ABSTRACT Dementia with Lewy bodies (DLB) is a common dementing syndrome with clinical and pathological features overlapping those observed in Alzheimer’s disease (AD) and Parkinson’s disease (PD). In common with PD, cases with DLB have Lewy body pathology and may also have clinical parkinsonism. In common with AD, cases with DLB have cortical senile plaques and a dominant dementia syndrome. As cases with AD may also have DLB, many cases with neurodegenerative dementia have both these diseases. A number of genes have been associated with AD and PD, although their relevance to DLB remains unknown and no independent genes have been associated with this disorder. The identification of several families with inherited DLB is an important step towards understanding the genetic contribution to this disease. In this review we examine familial cases with DLB and discuss the known gene associations with plaque and Lewy body formation, the core pathologic features found in DLB.
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