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Canada - New Brunswick monitoring more than 48 cases (10 fatal) of unknown neurological disease - 2015+ - officials say illnesses are due to unrelated

What can a medical mystery from Guam teach New Brunswick about its own strange, deadly disease?

A neurological syndrome that’s killed several New Brunswickers has stumped doctors, but researchers are looking at an algae-based toxin – and a decades-old, inconclusive debate about its effects – as a possible factor

WENCY LEUNGHEALTH REPORTER
PUBLISHED 7 HOURS AGO
UPDATED AUGUST 18, 2021

They called it lytico-bodig. As the Second World War drew to a close, U.S. military doctors stationed on Guam noticed many of the Pacific island’s Chamorro people were suffering from a baffling and devastating illness.

The local name for the progressive and fatal disease – said to have come from the Spanish “paralytico” – provided a label for what Islanders described as an illness that disconnects one from one’s family. It captured a constellation of symptoms, including muscle wasting, paralysis, muscle rigidity, shaking, confusion and memory loss. Doctors and researchers observed this strange illness shared characteristics of amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease), Parkinson’s disease and Alzheimer’s disease, and they clamoured to try to understand it, with the hope it would shed light on other neurodegenerative disorders.
...
The disease was still a mystery when Canadian chemist Susan Murch travelled to Guam as a postdoctoral fellow in 2003 to study a neurotoxin researchers believed might hold the key to solving the puzzle. What she and the scientists she worked with discovered there provided not only what they considered a possible source of the illness, but may also offer clues to a new medical mystery in New Brunswick, where dozens of people have developed unexplained neurological symptoms.

Dr. Murch, working with principal investigator Paul Cox, a U.S. ethnobotanist, found that a toxin produced by blue-green algae called beta-methylamino-L-alanine, or BMAA, was present in cycad trees, a source of food for the island’s residents. They found the same toxin in the animals residents consumed, such as pigs, deer and bats that ate the seeds, fruit and other parts of the cycad plants. And they also found it in the brain tissue of several Chamorro patients who died of the mysterious disease.

Dr. Cox, together with famed British neurologist Oliver Sacks, hypothesized that neurotoxic BMAA, the amounts of which were magnified as people consumed animals at higher levels of the food chain, might offer a long-sought explanation for what was causing damage to people’s bodies and brains. Today, that hypothesis is still debated. But investigators of the new illness in New Brunswick are revisiting the work of Dr. Murch and her colleagues.
...
“We’re not saying that we have that disease,” said Alier Marrero, a neurologist at Dr. Georges-L.-Dumont University Hospital Centre in Moncton, who is leading a special clinic for those affected. The symptoms of his own patients, which include pain, hallucinations, severe insomnia and visual disturbances, are not exactly the same as the ones experienced by those on Guam, he said. “But there could be a significant or similar link to an environmental factor that, if identified, it could be prevented or stopped. That’s our hope.”
...
https://www.theglobeandmail.com/can...-from-guam-teach-new-brunswick-about-its-own/
 
This is a mechanism for how this toxin might be producing this illness.

I'm renaming this thread now to get the incorrect diagnosis out of the title; if this was CJD, you'd have a lot more than 6 deaths in 48 cases as CJD is uniformly fatal. While a couple of these cases might be coincidental CJD infections, CJD is not the cause of the wider outbreak.

ViewPoint: Sault Ste. Marie , Blue-Green Algae and New Brunswick Neurological Desease | SaultOnline.com

[snip]

The body mistakes BMAA for the amino acid L-serine, a naturally occurring component of proteins.

When the toxin is mistakenly inserted into proteins in brain cells in place of L-serine, they become “misfolded,” meaning they no longer function properly, leading to neuronal meltdown.
 
Source: https://journals.lww.com/neurotoday...uestions_About_a_Mysterious_Neurologic.1.aspx


New Questions About a Mysterious Neurologic Cluster in Canada
By Dan Hurley
September 2, 2021

Article In Brief

Forty-eight people, including six who have died, have been identified as having a cluster of unknown neurologic disease in the province of Alberta in Canada. But independent experts question the validity of such a cluster, raising the possibility that the cases are functional neurologic disorders.

...Dozens of media outlets, local and international, have reported on news of the mysterious cluster, and some patients have expressed fears of living in a province with a “mysterious brain disease.”

Alier Marrero, MD, the neurologist who diagnosed and deemed the cases to be part of a cluster, told Neurology Today, “Currently our clinic is following over a hundred cases. And we have received dozens of communications. We've had referrals from other provinces in Canada, and also some in the United States.”

Dr. Marrero, an assistant professor of clinical neurology at Sherbrooke University and a member of the Canadian ALS Scientific Committee and the Canadian Network of Neuromuscular Diseases, said no cause has yet been found. “It's obvious that this is acquired,” he said. “Anybody who is exposed could have it.”

Yet an investigation by Neurology Today—including interviews with patients, family members, the president of the Canadian Institutes of Health Research (CIHR), and neurologists on the oversight committee appointed to review the matter—raises questions about the validity of the cluster.

Determining “whether this is a single neurological disorder or various disorders...is one of the main goals of the Oversight Committee,” stated an email from the committee in response to written questions from Neurology Today. “It is too early to answer this question.”...
 
This is the importance of getting a good case definition. If you're going to include any patient anywhere in North America with unexplained neurological symptoms, you're going to catch an awful lot of cancer, Alzheimer's, ALS, encephalitis, etc. in your definition. I would think the case definition should require exposure in New Brunswick itself.

While it is possible that this is a false cluster caused by unrelated illnesses in geographical proximity, several of these cases are unusual enough to require individual diagnosis; the 20 year old college student in particular is difficult to explain due to more common causes.
 
Source: https://globalnews.ca/news/8327861/mystery-brain-disease-deaths-new-brunswick-dr-gerard-jansen/


Cause of eight N.B. ‘mystery’ brain disease deaths identified by pathologist
By Kevin Bissett The Canadian Press
Posted October 26, 2021 8:09 pm

A neuropathologist who examined the deaths of eight people in New Brunswick initially described as having a mysterious neurological disease says the deaths were actually due to known diseases.

A summary of the study led by Dr. Gerard Jansen of the University of Ottawa, posted this month on the Canadian Association of Neuropathologists website, says the original cases were “misclassified clinical diagnoses.”

In March, New Brunswick health officials alerted the province’s doctors, nurses and pharmacists about a cluster of residents with an unknown and potentially new neurological syndrome with symptoms similar to those of Creutzfeldt-Jakob disease.

Jansen’s study indicates that those who died had diseases that included known neurodegenerative diseases and cancers...
 
New Brunswick’s Mystery Disease: Why Did the Province Shut Out Federal Experts?

The provincial government’s closed-door investigation has confused experts, stoked fears, and missed an opportunity to solve a possible new brain disorder

BY MATTHEW HALLIDAY
PHOTOGRAPHY BY CHRIS DONOVAN
Updated 20:24, Oct. 25, 2021 | Published 15:43, Oct. 22, 2021

IN JANUARY 2020, Alier Marrero, a neurologist in Moncton, New Brunswick, began examining a patient he’d been referred—an eighteen-year-old woman named Gabrielle Cormier from Dalhousie Junction, a small town in the province’s far north.

Her case was bewildering: as a high school student, she started to have difficulty reading, especially on computer screens, where letters appeared hazy and indistinct. By grade twelve, in 2019, her concentration flagged and she was losing strength in her lower body. A few months before graduating, she collapsed at school. An ER doctor chalked it up to a panic attack, but within weeks, she had developed tingling sensations in her legs; over the summer, they turned a sickly grey. In the fall, while studying biology at New Brunswick’s Mount Allison University, Cormier felt her disorientation and mental fog worsening, and her exhaustion grew debilitating.

That winter, Marrero performed a gauntlet of exams: cognition tests, memory tests, blood tests, and scans including EEG, MRI, VEP, and SPECT (a nuclear-imaging test in which a radioactive tracer is injected into the bloodstream). By then, Cormier was having difficulty walking and her vision problems had turned hallucinatory—a fluctuating field across her line of sight that she compares to TV static.

On Valentine’s Day 2020, Cormier underwent a spinal tap for signs of Creutzfeldt-Jakob disease (CJD), a fatal, swiftly developing disorder caused when brain proteins called prions misfold into an abnormal form. Cormier’s spinal fluid came back clean for CJD, however, as did the other tests, with two exceptions: an EEG showing diminished electrical activity in the brain and a SPECT showing reduced cerebral blood flow. Both suggested neurological impairment, but neither pointed, by itself, to any known illness.

Baffling as it was, Cormier’s condition was familiar to Marrero. A Cuban-born neurologist, he had worked in Moncton since 2012 and, in recent years, had seen more and more patients—often unusually young, equally men and women—displaying bizarre signs of neurological decline. In many cases, the symptoms developed with excruciating speed but began almost inconspicuously with behavioural changes, sleep disturbances, or inexplicable pain. Then came memory difficulties, muscle wasting, and difficulty balancing. Many patients experienced visual hallucinations—some relatively benign (Cormier’s TV static), others unsettling (looming shadows), some nightmarish. There were auditory hallucinations: music, breaking objects, distant voices. Eventually, dementia appeared; even some youthful patients experienced a state akin to late-stage Alzheimer’s. Some developed Capgras delusion, the belief that loved ones have been replaced by impostors. The only universal symptom was myoclonus: chronic muscle spasms so severe that spouses often couldn’t share a bed. Some patients eventually progressed to akinetic mutism: they were unable to speak or move but still experienced spasms.

The symptoms, terrifying and incapacitating, appeared to be expressions of a sickness with no name and no known provenance.
...
https://thewalrus.ca/new-brunswicks-medical-mystery/
 
Now 9 deaths. Eight of them confirmed due to known illnesses such as cancer and Alzheimers. Suspicion that this is not a single illness.

Public Health now questioning 'validity' of New Brunswick's mystery brain disease | CBC News

Also:

[snip]

The report also found 12 of the 34 people "had family members or close contacts who were experiencing similar symptoms," though it's not clear what the significance of that might be.

(Is this a sign of casting too wide a net, or a sign that despite the misdiagnosed fatalities, there might be something else going on here? - alert)
 
A whistleblower in the Canadian province of New Brunswick has warned that a progressive neurological illness that has baffled experts for more than two years appears to be affecting a growing number of young people and causing swift cognitive decline among some of the afflicted.

Speaking to the Guardian, an employee with Vitalité Health Network, one of the province’s two health authorities, said that suspected cases are growing in number and that young adults with no prior health triggers are developing a catalog of troubling symptoms, including rapid weight loss, insomnia, hallucinations, difficulty thinking and limited mobility.



The official number of cases under investigation, 48, remains unchanged since it was first announced in early spring 2021. But multiple sources say the cluster could now be as many as 150 people, with a backlog of cases involving young people still requiring further assessment.

https://www.theguardian.com/world/2022/jan/02/neurological-illness-affecting-young-adults-canada
 
Illness in a caretaker for a patient (including in an HCW below????) might suggest contagion, not an environmental trigger. But death counts are exactly where they were a few months ago. Case counts are in dispute, but it's not clear whether additional cases are occurring or whether unrelated illnesses are being lumped together.

Whistleblower warns baffling illness affects growing number of young adults in Canadian province | Canada | The Guardian

(snip)

Several new cases in New Brunswick involve caretakers of those afflicted, suggesting a possible environmental trigger

(snip)

At the same time, at least nine cases have been recorded in which two people in close contact – but without genetic links – have developed symptoms, suggesting that environmental factors may be involved.
  • One suspected case involved a man who was developing symptoms of dementia and ataxia. His wife, who was his caregiver, suddenly began losing sleep and experiencing muscle wasting, dementia and hallucinations. Now her condition is worse than his.
  • A woman in her 30s was described as non-verbal, is feeding with a tube and drools excessively. Her caregiver, a nursing student in her 20s, also recently started showing symptoms of neurological decline.
  • In another case, a young mother quickly lost nearly 60 pounds, developed insomnia and began hallucinating. Brain imaging showed advanced signs of atrophy.
------------

(This story continues getting odder. If this was an environmental exposure, I would expect larger clusters than just two cases. Entire families eat the same food. Could this be some kind of vector-borne pathogen causing encephalitis? Prion disease seems to be ruled out by autopsy, and this isn't the kind of clustering you would expect by contagion from contact. I find it at least a little odd that the only prospective diagnosis that seems to be under consideration is BMAA exposure.

Also, keep in mind the large number of contacts Ms. Cormier has had in multiple provinces, as well as the large number of cases Dr. Marrero has had contact with, neither one causing any apparent "transmission". - alert)
 
Last edited:
There are political overtones here as well:

Hospital worker blows whistle on spread of New Brunswick’s mystery illness: Bombshell report – NB Media Co-op (nbmediacoop.org)

(snip)

In a recent report by CBC’s Fifth Estate, several people within the cluster region who felt they were experiencing symptoms found it impossible to see a specialist because of long wait times. They also felt that practitioners dismissed their concerns.

The government, on the other hand, after continuously botching their COVID response, including over the Christmas holiday, has unsurprisingly offered no strategy to tackle this mystery illness.

Instead of calling for all hands on deck to investigate this mystery illness, the Premier is preventing scientists from doing their job, fighting healthcare workers who deserve fair wages, and forcing hospital closures.
 
This is the official report on 8 of the fatal cases. It was published in October, but I just found it now:

Abstract #16 | CANP-ACNP

There was one case of metastatic carcinoma, one case of FTLD-TDP43, one case of neocortical Lewy body pathology, one case of neocortical Lewy body pathology and AD, 2 cases of AD with vascular pathology, one case of mainly vascular pathology, and one case without significant pathology (consistent with patient’s previous history). In these 8 patients no evidence for a prion disease was found, nor novel pathology. We suggest that these 8 patients represent a group of misclassified clinical diagnoses.

------

(One additional thought...if there are currently nine suspected clusters, and about two months ago there was a report of 12 suspected clusters, does that mean that additional suspected cases have been excluded? If so, what were they diagnosed with? - alert)
 
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