[00:00:00] Jordan Heath-Rawlings: The world’s top doctors and top researchers and top scientists have been somewhat preoccupied over the past 18 months, and that is understandable. But as the third wave of COVID recedes, at least in Canada, and vaccinations take hold, some very smart people will soon have more time to investigate something very scary.
News Clip: Public health officials in Canada are closely monitoring a mysterious brain disease that has emerged out of New Brunswick. Now it’s not known what is causing the disorder or where it even came from. In a cluster of more than 40 cases have already been confirmed in the Atlantic province, five people sadly have died.
Jordan Heath-Rawlings: Thus far, whatever is happening in New Brunswick is really hard to explain, to identify, to treat, or to prevent. The numbers right now may be small, the cases may be localized, but the symptoms are devastating and [00:01:00] can be fatal. So what do we know about this brain disease? How much do we still need to understand? How concerned should we be right now? And how has COVID-19 impacted our ability to get the answers we need on medical mysteries like this one quickly? Because if there’s one thing we should have learned from recent history, it’s probably not to wait and see about stuff like this.
I’m Jordan Heath-Rawlings, this is The Big Story. Amanda Coletta covers Canada for The Washington Post. Hey Amanda.
Amanda Coletta: Hi Jordan.
Jordan Heath-Rawlings: Can you begin maybe by telling me about Alier Marrero and what he does?
Amanda Coletta: Sure. So Dr. Alier Marrero is a neurologist at a hospital in Moncton, New Brunswick, and [00:02:00] he is now sort of the lead person who is treating people in this cluster. Um, and also sort of leading the investigation into what is causing it.
Jordan Heath-Rawlings: When you say cluster, maybe back up a bit and tell us what’s happening out there. What are they looking at?
Amanda Coletta: So officials in New Brunswick and federal officials have identified what is being called the New Brunswick Cluster of Neurological Syndrome of Unknown Cause. Um, these are cases of a sort of mysterious neurological syndrome, and we can discuss sort of the symptoms that shortly. Um, but the cases are mostly in the Moncton area and the Acadian Peninsula, though officials haven’t identified to the public which specific towns or areas have cases. So far, there are 48 cases under investigation, six people have died, though [00:03:00] there are still some testing being done to determine sort of definitively whether this mysterious syndrome was the cause of those deaths. The youngest person in the cluster is 18, the oldest is 85. The cases are split evenly between men and women. The symptoms for most of the people in the cluster started in 2018, 2019 and 2020, but at least one case was identified sort of retrospectively last year, and that person sort of had symptoms well before that.
Jordan Heath-Rawlings: So, what are the symptoms of this neurological disorder?
Amanda Coletta: So, many of the symptoms are similar to those seen in patients with Creutzfeldt-Jakob disease, which we can also talk about in more detail. And in fact, it was a surveillance system that monitors for Creutzfeldt-Jakob disease that helped to detect the cluster in the first place.
Um, but in any [00:04:00] event, Dr. Marrero sort of outlined a constellation of symptoms, and he said that they were occurring in people who were previously healthy, highly functional, highly stable, um, and it usually begins with sort of atypical depression, irritability, and anxiety. Those patients then go on to develop sleeping disorders, and this can include insomnia that’s so severe that the patient stopped sleeping altogether or sleeps only a few nights a week even with medication. Others have no trouble falling asleep, the problem is waking them.
There’s trouble with memory, disorientation, uh, blurry vision, trouble with depth perception, coordination, balance. Many of the patients experience hallucinations of various forms, so there are auditory hallucinations where people hear, um, people or, or animals that aren’t present. There are tactile [00:05:00] hallucinations where people feel like they have insects crawling on them. And Dr. Marrero also noted that a number of the patients have these terrifying hallucinatory dreams that leave them afraid to sort of go to sleep to begin with.
Another symptom is these sort of uncontrollable and frequent muscle jerks. They’re so bad that sometimes the partner of the patient will have to sort of get out of bed at night and go sleep somewhere else. And what’s particularly troubling for Dr. Marrero is that he has also seen these jerks occur in people who are in palliative care, so. Really quite sick on quite, um, you know, high dosages of medication. Some of them are, they can’t talk, but they continue to have these, um, these jerks and he’s prescribed treatments to them that he might also use in patients with Parkinson’s disease, and they seem to have no effect. [00:06:00] There is muscle atrophy and weight loss that is both rapid and unexplained, dry skin and brittle nails, teeth chattering, some people lose entire clumps of hair.
Jordan Heath-Rawlings: Wow.
Amanda Coletta: Um, some patients also experience echolalia, which is, um, they repeat over and over the same words and phrases. So Dr. Marrero will ask them a question and the patients will repeat back to him that question over and over. And it’s quite frustrating for them because they don’t want to do this, but they can’t stop it.
Um, and there’s also another symptom that’s pretty devastating for the patients and their families, which is Capgras delusion. It’s, um, a false belief that loved ones or caregivers, people who are known to them, have been replaced by imposters. So some patients deteriorate really quickly. Others are stable for longer periods. It’s not clear to the doctors why this, why some are stable for longer.
Um, and I guess one thing I wanted to note is that, you know, Dr. Morrero sort of said, [00:07:00] the, the thing that’s really hard for the doctors and the patients and their families is that the pain isn’t just physical. There’s also these neuropsychiatric symptoms and they’re, you know, sort of tough to treat with medication. And it’s an awful syndrome for anyone regardless of their age. But you know, the youngest patient in this cluster is 18. And for some of the younger patients, it’s sort of beyond imagination.
Jordan Heath-Rawlings: Amanda, that is a terrifying list of symptoms. Like just one or two of those would be really scary.
Amanda Coletta: That’s right. And, um, Michael Strong, who is a neurologist who heads the Canadian Institutes for Health Research, he’s helping out with the investigation. Um, he said that, you know, if you look at the list of the symptoms and you pick three or four of them, you can say, okay, you know, these symptoms are common to X, Y, Z disease. But when you look at this whole list, the constellation of symptoms, that’s something that he hasn’t seen before.
[00:08:00] Jordan Heath-Rawlings: You mentioned earlier that it was similar to Creutzfeldt-Jakob disease. Do you want to just fill our listeners in on, you know, what that is and why those similarities pop up?
Amanda Coletta: Sure. So Creutzfeldt-Jakob disease is a rare brain disorder that is thought to occur around the world in humans and animals. And when we’re talking about humans, we’re talking about one, maybe two cases per 1 million people each year worldwide. And, um, CJD is the most common prion disease. And it’s sometimes used as an umbrella term to capture all prion diseases. Uh, prion diseases are rare, fatal rapidly degenerative brain disorders. So most people with CJD die within a year of the onset of symptoms.
Some other prion diseases that listeners might be familiar with are, um, Variant CJD, which, um, can be acquired by consuming meat that’s been infected with mad cow disease, for instance, or Chronic Wasting Disease, which you [00:09:00] sometimes see in deer and elk. And essentially prion diseases are caused by abnormal prion proteins in the brain that basically misfold. And then they accumulate and they infect healthy brain proteins, causing them to misfold and accumulate and affect other healthy brain, brain proteins. Um, and eventually sort of whole clusters of brain cells can be killed off. Under a microscope, um, if you look at the brain of a person or an animal with CJD, they kind of look like sponges with little holes in them.
There’s a bunch of causes. So some are subtypes of CJD are genetic, others occur more spontaneously, and some can be transmitted infectiously. So, you know, by consuming the meat of a, an animal that’s been infected, or if, um, someone has a brain surgery who has CJD and the tools aren’t or the instruments aren’t [00:10:00] sterilized properly, and then they’re used on another patient. Then that could be one way of transmitting CJD.
And the symptoms include sort of difficulty with balance and coordination, um, dizziness, vision problems, hallucinations, anxiety, trouble sleeping, depression, muscle spasms, memory loss, um, and the inability to speak. And most people in the sort of later stages can be bedridden as well.
Jordan Heath-Rawlings: And presumably, um, doctors have tested the patients suffering from this syndrome for CJD and it’s negative. And so where do they go after that? Do they just not know what this is? And how do you go about finding an explanation?
Amanda Coletta: So the doctors have, um, autopsied three brains of people who passed away in the cluster and doing a brain autopsy is sort of the best way to confirm a diagnosis of CJD or another prion disorder. And [00:11:00] so far those autopsies have shown no hint of CJD or another prion, another known prion disorder. But, um, Dr. Strong at the Canadian Institutes for Health Research said they’re still doing some molecular testing just to be able to rule out CJD and other known prion disorders 100% for good.
Um, and so now they’re really sort of trying to leave no stone unturned. There are a number of theories. Um, one of them is that this is an unknown or novel prion disorder. And then the other sort of grouping is that this is something that is caused by an environmental toxin.
Jordan Heath-Rawlings: You mentioned earlier when you described, uh, the people who are suffering from this, that it it’s a pretty wide array. Is there anything, um, that they have in common besides like a loose geographic, uh, connection?
Amanda Coletta: That’s one of the things that the investigators are [00:12:00] probing. So Dr. Strong said, you know, one of the things that they’ll look to do is try and identify three or four cardinal symptoms that are sort of common to everyone in the cluster, and then to go from there and see if there is, you know, if it tells you something about which part of the brain is most affected, and then that might tell you, uh, might lead you down one path over another. But at this point they’re sort of, kind of in the early stages of the investigation and everything is on the table.
Jordan Heath-Rawlings: This is maybe, uh, asking you to kind of use your own opinion a bit, but as you talk to doctors and scientists about this, like, are they freaked out by this? It sounds pretty freaky, especially, you know, when you use the term, maybe this is a novel, um, prion disease. Uh, we’ve heard about novel diseases recently and you know, it’s scary. How do they feel about it? Are they worried?
Amanda Coletta: I think they’re shocked that this is [00:13:00] something that is happening. Um, the, the cluster was detected by the CJD surveillance system, which is operated by the Public Health Agency of Canada, and Dr. Michael Coulthart, who heads the system has said that the system in his experience has never detected a cluster of CJD. So you can imagine how unusual it is for it to have picked up a cluster of something that is not CJD and that has no, a known cause.
Um, Dr. Marrero, you know, he said that he tries often to sound an optimistic note with family members and patients, um, because you know, he thinks hope is better than fear and he sort of said, look, around this time last year, what we were hearing from public health officials in Canada and around the world was that if there was going to be a safe and effective coronavirus vaccine, it would likely come sometime in the spring of [00:14:00] 2021. And now here we are in the spring of 2021, and not only do we have one safe and effective vaccine approved by Health Canada, we have several of them and we got them way earlier than we thought we would. So he sort of pointed at that as, as a reason for optimism.
And Dr. Coulthart, uh, from the CJD surveillance system said that since the existence of the cluster has been made public, there’s been a lot of, um, interest in reaching out from scientists and doctors from around the world. And so he’s hopeful that with more sort of eyes on it, they’ll be able to determine a cause, um, more quickly. At the same time, you know, doing the sort of investigations that you would need to do to determine if the cause is an environmental toxin is something that could take years.
Jordan Heath-Rawlings: Have those doctors developed any, uh, treatments for this at all? Um, do people ever [00:15:00] get better or do they just sort of continue to deteriorate? I know you’ve mentioned that several have died, but obviously, uh, dozens more have lived.
Amanda Coletta: There isn’t a cure because they don’t know what’s causing it. And so the best that they can do right now is sort of treat some of the symptoms. So if someone has a sleep disorder, there might be medications that they can prescribe to treat, uh, to treat that. So you’re really dealing sort of with the individual symptoms, um, w- which, you know, might bring some relief, but in some cases, the traditional treatments aren’t even working.
Jordan Heath-Rawlings: So far, we’ve been talking about doctors and scientists. Has this come on the radar of any level of government at all, and what have they said?
Amanda Coletta: Um, so the cluster’s existence, um, was noted in a memo from public health officials in New Brunswick to local physicians [00:16:00] in March, in early March of this year the public sort of learned about it. After that, after, um, Radio-Canada and the CBC obtained that memo, and the amount of information that the provincial government has provided about the cluster, um, hasn’t been very much, which has kind of frustrated some families. On the other hand, pu- what public health officials would say is we are telling you what we know, and right now we don’t know very much.
Um, but some family members have sort of complained that, you know, public health officials were supposed to get in touch with them several months ago to sort of, um, you know, conduct questionnaires and these types of interviews that you wouldn’t need to do to sort of get to the bottom of this. And so far there’s been radio silence. They have set up a website which gives, you know, basic details about the cluster. [00:17:00] Um, and they recently set up a clinic that will help to treat patients in the cluster and people who are maybe suspected of having this syndrome. Um, you know, this is all occurring in the middle of a pandemic, which has had some impact on just how quickly these investigations can take place.
Jordan Heath-Rawlings: That’s the next thing I was going to ask is just how many resources are there right now to throw at this? We’re talking about public health resources, which are obviously hugely in demand.
Amanda Coletta: Right, and New Brunswick is a relatively small province. So it, you know, it doesn’t have the same levels of public health resources as a province like Ontario, for instance. And we’ve seen how, how easily they can come under strain, uh, you know, during the pandemic. So every person that I spoke to outside of the government of New Brunswick, um, has basically said that the pandemic has impacted their work.
Um, and you know, it’s worth noting New Brunswick, like much [00:18:00] of Atlanta Canada has fared far better than many of the provinces to their west at containing the coronavirus. Um, and there’s a number of reasons for how it’s impacted, for why it’s impacting their work.
So if you think back to, you know, 2020, the early days and weeks and months of the, of the pandemic, many provinces and territories, including New Brunswick, um, temporarily put on hold or canceled a number of procedures, including diagnostic procedures like MRIs, um, x-rays, cat scans, and spinal taps. And, um, one of the ways that you can try and diagnosed, uh, CJD and other prion disorders while a person is alive is by testing their cerebrospinal fluid for elevated levels of certain protein markers. So you need to be able to do those spinal taps. So that kind of was one delay.
Dr. Marrero also said that a number of his [00:19:00] patients said that they’d had symptoms and had sort of wavered on whether or not to seek medical attention or go to the hospital because they were worried about contracting COVID-19, um, there.
And then the other sort of aspect of this is that you really need boots on the ground to do these types of epidemiological investigations. So, um, a number of the doctors that I spoke to said that they believe that the patients are being exposed or infected with whatever it is that is causing this syndrome approximately two years before they begin to exhibit symptoms. So you’re really needing to probe and, um, you know, mine through two years of someone’s life and you need to interview them, you need to interview their families, you need to interview their neighbours. And it’s kind of difficult to do these types of interviews in person in the middle of a pandemic in [00:20:00] which there’s an infectious respiratory virus spreading, and it spreads really easily when you’re in close contact with other people.
You know, epidemiologists will want to know about their diets, you know, what did you eat? When did you eat it? Where did you eat it? Um, their travel histories. Where did you go? When did you go? How long were you there? What did you do while you were there? Uh, you know, have you been in any lakes or rivers or bodies of water in the last, you know, however many years, for how long, which ones, um, have you had any contact with animals? Do you fish, do you hunt? When, where, what did you, you know, what did you catch? Um, so this is really sort of complex stuff.
And then the other aspect of it is that they’ll also want to be testing the environment. So testing soil, testing water, and so on, and pandemic restrictions in New Brunswick, which include sort of travel restrictions and then quarantine restrictions, have made it difficult to get [00:21:00] some of these people into the province as quickly as you might want them to be there. Um, and so now more people are allegedly arriving, but still not in the numbers that, um, are desirable.
Jordan Heath-Rawlings: So I have to ask this question, especially because you just kind of described the impact the pandemics having on it. And I know all our listeners will get mad if I don’t ask it, do we have any evidence that this is spreading? Um, are case numbers increasing more rapidly? Do we have any evidence that it can transmit human to human, et cetera?
Amanda Coletta: That’s a great question. And there is no answer.
Jordan Heath-Rawlings: Great!
Amanda Coletta: Um, Dr. Marrero basically said we can’t rule out that it’s not contagious because we don’t know what’s causing it.
Jordan Heath-Rawlings: Right.
Amanda Coletta: Um, and he said, what we can say is that it’s probably, if it is [00:22:00] contagious, it doesn’t seem like it’s as contagious as the coronavirus, which, um, we can, you know, because otherwise we’d have more cases than we do. Um, but he has seen sort of an increase in the number of patients year over a year that are presenting with these symptoms. But that doesn’t necessarily mean that it is, um, spreading from, from human to human. Without knowing the cause, it’s really difficult to determine if it’s contagious, what you need to do to protect yourself from it.
Jordan Heath-Rawlings: So what happens next? Uh, New Brunswick tries to stay safe while the scientists hopefully figure out what this is, or doctors hopefully figure out an effective treatment? Uh, what are we waiting for?
Amanda Coletta: Um, that seems like that’s really the only way forward, um, trying to sort of really narrow down the cardinal symptoms and getting people where they need to be just to start to do these types of, um, [00:23:00] investigations.
Jordan Heath-Rawlings: Amanda, thank you so much for explaining this to us. Now I have something else to worry about, but it is also, um, fascinating and obviously a story people should keep their eye on.
Amanda Coletta: Thanks for having me. And, um, yes, it’s definitely difficult for the families and the patients and, and, you know, the doctors who are treating them as well.
Jordan Heath-Rawlings: Amanda Coletta of The Washington post. That was The Big Story, for more from us, head to thebigstorypodcast.ca, you can find all of our episodes on there. You can talk to us anytime on Twitter at @TheBigStoryFPN. You can write us long essays or just short F-you emails at thebigstorypodcast, all one word, all lowercase, @rci.rogers.com [click here!]. And as always, we are in your favourite podcast players, in Apple, in Google, in Stitcher, in Spotify, in Amazon Music. And we are on all your smart speakers, just ask them to play The [00:24:00] Big Story podcast.
Thanks for listening. I’m Jordan Heath-Rawlings, we’ll talk tomorrow.
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