The federal government is scrapping a program that performed autopsies on the brains of people suspected of having Creutzfeldt-Jakob disease, the fatal neurodegenerative disorder that came to public prominence during the mad cow crisis of the late 1990s and early 2000s.
The Public Health Agency of Canada (PHAC) will stop funding the brain autopsies at the end of next month to save money, according to a copy of an e-mail sent to pathology labs across the country on Feb. 13 and obtained by The Globe and Mail.
“As you may be aware, increased fiscal restraint is being experienced across the Government of Canada,” said the message, signed by two senior officials with the country’s Creutzfeldt-Jakob Disease Surveillance System. “After careful consideration, we will be discontinuing financial support related to autopsy services for patients suspected of CJD.”
The budget for the autopsy program fluctuated, depending on the number of brains examined, and ran as high as $1.3-million a year. Fifty autopsies were completed in 2023-24 and 44 in 2024-25, according to Anna Maddison, a spokeswoman for PHAC.
Some doctors and scientists who study prion diseases, including CJD, slammed the decision because they fear it could leave Canada in the dark about possible human cases of chronic wasting disease. CWD is a different prion disease that is spreading among cervids such as deer, elk and moose mainly in Alberta and Saskatchewan.
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Chronic wasting disease (CWD) has not been found in humans. But if it were to make the jump from cervids to people, its symptoms could look like the loss of co-ordination and memory typical of CJD.
The best way to know the difference would be to conduct the type of brain autopsy that the federal government just stopped funding, said Joel Watts, a professor of biochemistry at the University of Toronto and the Canada Research Chair in Protein Misfolding Disorders.
“This is something that any scientist would say is a terrible idea,” Dr. Watts said. “It’s one of these cases where I don’t think the government sees the big picture.”
Ms. Maddison of PHAC said there are now reliable lab tests that can be performed on patients suspected of having CJD while they’re still alive. Those advances have lessened the need for confirmatory brain autopsies, she said by e-mail.
However, the experts The Globe spoke to said they were concerned that live tests may not be able to detect a CWD case since one has never been found in humans.
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As for chronic wasting disease, Ms. Maddison said the expert consensus is that it is “very unlikely” to be transmitted to humans. She added that testing performed at the National Microbiology Laboratory in Winnipeg, “is sensitive for detection of almost all subtypes of CJD, and surveillance for human prion diseases in Canada (including potential novel forms, like CWD) will continue.”
CWD and CJD are both incurable afflictions caused by transmissible misfolded proteins called prions. People who develop CJD can sometimes be mistaken for dementia or Parkinson’s patients as their memories fade and their movements become jerky. The disease is progressive and usually kills in less than a year. There is no treatment.
CJD is rare in Canada. Only 445 cases have been reported since 2020. The vast majority are a type known as sporadic CJD, where normal proteins in the brain misfold into infectious abnormal proteins for no discernable reason. A small number of cases are caused by an inherited genetic mutation.
The least common, but most famous, type of CJD is variant CJD, the kind caused by eating beef from cows infected with the animal prion disease Bovine Spongiform Encephalitis – better known as mad cow disease.
Variant CJD ceased to be much of an issue after the mad cow crisis, which hit hardest in Britain, was brought under control by rules barring the feeding of meat-and-bone meal to cows.
Gerard Jansen, a neuropathologist at the University of Ottawa, runs the laboratory where the federally funded autopsies were performed.
He learned about the funding cuts on Feb. 13, the same day the PHAC message went out. At the same time, he heard his lab would have to stop accepting brains or brain biopsy samples for the CJD program immediately.
“That basically means that every patient after last Friday is out in the cold, and that includes biopsies,” Dr. Jansen said. His lab is sometimes asked to examine tissue from a brain biopsy to confirm, rule out or subtype a CJD case, he explained.
Dr. Jansen is also worried about the risk, however remote, of missing an early case of CWD in humans. “I think that would be disastrous, frankly.”
Valerie Sim, a neurologist at the University of Alberta who sees patients with prion diseases, echoed those concerns.
“In an era of funding cuts, when a disease is rare or not immediately seen as affecting humans, it’s easy to look away,” Dr. Sim said. “But my concern is for the potential of a repeat of history. We didn’t do a very good job protecting people from mad cow disease.”