New CJD-type disease kills ten in US
Thursday, 10 Jul 2008 09:00
16 people have been affected by the CJD-type disease in the US
Scientists have discovered a new disease that is similar to CJD (Creutzfeldt-Jakob disease) and has killed ten people in the United States.
In total the disease is thought to have affected at least 16 people.
The form of fatal dementia causes patients to lose their ability to think, speak and move.
While these effects are similar to those caused by CJD, a report in the New Scientist claims that the new disease has features that make it distinct from known forms of CJD.
Dr Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Centre at Case Western Reserve University, Ohio, told the magazine that he believes the disease has been around for many years unnoticed, with some cases mistaken for other forms of dementia.
While six of the cases investigated by Dr Gambetti were from families with a history of dementia, suggesting a genetic cause, there were no mutations in their PrP (brain protein) genes.
Some very rare forms of CJD run in families and are caused by mutations in the gene for PrP.
Dr Gambetti is now conducting experiments in mice to see how the disease is transmitted, saying at present there is no cause for alarm.
"I believe the disease occurs naturally, and is not due to environmental causes," he said.