Abstract
BACKGROUND AND OBJECTIVES: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal neurodegenerative disorder with highly variable survival times, ranging from weeks to years. However, there are currently no tools for prognosticating a patient's survival time. This study aims to fill this gap by examining the relationship between CSF total tau (t-tau) levels and time to death in patients with CJD. METHODS: We use cases with CJD recorded in the electronic health record of a tertiary academic medical center from 2010 to 2022. RESULTS: We identified 29 cases with diagnosis of CJD. Using a Cox proportional hazards model, we find that elevated t-tau levels (>4,000 pg/mL) are associated with 9.62 (95% confidence interval: 1.93-47.92) times the hazard of death compared with CJD patients with t-tau less than 4,000 pg/mL. DISCUSSION: This finding supports the use of CSF t-tau as a prognostic biomarker for CJD.