Analysis of 2000 consecutive UK tonsillectomy specimens for disease-related prion protein.
Variant Creutzfeldt-Jakob disease (CJD) is thought to be caused by dietary or other exposure to bovine spongiform encephalopathy (BSE) prions. The prevalence of preclinical or subclinical prion infection in the UK is currently unknown. Since clinical variant CJD is uniformly associated with tonsillar prion infection, we screened 2000 anonymous surgical tonsillectomy specimens for disease-associated prion protein. Analysis by both high sensitivity immunoblotting and immunohistochemistry detected no positive cases. However, this negative result cannot provide reassurance that relevant community infection is unlikely because of the fairly small sample size, demographic and age-related factors, and unknown test sensitivity during the prolonged incubation period. Nevertheless, our findings establish a protocol for prevalence screening on a national scale.
MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
Adam
A
Frosh
Lorraine C
LC
Smith
Carl J
CJ
Jackson
Jacqueline M
JM
Linehan
Sebastian
S
Brandner
Jonathan D F
JD
Wadsworth
John
J
Collinge
D000818:Animals; D001923:Brain Chemistry; D002648:Child; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000453:epidemiology; D006224:Cricetinae; D006801:Humans; D015151:Immunoblotting; D007150:Immunohistochemistry; D014066:Palatine Tonsil / Q000737:chemistry*; D018620:PrPSc Proteins / Q000032:analysis; D015995:Prevalence; D017096:Prion Diseases / Q000175:diagnosis / Q000453:epidemiology; D011328:Prions / Q000302:isolation & purification*; D014068:Tonsillectomy; D006113:United Kingdom / Q000453:epidemiology
D018620:PrPSc Proteins; D011328:Prions