- Jessica Murphy
- BBC – Toronto
Doctors in Canada have recently received cases of patients with symptoms similar to those of Creutzfeldt-Jakob disease, a rare and dangerous disease that affects the brain. But after careful examination and examination of the cases, the doctors’ confusion increases.
About two years ago, Roger Ellis suddenly collapsed and spased on his 40th wedding day.
Ellis was in his early 60s, and was born and raised in rural New Brunswick, on the Acadian Peninsula. He was in good health until last June and is enjoying his retirement after working as a mechanic.
His son, Steve Ellis, says his father’s health has been deteriorating very quickly since that day.
“He has hallucinations and fantasies, and he loses weight, and he becomes aggressive, and he repeats his words a lot.”
He continued, “It reached the point that he could not speak at some point. Within three months, he was taken to the hospital where they told us that they thought he was dying, but no one knew the reason.”
In the beginning, doctors suggested that he was afflicted with “Creutzfeldt-Jakob” disease, which is a disease associated with prion, which is a protein molecule that causes infection with such diseases that affect the nervous system mostly. It causes a rare and dangerous disorder in the brain, symptoms of which include memory loss, change in behavior and difficulties in communication.
One of the stages of this disease is related to eating mad cow meat. Creutzfeldt-Jakob disease belongs to the category of brain diseases similar to Alzheimer’s, Parkinson’s disease and amyotrophic lateral sclerosis, which leads to protein imbalance and blockage in the nervous system.
But tests confirmed that Ellis did not have Creutzfeldt-Jakob disease, as well as other tests that doctors conducted to find the cause of his illness.
And his son says that the medical team did everything they could to overcome his father’s symptoms, but something that caused Ellis’s health to deteriorate remained like a little bit.
And last March, Ellis Jr. found an answer, perhaps in part.
Radio Canada obtained a copy of a public health note sent to health sector workers, warning them about the emergence of a group of patients with an unknown disease in the brain.
“The first thing that came to my mind was that this was my father’s condition,” he said.
Roger Ellis is now one of the most severe sufferers of unknown disease, and is under the care of neurologist Dr. Alier Marrero.
Marrero works at Dr. George El Dimon University Hospital in Moncton, and says that the first case doctors detected for this disease was in 2015, and that it was one “anomalous and unusual” case.
Cases have increased since then, enabling doctors to monitor a range of cases as a different disease “they had not seen before.”
The province says it has detected 48 cases so far, half of them men and the other half of women, in the age groups between 18 and 85 years. These cases are concentrated in the Acadian Peninsula and the city of Moncton in New Brunswick. Six people are believed to have died from this disease.
Most patients showed symptoms soon, starting in 2018, although one of them developed symptoms in 2013.
Dr. Marrero says symptoms vary widely from patient to patient.
At first, it may come in the form of a change in behavior, such as anxiety, frustration and distress, and then an unexplained pain, such as muscle pain and cramps, that affects previously healthy individuals.
Over time, patients experience sleep difficulties, whether severe insomnia and prolonged sleep, in addition to memory problems. It can be accompanied by increased speech problems, such as stuttering or repetitive speech, making it difficult to communicate and engage in a conversation.
Also among the symptoms are massive weight loss and muscle atrophy, as well as blurred vision, inability to communicate, and involuntary muscle twitching.
Some people may experience hallucinations in dreams, or hallucinations in waking life.
Many patients also developed Capgras syndrome, a psychological condition that makes the sufferer think that someone is impersonating someone close to him.
Dr. Marrero says, “It is very difficult. For example, a patient may tell his wife that she cannot share his bed with him because he is married. When she tells him that she is his wife, he tells her that she is not his real wife.”
Marrero leads the team of doctors looking into cases, with the help of a team of researchers and the Federal Public Health Agency.
Patients undergo a series of disease tests, tests to detect any genetic abnormalities, tests for autoimmune diseases or cancer, and scans for various types of viruses, bacteria, fungi, heavy metals and unusual antibodies.
Research also includes looking at environmental factors, lifestyle, travel, medical history, and sources of food and drink. This is along with samples from the spine to test for any infection or disease.
There is no treatment except to help relieve some of the symptoms. So far, it is likely that the disease is acquired and has no genetic cause.
“Our main idea so far is that there is a toxic agent acquired from the surrounding environment that causes these changes in the patient,” says Dr. Marrero.
Neurologist, Dr. Neil Cashman, of the University of British Columbia, is part of the team studying this medical practice.
Although there is no trace of a previous infection with prion disease (associated with a prion protein), the team does not rule out the possibility that it is the solution to the puzzle.
Another theory suggests the presence of permanent exposure to substances that cause excessive stimulation of neurons “oxytotoxin”, such as domoic acid, which was linked to the accident of food poisoning that occurred in 1987 due to contaminated shellfish with a toxin that leaked from neighboring Prince Edward Island province.
Besides irritation of the stomach and intestines, about a third of those infected at the time experienced symptoms such as memory loss, dizziness and confusion. Some patients fell into coma, and four died.
Dr. Cashman says they are also looking at another toxin, beta-methylamino-L-alanine (PMA), which is considered an environmental hazard that causes diseases like Alzheimer’s and Parkinson’s.
It secretes bacteria ( cyanobacteria) Or cyanobacteria, this substance, usually known as blue-green algae.
Some researchers believe that beta-methylamino-L-alanine is linked to a neurodegenerative disease that was detected in one of the indigenous communities on the American island of Guam in the middle of the decade and it was found in food pills that were part of their diet.
But Dr. Cashman warns that the theories currently put forward are “incomplete”.
“We must go back to the basic principles, to the starting point. At this stage, no possibility can be ruled out,” he says.
How many individuals who may be affected by this disease?
Dr. Marrero says it is possible that the phenomenon is widespread and is present in areas outside the two regions where they were discovered, namely the Acadian Peninsula, the fishing area and sandy beaches, and Moncton, where cases were recently discovered.
“Maybe what we are seeing is the tip of the iceberg. I hope we can reveal this quickly so that we can stop it,” he adds.
Residents of the affected areas live in a state of anxiety, which is understandable. But Marrero appeals to people to “act with hope, not fear, for fear paralyzes.”
Roger Ellis is now stable after he was getting better quickly, his son said.
Roger Ellis currently lives in a private care home, needs some help with his daily activities, has difficulties speaking and sleeping.
Steve Ellis runs a Facebook page to support families affected by the injuries, and says he wants government officials to be transparent about the disease.
Above all, he wants to know what made his father sick.
“I know they are working on it. But how did it happen?”
He added: “As a family, we are agonized by the idea that he will often die of this disease, and we only hope to know the answers, whether before or after his death, as well as holding the responsible accountable if it turns out to be something that could have been prevented.”