Anterograde and retrograde amnesia in a patient with probable corticobasal degeneration (pCBD) and dementia were studied in a university medical center setting. The patient with pCBD and four comparison patients of comparable global mental status (Mini-Mental State Exam) who met NINCDS-ADRDA criteria for Alzheimer's disease (AD) were included. Standard neuropsychological tests of naming, intelligence, achievement, verbal fluency, anterograde and remote verbal and visuospatial memory, and motor skill learning were given. The pCBD patient exhibited a progressive asymmetric akinetic-rigid syndrome, which was unresponsive to Sinemet. His initially mild, intellectual deficits consisted of apraxia, slowed speech, and word-finding and memory difficulties. Over a 2-year period, a dementia syndrome developed, which involved more-serious deficits in praxis and naming, as well as impairments in spelling, calculation, verbal fluency, IQ, anterograde verbal and visuospatial memory, and motor skill learning. When tested by recall methods, the pCBD patient exhibited marked deficits on several tests of remote memory; however, on recognition testing, he performed normally on the Famous Faces Test and on a test of geographical knowledge, which measures remote visuospatial memory. By contrast, the four AD patients, who showed equivalent naming difficulties, less-severe fluency deficits, and normal motor skill learning, showed severe impairments in recalling and recognizing the names of famous people from photographs. A magnetic resonance imaging (MRI) scan of the pCBD patient showed marked frontal and parietal lobe atrophy and central atrophy, with ventriculomegaly that was greater on the left side of the brain. The temporal lobes were relatively spared, and the amygdalae, hippocampi, and temporal horns were of normal size.(ABSTRACT TRUNCATED AT 250 WORDS)