Diagnostic and prognostic performance of CSF α‐synuclein in prion disease in the context of rapidly progressive dementia

Alzheimer’s & Dementia: Diagnosis, Assessment & Disease Monitoring

Andrea Mastrangelo¹  Simone Baiardi¹  Piero Parchi¹  Sabina Capellari¹  Anna Ladogana²  Anna Poleggi²  Corrado Zenesini³  Barbara Polischi³  Angela Mammana³ 

[1] Department of Experimental, Diagnostic and Specialty Medicine (DIMES) University of Bologna Bologna Italy;Department of Neuroscience Istituto Superiore di Sanità Rome Italy;IRCCS Istituto delle Scienze Neurologiche di Bologna Bologna Italy;

关键词: alpha‐synuclein;    cerebrospinal fluid;    Creutzfeldt‐Jakob disease;    diagnosis;    fatal familial insomnia;    prognosis;   

DOI  :  10.1002/dad2.12214

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown