Human prion disease, also known as Creutzfeldt-Jakob disease (CJD), is a neurodegenerative disease caused by abnormally folded prion proteins. The progression of CJD is rapid, with time from diagnosis to death being approximately 4 to 6 months. Currently, there is a need to more accurately predict disease duration in order to improve clinical management and stratify patients for clinical trials. Fluid biomarkers have been studied as a way to predict disease course in sporadic CJD (sCJD). In this cohort study, investigators performed neurological tests and collected cerebral spinal fluid (CSF) and blood from 188 participants with sCJD in order to examine the association between fluid biomarkers and disease progression. Investigators found that greater baseline levels of total tau (t-tau) were associated with shorter survival; this was true for both plasma (HR 5.8, 5% CI 2.3 to 14.8, p<0.001) and CSF (HR 1.6, 95% CI 1.2 to 2.1, p<0.001). Plasma and CSF t-tau levels were correlated (r=0.74, 95% CI 0.50 to 0.90 p<0.001). Plasma neurofilament light (NfL) levels, CSF nFL levels, total tau: phosphorylated tau ratios, and neuro-specific enolase levels were other fluid biomarkers associated with survival. Codon 129 genotypes of the PNRP gene and Barthel index were also associated with survival time. The results from this study suggest that using plasma and CSF t-tau levels could contribute important predictive information for patients with sCJD. It is important to note, however, that this study was limited with only a relatively small subsample of patients with all plasma and CSF biomarkers.
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