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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]

From: Case report of Lewy body disease mimicking Creutzfeldt-Jakob disease in a 44-year-old man

  Dementia with Lewy bodies Creutzfeldt-Jakob disease Case reported
Central feature
 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)
Core features
 Fluctuating cognition Yes No No
 Recurrent visual hallucination Yes Yes No (mainly delirious with few hallucinations)
 Spontaneous features of parkinsonism Yes Yes Yes
Suggestive features
 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)
Supportive features (Commonly present in DLB but not proven to have diagnostic specificity)
 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
Other
 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
  1. Probable DLB Two core features or one core feature and one or more suggestive features, Possible DLB Up to one core feature and one or more suggestive features
  2. Features for Creutzfeldt-Jakob disease are adapted from the MRI-CJD Consortium criteria for sporadic Creutzfeldt–Jakob disease [8]
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