Personal Injury Law

CJD-Like Form Of Fatal Dementia Discovered By Prion Center

We have to admit we do not see much of the New Scientist.

Since this article is sourced back to The National Prion Disease Pathology Surveillance Center (NPDPSC), we are going with it.   We'll report, but let you decide!

A NEW form of fatal dementia has been discovered in 16 Americans, ten of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

As in other spongiform encephalopathies, such as CJD and mad cow disease (BSE), the brain tissue of victims is full of tiny holes. This damage is thought to be caused by the accumulation of prions, misfolded versions of a brain protein called PrP that can convert normal PrP molecules into their own misshapen form.

Go here for the rest of the report.

According to its website, the Prion Center has examined only two CJD cases since 1997, one originated in Saudi Arabia and the other in the United Kingdom. 

Full post as published by Mad Cow Blog on July 10, 2008 (boomark / email).