B.C. officials confirm case of rare brain disease

VANCOUVER — 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 CreutzfeldtJakob 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 in hospital. None of the patients are related and they all resided in different towns, Van Buynder added.

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” the remaining three cases will be confirmed as CJD.

Cases can only be confirmed after a sample of brain tissue, taken from the patient post-mortem, is tested at a national laboratory, he said.

Before that happens, doctors make an initial diagnosis based on MRI images, blood tested for specific proteins and an electroencephalogram, 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 B.C., he said. “It’s not mad cow disease. It has nothing to do with the food chain. Neither the public nor anybody in our hospitals should be worried that they’re about to get this nasty disease,” Van Buynder said.

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