Diagnostic and prognostic performance of CSF α‐synuclein in prion disease in the context of rapidly progressive dementia
Open Access
- 1 January 2021
- journal article
- research article
- Published by Wiley in Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
- Vol. 13 (1), e12214
- https://doi.org/10.1002/dad2.12214
Abstract
Introduction Surrogate cerebrospinal fluid (CSF) biomarkers of neurodegeneration still have a central role in the first-line screening of patients with suspected Creutzfeldt-Jakob disease (CJD). Recently, CSF α-synuclein, a marker of synaptic damage, showed a close to optimal performance in distinguishing between CJD and other neurodegenerative dementias. Methods We evaluated the diagnostic value of CSF α-synuclein in patients with prion disease, non-prion rapidly progressive dementias, and non-neurodegenerative controls. Additionally, we studied its distribution across the different prion disease subtypes and evaluated its association with survival. Results CSF α-synuclein levels were significantly higher in patients with prion disease than in the other groups but showed a lower diagnostic value than CSF total tau or 14-3-3. Moreover, CSF α-synuclein was significantly associated with survival in the whole prion cohort and the most frequent clinicopathological subtypes. Discussion In the clinical setting, CSF α-synuclein does not exceed the diagnostic performance of currently used surrogate markers, but it might constitute a robust prognostic indicator.Keywords
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