Enhanced Sensitivity of a Modified Quaking‐Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study
These findings demonstrate that adapting EP-QuIC with an optimized substrate, termed enhanced sensitivity QuIC (ES-QuIC), significantly improves diagnostic performance for inherited and atypical prion diseases. By expanding the diagnostic reach of QuIC tests, this study strengthens antemortem surveillance, reduces reliance on postmortem confirmation, and improves opportunities for early intervention and clinical trial enrollment, particularly for genetic cases most likely to benefit from emerging therapeutic strategies. ANN NEUROL 2026.
Authors
- Rebecca Fox
- Clark Phillipson
- Dominic M. S. Kielich
- Jessy A. Slota (ORCID: https://orcid.org/0000-0002-9025-8368)
- Samuel Booth (ORCID: https://orcid.org/0000-0003-2450-8792)
- Lise Lamoureux
- Jennifer Myskiw
- Melanie Leonhardt
- Kristen Avery
- Sharon L. R. Simon (ORCID: https://orcid.org/0000-0001-8683-7749)
- Brian S. Appleby (ORCID: https://orcid.org/0000-0003-2275-3292)
- Kathy Frost
- Olivia Nykvist
- Ben A. Bailey‐Elkin
Institutions
- Manitoba Health (CA)
- Public Health Agency of Canada (CA)
- University Hospitals of Cleveland (US)
- University of Manitoba (CA)
- Case Western Reserve University (US)
Publication Details
- Journal
- Annals of Neurology
- Published
- 2026-01-26
- DOI
- https://doi.org/10.1002/ana.78162
- Primary Topic
- Prion Diseases and Protein Misfolding
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Public Health Agency of Canada
- Public Health Agency
- Centers for Disease Control and Prevention