Doctors investigate mysterious brain disease cluster in Canada

Creutzfeldt-Jakob disease is a degenerative brain disorder that causes personality changes, anxiety, depression and memory loss. The disorder, which can occur spontaneously or be inherited, can eventually lead to dementia and death.

Officials in Canada are trying to find the cause of a mysterious brain disease, which resembles Creutzfeldt-Jakob disease, and has affected more than 40 people in the New Brunswick province.

According to a report by Live Science, Canadian officials first alerted doctors in the New Brunswick area that they were evaluating 43 cases of an unknown neurological disease. Even though the first case was identified back in 2015, there have been a rising number of cases in recent years, with 24 cases reported in 2020 and six reported so far this year. Five deaths have also been associated with the disease insofar.

Although doctors first speculated the cases were Creutzfeldt-Jakob disease, tests conducted so far do not show any evidence of the disease. Creutzfeldt-Jakob disease is caused by prions, a type of protein that can cause normal proteins in the brain to fold abnormally. Prion diseases, which can affect both humans and animals, can sometimes be spread to humans through infected meat products.

“There is no evidence, not a hint — even in the three autopsies that have been performed — of a human prion disease,” Dr. Neil Cashman, a professor at the University of British Columbia, told CBC. “That came as a surprise to me, frankly.”

Patients with the illness have developed progressively worsening symptoms like unexplained pains, spasms, cognitive decline, muscle wasting and chattering teeth. Most of the cases are in the Acadian Peninsula in northeast New Brunswick and near Moncton, a city in southeast New Brunswick.

Multiple researchers are now racing to identify the cause of the disease, which may be due to a novel illness. 

Since the cases are localized to a certain region, the disease may be caused by environmental toxins, such as B-methylamino-L-alanine (BMAA) and domoic acid, two both toxins that can build up in fish and shellfish.

“It’s possible ongoing investigations will give us the cause in a week, or it’s possible it will give us the cause in a year,” Cashman noted.

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