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EnglishIntroduction: 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.
| Titolo: | Diagnostic and prognostic performance of CSF α-synuclein in prion disease in the context of rapidly progressive dementia | |
| Autore/i: | Mastrangelo, Andrea; Baiardi, Simone; Zenesini, Corrado; Poleggi, Anna; Mammana, Angela; Polischi, Barbara; Ladogana, Anna; Capellari, Sabina; Parchi, Piero | |
| Autore/i Unibo: | ||
| Anno: | 2021 | |
| Rivista: | ||
| Digital Object Identifier (DOI): | http://dx.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. | |
| Data stato definitivo: | 2022-02-27T19:34:49Z | |
| Appare nelle tipologie: | 1.01 Articolo in rivista |
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