Rapid Testing for Creutzfeldt-Jakob Disease in Donors of Cornea

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Abstract

Background

Creutzfeldt-Jakob disease (CJD) has been accidentally transmitted by contaminated corneal transplants. Eye donors are not ordinarily tested for CJD, in part because an easy test is not available. We propose a relatively simple postmortem procedure to collect brain samples without performing full autopsy and show that a test currently marketed for veterinary diagnosis would offer an effective screening test.

Methods

We selected 6 brains from confirmed cases of human sporadic CJD and sampled each in triplicate (18 specimens), 28 control brains of individuals with non-CJD neurodegenerative diseases and 10 normal brains. We also applied a procedure involving retro-orbital puncture after enucleation and biopsied the frontal lobes and optic nerves of a macaque experimentally infected with variant CJD. All samples were tested with the IDEXX HerdChek BSE-Scrapie Ag Kit to detect the abnormal prion protein, PrPTSE.

Results

The test discriminated between control and CJD-infected brains. All 18 infected brain samples diluted to 0.1%, except one, showed signals above cutoff, and a number of samples were reactive at even higher dilutions. These results suggest the test could detect the low concentrations of PrPTSE probably present in brains of donors at early stages of CJD. Our collection procedure obtained sufficient macaque brain and optic nerve tissues to detect PrPTSE.

Conclusions

We showed that a commercial test combined with rapid sample collection might offer a practical solution to screen brains of cornea donors for evidence of CJD. Such a test might enhance safety of corneal transplants and some other tissue-derived products.

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