As toll rises from N.B.'s mystery brain disease, Public Health pledges 'regular updates'

The death toll of a mysterious brain disease found only in New Brunswick has risen to six people from a previously reported five, according to the Moncton neurologist leading the province's investigation into it.

In an interview with CBC News, Dr. Alier Marrero also said there are now 44 cases overall, up from the 43 originally reported in March.

But neither Marrero nor the province's public health officials will confirm when or where that sixth person died, or where and when the 44th case was reported.

In an email Tuesday, Public Health confirmed that "since the first identified case, there have been 43 additional possible cases, for a total of 44 cases identified in the cluster, and there have been six deaths." But it did not disclose further details.

Public Health has also not shared the ages of the patients, although it has said the disease affects "all ages," and the agency has not held any public information sessions or updates since news of the cluster became public three weeks ago.

The disease has symptoms similar to those of Creutzfeldt-Jakob disease, a rare and fatal brain disease, according to an internal Public Health memo obtained by Radio-Canada dated March 5 and sent to medical professionals.

However, tests for Creutzfeldt-Jakob disease done on the patients have so far ruled out known prion diseases, and scientists are looking into the possibility that this is an entirely new disease, possibly caused by some sort of environmental toxin.

Aside from noting that most of the cases are concentrated in the Acadian Peninsula area in northeast New Brunswick and the Moncton region in the southeast, Public Health has not said specifically where the cases were identified.

The lack of public information about the cluster has been a source of concern for many since the disease first made headlines in mid-March.

"Residents are anxious, they're asking, 'Is it moose meat? Is it deer? Is it contagious?'" said Yvon Godin, the mayor of the northern Acadian Peninsula village of Bertrand and chair of the Forum of Acadian Peninsula Mayors, in an earlier interview.

"We need to know, as fast as possible, what is causing this disease."

Public Health promises website

On Tuesday, Public Health spokesperson Bruce Macfarlane said the office understands there is "confusion and concern in the community," particularly in the Acadian Peninsula and Moncton regions.

So far, he said, it is not known whether geographic area is linked to the neurological condition.

"Our investigation has not found any evidence suggesting that the residents of these regions are more at risk than those living elsewhere in the province," Macfarlane said.

He said Public Health is "dedicated to remaining transparent" as the investigation progresses, and noted that "regular updates will be provided" through a web page now being created.

The promised updates will be welcome for residents on edge, but Public Health is still facing criticism for what's been seen as a lack of transparency.

Steve Ellis, who said his father has displayed "every symptom" of the mystery disease described by Public Health for about two years now, is shocked the department didn't make the information public sooner.

The leaked Public Health memo stated that the first case dates back to 2015. There have been 11 additional cases in 2019, 24 more cases in 2020 and another seven in 2021.

In 2020, the cases were recognized as a cluster.

The memo was leaked on March 17 of this year, and Public Health confirmed the existence of the cluster the following day, when asked about it at a COVID-19 news conference.

"Why were we not told about this before now?" Ellis said. "Why didn't the government talk about this on their own, without having to have it leaked by the media?"

Symptoms' onset like flick of a switch

Ellis paused to collect his emotions when asked what kind of a father his dad, Roger Ellis, was before he got sick.

"He was great," he said. "He was a good dad, a very hard worker — he worked at the Brunswick mines in Bathurst from a very young age and brought the bacon home. He was understanding, he never let parenting fall to the wayside, he always did his very best — and he was that person right up until he got sick.

"That's how quickly the switch flipped."

Ellis, who now lives in Nova Scotia, vividly remembers the day that happened.

Two years ago in June, on the day of his 40th wedding anniversary, Roger Ellis collapsed and had a seizure.

Things escalated rapidly from there.

Within weeks, he was having delusions and hallucinations, was behaving aggressively and experiencing rapid weight loss. Within three months, he had lost 60 pounds. He couldn't eat, couldn't lift his head and could barely walk.

"We were called to the hospital to talk about end of life because they really thought he was dying," Ellis said. "We were preparing for his death without knowing what he was dying of."

'The lack of transparency has been really upsetting'

Roger was put on a series of medications and rallied a bit, but continued to experience debilitating symptoms that baffled doctors and the Ellis family alike.

That is, until a few weeks ago.

When Ellis heard the news that Public Health was alerting the province's doctors to a cluster of 43 cases of a mystery neurological disease, he jolted to attention.

"My dad had every one of the symptoms" they described, Ellis said.

He immediately reached out to Marrero, and an appointment was made for a consultation at the neurologist's Moncton clinic.

Marrero has now taken his father on as a patient and will conduct a series of toxicology, serology and other tests to determine whether he is suffering from the mystery disease.

It's the closest the family has come to answers in two years, Ellis said.

After reaching out to Public Health several times for information and to ask why New Brunswickers weren't told about the disease until this year, Ellis said he finally got a phone call last week from a senior adviser with the Department of Health.

"I was told that there would be more updates as updates become available," Ellis said. "And I'm hoping they'll stick to it, because the lack of transparency has been really upsetting."

He said "it took me going to multiple media outlets to get Public Health to call me back. And that shouldn't be the case."

Ellis has started a private Facebook page, titled Mystery Neurological Disease NB Support Group, for those who suspect they or a loved one has the disease.

"Our mission in sharing our story is to keep this going, and hopefully this brings answers to people," he said. " I know it will take time, but it's the most hope we've had since Dad got sick."

N.B.'s mystery disease: What we know so far

What is it?

An unknown neurological disease with similarities to Creutzfeldt-Jakob disease, a rare and fatal brain disease, or prion disease.

What's a prion disease?

A prion disease is a rare disease that pertains to a misfolded protein in the brain. The abnormal folding of the prion proteins provokes a chain reaction that destroys neurons and creates holes in the brain.

When was it discovered?

The first occurrence was retroactively found to have occurred in 2015, when the possible existence of a cluster of disease was first recognized by the CJD Surveillance System at the Public Health Agency of Canada in 2020. In 2019, 11 additional cases were identified, with 24 more in 2020 and seven so far in 2021.

When was it made public?

A March 5 internal memo from Public Health to health-care professionals was obtained by Radio-Canada and reported by Radio-Canada and CBC News on March 17.

Where are the cases?

The disease has so far only been identified in New Brunswick. It appears to be concentrated on the Acadian Peninsula in northeast New Brunswick and the Moncton region in the southeast.

How many cases are there?

Forty-four cases have been identified. Of those, Public Health has said 35 are on the Acadian Peninsula and eight are in the Moncton region. The location of the 44th case has not been revealed.

How many patients have died?

Six people have died of the mystery disease, according to neurologist Dr. Alier Marrero. The six are included in the 44 cases so far.

Who has been affected?

The disease affects all age groups and affects males and females equally, according to the Public Health memo. About half of the affected individuals are between 50 and 69 years of age.

What are the symptoms?

Symptoms include changes in behaviour, sleep disturbances, unexplained pain, visual hallucinations, co-ordination problems and severe muscle and brain atrophy.

Is it contagious?

Because the cause has not been determined, it is not yet known whether the disease is contagious.

What are the possible causes being researched?

Despite many similarities, tests for Creutzfeldt-Jakob disease have so far ruled out known prion diseases. Scientists are currently looking into the possibility that this is a new variant of a prion disease — or a new disease entirely. Neurologists and scientists suspect the cause might be exposure to an as-yet-undetermined environmental toxin.

Who's researching it?

The disease is the subject of investigation by an all-Canadian team of neurologists, epidemiologists, scientists, researchers and other experts. Moncton neurologist Dr. Alier Marrero is leading the research in New Brunswick. In Ottawa, senior scientist and Creutzfeldt-Jakob Disease Surveillance System director Michael Coulthart is leading the research.