosis. All they can do is attempt to gauge the severity of symptoms. This problem complicates the search for accurate statistics about the size and scope of the epidemic. The attempt to differentiate between CJD and Alzheimer’s represents a containment nightmare.
The Fore tribe of New Guinea unknowingly became the first known “Guinea Pigs” for prion disease (villagers call CJD “kuru”). Rumors made it out that many tribal members were getting sick and experiencing horrifying deaths from kuru. Ultimately, pioneering pathologists from the U.S. and Australia spent time with the Fore to understand the disease.
The research began back in 1955. After years of study, scientists determined that some tribal members (mostly women and children) contracted the prion disease after eating the brains of relatives who died. At some point in time, someone died of the natural form of dementia and then the tribe unknowingly consumed that person’s brains in a ritual—including the deadly prions that killed the person. Many of those who ate these infected brains also withered away and died of this brain wasting disease. As those people died, the surviving villagers consumed the brains of the deceased and so on. The disease kept recycling and intensifying itself through cannibalism.
It’s impossible to know exactly how many years it took for the disease to start snowballing among the Fore. Once it reached a tipping point, it almost destroyed the tribe. Thanks to the efforts of some dedicated researchers, they eventually isolated the problem and helped instill some social change to curb contamination and the epidemic.
I haven’t heard of any modern updates, but once infected, the soil in the area and personal items are still infected. Therefore, further outbreaks are possible just because of environmental contamination—even decades after the initial outbreak.
Elsewhere in the world, people have contracted the disease by eating meat from livestock and wildlife with prion disease. The UK had the most severe crisis back in the late 1980s and early 1990s. They killed almost 200,000 cattle in an attempt to eradicate mad cow disease.
Over the past 25 years, the world has seen several clusters of the disease in humans, which points toward areas of environmental contamination or contaminated food and water supplies. In Cleveland County North Carolina, for example, 17 patients died from CJD in just a few months in early 2013. In British Columbia, four people from the Fraser Valley were diagnosed with CJD in one day in 2013 (although one apparently died a few weeks earlier). Clusters of the disease have appeared before and they are still appearing thanks to sewage mismanagement and other forms of environmental contamination. A similar outbreak occurred in New Brunswick, Canada just a few years ago.
TSEs also mad cow disease and chronic wasting disease in deer. TSE has been found in mink, moose, mice, sheep, cats, elephants, dolphins and many other mammals. Sea mammals are extremely vulnerable, but they aren’t being tested. Sick mammals on land and at sea are a canary in a coal mine. Their sickness confirms an alarming epidemiological trend among humans. An environmental contagion is responsible for the spike among many mammals. The reckless management of infectious waste (sewage) is fueling the epidemic.
Governments’ Perspective On CJD
Sporadic CJD: the disease appears with no known risk factors. It accounts for about 85 percent of all cases. Despite the smokescreen of misinformation, these cases are actually acquired due to exposure to prion contamination, which is widespread and spreading further every day.
Hereditary CJD: person has a family history or tests positive for the gene mutation associated with CJD. Only about 5 percent to 10 percent of CJD cases in the U.S. are hereditary.
Acquired CJD: disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. Food and water contamination are now major pathways.
Initially, individuals with CJD experience problems with muscular coordination, impaired memory and judgment and vision, and personality changes. As the illness progresses, memory impairment becomes severe, and individuals develop involuntary muscle jerks and lose their sight. CJD causes more rapid deterioration of an individual’s abilities than Alzheimer’s disease.
Currently, there is no single diagnostic test for CJD.
A physician usually rules out treatable forms of dementia, such as encephalitis or chronic meningitis. It’s estimated that at least 25 percent of Alzheimer’s disease diagnoses are wrong. In these cases, the underlying disease is actually CJD, which means that millions of people around the world are walking incubators and prion distributors.
A spinal tap, an electroencephalogram (EEG), computerized tomography (CT) and magnetic resonance imaging (MRI) are all forms of testing and evaluations to make the diagnosis of CJD, but the only way to confirm CJD is by brain biopsy or autopsy.
Like Alzheimer’s disease, there is no effective treatment for CJD. Treatment is aimed at easing symptoms and making the individual as comfortable as possible. Medication can help relieve pain.
There are proven strategies to help avert neurodegenerative disease, including smart nutrition, exercise and prion aversion. There is not a cure for prion disease, but smart nutrition can ease the symptoms. Smart nutrition also can help you and your family avert neurodegenerative disease. Preview and order the eBook now to defend yourself and your family.
Gary Chandler is a prion expert. He is the CEO of Crossbow Communications, author of several books and producer of documentaries about health and environmental issues around the world. Chandler is connecting the dots to the global surge in neurodegenerative disease, including Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and other forms of prion disease. The scientific name for prion disease is transmissible spongiform encephalopathy.