'The answer cannot be nothing': The battle over Canada's mystery brain disease
12 hours ago Share Save Joel Gunter , senior international reporter, New Brunswick and Nadine Yousif , senior Canada reporter, New Brunswick Share Save
BBC
Five hundred people in a small Canadian province were diagnosed with a mystery brain disease. What would it mean for the patients if the disease was never real?
In early 2019, officials at a hospital in the small Canadian province of New Brunswick noticed that two patients had contracted an extremely rare brain condition known as Creutzfeldt-Jakob Disease, or CJD. CJD is both fatal and potentially contagious, so a group of experts was quickly assembled to investigate. Fortunately for New Brunswick, the disease didn't spread. But the story didn't end there. In fact, it was just beginning. Among the experts was Alier Marrero, a soft-spoken, Cuban-born neurologist who had been working in the province for about six years. Marrero would share some worrying information with the other members of the group. He had been seeing patients with unexplained CJD-like symptoms for several years, he said, including young people who showed signs of a rapidly progressing dementia. The number of cases was already more than 20, Marrero said, and several patients had already died. Because of the apparent similarity to CJD, Marrero had been reporting these cases to Canada's Creutzfeldt-Jakob Disease Surveillance System, or CJDSS. But the results had been coming back negative. Marrero was stumped. More worrying still, he was seeing a dizzying array of symptoms among the patients, according to his notes. There were cases of dementia, weight loss, unsteadiness, jerking movements and facial twitches. There were patients with spasms, visions, limb pain, muscle atrophy, dry skin and hair loss. Many said they were suffering with both insomnia and waking hallucinations. Patients reported excessive sweating and excessive drooling. Several exhibited Capgras Delusion, which causes someone to believe that a person close to them has been replaced by an identical-looking imposter. Others appeared to lose the ability to speak. One patient would report that she had forgotten how to write the letter Q. Marrero ordered test after test. But he was at a loss. "I just kept seeing new patients, I kept documenting new cases, and I kept seeing new people dying," he recalled. "And an image of a cluster became more clear."
Many of the cluster patients believe industrial poisoning has affected New Brunswick's environment. (Chris Donovan/BBC)
Over the coming months, Marrero and the CJDSS scientists began to suspect that instead of a small cluster of CJD patients, the province of New Brunswick might have on its hands a much larger cluster of people suffering from a completely unknown brain disease. Over the next five years, Marrero's cluster would balloon from 20 to an astonishing 500. But there came no scientific breakthrough, no new understanding of neurology, no expensive new treatments. Instead, last year, a bombshell research paper authored by several Canadian neurologists and neuroscientists concluded that there was in fact no mystery disease, and that the patients had all likely suffered from previously known neurological, medical, or psychiatric conditions. The New Brunswick cluster was, one of the paper's authors told the BBC, a "house of cards". To report this story, the BBC spent time with Marrero and spoke to a dozen of his patients or their relatives — some of whom are telling their story for the first time — as well as key scientists, experts, and government officials, and reviewed hundreds of pages of internal emails and documents obtained by freedom of information requests. We can reveal that at least one cluster patient has now opted for death via medical assistance in dying — legal in Canada since 2016. The diagnosis on the death certificate, according to the doctor who signed it off, was "degenerative neurological condition of unknown cause". At least one other cluster patient is currently considering assisted dying. The research paper published last year could have marked the end of a strange chapter in Canadian science. Except, hundreds of the patients disagree. Defiant, fiercely loyal to Marrero, and backed by passionate patient advocates, they argue that the paper is flawed and reject any notion that the cluster might not be real. Many believe instead that they have been poisoned by an industrial environmental toxin, and that the government of New Brunswick has conspired against them to cover it up. "I'm not a conspiracy theorist type person, at all, but I honestly think it's financially motivated," said Jillian Lucas, one of the patients. "There's all these different levels."
Lucas first met Marrero back in early 2020, after her stepfather, Derek Cuthbertson, an accountant and military veteran, began experiencing cognitive and behavioural problems including sudden rage and loss of empathy. He was referred to Marrero, who ordered a battery of tests but was unable to explain his symptoms. Cuthbertson became one of the early cluster patients — the so-called "original 48". Lucas had just gone through a divorce and suffered a bad concussion, and she moved back in with her mother and Cuthbertson in their rural community near the city of Moncton. Soon she began experiencing her own symptoms and went to see Marrero for herself. "He ran so many tests, so much blood work and scans and spinal taps," Lucas recalled. "We were trying to rule absolutely everything out and we just kept coming up with more questions." Short on answers, Marrero added Lucas to the cluster. Over the coming months, her symptoms worsened and new symptoms appeared. She experienced light sensitivity, tremors, terrible migraines and issues with her memory and ability to speak clearly, she said. She felt unexplained stabbing pains. Cold water felt scalding hot.
Jillian Lucas and her stepfather Derek Cuthbertson. Both were diagnosed by Marrero with the mystery illness. (Chris Donovan/BBC)
Marrero, though, was attentive and caring. He took her symptoms seriously. "He made me feel seen and that what I was experiencing was important," Lucas wrote in a Facebook post about her struggle. It was a sentiment that seemed to be shared by everyone who saw Marrero. He held their hands during appointments. He remembered them, cried with them. He was "the only one listening to them," said Lori-Ann Roness, one of the cluster patients. "He's an incredible human and physician," said Melissa Nicholson, whose mother died last year after being diagnosed with the mystery disease. "Watching our mom go through it was hard enough," Nicholson said. "But he was such a pillar of support." In March 2021, with Canada still in the grip of the Covid pandemic, the cluster suddenly became news. New Brunswick's chief medical officer had sent a memo to doctors alerting them to the apparent syndrome and suggesting they contact Marrero with possible cases. The memo leaked and the story hit the papers. Marrero found himself inundated with new patients. But he was also drawing support from the highest levels of Canadian science. The working group set up to respond to the original CJD cases had evolved into a multi-disciplinary group studying the cluster, and the possibility of a mysterious new neurological condition seemed, at times, irresistible to the scientists. "It's like reading a movie script," emailed one researcher to colleagues, about an early story in the Toronto Star. "We are all in the movie!", a senior federal scientist replied.
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