Table 1 Revised criteria for the clinical diagnosis of Dementia with Lewy bodies (DLB)

Central feature (essential for a diagnosis of possible or probable DLB)

• Dementia defined as progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function

• Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression

• Deficits on tests of attention, executive function and visuospatial ability may be especially prominent

Core features (2 core features or 1 core feature with 1 or more indicative biomarkers for a diagnosis of probable DLB; 1 core feature for possible DLB)

• Fluctuating cognition with pronounced variation in attention and alertness.

• Recurrent visual hallucinations that are typically well formed and detailed

• REM sleep behavior disorder, which may precede cognitive decline

• Spontaneous features of parkinsonism

Supportive features

• Severe neuroleptic sensitivity

• Postural instability

• Severe autonomic dysfunction

• Hallucinations in other modalities, delusions, apathy, anxiety, and depression

Indicative biomarkers (if 1 or more indicative biomarkers is present but there is no core clinical features, possible DLB can be made)

• Reduced dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET

• Abnormal (low uptake) iodine123 - metaiodobenzyguanidine (MIBG) myocardial scintigraphy

• Polysomnographic confirmation of REM sleep without atonia

Supportive biomarkers

• Relative preservation of medial temporal lobe structures on CT/MRI scan

• Generalized low uptake on SPECT/PET perfusion/metabolism scan with reduced occipital activity +/− the cingulated island sign on FDG-PET imaging

• Prominent posterior slow wave activity on EEG with periodic fluctuations in the pre-alpha/theta range

From McKeith IG, Boeve BF, Dickson DW et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017; 89(1): 88–100.