Prion diseases are a group of rare, invariably fatal brain disorders which occur both in humans and certain animals.
In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one person per million per year. In the United States this translates to 250-300 new cases per year.
It is well known that CJD is very difficult to diagnose leading to speculation that the one case per million report may be incorrect.
You can learn more about Creuzfeldt-Jakob disease from the pamphlet Creuzfeldt-Jakob Disease and Other Prion Diseases (in PDF format) from the Creuzfeldt-Jakob Foundation.
You will need the free Adobe Reader to view this pamphlet. If you do not already have it, click here to get it.

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