Forty-eight people in Canada have battled a rare and debilitating brain disorder.
It closely mimics the Creutzfeldt-Jakob disease, a fatal brain disorder that affects only 1 in 1 million people each year, according to a Washington Post report.
For those who have suffered the symptoms of this new disorder, tests for Creutzfeldt-Jakob disease have come up negative, and experts can’t seem to find a cause.
Patients have ranged in age from 18 to 85 and have lived in the Moncton area or the New Brunswick Acadian peninsula.
For now, it’s being referred to as the Neurological Syndrome of Unknown Cause. Officials are currently looking into the New Brunswick Cluster.
Of the 48 people who have been identified as having symptoms of the unknown syndrome, six have died, as of Monday.
“The suffering is immense … because it’s beyond physical,” said neurologist Alier Marrero, who works at Moncton’s Dr. Georges-L.-Dumont University Hospital Center. “There’s also the neuropsychiatric and moral suffering of the patients that is only partially relieved by medications.”
Some of the symptoms patients are developing include:
- Muscle aches or spasms
- Sleep disorders, including severe insomnia
- Blurred vision
- Memory problems
- Teeth chattering
- Hair loss
- Trouble with balance
- Rapid and unexplained weight loss
- Muscle atrophy
- Capgras delusion, which makes patients believe their family members have been replaced by imposters
“The rapidity in the constellation of features is something that -- I’ve not seen this before,” said Michael Strong, the neurologist who heads the Canadian Institutes of Health Research.
The Washington Post reports the cluster of cases was detected by the federal public health agency’s Creutzfeldt-Jakob disease surveillance system, which monitors for CJD and other prion disorders.
The Centers for Disease Control and Prevention describes prion diseases as a “family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.”
The brain of a person with a prion disease can resemble sponges with small holes.
The first case of this new disorder experts in the area were made aware of was in 2015, but Marrero could not find a diagnosis. From 2018 on, others presented with similar symptoms, and 11 new cases in 2019 would eventually be identified as part of the cluster. There were 24 more in 2020, at which time Marrero and Coulthart began thinking they could be dealing with something new.
“We say that they were resistant to diagnosis,” said Michael Coulthart, who heads the CJD surveillance system. “That’s what had to emerge as a pattern before we started talking about a cluster.”
It is believed the syndrome has a two-year incubation period.
Scientists have looked into many avenues for an explanation, including environmental exposures, travel histories, diet, as well as blood screenings for a presence of infectious diseases known to cause neurological symptoms, autoimmune disorders, metabolic deficits and cancer.
None of those tests have provided an answer.
A cerebrospinal fluid test for high levels of protein markers is what can help to diagnose CJD, but all of the patients have come up with a negative test result.
“These cases, for all intents and purposes by their description, should be CJD,” Strong said. “That’s what they sound like and are presenting like, and yet, the testing is negative.”
Two theories include that the syndrome could be tied to exposure to an environmental toxin, or that it is being caused by an entirely new prion disorder.
A spokesperson with the Department of Health said the department is working to identify all potential causes for the unknown disease.
Coulthart said he believes that attention will help them to arrive at an answer.
“Fear is understandable,” Marrero said. “But we are working for hope.”
To learn more about the unknown disease, click or tap here.