Estimate of variant CJD derived from pathology samples

A prevalence of variant Creutzfeldt-Jakob disease among people aged 10-50 in 1995-9 of 120 per million has been derived from a study of tonsil and appendix samples. David Hilton and colleagues tested stored samples of appendixes and tonsils from UK pathology departments, looking for the lymphoreticular accumulation of prion protein. They also examined 20 appendix samples removed at autopsy from patients with variant CJD, and found that 19 showed such accumulation of prion protein (p 633). Among the 8318 samples from people aged 10-50, one showed accumulation of prion protein, giving an estimated detectable prevalence of 120 per million (95% CI 0.5 to 900).

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Relevant Article

Accumulation of prion protein in tonsil and appendix: review of tissue samples

David A Hilton, Azra C Ghani, Lisa Conyers, Philip Edwards, Linda McCardle, Mark Penney, Diane Ritchie, and James W Ironside
BMJ 2002 325: 633-634. [Full Text] [PDF]