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Revised criteria for the clinical diagnosis of dementia with Lewy bodies (DLB)

Probable DLB	Two or more core clinical features of DLB are present, with or without indicative biomarkers; OR Only one core clinical feature is present, but with one or more indicative biomarkers Probable DLB should not be diagnosed on the basis of biomarkers alone
Possible DLB	Only one core clinical feature of DLB is present, with no indicative biomarker evidence; OR One or more indicative biomarkers are present, but there are no core clinical features
DLB is less likely	In the presence of any other physical illness or brain disorder including cerebrovascular disease, sufficient to account in part or in total for the clinical picture* If parkinsonian features are the only core clinical feature and appear for the first time at a stage of severe dementia^¶
Essential features	Dementia^Δ
Core clinical features (the first three typically occur early and may persist throughout the course)	Fluctuating cognition with pronounced variations in attention and alertness Recurrent visual hallucinations that are typically well formed and detailed REM sleep behavior disorder, which may precede cognitive decline One or more spontaneous cardinal features of parkinsonism (bradykinesia, rest tremor, rigidity)
Supportive clinical features	Severe sensitivity to antipsychotic agents Postural instability Repeated falls Syncope or other transient episodes of unresponsiveness Severe autonomic dysfunction (eg, constipation, orthostatic hypotension, urinary incontinence) Hypersomnia Hyposmia Hallucinations in other modalities Systematized delusions Apathy, anxiety, and depression
Indicative biomarkers	Reduced dopamine transporter uptake in basal ganglia by SPECT or PET Abnormal (low-uptake) ¹²³iodine-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 ± cingulate island sign on FDG-PET imaging Prominent posterior slow-wave activity on EEG with periodic fluctuations in the pre-alpha/theta range

DLB: dementia with Lewy bodies; REM: rapid eye movement; SPECT: single-photon emission computed tomography; PET: positron emission tomography; MIBG: metaiodobenzylguanidine; CT: computed tomography; MRI: magnetic resonance imaging; FDG: fluorodeoxyglucose; EEG: electroencephalography.
* These do not exclude a DLB diagnosis and may serve to indicate mixed or multiple pathologies contributing to the clinical presentation.
¶ DLB should be diagnosed when dementia occurs before or concurrently with parkinsonism. The term "Parkinson disease dementia" (PDD) should be used to describe dementia that occurs in the context of well-established Parkinson disease.
Δ Dementia is defined as a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational functions, or with usual daily activities. 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 visuoperceptual ability may be especially prominent and occur early.

Modified 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; 89:88. DOI: 10.1212/WNL.0000000000004058. Copyright © 2017 American Academy of Neurology. Reproduced with permission from Lippincott Williams & Wilkins. Unauthorized reproduction of this material is prohibited.

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