Ten Americans have died of a new form of fatal dementia which resembles Creutzfeldt-Jakob disease (CJD).

The disease, which sees patients gradually losing the capacity for thought, speech and motion, has been identified in a total of 16 patients.

Features of the new neurodegenerative disorder mark it out from known forms of CJD, and neither its provenance nor the conditions which allow it to spread are known.

"I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Centre at Case Western Reserve University in Cleveland, Ohio.

Gambetti's team first wrote a paper describing 11 cases referred to their centre for Annals of Neurology volume 63, and since then a further five have been discovered with the telltale tiny holes in the brain tissue.

In previous forms of the disease, prions which were thought to be responsible for the damage resisted degradation by proteases, but prions found in patients with the new disease are broken down by the proteases.

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