Dementia with Lewy bodies (DLB) accounts for 10-15 % of all dementia cases. Neuropathologically, DLB is characterized by the accumulation of aggregated protein α-synuclein in Lewy bodies and Lewy neurites, similar to Parkinson's disease (PD).
Extrapyramidal motor symptoms of PD are common in patients with DLB, but are not essential for a clinical diagnosis of DLB. Overlap with the pathology typical of Alzheimer's disease (AD) is also frequently observed both at the level of the clinical picture as well as neuropathological changes.
Consensus criteria for the diagnosis of DLB are based on typical clinical symptomatology and also on the presence of characteristic biomarkers. The presence of reduced dopaminergic neurotransmission in the basal ganglia or characteristic polysomnographic findings indicating a behavior disorder in REM sleep helps in the correct diagnosis of this serious neurodegenerative disease.
Unfortunately, there is currently no causal treatment for DLB. Acetylcholinesterase (AChE) inhibitors, approved for Alzheimer's disease, are also used in DLB therapy.
Caution is required when administering antidopaminergic agents, because of the risk of serious adverse effects.