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article imageWhat's happening today with N.B.'s mystery neurological disease

By Karen Graham     Mar 21, 2021 in Health
Experts in brain diseases say there are many unanswered questions about a possible degenerative neurological syndrome recently disclosed by New Brunswick health officials. Many people are worried and want to know how concerned they need to be.
At a news conference updating the COVID-19 pandemic on Thursday, the public learned about a mysterious neurological disease infecting a cluster of people in New Brunswick province.
The mystery illness has similarities to Creutzfeldt-Jakob disease, a rare and fatal brain disease, similar to Mad cow disease and other prion-caused disorders. Prion diseases are a group of rapidly progressive, fatal, and infectious neurodegenerative disorders affecting both humans and animals.
Here's what is known about the disease. The first case of the disease was diagnosed in 2015, according to the memo sent out by the chief medical officer of health to the New Brunswick Medical Society and to doctor and nurses associations.
After the initial diagnosis in 2015, three years later, in 2019, 11 additional cases were discovered, with 24 more cases discovered in 2020 and another six in 2021. Five people have died. The cases seem to be concentrated in the Acadian Peninsula in northeast New Brunswick and the Moncton region in the southeast.
Biopsy of the tonsil in variant  Creutzfeldt–Jakob disease.(CJD) prion protein immunostaining.
Biopsy of the tonsil in variant Creutzfeldt–Jakob disease.(CJD) prion protein immunostaining.
Sbrandner (CC BY-SA 4.0)
Questions galore but the answers are still elusive
People are curious, even scared. And that is to be understood. And questions abound - like what is this disease? Where does it come from? Is it something we are eating? All these questions and many more are all reasonable.
Yvon Godin, the mayor of Bertrand, a village further north on the peninsula, who also chairs the Forum of Acadian Peninsula Mayors, agrees. "We are very, very worried about it," Godin said. "Residents are anxious, they're asking 'Is it moose meat? Is it deer? Is it contagious?' We need to know, as fast as possible, what is causing this disease."
Yet the answers will only come after a thorough investigation into the disease itself. Theories have been put forward that the cause could be environmental, possibly some kind of toxin, especially seeing the disease is concentrated in specific areas, says Dr. Debbie McKenzie, with the University of Alberta.
“It would be actually stranger, in a sense, if it was spread out across all of New Brunswick or all of Atlantic Canada,” McKenzie said. “Then I think we're talking about something very, very different.”
Dr. McKenzie and Dr. Valerie Sim are both experts in brain diseases at the university where both doctors study prion diseases, which are caused by an abnormally folded protein in the brain. Those diseases cause the brain to waste away and the afflicted organs are often riddled with holes, Sim said.
Both Sim and McKenzie said it's unlikely the cluster of disease in New Brunswick is a previously unknown prion disease, though they have been known to pop up. In 1996, for example, a variant of Creutzfeldt-Jakob disease was discovered in the UK, McKenzie said in an interview Friday. Such diseases are easily identifiable in an autopsy because of the damage to the brain.
A call to action was answered
Dr. Neil Cashman is a professor in the University of British Columbia's faculty of medicine and is a neurologist with a special expertise in prion diseases. When Cashman first heard about the cases in New Brunswick, he says his first thought was, "We have a problem on our hands," according to CBC Canada.
Clearly, he said, "this was a call to arms to identify the cause."
California sea lion killed by domoic acid poisoning.
California sea lion killed by domoic acid poisoning.
NOAA/CSCOR/COP / Woods Hole Oceanographic Institution
Well, about a year ago, teams of researchers, scientists, and epidemiologists began gathering, at the federal and provincial levels to further investigate the disease. There is one thing the researchers do know, according to Dr. Cashman. "All the evidence, he said, points to this not being a prion disease such as Creutzfeldt-Jakob disease."
"There is no evidence, not a hint — even in the three autopsies that have been performed — of a human prion disease. That came as a surprise to me, frankly," he said. "So in essence, this is something new, and we need to get on the stick and figure out what this is."
Because they know that the disease is likely not a prion disease, then this leaves researchers looking at other - including unknown - neurological disorders and environmental toxins, says Cashman.
One possible culprit might be B-methylamino-L-alanine (BMAA), an environmental toxin made by certain bacteria that can accumulate in fish and shellfish.
β-Methylamino-l-alanine, or BMAA, is a non-proteinogenic amino acid produced by cyanobacteria. BMAA is a neurotoxin and its potential role in various neurodegenerative disorders is the subject of scientific research.
Microcystis algae grow in a large bloom in the Copco Reservoir on the Klamath River  posing health r...
Microcystis algae grow in a large bloom in the Copco Reservoir on the Klamath River, posing health risks to people, pets and wildlife.
Oregon State University
Domoic acid, another toxin produced by bacteria and that accumulates in shellfish, sardines, and anchovies is another possibility. So is lead, which can be responsible for clusters of neurodegeneration. Exposure to domoic acid affects the brain, causing seizures, and possibly death.
"All of these are speculation at this point," Cashman stressed. "A lot of scientific acumen will be required to pin it down to a cause. There's no sensible timeline I can provide on when we'll have an answer. It's just something that has to be the focus of scientific attention, and as rapidly as possible."
All the scientists have similar advice for the public: Try not to become consumed with worry or anxiety. Please believe that many experts are working to find the answer to what is going on. Additionally, people should take care and see a doctor if they experience any of the symptoms associated with the condition.
More about New brunswick, neuropogical disease, CreutzfeldtJakob disease, not human prion disease, jenvironmental toxin
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