%0 Journal Article
%T Diagnostic and prognostic performance of CSF α-synuclein in prion disease in the context of rapidly progressive dementia.
%A Mastrangelo A
%A Baiardi S
%A Zenesini C
%A Poleggi A
%A Mammana A
%A Polischi B
%A Ladogana A
%A Capellari S
%A Parchi P
%J Alzheimers Dement (Amst)
%V 13
%N 1
%D 2021
%M 34222611
暂无%R 10.1002/dad2.12214
%X <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.