A 71-year-old man presented with 6 weeks of progressive left-hand incoordination, gait unsteadiness, dysarthria, left facial weakness, and left-sided myoclonus. Examination also demonstrated slowed verbal responses, ideomotor apraxia, and hypometric saccades with a wide-based, unsteady gait and rigidity, marked dysmetria, and intention tremor of the left arm and leg.
Magnetic resonance imaging (MRI) of the brain showed diffusion restriction in the right frontal, parietal, occipital, and superior temporal lobes; the left parietal lobe; and the right caudate head. Fludeoxyglucose positron emission tomography (FDG-PET) imaging was undertaken to reconcile the normal MRI appearance of the cerebellum with its prominent clinical dysfunction. Hypometabolism was evident in the areas of cerebral diffusion restriction and in the left cerebellum (Figure 1). Cerebrospinal fluid was positive for 14-3-3 protein, supporting the diagnosis of Creutzfeldt-Jakob disease. The patient progressively deteriorated and died in hospice 1 month after presentation.