Editor’s Note: A deadly family of neurodegenerative disorders is impacting people and animals around the world in epidemic proportions. These prion diseases are known to us as Alzheimer’s, Creutzfeldt-Jakob, mad cow, chronic wasting disease and others that are linked by deadly proteins called prions. Medical professionals know very little about these diseases except that they are deadly and incurable. They also are highly contagious, which is why the family of disease is called Transmissible Spongiform Encephalopathies (TSE). An unbelievable amount of effort is going into the issue to explain that it isn’t the U.S. food supply, except that these industry explanations rarely include facts. While so much effort is going into this state of denial, we are opening up Pandora’s lunchbox even more as we ignore the transmissible aspect of the disease created by victims and everything that they touch (including Alzheimer’s patients). This man who just died in Texas is a good example. He died of a highly infectious disease, but no one knew what it was until his autopsy. In hindsight, his home, the hospital and the entire community were exposed to the pathogen behind his disease–even before he was diagnosed. In reality, the pathways that could have killed this man are numerous. It’s impossible to sterilize these pathways ever again. Find out more about prions and their pathways to you in our new eBook.
U.S. Blames Foreign Food Supplies Again
Laboratory tests have confirmed a diagnosis of variant CJD (a fatal brain disorder) in a patient who recently died in Texas. The confirmation was made when laboratory results from an autopsy of the patient’s brain tested positive for variant CJD, according to a Centers for Disease Control and Prevention news release today.
First described in 1996 in the United Kingdom, variant CJD is a rare, degenerative, fatal brain disorder in humans. It is believed to be caused by consumption of products from cows with the disease bovine spongiform encephalopathy (BSE, or mad cow disease).
Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom (177 cases) and France (27 cases). This case is the fourth to be reported in the United States. In each of the three previous cases, infection might have occurred outside the United States, including the United Kingdom (2 cases) and Saudi Arabia (1 case). The history of this fourth patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States (since the U.S. doesn’t seriously test for mad cow disease, it’s just a hopeful statement).
The last case confirmed in the US was in 2007 in a US resident in Virginia who was born and raised in Saudi Arabia and has lived in the United States since late 2005.
CDC assisted the Texas Department of State Health Services (DSHS)’s investigation of this case and will continue to help confirm further details of the patient’s history, including the potential source of infection. There are no Texas public health concerns or threats associated with this case.
A classic form of CJD, which is not caused by the BSE agent, occurs worldwide, including in the United States. Annually, for every 1 million people in the United States, 1 to 2 develops classic CJD.
Variant CJD has different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene. The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD. The median duration of illness for vCJD is 14 months, compared to 5 months for classic CJD.
Creutzfeldt-Jakob disease (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). CJD is highly infectious via all bodily fluids and tissue.