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In a large trial, a test to detect the abnormal prion protein associated with variant Creutzfeldt-Jakob disease (vCJD) performed well enough to conclude that it can be used to screen populations at risk for the disease, scientists reported in JAMA Neurology.
Variant CJD, a degenerative brain disease, is the human counterpart of bovine spongiform encephalopathy (BSE, or mad cow disease), which, the report notes, may have infected as many as 3 million cattle in the United Kingdom.
The authors, led by Graham S. Jackson, PhD, of the University College of London Institute of Neurology, used the test on blood samples from 5,000 US blood donors, 200 healthy British donors, 352 patients with nonprion neurodegenerative diseases, 105 patients deemed likely to have prion diseases, and 10 patients with confirmed vCJD.
The test showed 100% specificity (no false-positives) in the US donors (who were presumed to be free of prion disease) and in the healthy British donors. Likewise, it produced no false-positives among the patients with nonprion neurodegenerative diseases.
Among patients believed likely to have a prion disease, two who had sporadic CJD tested positive (98.1% specificity; 95% confidence interval [CI], 93.3% to 99.8%). And in a small set of samples from unaffected persons and patients known to have vCJD, the test showed 70% sensitivity (95% CI, 34.8% to 93.3%), which reconfirmed previous findings.
The authors said the test performed well enough to justify using it to screen a large sample of the British population and at-risk groups to estimate the prevalence of blood prions among British blood donors. Such an estimate would help policy makers decide whether routine vCJD screening is needed for blood, tissue, and organ donations and for patients facing high-risk surgical procedures.
Michael Wishart
CNC Infection Control
Holy Spirit Northside Private Hospital
627 Rode Road, Chermside, Qld 4032
t: (07) 3326 3068 | f: (07) 3326 3523
e: Michael.Wishart@hsn.org.au
w:www.holyspiritnorthside.org.au
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