Canadian doctors have come across patients with symptoms similar to Creutzfeldt-Jakob disease, a rare and deadly condition that attacks the brain. But when they scrutinized, what they found confused them.
Almost two years ago, Roger Ellis celebrated his 40th wedding anniversary and collapsed at home.
Born and raised on the idyllic Acadian Peninsula in New Brunswick in the early 1960s, Ellis was healthy until June and enjoyed retirement after decades of working as an industrial mechanic.
His son, Steve Ellis, says his father’s health declined rapidly after that fateful day.
“He had delusions, hallucinations, weight loss, aggression, and repetitive speech,” he says.
“At some point he couldn’t even walk, so during the three months we were taken to the hospital and told him he believed he was dying, for no reason. I did not know.”
Roger Ellis’s doctor first suspected Creutzfeldt-Jakob disease [CJD].. CJD is a human prion disease, a fatal and rare degenerative brain disorder in which patients present with symptoms such as memory loss, behavioral changes, and difficulty adjusting.
One of the most well-known categories is variant CJD, which is associated with eating contaminated meat infected with mad cow disease. CJD also belongs to a broader category of brain disorders such as Alzheimer’s disease, Parkinson’s disease, and ALS, where proteins in the nervous system are accidentally folded and aggregated.
However, Ellis’ CJD test returned negative, as did the barrage of other tests that doctors passed him when he tried to determine the cause of his illness.
His son says the medical team did his best to alleviate his father’s various symptoms, but the mystery still remains. What was behind Mr. Ellis’ decline?
In March of this year, young Ellis came across a partial but possible answer.
Public Broadcasting Radio Canada has obtained a copy of a public health memo sent to a state health professional warning a population of patients with unknown degenerative brain disease.
“The first thing I said was,’This is my dad,'” he recalls.
Roger Ellis is now believed to be one of those suffering from the disease and is under the control of Dr. Arie Marello.
A neurologist at the Dr Georges-L-Dumont University Hospital Center in Moncton states that doctors first encountered a mysterious illness in 2015. At that time, it was a patient, an “isolated atypical case.”
However, since then, the number of patients like the first has increased. Doctors can now identify the cluster as another condition or syndrome that has never been seen before.
The state says it is currently tracking 48 cases, evenly divided into men and women, between the ages of 18 and 85. These patients come from the Acadian Peninsula and Moncton region of New Brunswick. It is believed that six people died of illness.
Most patients have recently begun to experience symptoms since 2018, but it is believed that they did appear as early as 2013.
Dr. Marello states that the symptoms are widespread and can vary from patient to patient.
Initially, there are behavioral changes such as anxiety, depression, and irritability, followed by unexplained pain, muscle aches, and cramps in previously healthy individuals.
Patients often develop sleep disorders (severe insomnia or hypersomnia) and memory problems. Problems such as stuttering and word repetition can lead to rapidly developing speech disorders that make communication and fluent conversation difficult.
Other symptoms are rapid weight loss and muscle atrophy, visual impairment and coordination problems, and involuntary muscle spasms. Many patients need the help of pedestrians and wheelchairs.
Some people develop disturbing hallucinatory dreams, or awakening hallucinations.
Some patients showed transient “capgras delusions”. This is a mental illness that people believe has been replaced by fraudsters.
“For example, I’m very anxious because the patient tells his wife,” I’m sorry, I can’t go to bed. I’m a married man. ” Even if his wife gives her a name, he says: “It’s not real,” says Dr. Marello.
A Moncton-based neurologist is leading the investigation of the condition with the help of a team of researchers and a federal public health agency.
Suspicious patients are screened for prion disease and genetic status, panels for autoimmune disease or cancer morphology, viruses, bacteria, fungi, heavy metals, and abnormal antibodies.
They are asked about environmental factors, lifestyle exposure, travel, medical history, food and water sources. They undergo a spinal puncture to test for a variety of possible infections and disorders.
There is no cure other than helping to relieve the discomfort of some symptoms. For now, the theory is that the disease is acquired rather than genetic.
“Our first general idea is that the toxic elements acquired in this patient’s environment cause degenerative changes,” says Dr. Marello.
Dr. Neil Cashman, a neurologist at the University of British Columbia, is one of the researchers trying to unravel the mysteries of medicine.
He says that even though the patient shows no evidence of known prion disease, it has not been completely ruled out as a cause.
Another theory is chronic exposure to what is called “excitotoxicity”, such as domoic acid. This was related to a 1987 food poisoning incident with toxin-contaminated mussels from the nearby state of Prince Edward Island.
In addition to gastrointestinal upset, about one-third of affected people showed symptoms such as amnesia, dizziness, and confusion. Some patients fell into a coma and four died.
Dr. Cashman said he is also considering another toxin, betamethylamino-L-alanine (BMAA), which is associated with environmental risk in the development of diseases such as Alzheimer’s disease and Parkinson’s disease.
BMAA is produced by cyanobacteria, commonly known as blue-green algae.
Some researchers also found that BMAA was associated with neurodegenerative diseases found in seeds that were recorded in the territory of the US islands of the Pacific Ocean in Guam in the mid-20th century and were part of their diet. I believe there is.
Dr. Cashman warns that the current list of theories is “not complete.”
“We have to go back to first principles and back to square principles,” he says. “At this point, basically nothing can be excluded.”
So how many more people are affected by this disease?
Dr. Marello says it could be a broader phenomenon seen outside the two regions-the fishing community and the sandy Acadian Peninsula, and Moncton in the city center where patients are currently identified.
“Are we looking at the tip of the iceberg? Maybe,” he says. “I want to be able to capture this very quickly and stop it.”
People living in affected communities are naturally worried, but Dr. Marello encourages people to “work with hope without fear. Paralysis of fear.”
Roger Ellis’s condition has been stable since the early rapid progression, his son says.
He is in a specialized care facility, needs help in daily life and suffers from conversation and sleep problems.
Steve Ellis, who runs a Facebook support group for sick-affected families, wants government officials to promise transparency about their illness.
Best of all, he wants to know why his father got sick.
“I know they’re working on it, but how did this happen?” He says.
“As a family, we are very aware of the fact that he could die from this. The answer was whether it happened before or after he died, which could be prevented. If so, I hope you are accountable. “”