1. Dementia with Lewy Bodies
Jae Woo Kim, M.D.
Department of Neurology
Dong-A University Hospital

2. Dementia with Lewy Bodies
Second most common cause of degenerative dementia
: prevalence 19% (52% for AD, McKeith, 1992)
Lewy body pathology
: third most common cause of dementia, 7.7%
(71% for AD change & 34% for CVD)

3. Lewy body

4. Classification of Lewy body disease (Kosaka et al, 1993)
Cortex brainstem
Diffuse type
Common form : DLB + AD
Pure form : DLB
Transitional type
Brainstem type : Parkinson’s disease

5. Diagnostic Criteria of DLB
Progressive cognitive decline
Core features
fluctuating cognition
recurrent visual hallucinations
parkinsonism
Supportive features
repeated falls
syncope
transient loss of consciousness
neuroleptic sensitivity
systematized delusions
hallucinations in other modalities

6. Neuropathology Predilection areas
cortex - ant. frontal & temporal, cingulate, insula
subcortex - substantia nigra, nucleus basalis of Meynert
locus ceruleus, raphe N, amygdala
More neuronal loss than in AD
SN, nb Meynert, frontal lobe
Neurofibrillary tangles
negative correlation btw neocortical LB & NFT density
scarcity of NFT - distinct from AD

7. Neurochemistry Acetylcholine ChAT - more severe deficit than in AD
correlated with visual hallucination
& cognitive impairment
Increased postsynaptic muscarinic receptors
Dopamine
40-60% loss in striatum (80% in PD)

8. PET Studies Fluorodopa reduced uptake in striatum FDG
more severe glucose hypometabolism in T-P-O
association cortices (esp. medial & lateral occipital lobes) in DLB and medial temporal & cingulate areas in AD

9. DLB vs Parkinson’s Disease
Less DA neuronal loss in SN
Less DA depletion in striatum
Decreased HVA / DA ratio : turnover rate
Reduced ChAT activity in striatum
: rare rest tremor, milder parkinsonism
Reduced D2 receptors
: less responsiveness to LD

10. Parkinsonian Features
Less responsiveness to LD
Less asymmetricity
Neuroleptic sensitivity
Rare rest tremor

11. DAT SPECT
normal
DLB

12. DAT SPECT
HMPAO-SPECT

13. Clinical Characteristics of Patients with DLB
Case No.
(n=10)
Sex/Age
FC / VH / P
K-MMSE
(min / max)
SPECT
(Hypoperfusion
area)
IPT SPECT 1
M/66
+/-/+
23/29
Both F-T-P-O
0.77/0.54 2
M/61
+/+/+
15/19
0.94/1.07 3
M/75
6/11
0.44/0.48 4
M/79
10/15
0.76/0.73 5
M/60
6/9
Both T-P-O
0.88/0.61 6
M/70
17/22
Left T-P-O
0.43/0.70 7
M/58
17/25
0.47/0.54 8
0.72/0.86 9
18/25
Both F-T-P
0.36/0.54 10
M/80
23/28
1.16/1.13

14. Responsiveness to Levodopa

15. Responsiveness to Acetylcholinesterase Inhibitor