New Brunswick October 27, 2021 - Health minister availability Report on cluster of unknown neurological disorders update
October 27 - Health minister availability Report on cluster of unknown neurological disorders update
News Release
Health
Food and environmental exposures ruled out as common factor in cases of neurological syndrome of unknown cause
27 October 2021
FREDERICTON (GNB) – An epidemiological report has found that there is no known exposure in the province that could have triggered the symptoms of a potential neurological syndrome of unknown cause.
“Based on the findings of the report, we have learned that New Brunswickers are not at risk from any behaviours, foods or environmental exposures within the province,” said Health Minister Dorothy Shephard.
Shephard released the report and its findings today as part of Public Health’s investigation into the cluster of patients who have a potential neurological syndrome of unknown cause. The investigation was to determine whether the condition is an illness of unknown cause, and if so, its potential causes, risk factors and prevention measures.
Public Health’s epidemiology and surveillance branch developed an enhanced surveillance questionnaire for the investigation to better understand potential exposures. Thirty-four of the 48 patients in the cluster, or their family members (proxies), participated in the enhanced surveillance interviews. Nine patients declined to participate, and five could not be contacted.
Other findings include:
a total of 48 patients were identified to have some clinical signs and symptoms that were similar to those associated with Creutzfeldt-Jakob Disease (CJD) yet presented atypical characteristics. To date, these patients all tested negative for known forms of human prion diseases, which are a family of rare progressive neurodegenerative disorders.
forty-six of the 48 identified patients in the cluster were referred by a single neurologist and the two other cases were referred by two separate neurologists. all of the patients who participated in the enhanced surveillance interviews were referred to the Creutzfeldt-Jakob Disease Surveillance System (CJDSS) by the same neurologist.
the potential neurological syndrome appears to be limited to New Brunswick, with no similar cases being referred from other provinces or territories. Clinical and diagnostic information is currently being reviewed by an oversight committee composed of six neurologists to help determine whether there are alternate diagnoses for the patients included in this cluster or whether more investigation is required.
“While we have ruled out food and other common exposures, we are still working to determine what took place,” said Shephard. “The role of the oversight committee is to provide a professional second opinion to ensure due diligence and rule out other plausible diagnoses, and they will continue to review all case files, medical charts and records and will re-interview and re-evaluate patients if needed.”
The oversight committee is expected to complete its clinical review sometime in early 2022.
27-10-21
Investigation into a Neurological Syndrome of Unknown Cause: An Epidemiological Summary of Enhanced Surveillance Interviews
Epidemiology and Surveillance Branch Public Health New Brunswick
Date: October 26th, 2021
Overview and Highlights
• The aim of this report is to provide an epidemiological review regarding a cluster of individuals identified as having a potential neurological syndrome of unknown cause in New Brunswick. It is important to note that the exploratory findings presented here do not indicate causality. These epidemiological results are also independent of the clinical review that is currently being undertaken by an oversight committee that has been established for this purpose, and no conclusions can be drawn with regard to the identification of a novel syndrome or disease from this report.
• At this stage of the investigation, based on the current findings, there are no specific behaviours, foods, or environmental exposures that can be identified as potential risk factors with regard to this cluster of cases.
• Since early 2020, three neurologists in New Brunswick have identified a number of individuals who were reported to have an unusual combination of neurological symptoms. Forty-six of the 48 identified cases were referred by a single neurologist and the two other cases were referred by two separate neurologists.
• The first of these cases was referred to the Creutzfeldt-Jakob Disease Surveillance System (CJDSS) in 2015, followed by 12 cases in 2019, 25 cases in 2020, and 10 cases in 2021. The CJDSS is managed by the Public Health Agency of Canada (PHAC) and performs nationwide monitoring for human prion diseases which cause neurologic manifestations. These referrals were made in relation to suspected diagnoses of Creutzfeldt-Jakob Disease (CJD).
• The cluster was identified by the main referring New Brunswick neurologist and its existence was first tentatively recognized by the CJDSS in early 2020. Several cases, including one that was referred to the CJDSS in 2015, were later identified through a retrospective review and added to the cluster on the recommendation of the principal neurologist, due to similarities in clinical presentation and lack of a confirmed diagnosis. The review of older files was completed on cases for which a diagnosis of CJD was suspected but later set aside. It was only through this retrospective review that the older cases were identified.
• As of April 30, 2021, there were a total 48 individuals who were identified to have some clinical signs and symptoms that were similar to those associated with CJD yet presented atypical characteristics. These patients all tested negative for known forms of human prion diseases.
• Difficulties in diagnosing these individuals led to their classification as a potential cluster with a neurological syndrome of unknown cause in New Brunswick in early 2020 by the principal referring neurologist and CJDSS. Public Health New Brunswick (PHNB) was made aware of this matter by the CJDSS in late 2020 and began to investigate in early 2021. The investigation regarding this condition is still active and PHNB continues to collaborate with local and national subject matter experts.
• The potential neurological syndrome appears to be limited to New Brunswick, with no similar cases being referred from other provinces or territories.
• PHNB is still actively investigating the cluster in order to determine whether the condition is an illness of unknown etiology, and if so, its potential causes, risk factors and prevention measures. The investigation is likely to last several months, and regular updates on the investigation will be provided. An oversight committee, with six New Brunswick neurologists, has been established to provide a professional second opinion to ensure due diligence and rule out other plausible diagnoses.
• As of September 2021, 34 of the 48 cases (71%) participated in the PHNB enhanced surveillance interviews. Nine cases (19%) declined an interview. Five (10.4%) cases could not be contacted after multiple attempts. Interviews with identified individuals or their representatives began in May of this year.
• All of the cases that participated in the enhanced surveillance interviews were referred to the CJDSS by the same neurologist.
• Among the interviewed cases, there were 18 females (53%) and 16 males (47%). Approximately half of those cases (59%) were between 18-59 years of age, and the median age was 57 years. Overall, females were younger than males. The average age of female cases was 51 years, while the average age of male cases was 59 years.
• Among the 48 cases in the cluster, nine are deceased. Three of the deceased cases were females, the other six were males. There was one death in 2019, four in 2020, and four more in 2021. Representatives for five of the nine deceased cases agreed to participate in enhanced surveillance interviews conducted by experienced PHNB nurses as part of the epidemiological investigation.
• Most of the individuals interviewed, at the time of reported symptom onset, were living in southeastern New Brunswick, around the Moncton area, and in the northeastern part of the province, near the Acadian Peninsula. The geographic distribution of the cases may have been influenced by the catchment area of the referring neurologists.
• PHNB has adopted a thorough, transparent, and open approach and has considered a wide range of hypotheses that include food safety, environmental factors, and lifestyle practices. Further epidemiological research may be required once the clinical case reviews are completed by the Oversight Committee.
Neurological Syndrome of Unknown Cause Epidemiological Summary Epidemiology and Surveillance Branch October 2021 Page 6 of 36 Public Health New Brunswick
see full report;
New Questions About a Mysterious Neurologic Cluster in Canada
FOR YOUR PATIENTS
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.
A neurologist in the Canadian province of New Brunswick has diagnosed 48 individuals there, including six who have died, as belonging to a cluster of a novel, previously unknown neurologic disease.
The symptoms and history of the cases vary dramatically—and the patients live in a geographic span of well over 100 miles—but all involve what the neurologist and provincial health authorities say are unexplained, progressive neurologic symptoms.
One patient died at the age of 81, four months after developing paranoid delusions and seizures that led him to become violent while hospitalized.
Another, aged 20, developed headaches, brain fog, fatigue, blurry vision, muscle weakness and memory challenges during her first semester of college last year.
A third, aged 63, thought he was having a heart attack the day before his 40th wedding anniversary, in June of 2019, but was sent home from the hospital after tests showed normal cardiac function. He then developed symptoms that convinced his neurologist he had Creutzfeldt-Jakob disease (CJD). That, however, was excluded by testing, as was amyotrophic lateral sclerosis (ALS), Parkinson's disease, Alzheimer's disease, and autoimmune encephalitis. His symptoms have since waxed and waned.
A fourth patient, now 41, was stopped at a red light when his vehicle was totaled by a car that rammed him from behind in October of 2018. He showed no signs of a brain injury on the MRI at the hospital, but was later found to have strabismus and prescribed prism glasses. Nevertheless, he continues to have memory problems, headaches, muscle spasms, and pain in his shoulder and neck. Unable to work, he sleeps up to 16 hours a day.
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.”
Asked whether he is convinced that New Brunswick does in fact have a cluster of a single, as-yet unknown neurologic disorder, Michael J. Strong, MD, president of the CIHR said: “That's the million-dollar question right now. In any sort of an event like this, where you're asking is this a cluster? Is this a new disease? Whatever it might be, you've got to do the groundwork. You have to go through and look at every single case. You have to agree on primary and secondary criteria for a diagnosis. That's where the process is at the moment.”
Dr. Strong, who has spent decades studying and treating ALS and remains a distinguished professor in the department of clinical neurological sciences at the Schulich School of Medicine & Dentistry at Western University, emphasized that he takes “at face value” Dr. Marrero's assertion of a mysterious neurologic cluster.
But, he added, “It now behooves somebody, and we know this committee has been established, to look at all the information—all the notes, all the test results—reexamine the patients who are still alive, and have two or three neurologists agree that they're seeing the same thing. Until then, as a researcher who has expertise in these unusual syndromes, I am just sitting tight until I've got that information.”
The Case for a Cluster
In an interview with Neurology Today, Dr. Marrero explained how he became convinced that a mysterious neurologic disease was present in the province of New Brunswick, and possibly beyond.
“I met the first patient who had this condition in 2014,” Dr. Marrero said. “The patient was in their early thirties, but had a rapidly progressing dementia with no explanation. It was fully investigated. We thought the patient had CJD [Creutzfeldt-Jakob disease]. With all of the cases, we thought they had prion disease.”
Prion disease has not, in fact, been confirmed in any of the patients by CSF testing or on autopsy. But Dr. Marrero said that a common picture has emerged of a neurologic disease unexplained by most tests.
“Most frequently, at first the symptoms are non-specific,” he said. “These patients develop behavior changes, severe anxiety, unexplained severe pains, muscle spasms. But when you do nerve conduction tests, they are normal. They don't have diabetes, they don't have an infection. Initially they are seen by family physicians, sometimes in psychiatry. They do not respond well to therapy.”
All the patients in the cluster, he said, “have developed some type of sleep disorder and mostly severe insomnia. I have some patients who barely sleep at all. Coinciding with that, they develop memory difficulty, word finding difficulty, disorientation. One hundred percent of them develop visual disturbances. Initially, in nearly all of them, it's blurry vision, but it's unexplained. They become dizzy and bump into objects. In nearly all cases, this evolves into visual hallucinations. They will see shadows. A shadow of a person, or shadows of cats. After that, they do have in many cases obvious, frank, complex hallucinations that coincide with the appearance of frightening nightmares. The nightmares are very common, they are very upsetting. At some point they develop prominent myoclonus. And they develop ataxia. Patients end up in a wheelchair quite fast.”
Figure
“Most of the times when I hear things like this, especially in my experience with epidemics of prion disease, they almost always turn out to be incorrect. They usually turn out to be multiple disorders that either had not been diagnosed yet, or had been incorrectly diagnosed.”—DR. MICHAEL D. GESCHWIND “I have seen records that indicate that some of the people in the cluster definitely have a neurodegenerative syndrome, but whether it is a novel disease would depend on further investigation and necropsy.”
—DR. NEIL CASHMAN
Some patients develop atypical parkinsonism that does not respond to levodopa, and some lose a great deal of weight. “They just lose their muscles and become very weak with prominent atrophy,” Dr. Marrero said. Many, he added, begin drooling uncontrollably.
As for tests, he said, only one finding consistently appears. “In many cases, we can document brain atrophy. Initially the MRIs are normal, absolutely normal. But as it advances, the brain atrophy progresses.”
The Cluster Goes Public
Neil Cashman, MD, professor of neurology at the University of British Columbia and academic director of the Coastal Health ALS Clinic, said that he has been reviewing and discussing case files with Dr. Marrero for over a year.
“I have seen records that indicate that some of the people in the cluster definitely have a neurodegenerative syndrome, but whether it is a novel disease would depend on further investigation and necropsy,” Dr. Cashman said.
Asked whether he believes that all 48 patients in the cluster fit the criteria that was established, he said: “I would say there are certain individuals who don't seem to fit the case definition as I understand it. Perhaps there is a certain amount of over-inclusion.”
After Dr. Marrero's repeated pleas to public health authorities to open an investigation, Cristin Muecke, MD, New Brunswick's deputy chief medical officer of health, sent out a memo on March 5 of this year to health care professionals in the province.
“Public Health New Brunswick wishes to make you aware of a cluster of progressive neurological syndrome of unknown etiology in the province,” the memo stated. “To date in New Brunswick, there have been 40 cases referred to the national Creutzfeldt Jakob Disease Surveillance System (CJDSS) as part of this cluster under investigation: one case in 2015 (retrospectively identified), 11 cases in 2019, 24 cases in 2020, and four cases thus far in 2021. Five cases (13 percent) have died; all met the case definition for a confirmed case of this syndrome.
“Preliminary investigation conducted in late 2019/early 2020 determined this to be a distinct atypical neurological syndrome,” the memo continued. “While these cases have many similarities to Creutzfeldt Jacob [sic] disease, testing for CJD so far has ruled out known prion diseases. We are collaborating with different national groups and experts; however, no clear cause has been identified at this time.”
The case definition for confirmed cases, the memo stated, includes progressive neurologic syndrome involving rapidly progressing dementia and at least four of the following clinical features, verified directly where possible by a physician:
cerebellar ataxia (gait ataxia, truncal ataxia, cerebellar dysarthria or dysmetria), abnormal cerebellar function test or cerebellar symptoms (dysdiadochokinesis, intention tremor etc.)
psychiatric symptoms (agitation/irritability, aggressiveness, apathy/withdrawal, anxiety or obsessive behavior)
visual hallucinations, cortical blindness or other cortical visual symptoms
pyramidal or extrapyramidal signs, including atypical parkinsonism
myoclonus
painful sensory symptoms (limb pain, dysesthesia or paresthesia) persisting for six months or more, in absence of peripheral nervous system dysfunction
muscle atrophy
In addition, according to the case definition described in the memo, the majority of cases should manifest during the first 18-36 months of illness, and one or more of the following findings should be seen on testing:
atrophy, greater than expected for age on MRI
EEG slowing or hypoperfusion on SPECT (CT) or hypometabolism on PET-CT scan. The New Brunswick health department has since conducted lengthy telephone interviews with many of the patients or survivors to determine if any environmental exposure could be at play.
Expert Commentary
Valerie Sim, MD, an associate professor of neurology in the Centre for Prions and Protein Folding Diseases at the University of Alberta, said that without the sort of detailed review of cases now being conducted by the oversight committee, “It's impossible to conclude anything. The age range of people affected is extreme, from 18 to 85, and from what I gather, there's a whole host of different symptoms. Some are very mild and some are very severe. My question is: why is this a syndrome, and what do these patients have in common?”
Anthony E. Lang, MD, FAAN, professor and Jack Clark Chair for Parkinson's Disease Research at the University of Toronto, stated in an email: “I am not involved in the assessment of these cases. I have tried very hard to learn more about them but without any success. To be honest, I don't know that there really is a cluster of one single disorder since no information is available to confirm this claim.”
Figure
“Its impossible to conclude anything. The age range of people affected is extreme, from 18 to 85, and from what I gather, theres a whole host of different symptoms. Some are very mild and some are very severe. My question is: why is this a syndrome, and what do these patients have in common?”—DR. VALERIE SIM Michael D. Geschwind, MD, PhD, FAAN, professor of neurology at the Memory and Aging Center of the UCSF Weill Institute for Neurosciences, also expressed doubts about there being a cluster of a specific yet unknown neurologic disorder.
“I have not been involved in the evaluation of any of these patients,” he said. But, having communicated with two of the neurologists on the oversight committee, he said: “Most of the times when I hear things like this, especially in my experience with epidemics of prion disease, they almost always turn out to be incorrect. They usually turn out to be multiple disorders that either had not been diagnosed yet, or had been incorrectly diagnosed.”
Since March, when news of the suspected cluster first appeared, Dr. Geschwind added: “I've had numerous patients thinking they have it. One patient's medical team contacted me because a patient of theirs had been in that area for a day, years ago. They thought, ‘This is it.’ We don't even know what ‘it’ is.”
One possible explanation for some of the patients' symptoms raised by neurologists is that they could be due to functional neurologic disorders (FNDs). Although obviously not a cause of death, FNDs have long been associated with bewildering cases of motor and other neurological symptoms that defy a purely organic explanation. Often triggered by a frightening or stressful event, they are diagnosed by excluding all possible organic causes and observing signs or symptoms that are inconsistent or discordant with known disorders.
“I'm not saying any of these cases are necessarily functional. But we all know that functional neurological disorders are real,” said Dr. Sim. “We see so much of it in neurology, but the treatment for it lies outside of our training.”
Dr. Geschwind also speculated that at least some of the patients might have an FND. “The good thing is that those can be treatable, whereas almost any other neurodegenerative disease has no disease-modifying treatment.”
Dr. Strong said he would not be surprised if some of the patients turned out to have an FND. But, he added: “I have seen cases where I thought it was a functional disorder, but when we sorted through it, we found an etiology. I'm incredibly cautious about ascribing a person's symptoms to a functional disorder, and not until I have seen everything else dealt with.”
Even Dr. Marrero said he had considered the possibility of FND at the early stages of some of his patients' illness.
“In some of our patients, their first presentation is non-specific behavioral changes,” he said. “At that stage, a functional illness is part of the differential diagnosis. But as the disease progresses, it becomes obvious that it is not. Then you have objective findings of brain atrophy, the myoclonus, the muscle wasting.”
Whatever the explanation or explanations for the patients' symptoms turn out to be, they and their family members expressed sharply different views on the care they have been receiving from Dr. Marrero.
In response to written questions from Neurology Today, the oversight committee stated: “All the efforts have been made to expedite the process to get answers as early as possible. We understand the concerns and fears of patients and their families, but our ultimate goal is to provide them with as accurate and precise an answer as possible....The diagnosis of complex neurological and neurodegenerative conditions can take a significant amount of time and needs to be performed in a careful and systematic way.”
Figure
“Currently our clinic is following over a hundred cases. And we have received dozens of communications. Weve had referrals from other provinces in Canada, and also some in the United States.”—DR. ALIER MARRERO
Disclosures
Drs. Marrero, Strong, Lang, and Sim had no disclosures related to this story. Dr. Geschwind receives research support from the NIH/NIA and the Michael J. Homer Family Fund. He has consulted for Adept Field Consulting (Backay consulting), Ascel Health LLC, Anderson Boutwell Traylor, Best Doctors Inc., Biohaven Pharma Inc., Bioscience Pharma Partners, LLC (BPP), Clarion Consulting, First Thought Consulting, Grand Rounds Inc./UCSF Second Opinion Inc., Maupin Cox Legoy, Quest Diagnostics, Smith & Hennessey LLC and Trinity Partners LLC. He has received speaking honoraria for various medical center lectures, Oakstone Publishing and Wolters Kluwer. He has received past research support from Alliance Biosecure, CurePSP, the Tau Consortium, Quest Diagnostics, and NIH. Dr. Geschwind serves on the board of directors for San Francisco Bay Area Physicians for Social Responsibility and on the editorial board of Dementia & Neuropsychologia.
Dr. Lang has served as an advisor for Abbvie, Acorda, AFFiRis, Biogen, Denali, Janssen, Lilly, Lundbeck, Maplight, Paladin, Retrophin, Roche, Sun Pharma, Sunovion, Theravance, and Corticobasal Degeneration Solutions; received honoraria from Sun Pharma, AbbVie and Sunovion; received grants from Brain Canada, Canadian Institutes of Health Research, Corticobasal Degeneration Solutions, Edmond J Safra Philanthropic Foundation, Michael J. Fox Foundation, the Ontario Brain Institute, Parkinson Foundation, Parkinson Canada, and W. Garfield Weston Foundation; received publishing royalties from Elsevier, Saunders, Wiley-Blackwell, Johns Hopkins Press, and Cambridge University Press.
Vol. 21, Issue 17 - p. 1-14
New Brunswick Cluster of Neurological Syndrome of Unknown Cause
The Province of New Brunswick is collaborating with local and national subject matter experts and health-care providers to investigate a group of individuals who are experiencing signs and symptoms of a neurological syndrome of unknown cause (NSUC).
At this time, the investigation is active and ongoing to determine if there are similarities among the reported cases that can identify potential causes for this syndrome, and to help identify possible strategies for prevention. The investigation team is exploring all potential causes including food, environmental and animal exposures.
Investigation overview
Since early 2020, physicians in New Brunswick have been identifying a number of individuals with an unusual combination of neurological symptoms. Despite extensive medical investigation, a diagnosis for these individuals has not yet been determined.
Local health-care providers in New Brunswick have engaged the Public Health Agency of Canada’s Creutzfeldt-Jakob Disease Surveillance System (CJDSS) to actively investigate the possibility of human prion disease, but to date, all test results have been negative for known forms of human prion disease. Due to commonalities in signs and symptoms and the lack of a confirmed diagnosis among cases, a cluster of NSUC has been identified.
At the time of referral by their health-care provider, most of the individuals under investigation were living in the southeastern and northeastern regions of New Brunswick, around the Acadian Peninsula and Moncton areas. However, so far our investigation has not found any evidence suggesting that the residents of these regions are more at risk than those living elsewhere in the province.
Canadian health-care providers have been alerted to this investigation and are advised to contact New Brunswick Public Health for further information or to make referrals for individual cases.
Investigation status
Active
Cases under investigation
48
Deaths
6*
Illness onset date range in years
2013 – 2020**
Gender
50% female – 50% male
Age range in years
18 – 85
* In some cases, additional information is needed to determine if the cause of death was a result of this syndrome. ** Symptoms started in 2018, 2019 or 2020 for most cases. Only one case identified retrospectively in 2020 was found to experience symptoms in 2013. Timeline
The Creutzfeldt-Jakob Disease Surveillance System (CJDSS) routinely works in partnership with the Regional Health Authorities in New Brunswick to detect all types of human prion disease.
Through routine case management, the CJDSS noted common symptoms and similar potential diagnostic profiles among some recent New Brunswick referrals to the surveillance system. These referrals tested negative for Creutzfeldt-Jakob Disease and it became apparent that these atypical referrals represented a cluster of cases that were worth further investigation.
In December 2020, the CJDSS notified Public Health New Brunswick to actively include us in the investigation.
The first draft for the potential case definition was prepared at the end of January 2021, and a memo regarding this potential case definition was sent to physicians in New Brunswick on March 5.
The memo’s purpose was to inform and encourage any health-care provider with patients that may meet the case definition for this neurological syndrome of unknown cause, to please contact Public Health, the CJDSS or the Mind Clinic for more information.
In April, Public Health began collecting consent and contact information from those impacted by the investigation to undertake an investigative survey.
With input from local and national subject matter experts, Public Health drafted an epidemiological questionnaire to gather information from New Brunswickers suspected of being part of this cluster. This questionnaire asks for lots of information including but not limited to, the persons’ environment, residence, potential exposure and travel history. Detailed information is needed to better understand and may help determine if there is an environmental or infectious source of these cases and if so, to help identify ways to prevent more cases from occurring. Data collected will be incorporated into the investigation to help identify potential sources of the syndrome.
In May, Public Health began calling individuals to schedule appointments to conduct the surveys.
The first interview was completed on Wednesday, May 26, 2021.
To date, 23 surveys have been conducted, and several more are being scheduled and followed up on. Symptoms
Some symptoms include, but are not limited to:
memory problems
muscle spasms
balance issues,
difficulty walking or falls
blurred vision or visual hallucinations unexplained,
significant weight loss
behaviour changes
pain in the upper or lower limbs
What you can do
If you suspect that you, or your loved one, may be experiencing changes in personal health that may be similar to those described above, please speak with a health-care provider.
As the cause of the neurological syndrome is currently unknown, only a health-care provider can assess if the symptoms an individual is experiencing may be related to this NSUC investigation.
What the Government is doing
The Government of New Brunswick is committed to protecting the health of all citizens from new and emerging diseases in the province. The investigation is ongoing and Public Health New Brunswick will continue to investigate, working with the following partners:
New Brunswick Department of Agriculture, Aquaculture and Fisheries New Brunswick Department of Natural Resources and Energy Development New Brunswick Department of Environment and Local Government. Public Health Agency of Canada Canadian Food Inspection Agency Frequently Asked Questions
What is a cluster?
A cluster is defined as several things of the same kind, and/or a group of things or persons close together. Occasionally, an outbreak or cluster of cases is discovered, for which the cause is unclear, and epidemiologists and biostatisticians must work diligently to asses whether a true cluster of disease exists.
In this investigation, the term cluster is being used because the ill individuals have similar clinical signs and symptoms that do not have a known cause and are located in the same geographical area.
Is it unusual to see this many cases of unknown neurological disease in New Brunswick?
Occasionally, an outbreak or cluster of cases is discovered, for which the cause is unclear, and epidemiologists and biostatisticians must work diligently to asses whether a true cluster of disease exists. This cluster of individuals experiencing signs and symptoms of a neurological syndrome of unknown cause in New Brunswick is unexpected, which is why the cluster is being actively investigated by Public Health New Brunswick in collaboration with the established Oversight Committee comprised of Neurologists and chaired by VPs from both RHAs. In addition other local and national subject matter experts and health-care providers will be engaged to identify potential causes.
Are there cases in other provinces or countries?
To date, no unusual clusters of individuals with neurological syndrome of unknown cause have been identified outside of New Brunswick. However, public health officials across Canada have been informed of this investigation and advised to contact New Brunswick Public Health for further information. The Public Health Agency of Canada has been in contact with other countries to provide further information and will be notified if cases are identified outside of Canada.
Do other cases under investigation have the same symptoms and/or severity of symptoms as (case)?
Individuals under investigation have similar symptoms that do not have a known. The most frequently reported symptoms include memory problems, muscle spasms, balance issues (e.g., difficulty walking or falls), blurred vision or visual hallucinations, unexplained, significant weight loss, behaviour changes and pain in the upper or lower limbs. The severity of symptoms varies among individuals.
What is being done to identify the cause? Are you doing any testing to find out if there are hazards in the environment?
Public Health New Brunswick has developed, with input from local and national subject matter experts, an epidemiological investigation questionnaire to gather exposure information from individuals in NB suspected of being part of this cluster. The information collected from these interviews will be analyzed to look for common exposures that can be investigated further.
The investigation team is exploring all potential causes, including food, environmental and animal exposures. At this time, there are no specific behaviours or foods that have been identified to avoid. As new information becomes available in this investigation, this will be communicated to the public through the New Brunswick Public Health webpage.
Is it safe to live in the Acadian peninsula or Moncton areas if cases who developed this disease lived here too?
At the time of referral by their health-care provider, most of the individuals under investigation were living in the southeastern and northeastern regions of New Brunswick, around the Acadian Peninsula and Moncton areas. However, so far the investigation has not found any evidence suggesting that the residents of these regions are more at risk than those living elsewhere in the province. As new information becomes available in this investigation, this will be communicated to the public through the New Brunswick Public Health webpage.
As the investigation evolves, this webpage will be updated to provide new information about the ongoing investigation.
Last updated: 2021-07-08 - Updates made to “Timeline” section.
STILL no answers. no cause. very concerning. ruled out tse prion disease to date.
***>These patients all tested negative for known forms of human prion diseases.
key word here is _known_.
i believe the verdict is still out on this, and we would hope they checked vpspr tse prion, because i see know vpspr documented in Canada.
HOLEY SMOKES, VPSPR CASES SEEM TO BE RISING, no one with a clue if it's zoonotic from cwd, atypical bse, scrapie, iatrogenic there from, or all of the above, take your pick, but with Canada having this outbreak of an neurological disorder similar to cjd, but yet, unlike anything they have seen, and cjd ruled out, yet still no answers, and all these cases of TYPE DETERMINATION PENDING IN THE USA, IN WHICH NVCJD HAS BEEN RULED OUT, AND VPSPR, WHAT'S GOING ON HERE?? WHAT THE HELL IS GOING ON???
***> CANADA, I find it very odd that Canada has NO recorded or documented cases of Variably Protease-Sensitive Prionopathy (VPSPr)?
CANADA Creutzfeldt-Jakob disease surveillance system (CJDSS) report
Definite and probable CJD, 1998-2020
As of 31 October, 2020
Year Sporadic Iatrogenic Familial GSS FFI vCJD Total
1998 22 1 0 1 0 0 24
1999 27 2 2 1 0 0 32
2000 32 0 0 3 0 0 35
2001 27 0 2 1 0 0 30
2002 31 0 2 2 0 1 36
2003 27 1 1 0 0 0 29
2004 42 0 1 1 0 0 44
2005 42 0 1 1 0 0 44
2006 39 0 1 3 1 0 44
2007 35 0 0 4 0 0 39
2008 48 0 1 0 0 0 49
2009 48 0 3 2 0 0 53
2010 35 0 3 0 0 0 38
2011 46 0 3 1 0 1 51
2012 62 0 1 0 0 0 63
2013 50 0 0 0 1 0 51
2014 51 0 4 0 1 0 56
2015 44 0 5 1 2 0 52
2016 57 1 5 1 0 0 64
2017 82 0 2 1 1 0 86
2018 74 1 4 0 1 0 80
2019 76 0 2 0 0 0 78
2020 30 0 2 0 0 0 32
Total 1027 6 45 23 7 2 1110
Cases with definite and probable diagnosis to date.
Gerstmann-Sträussler-Scheinker disease (GSS)
Fatal familial insomnia (FFI)
Variant CJD (vCJD)
NO CASES VPSPR TSE PRION DISEASE CANADA ???
COMPARED TO USA;
Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated quarterly.
Last updated on: July 9th, 2021
Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD
1999 & earlier 382 231 201 27 3 0
2000 145 102 90 12 0 0
2001 209 118 110 8 0 0
2002 241 144 124 18 2 0
2003 259 160 137 21 2 0
2004 315 180 163 16 0 1³
2005 328 179 157 21 1 0
2006 365 179 159 17 1 2⁴
2007 374 210 191 19 0 0
2008 384 221 205 16 0 0
2009 397 231 210 20 1 0
2010 401 246 218 28 0 0
2011 392 238 214 24 0 0
2012 413 244 221 23 0 0
2013 416 258 223 34 1 0
2014 355 208 185 21 1 1⁵
2015 401 263 243 20 0 0
2016 395 277 248 29 0 0
2017 375 266 247 19 0 0
2018 308 221 202 18 1 0
2019 432 281 259 22 0 0
2020 358 249 223 22 1 0
2021 150 74 36 5 0 0
TOTAL 77966 47807 42698 4609 14 4
1 Listed based on the year of death or, if not available, on the year of referral;
2 Cases with suspected prion disease for which brain tissue was submitted;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 39 case in which the diagnosis is pending (1 from 2018, 1 from 2019, 1 from 2020 and 19 from 2021), and 19 inconclusive cases;
7 Includes 33 (33 from 2021) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 4158 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 76 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 282 Familial cases diagnosed by blood test only.
SO SURELY, VPSPR TSE PRION WAS INCLUDED INTO THEIR INVESTIGATION OF THE CLUSTER OF UNKNOWN NEUROLOGICAL DISORDERS...surely?
still and all, IF this is not a good case/argument for the misdiagnosis of CJD and other dementias, from this investigation, even though there are no answers yet, i don't know what is...
so, i bring to your attention;
''Sporadic Creutzfeldt-Jakob disease (sCJD) in a 21-year-old patient was the right diagnosis, but hardly a satisfactory explanation for either the doctor or patient and his family in the Clinical/Scientific Note by Appleby et al.1 in this week's issue of Neurology®. How can a typically late-onset disorder occur in someone so young?''
Exactly, absolutely, Yes Sir, and here is why it is not acceptable, imo, the science has been there, but political, legislative, junk science, does not allow sound science to make this call yet, i.e. the industries involved, and they are many. you just had to have been there to see it over the last 23 years. so please let me plead my case of urgency, it's long, it's 23 years, daily, as i know it, but the science is there, but nobody seems to care about the truth, they would rather stick their heads in the sand and call it spontaneous or sporadic $$$
FIRST OFF, spontaneous/sporadic CJD and a brief history of spontaneous sporadic cjd science put forth to date, history there from.
let's compare apple to oranges first I.E. spontaneous sporadic TSE Prion Primates Research Laboratories
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
https://www.nature.com/articles/srep11573
NEXT, just what does spontaneous/sporadic, mean?
sporadic, spontaneous CJD, 85%+ of all human TSE, just not just happen. never in scientific literature has this been proven.
if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
sporadic = 68,704 results for definition of various diseases https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic
spontaneous = 444,312 results for definition of various diseases https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous
SO PLEASE, the least we could do is define what spontaneous and sporadic, in terms of Transmissible Spongiform Encephalopathy TSE Prion disease are, define exactly what that means, for those that still think 85%+ of all human TSE Prion disease i.e. and this is a very broad term including vpspr, sffi, sgss, and SPORADIC CJD, for those that still believe all sCJD is just a happening of a spontaneous even of a protein flipping out on it's own, with no cause, just happens, in 85%+ of all human TSE Prion, without a shread of documented evidence to prove this. the UKBSEnvCJD only theory is, was, and will be, not scientific, and we must move away from that. With the science at hand, animal transmission studies, molecular studies, there is enough evidence to date, (as much today, as there was back in 1995 linking nvCJD to c-BSE), to declare that there is evidence that if not all, some of the sporadic CJD strains are indeed a zoonosis disease, or, are a iatrogenic event from either a zoonotic source by friendly fire, pass it forward, from a subclinical host from said sporadic CJD cases, from either CWD in cervid, BSE in cattle, or Scrapie in sheep and goats, both typical and atypical, and all of the above have now been linked by science to the infamous spontaneous/sporadic CJDs. we have wasted too much effort on the UKBSEnvCJD only theory imo. for the announcement for science to link nvcjd to humans from UK c-BSE in 1984 or 1985, it took an additional 10 years to finally make the call that nvcjd was infecting humans in 1995. i think BSE started actually before Carol Richardson and the case there (see source references below) , but we can't wait for another 10 years for a call to be made for zoonosis of CWD, BSE, and Scrapie, for some of the cases, if not all, of the sporadic CJDs as zoonosis disease. to continue this thinking, will only allow the TSE Prion disease to continue to be spread via iatrogenic events. we cannot wash our hands of these events, with a spontaneous or sporadic ideology. WE MUST MOVE FORWARD WITH THE ZOONOISIS science of sporadic CJD as zoonosis, and work ourselves down from there, trying to prove it's not, instead of spreading to hell and back around the globe for 5 decades or so, saying it is not, without proof. i think science has been screaming for over a decade that indeed, sporadic CJD, is zoonosis, and govern from there. THIS CHARADE MUST END!
CJD is not reportable in all states, but no evidence of increase in the number of cases has been observed in the U.S. However, the NPDPSC examines about 64% of the approximately 300 cases expected to occur in the U.S. per year...
https://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
expected to occur in the U.S. per year???
oxymoron
you must have _all_ key components of a math formula to correctly solve a math problem, or in this case, figuring statistics. yet we don't have all the key components.
not all states report cjd tse prion, and some that do, only report in 55 and older, yet some do make in reportable of all ages. plus, you figure in misdiagnosis, that's another key element/component, it happens.
for example;
CJD is not a reportable disease in West Virginia. Because the disease is 100% fatal the occurrence of CJD is tracked through death certificates.
I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;
routine passive mortality CJD surveillance USA ?
THIS has been proven not to be very useful in the U.K.;
THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)
snip...
One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...
snip...
Draft Proposal For The Monitoring of Creutzfeldt-Jakob Disease 1989 Dr. R. Will
snip...
IDENTIFICATION OF CASES
Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...
full text;
http://web.archive.org/web/20090505230809/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf
Confucius is confused again? how in 1996 and earlier can the 28 sporadic CJD victims and the one-in-a-million there from, how can it still be one in a million in 2008, with the sporadic CJD count rising to 205, still be one-in-a-million? and the years in-between, steady rise just about every year, and it still be only one-in-a-million, year after year after years? I suppose just more of that fuzzy math, which you can see here;
CJD TSE PRION ONE IN A MILLION ???
Prion disease is not one in a million Dec 6, 2018 • ericminikel • Cambridge, MA
snip...
Ryan Maddox at the CDC has been doing this for the U.S., and he reported in 2016 that about 1 in every 6,000 deaths is due to prion disease. This figure probably captures some cases that were diagnosed only postmortem or never got referred to the surveillance center, although there might still be some underdiagnosis at work here.
Simon Mead has announced a figure of 1 in every 4,700 deaths in the U.K. Coming at it from several different angles and data sources, then, we converge on an answer that roughly 1 in every 5,000 people dies of prion disease, or in other words, the general population’s lifetime risk of prion disease is 1 in 5,000.
If you’re a researcher studying prion disease or a family affected by prion disease, people probably often ask you “oh, is that super rare?”. Next time this happens, give the best answer you can give: “it kills about 1 in 5,000 people.” Not super common, but not 1 in a million.
older stats;
55 year old and greater, the rate of death for sporadic cjd is one in 9,000.
There is one CJD death per every 6,000 to 10,000 deaths in the U.S. each year. Eighty-five percent of CJD cases are sporadic, meaning there is no known cause at present.
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
I kindly would like to bring to everyone's attention;
***> 6 Includes 39 case in which the diagnosis is pending (1 from 2018, 1 from 2019, 1 from 2020 and 19 from 2021), and 19 inconclusive cases;
WOW, 2021 is showing 19 cases where the diagnosis is pending, and 19 inconclusive cases, what's that all about???
***> 7 Includes 33 (33 from 2021) cases with type determination pending in which the diagnosis of vCJD has been excluded.
HOLEY COW, WITH 33 ADDITIONAL CASES FROM 2021, WITH TYPE DETERMINATION PENDING, IN WHICH DIAGNOSIS OF VCJD HAS BEEN EXCLUDED, WHAT'S ALL THAT ABOUT???
***> 8 The sporadic cases include 4158 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 76 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI).
HOLEY SMOKES, VPSPR CASES SEEM TO BE RISING, no one with a clue if it's zoonotic from cwd, atypical bse, scrapie, iatrogenic there from, or all of the above, take your pick, but with Canada having this outbreak of an neurological disorder similar to cjd, but yet, unlike anything they have seen, and cjd ruled out, yet still no answers, and all these cases of TYPE DETERMINATION PENDING IN THE USA, IN WHICH NVCJD HAS BEEN RULED OUT, AND VPSPR, WHAT'S GOING ON HERE?? WHAT THE HELL IS GOING ON???
IATROGENIC, IATROGENIC, IATROGENIC.
LET'S COMPARE TO LAST REPORTS HERE;
USA Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated quarterly.
Last updated on: October 8th, 2020
Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD
1999 & earlier 382 231 200 27 3 0
2000 145 102 90 12 0 0
2001 209 118 110 8 0 0
2002 241 144 124 18 2 0
2003 259 160 137 21 2 0
2004 316 181 164 16 0 1³
2005 327 178 156 21 1 0
2006 365 179 159 17 1 2⁴
2007 374 210 191 19 0 0
2008 384 221 205 16 0 0
2009 397 231 210 20 1 0
2010 401 246 218 28 0 0
2011 392 238 214 24 0 0
2012 413 244 221 23 0 0
2013 416 258 223 34 1 0
2014 355 208 185 21 1 1⁵
2015 401 263 243 20 0 0
2016 396 277 248 29 0 0
2017 375 266 247 19 0 0
2018 309 223 204 18 1 0
2019 422 274 252 21 0 0
2020 252 159 125 11 1 0
TOTAL 75316 46117 41268 4439 14 4
1Listed based on the year of death or, if not available, on the year of referral;
2Cases with suspected prion disease for which brain tissue was submitted;
3Disease acquired in the United Kingdom;
4Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;
5Disease possibly acquired in a Middle Eastern or Eastern European country;
6Includes 12 cases in which the diagnosis is pending, and 19 inconclusive cases;
7Includes 24 (1 from 1986, 1 from 2019, 22 from 2020) cases with type determination pending in which the diagnosis of vCJD has been excluded.
8The sporadic cases include 4020 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 71 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI).
9Total does not include 277 Familial cases diagnosed by blood test only.
snip...see full text;
Creutzfeldt-Jakob Disease TSE Prion Questionnaires a review 2021
Please see my complete comment to this synopsis here ;
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010
snip...see full report;
TUESDAY, OCTOBER 26, 2021
Sporadic Creutzfeldt-Jakob Disease in a Very Young Person Singeltary Reply 2021
Terry S. Singeltary Sr.
