ArticleMolecular neurobiology2018
Validation of α-Synuclein as a CSF Biomarker for Sporadic Creutzfeldt-Jakob Disease.
Article in Molecular neurobiology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 44 citations in OpenAlex.
- Cerebrospinal Fluid Biomarkers in Creutzfeldt-Jakob Disease: Diagnostic Value, Limitations, and Future Multi-Omics Strategies.International journal of molecular sciences · 2026Review
- Prion protein (PrP) profiles in blood and CSF: insights into pre-symptomatic and symptomatic prion disease.Brain communications · 2026Article
- Ubiquitin C-hydrolase L1 as a cerebrospinal fluid and blood biomarker for prion diseases.Brain communications · 2026Article
- Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety.Sub-cellular biochemistry · 2025Review
- Diagnosis of Prion Diseases.Sub-cellular biochemistry · 2025Review
- Baseline Cerebrospinal Fluid α-Synuclein in Parkinson's Disease Is Associated with Disease Progression and Cognitive Decline.Diagnostics (Basel, Switzerland) · 2022Article
- A new paradigm for diagnosis of neurodegenerative diseases: peripheral exosomes of brain origin.Translational neurodegeneration · 2022Review
- Diagnostic accuracy of cerebrospinal fluid biomarkers in genetic prion diseases.Brain : a journal of neurology · 2022Article
- Role of Biomarkers for the Diagnosis of Prion Diseases: A Narrative Review.Medicina (Kaunas, Lithuania) · 2022Review
- Comparison of cerebrospinal fluid tau, ptau(181), synuclein, and 14-3-3 for the detection of Creutzfeldt-Jakob disease in clinical practice.Journal of neural transmission (Vienna, Austria : 1996) · 2022Article
- Sporadic Creutzfeldt-Jakob disease: Real-Time Quaking Induced Conversion (RT-QuIC) assay represents a major diagnostic advance.European journal of histochemistry : EJH · 2021Review
- TREM2 expression in the brain and biological fluids in prion diseases.Acta neuropathologica · 2021Article
- Review
- Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease.The Lancet. Neurology · 2021Review
- Cerebrospinal Fluid and Blood Neurofilament Light Chain Protein in Prion Disease and Other Rapidly Progressive Dementias: Current State of the Art.Frontiers in neuroscience · 2021Review
- Diagnostic and prognostic performance of CSF α-synuclein in prion disease in the context of rapidly progressive dementia.Alzheimer's & dementia (Amsterdam, Netherlands) · 2021Article
- Blinded RT-QuIC Analysis of α-Synuclein Biomarker in Skin Tissue From Parkinson's Disease Patients.Movement disorders : official journal of the Movement Disorder Society · 2020Article
- A new tetra-plex fluorimetric assay for the quantification of cerebrospinal fluid β-amyloid42, total-tau, phospho-tau and α-synuclein in the differential diagnosis of neurodegenerative dementia.Journal of neurology · 2020Article
- Fluid Biomarkers for Synaptic Dysfunction and Loss.Biomarker insights · 2020Review
- Challenges and Advances in Antemortem Diagnosis of Human Transmissible Spongiform Encephalopathies.Frontiers in bioengineering and biotechnology · 2020Review
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Authors and funding
17 authors at 6 institutions in 3 countries.
Funding
Abstract
The analysis of cerebrospinal fluid (CSF) biomarkers gains importance in the differential diagnosis of prion diseases. However, no single diagnostic tool or combination of them can unequivocally confirm prion disease diagnosis. Electrochemiluminescence (ECL)-based immunoassays have demonstrated to achieve high diagnostic accuracy in a variety of sample types due to their high sensitivity and dynamic range. Quantification of CSF α-synuclein (a-syn) by an in-house ECL-based ELISA assay has been recently reported as an excellent approach for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), the most prevalent form of human prion disease. In the present study, we validated a commercially available ECL-based a-syn ELISA platform as a diagnostic test for correct classification of sCJD cases. CSF a-syn was analysed in 203 sCJD cases with definite diagnosis and in 445 non-CJD cases. We investigated reproducibility and stability of CSF a-syn and made recommendations for its analysis in the sCJD diagnostic workup. A sensitivity of 98% and a specificity of 97% were achieved when using an optimal cut-off of 820 pg/mL a-syn. Moreover, we were able to show a negative correlation between a-syn levels and disease duration suggesting that CSF a-syn may be a good prognostic marker for sCJD patients. The present study validates the use of a-syn as a CSF biomarker of sCJD and establishes the clinical and pre-analytical parameters for its use in differential diagnosis in clinical routine. Additionally, the current test presents some advantages compared to other diagnostic approaches: it is fast, economic, requires minimal amount of CSF and a-syn levels are stable along disease progression.
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