Dementia with Lewy bodies (DLB) is a clinical syndrome characterized by progressive dementia, cognitive fluctuations, visual hallucinations (VH), and parkinsonism. In 1961, Okazaki, Lipkin, and Aronson reported two patients with dementia and parkinsonism with cortical neuronal inclusions similar to the brain-stem Lewy bodies (LB) seen in Parkinson’s disease (PD). LBs are intra-cytoplasmic neuronal inclusions containing α-synuclein and ubiquitin. There are other associated pathological features in DLB such as spongiform change neuronal loss, and Alzheimer’s disease (AD) pathology includes amyloid plaques and neurofibrillary tangles (NFTs). DLB and other entities such as PD and multiple system atrophy (MSA) have been grouped under the term synucleinopathies due to the existence of α-synuclein inclusions in the brain. The central feature required for a diagnosis of DLB is the presence of dementia: a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function.
