Prion disease mimicking rapidly progressive Alzheimer disease: case series and systematic review.
J NeurolSep 7, 2026 (epub)
Anas Elgenidi, Dror Shir, Yoav D Piura et al.
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From the abstract
Prion disease and Alzheimer disease (AD) are common causes of rapidly progressive dementia (RPD). Although most patients with prion disease are distinguished by MRI and CSF findings, selected cases mimic rapidly progressive AD.
Reported results
Of 204 patients with RPD, five (2.5%) were initially diagnosed with clinically probable AD but ultimately determined to have prion disease. Systematic review identified 10 additional cases (n=15, median age-at-onset, 59 years; 67% male).
Authors' conclusions
Prion disease may rarely mimic rapidly progressive AD. Disproportionate elevations in CSF total-tau levels or total-tau/p hosphorylated-tau181 ratios should prompt consideration of prion disease.