April 15th, 2011 by Rasheed
April 2011: Canadian health officials have confirmed the country’s second-ever case of variant Creutzfeldt-Jakob disease, the rare human form of the so-called “mad cow disease” usually caused by eating infected beef. The head of the federal agency that monitors the disease states the man, now an Ontario resident, is believed to have been infected in Saudi Arabia.
Information released by Health Canada in both Arabic and English states the man began experiencing symptoms just prior to immigrating to Canada from Saudi Arabia in 2010.
Outbreaks of the human form of the disease also led to changes in blood donor regulations in a number of countries. The discovery of a new case of variant Creutzfeldt-Jakob last month prompted an almost immediate change in Canada’s blood donation policies, which now restrict anyone who was in Saudi Arabia for six months or longer in the period from 1980 to1996 from giving blood. The restriction previously related only to the United Kingdom, France and Western Europe.
The disease first emerged in humans in the mid-1990s and peaked in 2000 in the U.K, where it has been the most prevalent. According to the World Health Organization, the human form of mad cow disease is an aggressive condition that begins with psychiatric symptoms and dementia, and progresses quickly to complete lack of physical control, leaving the person unable to move or speak. The WHO states the disease leaves “daisy-like” holes in the brain.
Variant Creutzfeldt-Jakob disease is the human form of bovine spongiform encephalopathy, or BSE. It is most commonly caused by eating meat from BSE-infected cows, though there have also been documented transmissions through blood transfusions and the potential for infection through medical and dental tools.
Dr. Michael Coulthart, director of the Canadian Creutzfeldt-Jakob disease surveillance system at the national microbiology lab in Winnipeg, Canada stated, “It’s one of those cases where long-term vigilance is going to be required to definitively deal with the issue. Every case is potentially significant, and you don’t want to miss any. Any individual case can have large implications, potentially, and you want to know as much as you can about the origins of each case that does occur, whenever it occurs. So, that’s one reason to stay vigilant.”
Because of the disease’s long incubation period, Coulthart states there are also concerns about the “carrier effect,” where people could have the disease for years, even decades without knowing it, then pass it on through blood transfusions or medical or dental tools. In that scenario, he states there could be a resurgence in the human form of the disease in the future, particularly given that the prion agent of the disease is extremely resistant to normal methods of decontamination and disinfection.
One pathologist who studied the disease in the 1990s described it at the time as being “like something out of the X-Files, the indestructible thing from outer space.” Despite a relatively small number of human deaths, the emergence of mad cow disease in animals and humans in the 1980s and 1990s had a huge impact around the world, prompting the large scale culling of animal herds, leading to massive changes in animal feeding guidelines, and devastating cattle operations and trade globally.
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