Neurologist Alier Marrero of Dr. Georges-L.-Dumont University Hospital in Moncton sees more and more patients each year affected by what federal public health authorities have called neurological syndrome without a known cause in Moncton and the Acadian Peninsula. in the Canadian province of New Brunswick. They started an investigation of a cluster of 48 patients (six of whom died) who showed similar symptoms.
According to Dr. Marrero, the disease begins with atypical anxiety, depression, pain and muscle spasms. Then there are sleep disorders and severe insomnia that do not respond to medication.
CT scans show brain atrophy. Many of those who are ill have blurred vision, memory problems, gnashing of teeth, hair loss and balance problems. Some of them, including those in palliative care and heavily medicated, have uncontrollable muscle spasms. Others lose weight suddenly and quickly and develop muscle atrophy.
Symptoms include hallucinations, including tactile, and “terrifying hallucinatory dreams.”
Patients are between 18 and 85 years old and almost all of them had their first symptoms in 2018. Only one of them became ill in 2015, but then he could not be diagnosed with any disease.
“Their suffering is immense, because it goes beyond physical ailments. They also experience a neuropsychiatric and moral suffering that can only be partially alleviated by medication, ”says Marrero.
“The speed with which these constellations of symptoms developed is something I have never seen before,” said Michael Strong, who leads the Canadian Institutes of Health Research.
The daughter of a 75-year-old patient who arrived at the emergency room last year says the woman began to lose weight and have a “trembling sensation” in her body, her legs were heavy and one arm was shaking involuntarily.
“My mother goes to bed every night thinking, ‘I’ll wake up tomorrow, and if I’m still alive, can I go and talk?’ Because no one has answers, no one knows anything about the disease. There is no logic, we have nothing “.
The case cluster was detected by the federal public agency that monitors Creutzfeldt-Jakob disease and other prion degenerative diseases triggered by prions, infectious agents made up only of proteins with an abnormal structure that can also affect healthy proteins. They accumulate in the brain and cause abnormal folding of healthy proteins.
Under the microscope, the brains of animals and people with prion diseases look like porous sponges.
Michael Coulthart, head of the monitoring system, said he reported suspicious cases each year, but only a few cases were confirmed to be Creutzfeldt-Jakob syndrome: A total of 36 “clear and probable” cases of the disease were identified in New Province. Brunswick since 1998.
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Marrero saw the first case in 2015, but could not diagnose it. From 2018, patients with similar symptoms began to appear. In 2019, another 11 cases were identified from the unknown neurological syndrome cluster. In 2020, the number of patients will reach 24.
“We say there are cases that are resistant to diagnosis. They appeared as a pattern before we talked about a cluster, “says Coulthart.
Researchers believe the syndrome has been incubating for two years. They have not yet determined a cause and are considering any clue to find it, from exposure to something in the environment to something they have taken on a journey and food style.
Marrero tested them for infectious diseases transmitted from animals, looked for autoimmune diseases, metabolic deficits, cancer cells and genetic diseases.
And the protein markers in the cerebrospinal fluid came out negative for Creutzfeldt-Jakob syndrome.
Brain autopsies of three of the patients who died did not provide any evidence of a known prion disease. Molecular tests are underway.
“All these cases, given their description, should be CJD. That’s how it sounds and that’s how it looks, and yet the tests are negative, “Strong explained.
Among the advanced theories are a new prion disease and an environmental toxin. One of the toxins investigated is β-Methylamino-L-alanine, produced by cyanobacteria or blue-green algae.
Another is domoic acid, a toxin that is naturally present in the environment and produced by certain types of algae – in fact, it caused a deadly outbreak of intoxication in Canada in 1987.
In Caraquet, a town of about 4,200 people on the Acadian Peninsula, Mayor Kevin Haché said “the biggest problem is the unknown.”
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