
Sporadic is the most prevalent of the Human Prion Diseases, accounting for ~85% of all those identified. The cause of Sporadic human Prion Disease is not known, but it is thought to be due to spontaneous misfolding of the normal prion protein (PrPc).
Transmitted by infection due to exposure to the infectious prion (PrPSc) from transplant of contaminated tissues, or use of contaminated instruments during specific surgical procedures. Iatrogenic internationally account for less than 0.1%.
Since the first report in 1996, a total of 229 vCJD cases have been reported worldwide: 177 in the United Kingdom; 27 in France; and 25 distributed in 10 other countries, including Ireland (4).
Genetic forms of human Prion Diseases (also called familial or inherited) are caused by mutations in the prion protein gene PRNP. They account for approximately 15% of all human Prion Diseases.