A pure case of autopsy-confirmed dementia with Lewy bodies (DLB) is describ
ed. The patient presented with distinctive verbal fluency deficits in the c
ontext of mild language impairment, intact recognition memory, and impaired
paragraph recall. Neuroimaging (CT nd SPECT) showed progressive medial tem
poral lobe atrophy. Neuropathology revealed Lewy bodies, degeneration in th
e substantia nigra, nucleus basalis of Meynert (Nakano & Hirano, 1984), and
locus ceruleus, but no pathology characteristic of Alzheimer's disease. It
is in this sense that the case is "pure" DLB. Early neuropsychological dia
gnosis of DLB is essential (Salmon et al., 1996) given the potentially fata
l hazard of neuroleptics (McKeith et al., 1992) and the difficulties associ
ated with clinical neurological diagnoses (Litvan et al., 1998). (C) 2000 A
cademic Press.