MLAs call for “interprovincial inquiry” to “address surge of atypical neurogenerative illness.”

Early last week, two MLAs issued a joint press release calling for federal and provincial “cooperation” to address a “surge of atypical neurodegenerative illness.” Elizabeth Smith-McCrossin, MLA for Cumberland North in Nova Scotia, and Megan Mitton, MLA for Memramcook-Tantramar, in New Brunswick, say there are patients on “both sides” of the border and the Public Health Agency of Canada (PHAC) should step in and lead an interprovincial inquiry.

While the cases of a “mysterious neurological disease” had been accumulating for years, the public only became aware of the situation in March of 2021, when Radio-Canada reported that a leaked memo from the New Brunswick government’s public health department advised medical professionals to be on alert for patients presenting with symptoms similar to Creutzfeldt-Jacob disease (CJD)— a prion disease with severe neurological manifestations, including memory loss, loss of coordination, vision problems and dementia.¹

In a recent news report, New Brunswick neurologist Dr. Alier Marrero—the referring specialist for the vast majority of the affected patients—said he’s seen “more than 320 patients in six provinces with atypical neurological symptoms.”

Early on, there was a concerted effort to get to the bottom of the unusually high number of patients presenting with CJD-like symptoms – but testing negative for CJD. Federal and provincial governments began collaborating in the spring of 2021 with a focus on discovering what was believed at the time to be the “environmental cause” of the syndrome. Five-million dollars in federal research funding was being fast-tracked to explore the cluster and its potential environmental triggers.

Experts in the fields of toxicology, zoonoses, food safety, and fisheries were being engaged. Investigative reporting on the story, based on a trove of internal documents, revealed that PHAC distributed summary papers, internally, about potential environmental causes, stating:

Primary data shows that the disease is not genetic, and could be contracted from water, food, or air. It is not known if the disease is contagious… Other possibilities include an environmental toxin similar to domoic acid, which caused the Canadian amnesic shellfish poisoning cases in 1987, or beta-Methylamino-L-alanine (BMAA).

BMAA is a neurotoxin produced by different types of cyanobacteria, or blue-green algae, which can be found in shellfish. Paralytic Shellfish Poison has been found in lobster in Canada since 2008. According to the internal documents, the primary concern with eating lobsters is the tomalley (the green paste-like substance that functions as its liver and pancreas). There was also discussion that the neurodegeneration could be caused by the presence of multiple causative agents in combination with BMAA, such as metals and pesticides.

According to the reporting on the internal documents, the effects of blue-green algae toxicity on water consumption and shellfish was the leading theory among the researchers for what was causing the cluster.²

Since then, others in New Brunswick have raised the possibility that glyphosate, the active ingredient in the most heavily used herbicide in the world, is behind the rapid neurological degeneration. Despite the facts that the product is now considered a probable carcinogen, is able to cross the blood-brain barrier, and is at the centre of roughly 165,000 legal claims—including a class-action lawsuit in Ontario— it continues to be used in astonishing quantities in both forestry (Vision) and agriculture (Roundup).

According to an open letter reported by the New Brunswick Media Coop, many of the patients presenting with the mystery illness had high urine levels of glyphosate and other pesticides.

But, despite the promising momentum of the federal/ provincial collaboration, and its search for a potential environmental trigger, it all “came to a grinding halt” on May 6, 2021 when New Brunswick cancelled all further meetings between federal and provincial researchers, and never re-scheduled them.

The province decided it was going to take the lead with its own truncated version of an investigation—a subject we’ll return to—and in early 2022, it came to a rather startling conclusion: there is no cluster, there is no neurological syndrome of unknown cause, and no such syndrome exists.

According to the MLAs’ recent joint press release, the federal-provincial investigation needs to be re-opened: “The data must be collected, environmental testing must be done, and answers must be found for these patients, their families, and our communities,” says New Brunswick MLA Megan Mitton.

Cumberland-North MLA Smith-McCrossin says she contacted Dr. Robert Strang, Nova Scotia’s Chief Medical Officer of Health urging him to “engage” with his counterpart in New Brunswick. Smith-McCrossin says patients from her riding who require neurological referrals are typically seen by specialists in Moncton, and that because they are being seen in another province, the issue might not be on Strang’s radar. “[He] may not fully grasp the extent of this atypical illness affecting Nova Scotians,” says the release.

I spoke to Smith-McCrossin this week, while she was in break at the NS Legislature. She says she has constituents coming to see her that have “this atypical neurodegenerative-like illness with no definitive diagnosis.”

“My concern is that this has mainly been focused on New Brunswick and we may not have a full picture of the severity of this.”

Smith-McCrossin says the normal referral pattern in Cumberland County for neurology is to send people to the neurologist in Moncton, and “our Department of Public Health may not be fully aware of the number of people that may be experiencing this.”

When asked about numbers, Smith-McCrossin wouldn’t disclose numbers for “privacy reasons,” but she says, “the fact that I've had some people come to me, it makes me question, what is the severity, what is the incidence?”

Smith-McCrossin sent a request to Dr. Robert Strang about a week and a half ago, and is still waiting to hear back.

According to CANADALAND reporting on this, federal meeting minutes from May 2021 indicate there was at least one referral to the cluster from Nova Scotia at that time, noting, “it does not appear to have originated in New Brunswick, as the patient has not been a past resident of [that province].” According to the minutes obtained, patients had also been referred from other provinces, including Quebec, Newfoundland, Saskatchewan and Alberta and some “had previously lived or spent a significant amount of time in New Brunswick.” By February 2022, patients had also been identified in P.E.I and Ontario, as well as one Canadian living in Australia.*

Dr. Alier Marrero, the referring neurologist for the vast majority of the affected patients—said he’s seen “more than 320 patients in six provinces with atypical neurological symptoms.”

Background

In early 2020, three neurologists in New Brunswick had identified several patients aged 17-80, presenting with an “unusual combination” of neurological symptoms, including dementia, severe pain syndrome, and brain and muscle atrophy.

The cases had actually been accumulating since 2015, when the first one was referred to the Creutzfeldt-Jacob Disease Surveillance System (CJDSS), a nation-wide monitoring system part of the Public Health Agency of Canada. By 2019, 12 more cases had emerged, in 2020 there were 25 more, and in 2021 another ten.

Overall, forty-six of the 48 cases identified were referred by Dr. Alier Marrero, and the rest by two other neurologists.³

All the patients had eventually tested negative for CJD, leading to a determination that they might instead form a “cluster” with “unknown etiology” or cause.⁴

The “cluster” of cases, determined by the geographical location of the main residence at the time of reported symptom onset, was originally determined to be primarily in the Moncton and Acadian Peninsula regions of the province, indicating a “common source exposure.” That is, “most of the interviewed cases were living in areas in southeastern New Brunswick around Moncton area (65%), followed by the northeastern region around the Acadian Peninsula (23.5%).⁵

In early 2021, members of the CJD surveillance team and Marrero agreed on a “case definition” for the unknown illness – that is, the clinical signs and symptoms being exhibited by the patients. The definition was accepted by NB Public Health. By this point, efforts to get to the bottom of it started to coalesce: a multidisciplinary team from federal and provincial agencies as well as academia came together with NB Public Health leading the investigation, and, as previously stated, by March a memo was sent to NB physicians advising them of the cluster. An alert that goes out to public health officials across Canada was also posted on the Canadian Network for Public Health Intelligence.

In April 2021, the Moncton Interdisciplinary Neurodegenerative Diseases (MIND) Clinic opened with funding by the NB Department of Health and the Horizon Health Network and Marrero was the clinic’s neurologist.⁶ A few months later, the province issued a memo to the province’s health-care professionals advising them to send all the patients presenting with the case definition directly to the MIND clinic, and that new referrals to the cluster now require “sign-off” by two specialists.

In an excellent piece published in The Walrus, Matthew Halliday describes what happened next:

As with most matters related to health, outbreak response in Canada falls under provincial jurisdiction, but in this case, New Brunswick asked the [Public Health Agency of Canada] PHAC for help. Federal colleagues began assembling a nationwide working group, which eventually numbered about two dozen. It included Michael Coulthart, head of the CJDSS, as well as Neil Cashman, a University of British Columbia neurologist, and Strong. Across the country, consultations began with experts in prion disease, environmental neurotoxins, and food- and water-borne illness. According to documents obtained by a freedom of information request, the CIHR [Canadian Institutes of Health Research] and the PHAC were meeting weekly, and a clinic was being put together in Moncton as a clearing house for patients, which would be partly headed by Marrero. By then, a posting on the Program for Monitoring Emerging Diseases—a global outbreak-monitoring system that publicized the first cases of SARS and Ebola—had brought the illness to global attention. Experts from Johns Hopkins University, the Mayo Clinic, and the Cleveland Clinic reached out. As rapidly as the cluster had appeared, so did the expertise to combat it. Then, on June 3, New Brunswick abruptly changed tack. The province told the emerging national working group to stand down.

In place of what appeared to be a formidable federal collaboration, the province devised its own in-house probe: it conducted a telephone questionnaire to gather information about those included in the original cluster, and it created an oversight committee, tasked to rule out other potential diagnoses. 

It should also be noted that by the spring of 2021 the province had stopped adding patients to the cluster, and had capped the number at 48, despite there being more presenting with the unexplained syndrome.

For the questionnaire, thirty-four of the 48 affected individuals or someone representing them were interviewed over the phone and the results of these 4-hour long “enhanced surveillance interviews” were reported in an epidemiological summary, published in the fall of 2021.⁷

“The questionnaire included both closed and open-ended items that focused on topics related to demographics, food exposures, environmental exposures, occupational and work details, recreational exposures, family and close contact exposures, and additional information that participants felt might be relevant to their symptoms.”

According to the summary, some of the most common identified exposures in the cluster were the consumption of lobster, wild berries, or moose meat. The summary says these are “typical of the region, which is strongly influenced by Maritime cultural practices.”

Despite the fact that the report is clear in stating that its results “represent a first step [italics mine] in identifying potential commonalities between members of the cluster,” it instead went on to draw the following conclusion:

“There is no evidence from this report that a particular food… is linked to the development of neurological symptoms, as more cases would have been identified within New Brunswick and in neighbouring provinces or states.”

The summary went on to say, “there are no specific behaviours, foods, or environmental exposures that can be identified as potential risk factors with regard to this cluster of cases with a potential neurological syndrome of unknown cause. As such, residents should feel confident that they are not considered to be at risk of any food or environmental exposures within the province.”

This seems to me an obviously bizarre statement to make. As the summary itself states, the survey could only have “identified potential commonalities,” and had no way of drawing any conclusions about whether they might be linked to neurological decline without further study. The patients had not undergone any systematic biological testing – blood, urine, fat biopsies – to exclude the possibility of an exposure, through diet or otherwise, to a contaminant or pesticide, for instance.

This is not to say there has been an environmental exposure of some kind, but this “epidemiological summary” does nothing to rule out one.  

James Paddle, a medical student living in Moncton, said he authored and circulated the letter after witnessing first-hand the suffering of patients with severe neurological symptoms, some of them teenagers. Screen grab from video posted by New Brunswick Media Co-op.

As previously mentioned, by the summer of 2021, in lieu of collaborating with the feds, the province created its own “oversight committee” composed of six provincially appointed neurologists. According to Halliday’s reporting, none “appear to possess epidemiological experience in neuropathology—skills essential for investigating a cluster of this complexity.” A provincial news release stated that the committee’s mandate was to “provide second opinions” on the files of affected patients in order to “ensure due diligence and rule out other potential causes.”

In February 2022, the oversight committee released its findings and noted, with an astonishing lack of irony, that “the lack of a defined federal/ provincial process for the identification and investigation of a novel disease or condition has caused much speculation and public distrust.”

But it also concluded that “the individuals who were included in this cluster do not represent a neurological syndrome of unknown cause,” and that “no such syndrome exists.”

“No individual met the case definition in full and many were found to have other, more probable diagnoses.”

For the current New Brunswick government, the case appears to be closed, but an upcoming provincial election might change that, as Liberal leader Susan Holt promises to re-engage with the feds and open up the investigation if elected.

The Quaking Swamp Journal will be following this story.

 

Postscript: For this piece I contacted the NS Department of Health and Wellness to find out if the province has any way to capture the cases that may be occurring here, particularly if the patients are being treated in New Brunswick. I also asked if Dr. Strang was aware of any cases of this “neurological syndrome of unknown cause” here in Nova Scotia. I did not receive a response in time for publication, but did receive this response on October 2nd.

According to Dr. Robert Strang, Chief Medical Officer of Health for Nova Scotia:

“Our public health teams have a long-standing surveillance system to detect communicable and emerging diseases. Both clinicians and laboratories report into the system. I have been in touch with my counterpart in New Brunswick, and New Brunswick is looking into this reported situation. Our teams will continue to stay connected.”

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1. CJD is a neuro-degenerative disease that’s part of the family of Transmissible Spongiform Encephalopathies (TSE). These diseases incubate silently, some say for up to 30 years, kill every victim they infect and are indestructible. Neurologist Stanley Prusiner was the first to hypothesize in 1995 that a rogue protein called a prion (short for proteinateous infectious particle) was responsible for the transmission of spongifying diseases by converting normal proteins into dangerous ones by inducing them to change their shape. The virtually indestructible prion is responsible for a number of SEs including scrapie (sheep), mad cow disease, and similar brain wasting diseases in mink, deer, elk, and even cats. The disease is not contagious, but can be spread by eating infected tissue, and hence the shock waves that were felt back in March of 1996 when it was discovered in the UK that there was a link between “mad cow disease” or bovine spongiform encephalopathy (BSE) and a strain of the human CJD. The disease was being spread among livestock through the feed: waste ruminant tissues were being fed to live ruminants. But BSE can only be transmitted to humans through eating cow brain, spinal cord, the retina of the eye or the small intestine, or if these tissues contaminate “high risk” tissues like muscle, through the slaughtering process. In 1997, Canada banned most mammalian proteins from ruminant feed. Exceptions are that pig and horse meat can still be used in ruminant feed. However, pigs are known to get spongiform disease.

2. CANADALAND did some excellent investigative reporting on this story, based on thousands of pages of internal documents obtained through Access to Information requests with the help of the non-governmental organization BloodWatch.

3. From “Investigation into a Neurological Syndrome of Unknown Cause in New Brunswick.”

4. See Timeline provided by NB Public Health. You can find all the documents associated with the province’s investigation here.

5. From the Epidemiological Summary, p. 14.

6. By August 2022, Marrero was no longer with Moncton Hospital’s MIND clinic. According to this news report, his patients had the option of following him to Moncton’s Dr. Georges-L-Dumont University Hospital Centre for continuing care at his primary practice.

7. According to the Epidemiological Summary, the questionnaire was administered between May and September 2021, “and the responses were analyzed using a comprehensive approach involving quantitative and qualitative methods. The findings presented in the report are based solely on these responses and represent a first step in identifying potential commonalities between members of the cluster.”