Table 2 Early diagnostic profile of the case according to the revised criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) – adapted from McKeith et al., Neurology 2005 [3]

 Cognitive decline

Progressive, insidious onset

Acute or rapidly progressive

Acute

 Prominent or persistent memory impairment

Not early: usually evident with progression

Early

Early

 Prominent deficits of attention, executive function, and visuospatial ability

Yes

No (global dementia)

No (global cognitive impairment)

 Fluctuating cognition

Yes

No

No

 Recurrent visual hallucination

Yes

Yes

No (mainly delirious with few hallucinations)

 Spontaneous features of parkinsonism

Yes

Yes

Yes

 REM sleep behavior disorder

Yes

No

Not reported by the wife

 Severe neuroleptic sensitivity

Yes

No

No

 Low dopamine uptake in basal ganglia on imaging

Yes

Very rare (one case reported [22])

Not performed (the case was parkinsonian)

 Repeated falls and syncope

Yes

No

Yes

 Transient loss of consciousness

Yes

No

No

 Severe autonomic dysfunction

Yes

No

No

 Hallucinations in other modalities

Yes

No

No

 Systematized delusions

Yes

No

Yes

 Depression

Yes

Yes

Yes

 Relative preservation of medial temporal lobe structures on MRI

Yes

Yes

Yes

 Generalized low uptake on PET perfusion scan with reduced occipital activity

Yes

No

Extensive hypometabolism

 Prominent slow wave activity on EEG with temporal lobe transient sharp waves

Yes

No (biphasic and triphasic periodic complex)

Slow but without temporal lobe transient sharp waves

 Mean age of onset (years)

75

70

42

 Mean duration (years)

7

0.5

5

 Detection of 14.3.3 protein

Rarely (3 cases reported)

Yes

Yes