Lewy bodies (LBs) are found throughout the brain stem, limbic and neoc
ortical areas in Parkinson's disease. Lewy bodies are also associated
in these areas with dementia and the substrate of 'dementia with Lewy
bodies' is thought to include Lewy body pathology in limbic and neocor
tical areas with or without Alzheimer-type changes. In order to determ
ine whether dementia with Lewy bodies is characterised primarily by co
rtical or limbic LB pathology, we have measured the density of Lewy bo
dies, neuritic plaques and neurofibrillary tangles in 12 neocortical,
limbic and brain stem sites in 10 patients who were pathologically dia
gnosed with diffuse Lewy body disease (DLBD) (64.7+/-2.7 years). The m
ean LB density in limbic areas (3.00+/-0.61/mm(2)) was significantly g
reater than that of neocortical areas (1.13+/-0.22/mm(2); P < 0.001).
The greatest density of LBs was found in the amygdala (4.1+/-0.7/mm(2)
) and the lowest in the occipital cortex (0.3+/-0.1/mm(2)). In limbic
areas, LB formation positively correlated with neuritic plaque formati
on (r=0.51, P < 0.01) but not with neurofibrillary tangle densities. T
hese data indicate that dementia with Lewy bodies is characterised pri
marily by limbic, and secondly by neocortical, LB pathology. It remain
s to be determined why limbic areas are selectively vulnerable to LB p
athology in dementia with Lewy bodies.