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Updated: Feb 28, 2026
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Prion Safety Laboratory Swipe Test
Published on: February 14, 2025
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Human Prion Disease: Pathogenesis, Diagnosis and Public Health
Paola Bellini1, Francesco Ruggiero2, Andrea Benedetti3
1Division of Infectious Diseases, Ente Ospedaliero Cantonale, 6900 Lugano, Switzerland.
Viruses
|February 27, 2026
Summary
Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins (PrPSc). While diagnostic tools like RT-QuIC improve detection, effective treatments remain elusive, necessitating ongoing research and surveillance.
Area of Science:
- Neurology
- Infectious Diseases
- Protein Misfolding Diseases
Background:
- Prion diseases are rare, progressive, and fatal neurodegenerative disorders.
- Characterized by infectious misfolded prion protein (PrPSc) inducing conformational changes in physiological PrPC.
- Significant clinical and therapeutic challenges persist despite scientific advances.
Purpose of the Study:
- To review the current scientific literature on prion diseases.
- To highlight diagnostic advancements and therapeutic challenges.
- To discuss the implications of iatrogenic cases and infection control.
Main Methods:
- Conducted a narrative literature review across major biomedical databases (PubMed, Scopus, Web of Science, Google Scholar) up to January 2025.
- Included an illustrative clinical case of iatrogenic Creutzfeldt-Jakob disease.
- Focused on diagnostic uncertainty and infection-control aspects.
Main Results:
- PrPSc is confirmed to drive neurodegeneration and transmissibility, influenced by genetic and phenotypic variants.
- Neuroimaging and cerebrospinal fluid biomarkers have improved, with RT-QuIC showing high specificity and sensitivity.
- No current treatment significantly impacts survival, but novel strategies are under investigation.
Conclusions:
- Prion diseases present a complex challenge at the intersection of neurology and infectious diseases.
- Earlier diagnosis via advanced biomarkers and development of targeted therapies are crucial for patient management.
- The persistence of iatrogenic cases emphasizes the need for continued surveillance and prevention strategies.
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