Mysterious brain disease baffles doctors


Doctors in Canada have been coming across patients showing symptoms similar to that of Creutzfeldt-Jakob disease, a rare fatal condition that attacks the brain. But when they took a closer look, what they found left them stumped.

Almost two years ago, Roger Ellis collapsed at home with a seizure on his 40th wedding anniversary.

In his early 60s, Mr Ellis, who was born and raised around New Brunswick’s bucolic Acadian peninsula, had been healthy until that June, and was enjoying his retirement after decades working as an industrial mechanic.

His son, Steve Ellis, says after that fateful day his father’s health rapidly declined. “He had delusions, hallucinations, weight loss, aggression, repetitive speech,” he says. “At one point he couldn’t even walk.

So in the span of three months we were being brought to a hospital to tell us they believed he was dying – but no one knew why.”

Roger Ellis’ doctors first suspected Creutzfeldt-Jakob disease [CJD]. CJD is a human prion disease, a fatal and rare degenerative brain disorder that sees patients present with symptoms like failing memory, behavioural changes and difficulties with co-ordination.

One widely known category is Variant CJD, which is linked to eating contaminated meat infected with mad cow disease.

CJD also belongs to a wider category of brain disorders like Alzheimer’s, Parkinson’s and ALS, in which protein in the nervous system become misfolded and aggregated.

How BSE crisis shook our faith in food But Mr Ellis’ CJD test came back negative, as did the barrage of other tests his doctors put him through as they tried to pinpoint the cause of his illness.

His son says the medical team did their best to alleviate his father’s varying symptoms but were still left with a mystery: what was behind Mr Ellis’s decline?—AP