One case of rare brain disease confirmed in B.C.

One case of a rare, degenerative brain disease has been confirmed in British Columbia's Fraser Valley, and a health official says two other cases are now suspected.

However Paul Van Buynder, chief medical officer for the Fraser Health authority, said late Friday that even if the two suspected cases end up being Creutzfeldt-Jakob disease it's not "drastically unusual."

The confirmed case of Creutzfeldt-Jakob disease, or CJD, was a resident in a long-term care home who died a year ago, Van Buynder said.

He said the two other patients suspected of having Creutzfeldt-Jakob disease are currently hospitalized. None of the patients are related; all lived in different towns.

The findings stem from a review officials conducted after they initially believed there were six CJD cases in the Fraser Valley, east of Vancouver, over the past year.

Buynder said it's "highly unlikely" that the remaining three cases will be confirmed as CJD.

Cases are only confirmed after a sample of , typically taken from the patient post-mortem, is tested at a national laboratory, he said.

Before that happens, doctors make an initial diagnosis based on , blood tested for specific proteins and an , a test that measures the brain's activity.

Only 30 to 50 cases are reported annually in Canada, and five of those cases are expected to occur in British Columbia, he said.

"I think the public really need to know that this is probably one or two more than I expect, but it's not drastically unusual," said Van Buynder.

"It's not . It has nothing to do with the . Neither the public nor anybody in our hospitals should be worried that they're about to get this nasty disease."

Provincial health officer Dr. Perry Kendall said CJD causes the rapid onset of dementia, leading to a coma usually within a six-month time frame, because agents known as preons destroy the brain.

One strain has been linked with mad cow disease, but Van Buynder said more than 90 percent of the cases are sporadic and health officials don't know its cause, and they have no treatment or cure.

"It's a tragic disease that is rapidly progressing," he said. "It's a degenerative disease of the brain and usually within six months they've passed away. It's a tragic disease for families, and we just really don't understand it."

not rated yet
add to favorites email to friend print save as pdf

Related Stories

Blood test for human form of mad cow disease developed

Jan 16, 2012

(Medical Xpress) -- Mad cow disease is serious business in the U.K., the human form, known as Creutzfeldt-Jakob after Hans Gerhard Creutzfeldt and Alfons Maria Jakob (CJD), who independently first described its existence ...

Recommended for you

In the battle against Ebola, a double-layer solution

2 hours ago

When working with Ebola patients, protective gear works, but removing it can be harrowing. Seeking to protect health care workers from the precarious nature of taking off soiled gloves, Cornell students have ...

New hope for rare disease drug development

13 hours ago

Using combinations of well-known approved drugs has for the first time been shown to be potentially safe in treating a rare disease, according to the results of a clinical trial published in the open access Orphanet Journal of ...

Three weeks since last Ebola case in Mali: WHO

16 hours ago

Mali has not had a case of Ebola for three weeks, the World Health Organization said Wednesday, completing one of the two incubation periods the country needs to be declared free of the virus.

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.