Transcript Lewy Body Dementia

59 year old man w visual
hallucinations
Jesse C James MD
AM Report, September 2009
University of North Carolina Hospitals
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.
OUTLINE
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Epidemiology
Pathophysiology
Clinical Features
Management
Summary
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
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.
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.
CLINICAL FEATURES:
Revised Criteria
 Central Features: Must be established for
diagnosis of possible or probable DLB
 Cognitive Decline
 Memory Impairment
 Attention Deficit
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
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.
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.
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.
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
CLINICAL FEATURES:
Supportive Criteria
 Commonly present but of poor diagnostic
specificity
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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.
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.
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.