2 Quebec deaths being investigated for CJD: reports

The deaths of two people in Quebec's Saguenay-Lac St. Jean region in the last few months are being investigated to determine whether they might have contracted a form of Creutzfeldt-Jakob disease, reports from the area indicate.

An official with the Public Health Agency of Canada told CBC News on Thursday that the agency has been aware of the cases.

"This case is not new to us," said Dr. Michael Coulthart, adding it generally takes at least three to four months to get test results.

So-called classic Creutzfeldt-Jakob — which appears only in humans — kills one in a million Canadians every year. The other form of the disease, variant CJD, has only been reported once in Canada and is thought to be contracted by eating beef from cattle infected with mad cow disease, or bovine spongiform encephalopathy (BSE). 

Both forms of CJD cause a fatal degenerative neurological condition.

According to CKRS-FM radio in Chicoutimi, Que., the deaths there of a person in December and another in February are being treated with extreme caution by federal health authorities amid concerns they possibly had a form of CJD.

The radio report, which first aired Wednesday, said two patients have never died of CJD within such a short period of time in one area of Canada.

In the hours following the death of the second person, a woman in her 50s, federal health officials asked her family to authorize an autopsy as soon as possible, and gave strict guidelines about how to dispose of the body, the report said.

The body was sent to Quebec City for a post-mortem and then returned to Chicoutimi, where it was cremated.

An undertaker at l'Alliance funéraire du Royaume funeral home in Chicoutimi told Le Quotidien newspaper that an autopsy had been performed in mid-February under strict conditions.

"Since it's possible for the disease to be transmitted during the autopsy, family members couldn't see the body," Stéphane Cantin said.

Coulthart, the Public Health Agency of Canada's principal investigator for suspected incidences of CJD, wouldn't comment on the details of the two cases, and would only say these kinds of procedures are "normal protocols."

"We see no aspect of this situation that is outside the bounds of our normal operation," he said. 

It could take six months before health officials have final results from the post-mortem, and can confirm or deny whether the woman had any form of CJD.

Britain has had about 160 cases of variant CJD. The one confirmed death in Canada was a person who had lived in England.