The Dementia with Lewy Body (DLB) Consortium revised criteria for the disorder in 2005.
Synonym: alpha-synucleinopathy
Central feature (required for diagnosis): progressive cognitive decline that results in significant impairment in social and/or occupational function.
where:
• Memory impairment may be absent initially but eventually appears.
• The patient may show significant deficits in attention, visuospatial ability and executive function.
Core features
(1) fluctuating cognition with variation in alertness and/or attention
(2) recurrent visual hallucinations that are usually detailed and well-formed
(3) spontaneous features of parkinsonism
Suggestive features:
(1) REM sleep behavior disorder
(2) severe sensitivity to neuroleptics
(3) low dopamine transporter uptake in basal ganglia demonstrated on PET or SPECT imaging
Supportive features:
(1) syncope and repeated falls
(2) transient, unexplained loss of consciousness
(3) nonvisual hallucinations
(4) depression
(5) systematized delusions
(6) relative preservation of medial temporal lobe structures on MRI or CT scan
(7) low uptake on MIBG myocardial scintigraphy
(8) EEG with prominent slow wave activity and temporal lobe transient sharp waves
(9) severe autonomic dysfunction (urinary incontinence, orthostatic hypotension, etc)
Findings making the diagnosis of DLB less likely
(1) cerebrovascular disease with focal changes (neurologic signs, imaging findings)
(2) onset of parkinsonism after onset of severe dementia
(3) alternative explanation that can explain all of the findings
Criteria for probable DLB - both of the following:
(1) central feature
(2) one of the following:
(2a) 2 or more core features
(2b) 1 or more core features AND 1 or more suggestive features
Criteria for possible DLB - both of the following:
(1) central feature
(2) one of the following:
(2a) 1 core feature
(2b) 1 or more suggestive features
Specialty: Neurology
