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. “It is literally something heartbreaking,” Marrero says, “because you need to provide answers, and every time you go back, it’s ‘Okay, you don’t have multiple sclerosis, you don’t have Parkinson’s disease, you don’t respond to therapy.’”

Because they ticked so many boxes associated with CJD, each case was reported to the Ottawa-based Creutzfeldt-Jakob Disease Surveillance System. The CJDSS was set up, in 1998, by the Public Health Agency of Canada (PHAC) in the aftermath of the UK’s outbreak of “variant” CJD—more commonly known as mad cow disease—caused by prion-tainted beef, which ultimately killed at least 178 people.

Most CJD, however, is of the so-called sporadic variety, in which prions misfold without warning in otherwise healthy individuals. It is incredibly rare, striking about one or two people per million. (Canada saw sixty-five CJD cases last year, a typical annual number.) The volume of referrals coming from a province as small as New Brunswick was surprising and led to a deeper look. Some patients did turn out to have CJD, and several more received diagnoses of other established brain conditions. But the majority, totalling forty cases mostly between 2017 and early 2021, stumped Marrero and his federal colleagues, some of the country’s leading experts on neurodegenerative diseases. In the twenty-year history of the CJDSS, such a large group of patients in one small area—afflicted with a CJD-like disorder seemingly impervious to diagnosis—was unprecedented. According to one senior scientist in the federal public health infrastructure, “Nothing like this phenomenon has ever been seen before.”

In late 2019 and early 2020, as Marrero was working with Cormier and other patients, he and PHAC scientists came to believe that they may be looking at a distinct, previously undescribed neurological disease. They took the extraordinary step of grouping the undiagnosed cases together as the Cluster of Progressive Neurological Symptoms of Unknown Etiology in New Brunswick. On March 5, the province’s deputy chief medical officer of health sent a memo to provincial physicians, urging them to watch for symptoms.

The cluster came to public attention that month, when the CBC reported on the memo. On March 18, New Brunswick’s chief medical officer of health, Jennifer Russell, told reporters it was too early to venture a cause. With nowhere certain to settle, speculation found any perch: seafood contamination, old mine operations, well water, wild game, industrial herbicides, even pollution from a shuttered lead smelter in northern New Brunswick. By this time, the case count had risen to forty-three—eight in and around Moncton and thirty-five on the Acadian Peninsula, a rural francophone region of fewer than 60,000 people in the province’s northeast, which appeared to be the cluster’s hub.

In April, Michael Strong, a neuropathologist and the president of the Canadian Institutes of Health Research (CIHR), told the Globe and Mail that the medical mystery demanded “boots on the ground”—epidemiological investigators conducting in-person field research. And, later that spring, a concerted response started to coalesce.

As with most matters related to health, outbreak response in Canada falls under provincial jurisdiction, but in this case, New Brunswick asked the 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 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. The investigation “was pulled up to the highest levels of the New Brunswick government, and they took control,” says the senior scientist, who is intimately familiar with the workings of the PHAC investigation and has asked for anonymity, claiming federal scientists have been “muzzled” by federal health authorities at the request of the province. Cashman declined to speak for this story, indicating that he needed clearance from the New Brunswick government. Strong was permitted by the CIHR to speak only if the conversation avoided New Brunswick and instead focused on cluster epidemiology in general.

The New Brunswick government didn’t announce its suspension of the federal collaboration at the time. Instead, what the province has done is create its own oversight committee composed of six provincially appointed neurologists, none of whom appear to possess epidemiological experience in neuropathology—skills essential for investigating a cluster of this complexity. The committee mandate, according to a June 3 news release, is to “provide second opinions” on the files of affected patients in order to “ensure due diligence and rule out other potential causes.”

New Brunswick has a long history of withholding information from its citizens.

In other words, rather than collaborating with the country’s top experts in a methodical, robustly funded investigation aimed at digging into potential causes, the province has put its modest resources toward relitigating the question already addressed by PHAC scientists: whether this is a true disease cluster, linked by a common cause. Since June, a pall of secrecy has descended over the committee’s work, and federal collaborators have been left largely in the dark. Right before the province unilaterally suspended its relationship with the PHAC, forty-eight cases were being investigated with thirty-nine confirmed—six of which had proven fatal. As of this writing, the provincial government hasn’t issued any updates on current patients or provided information about additional cases under investigation. (I made multiple requests to speak to the province’s chief medical officer of health as well as its health minister. Neither was made available.)

This isn’t necessarily unexpected. New Brunswick’s provincial elite, in both government and business, has a long history of withholding information from its citizens. But some of the highly regarded epidemiologists and neurologists I spoke to for this story agree that, whatever the province’s motivation, the cluster isn’t being tackled with the urgency it deserves. “We have an unbelievably capable set of tools to look at biological and epidemiological and environmental characteristics,” says the senior scientist of the expertise being inexplicably sidelined. “It’s amazing, the potential that is not being tapped.” By spurning that help, New Brunswick appears to be abdicating its responsibility to the sick, to the public, and to medical science itself.

Neurologists and epidemiologists have long flocked to unusual disease clusters, drawn by the prospect of discoveries that can shed light on afflictions affecting millions of people, such as Alzheimer’s or ALS. We know the devastating effects of these illnesses on the brain. But, as common as they are, they remain largely mysterious: we don’t know why they develop or how to prevent or reverse their course. “Hyperendemic foci,” as these clusters are called, promise something like real-world laboratories stocked with natural controls: a lot of sick people living in a similar environment, eating similar food, often with similar genetic backgrounds. Researchers hope to find causal patterns that may otherwise elude them.

For neurologists, the granddaddy of all hyperendemic foci—the most researched and among the longest lasting—occurred on Guam. Since at least the early twentieth century and probably long before, the Indigenous Chamorro people of the western Pacific island had been stricken, at extraordinarily high rates, by a neurodegenerative disease called lytico-bodig. (At one point, it accounted for 10 percent of adult deaths.) The mystery of lytico-bodig has never been solved, but other clusters have been cracked. In the 1960s, when American researcher Carleton Gajdusek went chasing reports of a fatal neurological illness among Papua New Guinea’s Fore people, he found a disease very much like CJD, called Kuru. It turned out to be transmitted by funerary cannibalism: rites of mourning that involve consuming the dead.

Some clusters have persisted for centuries, such as Muro disease, an ALS-like condition afflicting residents of Japan’s Kii Peninsula. Others are shorter lived, such as an outbreak of Progressive Supranuclear Palsy in northern France in the early 2000s, which was suspected to be linked to heavy-metal contamination. But most, even those that remain mysteries, have at least led to new knowledge and hypotheses about the function and dysfunction of our most complex, least understood organ—especially with regards to how the brain responds to environmental factors.

That potential is also present, says Marrero, in New Brunswick. “We might actually have the possibility in Canada, with all our experts contributing, to better understand other conditions.” Critical to developing that understanding, says Ralph Garruto, a former researcher with the US National Institutes of Health, is “shoe-leather epidemiology,” in which investigators spend time in communities, gathering detailed information through careful exploratory work with patients. Garruto himself has spent years in the field, examining disease clusters in the US, Ukraine, and the western Pacific—including Guam. There, he says, community engagement led to discoveries that would have been impossible remotely. A casual conversation uncovered new leads, someone’s stray limp became a diagnosed case, and so on. “You have to be on the ground to assess what’s happening,” says Garruto. “You have to move from one area to another, talk to people extensively and ferret out any cases which may not come to clinical attention.”

New Brunswick, by contrast, is conducting its investigation behind closed doors, at a remove from the patients it’s trying to better understand, and at what might be called a leisurely pace. According to Édouard Hendriks, one of the original two co-chairs of the provincial oversight committee, members are putting in “a few hours per week.” (Hendriks retired in August.)

The purpose of the internal review is to test for what scientists call a null hypothesis: the possibility that the PHAC’s experts mistook dozens of difficult-to-diagnose cases as representing a pattern that doesn’t exist—the possibility that each case is a one-off. The committee, says Hendriks, is focusing on reviewing the cases with an open mind. “Perhaps what we think is unknown at this point in time, in six months, one year, depending on the evolution of the patient, we’ll be able to see this is purely Alzheimer’s, this is purely this or that, and so forth.”

It’s true that medical history is littered with unexplained disease clusters that turn out to be statistical blips mistakenly identified by overzealous investigators. But others have entered the annals of medical lore and literature. Experts I’ve spoken to believe that what is happening in the province is too big to be explained away as a statistical illusion—that the existing data simply doesn’t lend itself to misreading. One example is the number of cases contrasted with total population. The Acadian Peninsula has seen at least thirty-five cases. That’s an extremely high prevalence for an undiagnosable neurodegenerative condition. That the syndrome is affecting people in their twenties and thirties is another factor, argues Marrero, resembling clusters like those in Papua New Guinea or Guam.

One important part of the investigation is in place: a questionnaire, which the PHAC helped design before it was cut off. It focuses on patients’ environments, job histories, travel habits, diets, etc. It was meant as a starting point, establishing common links between those affected—but, the senior scientist cautions, it’s “not highly sophisticated.” It not only appears to be behind schedule but is being conducted in secret, with some family members required to sign non-disclosure agreements before participating. It’s also being carried out by telephone rather than in person. Garruto calls that an amateur mistake, suggesting a “low-budget situation.” (According to Hendriks, COVID-19 protocols haven’t been a factor in limiting the investigation.)

“You cannot solve this with questionnaires,” says Hermann Schaetzl, a Calgary-based expert in animal prion diseases who was consulted last year by the PHAC on the then burgeoning investigation. Months ago, he anticipated a research call centred on New Brunswick, bringing together experts in multiple fields. No such thing emerged.

Another aspect of fieldwork not underway is environmental testing for neurotoxins in food, water, and soil. “If we’re going to do assays on the food web,” says Susan Murch, a professor of chemistry at the University of British Columbia, “then we need to do it before people get sick, not after.”

Murch was contacted by federal authorities this spring as a possible participant in the investigation. Her expertise lies in certain amino acids that are theorized to kick-start neurodegeneration. She was instrumental in suggesting a possible link between lytico-bodig (the Guam disease) and an amino acid called BMAA, which is present in the seeds of the island’s cycad trees. BMAA can also accumulate in seafood and develops in the kind of blue-green algae blooms that are increasingly common in New Brunswick’s lakes and rivers. (It’s been tentatively linked to elevated levels of ALS in the United States.) Murch’s lab at UBC’s Okanagan campus is the only one in the country capable of testing for BMAA in human tissues. Initial discussion with the PHAC fell silent, however, after New Brunswick asked its federal counterparts to stand aside.

Maybe the most crucial aspect of this kind of investigation, according to experts, is open communication. This is a cornerstone of the approach recommended by the US Centers for Disease Control and Prevention in its step-by-step guide to outbreak investigations, which advocates transparency with media and regular information sharing with colleagues and the public on ongoing cases. This, too, is not happening. The province may even have fallen silent on news of additional deaths. On May 8, seventy-seven-year-old Sylvia Curtis—who, according to her daughter, was a confirmed case under Marrero’s care—died after a swift three-month decline. If the syndrome is responsible, she would be the seventh death. The official number, however, still stands at six.

The total case number has also stalled, at forty-eight, though this, too, may change. “I’ve been required by public health not to update anything until we finish this initial investigation, because we have not diagnosed everybody yet,” Marrero told me. But, “in the clinic right now, we have over 100 patients in different stages of investigation.”

Photograph of Alier Marrero at his home in Allison, NB.
Alier Marrero at his home in Allison, NB.

In the absence of information, rumours and anger have spread. Steve Ellis has had a front-row seat to much of it. On a Sunday night in June 2019, his sixty-one-year-old father, Roger, collapsed with a seizure. Roger spent the next six weeks in hospital. Within the first week, his muscles began to atrophy, his personality turned inexplicably aggressive, his balance faltered, and his memory became hazy. As with Cormier, Roger’s CJD test came back negative, and he was then transferred to Moncton’s Dr. Georges-L.-Dumont University Hospital Centre. Test after test turned up nothing. By the end of September, he had lost sixty pounds and descended into a dense cognitive fog. Roger’s decline was a rapid, catastrophic cascade from health to infirmity in less than four months. In June 2020, his case was counted as part of the New Brunswick cluster, and today he lives in long-term care.

As his father has deteriorated, thirty-nine-year-old Ellis has become an advocate for patients and families living with the condition. Last March, when the cluster was publicly announced, he made a Facebook page, hoping to find other affected families. It’s since become a beacon for connection and commiseration—as well as grievance, frustration, and a fair amount of speculation.

Ellis tries to keep the page out of tinfoil-hat territory. (Everything from vaccines to electromagnetic radiation has cropped up.) But he empathizes with the urge to find answers, particularly as his father’s health worsens and his frequent missives to government, when acknowledged at all, yield vague responses. “The less the government says and the longer it goes on,” he says, “the more it looks like they’re hiding something.”

Lest that sound like conspiracy mongering, note that New Brunswick has long exhibited a penchant for paternalism and secrecy on matters of public health, and there’s no shortage of high-profile examples. For years, Parlee Beach—a bustling summertime attraction near the tourist town of Shediac—was plagued by sewage dumping and high E. coli counts. The province eventually devised a unique water-testing system that undercounted fecal contamination. A 2017 CBC investigation revealed that the system was largely intended to ensure that the beach, and the Shediac-area economy, remained open and humming. In 2019, a Legionnaires’ disease outbreak in Moncton sickened sixteen people and killed one. For sixteen months, health authorities refused to disclose its source, until another CBC investigation traced the outbreak to bacteria in a cooling tower at a cannabis plant owned by Organigram—a company that has received substantial government funding and supplies the government-owned Cannabis NB.

Maybe the most notorious recent example is the firing of the province’s chief medical officer of health, Eilish Cleary, in 2015. At the time, Cleary was researching the human-health effects of glyphosate, a powerful herbicide (and potential carcinogen, according to the International Agency for Research on Cancer) used in abundance by NB Power and J.D. Irving, the forestry arm of the wealthy Irving family business. No reason was given for Cleary’s firing, but environmental advocates and opposition politicians have suggested corporate interference. In 2016, Cleary received a $720,000 severance package in exchange for signing a nondisclosure agreement. Speaking to the CBC in 2017, Mario Levesque, a political science professor at Mount Allison University, called the payment “hush money.”

Kat Lanteigne, executive director of BloodWatch, a Toronto-based group that advocates for a safe blood-donation system in Canada, is concerned that a large pool of possibly contaminated people in an area will lessen the number of healthy donors available to give blood. She’s also from the Acadian Peninsula and suspects that the province’s ousting of national collaborators is in keeping with a longer tradition of shutting down chatter that could be politically or economically damaging. “I think what this points to is that classic nannyism of the government,” she says, “putting profit and industry before public safety.”

New Brunswick’s northeast, in particular, where the majority of cases have been identified, is already struggling economically. Dark whispers about local food, water, or industry are not in anyone’s financial interest. But that’s the very reason we have a federal health minister, Lanteigne argues. “You need the lever to be from the federal agency, with the appointed leader from New Brunswick, and they all sit at a command table with a patient representative for the public,” she says. “Otherwise, this stuff happens in a vacuum.”

Hendriks, former co-chair of the provincial oversight committee, reassures me that the group was created, in part, to counter runaway speculation. “I think we’re trying to calm the anxiety of people by saying, ‘We’re going to look into this and say, No, it’s not related to, I don’t know, the lobster in New Brunswick or anything specific.’ Our hope is probably that, over time, we will realize it’s part of the usual neurodegenerative pattern of all the diseases we see.”

Hendriks is referring to the null hypothesis, which remains a possibility. It’s also possible that, just as happened on Guam, an environmental trigger may elude detection no matter how much expertise is—or isn’t—brought to bear. One out-of-province researcher, who was consulted as a potential collaborator on the suspended federal investigation (and requested anonymity, not wishing to compromise their reinvolvement should the federal-provincial collaboration resume), believes the province may already have an endgame in mind. “I think what might happen is they’ll get to the end and announce that they have failed to find a clear diagnostic feature, or a link between the cases, so they don’t really have problem.”

Whatever the results, the secretive, expertise-eschewing nature of the process will for many be enough to cast doubt on its conclusions and leave open the question of political interference. Even if the province decides to reopen communication with its federal counterparts, months have now been lost, during which some of the country’s—and perhaps the world’s—most knowledgeable experts could have been trying to solve the deadly riddle.

In the meantime, New Brunswickers, especially those in the small communities dotting the province’s far north, have been answered with silence. Few likely struggle with the uncertainty more than those who have to contend with the mysterious condition. “I still have hope that there will be a cure or a treatment or answers,” Gabrielle Cormier said in late August. Her speech had become halting, and she needed time to collect her thoughts before each answer. “But I’m still realistic—it may not go that way.”

Matthew Halliday
Matthew Halliday is a writer and editor in Halifax. He has written for the Globe and Mail, Hakai Magazine, and Chatelaine.

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