Teaching NeuroImages: DWI and EEG findings in Creutzfeldt-Jakob disease

A 62-year-old woman, previously healthy, presented with 3 weeks of progressive short-term memory loss, dyscalculia, dysgraphia, appetite loss, and frequent episodic left arm dystonic posturing and eyebrow elevation. On examination, she also had finger agnosia, dysarthria, agraphesthesia, and astereognosis bilaterally, with myoclonus, ideomotor apraxia (left worse), and wide-based gait. EEG showed slowing and periodic lateralized discharges over the right hemisphere with triphasic morphology, less often involving the left, reflecting clinical asymmetry (figure 1).1 MRI brain showed diffusion restriction in the bilateral basal ganglia with cortical ribboning more prominent on the right involving several gyri, consistent with Creutzfeldt-Jakob disease (figure 2).2