A 66-YEAR-OLD WOMAN WITH A RAPIDLY PROGRESSING DEMENTIA AND BASAL GANGLIA INVOLVEMENT

A 66-year-old woman presented with a rapidly progressive dementia init ially characterized by an auditory agnosia. She experienced a rapid pr ogression of her aphasia and developed ataxia and myoclonus. An initia l neurological evaluation suggested a left parieto-temporal lesion, ho wever, neuroimaging did not reveal any. An MRI of her brain demonstrat ed highly focal T2 hyperintensities in her basal ganglia. The patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) on the basis of the presence of two proteins in the CSF that are highly sensitive and spe cific for CJD. Pathological examination confirmed the diagnosis. The d ifferential diagnosis and utility of MRI in patients with CJD is discu ssed.