Neuropathology of three clinical cases prospectively diagnosed as dementiawith Lewy bodies

Concensus guidelines for the clinical and pathological diagnosis of dementi a with Lewy bodies have recently been proposed based on retrospective studi es (McKeith IG, Galasko D, Kosaka K et at. Consensus guidelines for the cli nical and pathologic diagnosis of dementia with Lewy bodies (DFB): report o f the consortium on DLB international workshop. Neurology 1996; 47: 113-112 4.) The present study tests these criteria prospectively in three cases whi ch came to autopsy: a 57-year-old female followed for 5 years, an 86-year-o ld 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 au topsy all had pathologically confirmed Lewy body disease. However, the Lewy bodies found in the 57-year-old were scanty and she also had marked Alzhei mer-type pathology, making the contribution of Lewy bodies to the dementia uncertain. The 66-year-old had unusual cortical Lewy body pathology, previo usly only described in one other case (Masliah E, Galasho D, Wiley CA, Hans en LA. Lobar atrophy with dense-core (brain stem type) Lewy bodies in a pat ient with dementia. Acta Neuropathol 1990; 80: 453-458.) While this study p rospectively validates the current criteria for dementia with Lewy bodies, there was considerable pathological variability in the cases.