Mysterious neurological disease haunts Atlantic Canada | Canada


When Roger Ellis fell ill two years ago, his family rushed to hospital fearing he would have a heart attack. Doctors quickly ruled out this, but a few days later he had a seizure.

In the weeks that followed, the retired industrial mechanic, 64, who lived in the city of Bathurst, in eastern Canada, became increasingly anxious and disoriented, and repeated himself often.

His condition deteriorated rapidly. In his first three months in the hospital, he lost 60 pounds and had to eat from a tube and use a wheelchair.

“We almost lost him a few times,” said his son Steve.

The doctors were puzzled. They excluded epilepsy, stroke, Creutzfeldt-Jakob disease, autoimmune encephalitis and cancer. Roger spent over a year in hospital before being transferred to a nursing home.

“We unfortunately accepted the fact that we weren’t going to know what he had until his death,” Ellis said.

Roger remains in the care home and his family now believe he is part of a group of people suffering from a mysterious progressive neurological disease.

The cases, largely concentrated in a sparsely populated area of ​​New Brunswick, have baffled experts. And the mystery has sparked a heated row between officials who suggest the cases are unrelated and scientists who argued they may all have been triggered by environmental factors or contaminants.

Victims experience unexplained pain, spasms and changes in behavior, said Dr Alier Marrero, the neurologist who identified the first cluster. Many then showed signs of cognitive decline, muscle wasting, drooling and chattering, and frightening hallucinations.

An illustration of brain synapses. Illustration: Alamy

So far, 48 cases have been publicly acknowledged, but officials familiar with research on the cluster have told the Guardian that the number of people affected now exceeds 100.

Public health officials have been following the cases for almost a year and have given little indication that they are close to a solution.

But in an unexpected turn of events, New Brunswick Health Minister Dorothy Shephard announced in late October that an epidemiological report had found no significant evidence that a known food, behavior or environmental exposure could. to be responsible.

The press conference followed a controversial article presented to the Canadian Association of Neuropathologists who claimed that eight deaths attributed to the group were “misdiagnoses” of known diseases, such as Alzheimer’s disease and cancer.

New Brunswick officials have begun to float the idea that the group itself may be an amalgam of unrelated cases. But the sudden change has raised concern among families of the victims that the province is rushing to declare the case closed.

“It was just a demonstration of the incompetence and disorganization of the province,” said Ellis, who said other families he had spoken with were “devastated” by the province’s response. “It’s too bad my dad and so many others are involved in this mess.”

The episode also revealed the severed relationship between the province and federal health officials, who were asked not to participate in the investigation – and said they were barred from testing tissue samples from deceased patients. .

Staff at Canada’s public health agency said they were caught off guard by the press conference and the controversial document – and cast doubt on the province’s suggestion that there was no connection between the cases.

“It was amazing,” a scientist told The Guardian. “Even a layman understands that you will never find anything just by looking at the phenomenon itself. You must have a control group … It was just rookie epidemiology.

RResidents learned of the mysterious group almost a year ago, when a memo leaked by the province’s public health agency asked doctors to look for symptoms similar to Creutzfeldt-Jakob disease (CJD) – a rare and fatal brain disease caused by malformed proteins called prions.

But at the end of October, an article presented by Dr Gerard Jansen, the Canadian Association of Neuropathologists shocked families by suggesting that eight of the victims had died of unrelated brain diseases.

“We have unequivocally called for this study to be withdrawn and for an apology,” said Kat Lanteigne, leader of the nonprofit advocacy group BloodWatch. “Every scientist that our organization has ever worked with or contacted is absolutely mortified. “

Jansen’s article was also condemned by Canada’s public health agency, which accused him of misusing private data.

Jansen did not respond to a request for comment, but previously told CBC News that the federal agency does not have the data. He also said that after presenting his findings, his colleagues “unanimously endorsed my findings in the eight autopsies and my findings. [regarding] this cluster ”.

The Public Health Agency of Canada has stated that it “reserves the right to take corrective action, to ensure that this type of situation does not recur and, if necessary, to remedy any misinterpretation of the resulting facts in the context of public health “.

And other scientists have also questioned Jansen’s findings.

Man in red jacket leaning against a tree, eyes closed
Steve Ellis has tried to find answers to what caused the mental and physical deterioration of his 64-year-old father. Photograph: Darren Calabrese / Darren Calabrese / The Guardian

“[Jansen] deeply, deeply misinterpreted the significance of his findings, ”said a federal scientist, who argued that even if the victims suffered from known brain diseases, this would not rule out the impact of environmental factors.

Increasingly, experts believe that β-methylamino-L-alanine (BMAA) – a neurotoxin found in blue-green algae blooms across the province – could help explain the various symptoms.In a study, high concentrations of BMAA were found in lobster. The lobster fishery is one of New Brunswick’s biggest economic engines – suggesting that efforts to exclude the existence of a cluster may be motivated by political decision-making.

Federal scientists would like to test the brain tissue of eight people in the group who died from potential environmental toxins. But the province refused permission for such studies.

Families and scientists are also increasingly troubled by the New Brunswick government’s apparent efforts to distance itself from Marrero, the neurologist who identified the group.

New Brunswick Health Minister Dorothy Shephard recently told reporters there had been “issues” in the reporting process that allowed the situation to escalate “often unattended” and failed to say that Marrero had worked with federal scientists, as well as neurologists from other provinces, when the cluster was first identified.

The provincial health ministry did not respond to a request for comment from the Guardian.

Steve Ellis holds photos of his father and himself near his home in Bridgewater, Nova Scotia.
Steve Ellis holds photos of his father and himself near his home in Bridgewater, Nova Scotia. Photograph: Darren Calabrese / Darren Calabrese / The Guardian

But for brain disease experts, the age range of patients in the group, the volume of cases, and the geographic location of those with pain suggest that further investigation is needed.

This stuff doesn’t easily lend itself to the explanation that this is just a random collection of sporadically occurring cases that have been artificially bundled together by an overly enthusiastic neurologist, ”the scientist said. “It just doesn’t wash. “

A second investigation, led by a committee of neurologists from across the province – but probably not Marrero – is expected to release a second report early next year after examining all 48 patients, nearly all of whom were treated with Marrero.

“They really seem to discredit him without using his name,” Ellis said. “This is unacceptable. Of all people, he has been the most communicative over the past few months. His work ethic, professionalism and empathy are simply top notch.

Ellis says if it hadn’t been for the CJD memo leak last year, the audience would still be in the dark.

“It makes me sick to think that they are trying to make this happen,” he said. “And you know what? Ultimately, if it’s nothing, then we still have to figure out how to help people. Because they’re still suffering.


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