A 60-year-old man presented with diplopia and neurocognitive deficits, which progressed rapidly over several months. Magnetic resonance imaging of the head revealed bilateral signal abnormalities and diffusion-weighted imaging restriction in bilateral basal ganglia, thalami, mesial temporal regions, and periaqueductal gray matter. Cerebrospinal fluid analysis was positive for 14-3-3 and tau proteins. The patient developed progressive neurocognitive decline followed by sleep disturbance and myoclonic jerking consistent with probable Creutzfeldt-Jakob disease.
