Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia.

OBJECTIVE To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia. DESIGN Case report. SETTING Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease. PATIENT Patient presenting with logopenic variant primary progressive aphasia initially thought to be due to Alzheimer disease. RESULTS Despite the long, slow 3.5-year course, the patient was shown to have pathology-proven sporadic Jakob-Creutzfeldt disease. CONCLUSIONS These findings expand the differential of primary progressive aphasia to include prion disease.