New Brunswick ramps up efforts to solve mystery behind unknown brain disease

FREDERICTON — New Brunswick is redoubling its efforts to find the cause of a baffling neurological disease that has killed six and infected 48 others, announcing Thursday that an expert committee will lead the ongoing investigation.

“The discovery of a potentially new and unknown syndrome is scary,” Health Minister Dorothy Shephard told a new conference. “I know that New Brunswickers are concerned and confused about this potential neurological syndrome.”

Shephard also announced that health officials have drafted a new, comprehensive questionnaire for patients and their families. The questionnaires, which can take up to four hours to complete, will be filled out over the next six to eight weeks, and the new committee has a four-month schedule to complete a clinical review of every patient’s medical records.

Part of the committee’s mandate is to provide second opinions and to rule out potential causes.

“We have faith that this diligence will pay off,” Shephard said.

Dr. Edouard Hendriks, one of committee’s co-chairs, said the nine-member group is facing a tough challenge. “There are many diseases that lead to these kind of problems, including Alzheimer’s disease, vascular dementia and other types of dementia,” said Hendriks, vice-president of medical, academic and research affairs at the Horizon Health Network.

“All of these diseases start pretty much the same way,” he added, “with a little bit of cognitive disorder, some strange muscle movements. And it’s only over time … that specialists can say, ‘This seems to be more this than that.'”

The symptoms for the disease include rapidly progressing dementia, muscle spasms, atrophy, memory loss and hallucinations. Fifty-one per cent of the cases have involved women and 49 per cent men, and the age range has been between 18 and 85. 

“I know that many people in the province feel frustrated and I understand that, too,” Shephard said, acknowledging that the families of some victims have complained that the government is doing a poor job of sharing information.

The provincial Health Department says the first case of the disease dates back to 2015, but a potential cluster of cases wasn’t identified by federal officials until December 2020.

Shephard said the federal Creutzfeldt-Jakob Disease (CJD) Surveillance System spotted a pattern of symptoms among patients last year and then ruled out the possibility that the syndrome was a human prion disease like CJD.

News of the unknown syndrome first emerged through a March 5 memo from the province’s deputy chief medical officer of health, Dr. Cristin Muecke, to various medical professional associations in New Brunswick.

At the time of their referrals, most of the existing patients were living in and around the Moncton, N.B., area and the Acadian Peninsula in northeastern New Brunswick. But there is no hard evidence to suggest the syndrome is linked to geography.

Shephard declined to be more specific when asked to describe where the patients are from. “They’re not just secluded to two areas,” she said. “It would be short-sighted to start targeting areas.”

Last week, the province opened a special clinic for patients. The Special Neurodegenerative Disorder Clinic is located at the Moncton Hospital. Shephard confirmed that 40 patients had already been seen at the facility.

As well, the Horizon Health Network launched a website that offers details about what is known about the illness. The website says an investigation team is exploring all potential causes including food, environmental and animal exposures.

In March, a researcher with the Public Health Agency of Canada said a potential cause could be some kind of environmental exposure. 

Michael Coulthart, the head of the Creutzfeldt-Jakob Disease Surveillance System, said many neurological disorders have features that overlap, but he said he has not seen anything like the New Brunswick cluster before. 

He said the fact that the cases have been spread over a number of years will make it more difficult to pinpoint a source.

On Thursday, Shephard said the committee has to remain open to all possible causes.

This report by The Canadian Press was first published June 3, 2021.

— By Michael MacDonald in Halifax.

The Canadian Press