A minority of neuropathologically confirmed Alzheimer disease (AD) brains lack neocortical neurofibrillary tangles or have very few, constituting a form of “plaque-only AD.” A significant percentage of clinically diagnosed AD patients are found at autopsy to have both AD and brainstem and neocortical Lewy bodies. Many of these Lewy body variants of AD (LBV) have numerous senile plaques but no neocortical neurofibrillary tangles, and so resemble plaque-only AD. In this study, we sought to determine if plaque-only AD was usually LBV, and, conversely, if LBV was usually plaque-only AD. We analyzed 147 consecutively accessioned cases of neuropathologically confirmed AD, diagnosed according to criteria from the National Institute on Aging and the Consortium to Establish a Registry for Alzheimer's Disease. Twenty-five percent of all AD cases in this scries were plaque-only AD, and 75% were plaque and tangle AD. Twenty-eight percent of AD cases in this series were LBV, and 72% were pure AD. Of the plaque-only AD cases, 75% were LBV and only 25% were pure AD. Of the LBV, 66% were plaque-only AD and only 33% were plaque and tangle AD. These results indicate that most plaque-only AD is LBV, and, conversely, that most LBV is plaque-only AD.