Alexia without agraphia in Creutzfeldt-Jakob disease.

Progressive dementia uncommonly presents with focal deficits referable to posterior cortical dysfunction. We describe a 62 year-old man who presented with progressive visual disturbance in whom detailed cognitive testing documented alexia without agraphia. The only finding from diagnostic investigations was hypoperfusion of the posterior left temporoparietal region on brain SPECT scan. He rapidly progressed and Creutzfeldt-Jakob disease (CJD) was confirmed at autopsy. Although reading disorders may develop in association with posterior cortical atrophy due to CJD, this is the first reported autopsy-confirmed case presenting as alexia without agraphia.