1. 59 year old man w visual hallucinations
Jesse C James MD
AM Report, September 2009
University of North Carolina Hospitals

2. HPI
59 yo WM PMHx HTN, DM
Three week hx of daily visual hallucinations involving home invasion “knee-high people”. Had “orgies…more than two people having sex”. Trip to N.H.
No past hx psychiatric disorder, delerium/dementia, recent illness, illicit drug use or STD.

3. OUTLINE Epidemiology Pathophysiology Clinical Features Management
Summary

4. Epidemiology Second most common diagnosis of dementia
DLB accounts for 10-22% dementia cases in US
Prevalence estimated at .7%
Increases w age
Mean age of presentation is 75 yo
More prevalent in men than women
aGE ONLY RELIABLE RF
More prevalent in men, than women
Rare cases reported in literature of famillies w triplet repeats

5. PATHOLOGY
Lewy Bodies are round, eosinophillic intracytoplasmic neuronal inclusions.
Predominantly located in cortex, anterior frontal lobes and temporal lobes.
Neuronal loss greater in frontal lobes, substantia nigra and locus ceruleus.
Neurofibratory tangles (typical of AD) sparse or absent.
Presence of visual hallucinations correlates with density of lewy bodies.
High Lewy bodies density in ANT AND TEMPORAL LOBES/AMYGDALA CORRELATE well w VISUAL HALLUCINATIONS

6. CLINICAL FEATURES
Consensus criteria for diagnosis developed by Consortium on Dementia with Lewy Bodies.
Defined as progressive cognitive decline, persistent memory impairment, and attention/ visuospatial deficits.
Distinguished by visual hallucinations, cognitive fluctuations, parkinsonism and nueroleptic sensitivity.
Diagnosis is based upon heirarchy of central, core, suggestive, and supportive features.

7. CLINICAL FEATURES: Revised Criteria
Central Features: Must be established for diagnosis of possible or probable DLB
Cognitive Decline
Memory Impairment
Attention Deficit
Progressive cognitive decline sufficient to in interfere with normal function
Prominent memory impairment, often not evident in early course but eventually evident
Attention, executive function and visuospatial deficits explicit.

8. CLINICAL FEATURES: Core Criteria
At least two core features are sufficient for probable DLB, one for possible DLB
Cognition Fluctuation
Recurrent Visual Hallucinations
Spontaneous Parkinsonism

9. CLINICAL FEATURES: Cognitive Impairment
Cognitive dysfunction typically the presenting symptom and eventually occurs in nearly all cases
Visuospatial and attentive deficits
Compromised executive task (job loss)
MMSE unreliable for distinguishing subtype dementias
Fluctuations in cognition range from brief or subtle inattention to frank syncope and last from seconds to days.
COGNITION: UNLIKE AD WHICH MEMORY LOSS EARLY, MEMORY OFTEN PRESERVED
DRAWING TEST, SERIAL SEVENS, WORLD

10. CLINICAL FEATURES: Visual Hallucinations
Visual hallucinations distinguish DLB from AD and VD.
Occur in nearly two thirds of DLB patients
In study visual hallucination most reliable feature unique to DLB vs AD, 83% PPV
Range from abstract shapes to well described animals/humans. Home invasion is common and may involve complex dialogue.
VISUAL HALLUCINATIONS ARE UNDERREPORTED

11. CLINICAL FEATURES: Parkinsonism
A variety of Parkinsonian symptoms occur in approximated three fourths of DLB.
Typically bilateral and less severe than in PD.
Symptoms range include tremor, rigidity, and gait disturbance.
Anecdotally and unreliably, dementia should precede Parkinsonism.
“One year rule” not without controversy.

12. CLINICAL FEATURES: Suggestive Criteria
At least one core and one suggestive required for probable; at least one suggestive required for possible DLB
REM sleep disorder
Severe Neuroleptic Sensitivity
Low Dopamine Transporter uptake in BG on SPECT or PET

13. CLINICAL FEATURES: Supportive Criteria
Commonly present but of poor diagnostic specificity
Repeated fall/syncope (33%)
Transient loss of consciousness (50%)
Systemized delusions (75%)
Hallucinations of various modalities
Depression
Preservation of temporal lobes on CT/MRI
Prominent slow wave EEG activity w temporal lobe transient sharp waves.

14. MANAGEMENT Goal is symptomatic treatment
Donepezil and other cholinesterase inhibitors considered first line. Pt on rivastigmine significantly reduced delusions and hallucinations vs placebo.
In general, avoid neuroleptics. If required for psychotic features, start with atypicals.
Parkinsonism treated with levodopa, but parkinsonism of DLB is less responsive than PD.
aricept

15. SUMMARY
DLB is probably under-diagnosed and requires a high index of suspicion.
Optimal treatment unique from AD w psychotic features and PDD.
Visual Hallucinations distinguish from AD and VD verified in multiple neuropathologic studies.
If concurrent w rigidity/dyskinesia, diagnosis DLB if dementia preceded parkinsonism. PDD describes dementia occurring w established PD.