Abstract
Real‐time quaking‐induced conversion (RT‐QuIC) has been proposed as a sensitive diagnostic test for sporadic Creutzfeldt–Jakob disease; however, before this assay can be introduced into clinical practice, its reliability and reproducibility need to be demonstrated. Two international ring trials were undertaken in which a set of 25 cerebrospinal fluid samples were analyzed by a total of 11 different centers using a range of recombinant prion protein substrates and instrumentation. The results show almost complete concordance between the centers and demonstrate that RT‐QuIC is a suitably reliable and robust technique for clinical practice. Ann Neurol 2016;80:160–165
Highlights
A new approach to the premortem diagnosis of sporadic CJD (sCJD) has been to exploit the ability of small amounts of cerebrospinal fluid (CSF) PrPSc to convert native prion protein (PrP) into PrPSc in a newly described protein aggregation assay known as real-time quaking-induced conversion (RT-QuIC)
A negative Real-time quaking-induced conversion (RT-QuIC) result was obtained in the CSF of 1 sCJD case, which had a disease duration of 12 months
In the first ring trial, 6 of 7 laboratories correctly identified all sCJD cases; 1 laboratory obtained a negative CSF RT-QuIC result from an sCJD patient with a disease duration of 12 months
Summary
Current diagnostic criteria for sCJD rely on clinical features, the results of electroencephalography and magnetic resonance imaging, and the presence of 14-3-3 protein in the cerebrospinal fluid (CSF).1,2 These tests are not specific for CJD, and none is able to detect all forms of CJD.. A new approach to the premortem diagnosis of sCJD has been to exploit the ability of small amounts of CSF PrPSc to convert native PrP into PrPSc in a newly described protein aggregation assay known as real-time quaking-induced conversion (RT-QuIC). This technique uses recombinant PrP (rPrP) as a substrate, which is induced to aggregate by the addition of CSF containing
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