Neuropathology of three clinical cases prospectively diagnosed as dementia with Lewy bodies

Concensus guidelines for the clinical and pathological diagnosis of dementia with Lewy bodies have recently been proposed based on retrospective studies (McKeith IG, Galasko D, Kosaka K et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology 1996; 47: 113-1124.) The present study tests these criteria prospectively in three cases which came to autopsy: a 57-year-old female followed for 5 years, an 86-year-old male followed for 3 years and a 66-year-old male followed for 11 years. All were considered to have dementia with Lewy bodies clinically, and at autopsy all had pathologically confirmed Lewy body disease. However, the Lewy bodies found in the 57-year-old were scanty and she also had marked Alzheimer-type pathology, making the contribution of Lewy bodies to the dementia uncertain. The 66-year-old had unusual cortical Lewy body pathology, previously only described in one other case (Masliah E, Galasho D, Wiley CA, Hansen LA. Lobar atrophy with dense-core (brain stem type) Lewy bodies in a patient with dementia. Acta Neuropathol 1990; 80: 453-458.) While this study prospectively validates the current criteria for dementia with Lewy bodies, there was considerable pathological variability in the cases. Copyright 1999 Harcourt Publishers Ltd.