A DIAGNOSTIC PROFILE FOR DEMENTIA WITH LEWY BODIES VS. PURE ALZHEIMER'S DISEASE
P.K. Ogrocki, T. Fritsch, D.S. Geldmacher, M.B. Patterson
University Alzheimer Center, University Hospitals of Cleveland,
Case Western Reserve University, Ohio
ABSTRACT
Many cases of dementia with Lewy bodies (DLB) meet all the criteria for probable Alzheimer's disease (AD) at the time of presentation. The clinical and neuropathologic features of each disease can occur concomitantly, as in the Lewy body variant of AD (LBV), making prospective clinical differentiation of pure AD and LBV difficult. Pathologically defined groups of pure AD (n=66) and AD with Lewy bodies, LBV (n=14) were compared on clinical history and presentation, cognitive functioning, noncognitive symptoms, and functional abilities. Diagnostic agreement was 98% for pure AD and 28% for LBV, with 72% of LBV cases diagnosed as probable AD. Initial symptoms for both groups included memory and functional impairment. A higher proportion of LBV had a clinical history with early symptoms of delusions/hallucinations (p<.01) and delirium (p<.08), as well as masked facies and stooped posture (p<.05) on clinical exam. After controlling for demographic variables and dementia severity (CDR), LBV was associated with more apathy (p<.01), psychotic (p<.01), and vegetative (p<.06) symptoms on the CERAD BRSD. Groups were equally impaired on CERAD tests of language and word list learning. LBV exhibited better delayed recall (p<.001) and recognition memory (p<.01), and more impaired visuospatial abilities (p<.05) (Trailmaking A and Visual Design Copy Test). Despite different cognitive profiles, functional status (ADLs) was equivalent. The assessment of both cognitive and noncognitive symptoms enhances the clinical differentiation of LBV from pure AD.
BACKGROUND
- Cases of Lewy body dementia often meet all the criteria for probable AD at the time of clinical presentation
- AD plaques and tangles and Lewy bodies can occur concomitantly, as in the Lewy body variant of AD (LBV)
- Prior attempts at the neuropsychological characterization of LBV have not yielded a sufficiently diagnostic profile
PURPOSE OF THE STUDY
To develop a diagnostic profile to differentiate pure AD and LBV across multiple domains, including:
- clinical history and presentation
- cognitive functioning
- noncognitive symptoms
- functional abilities
METHODS
Sample
- Pathologically defined groups of pure AD (n=66) and LBV (n=14) from the research registry of the ADRC*
- Agreement between clinical diagnosis and neuropathological diagnosis was 98% for pure AD and 28% for LBV (72% of LBV diagnosed as pure AD)
- All LBV cases had both AD neuropathology and cortical Lewy bodies
*Groups did not differ with respect to age, education, gender, disease stage, or MMSE
Procedure for Subject Evaluations
- Clinical history and presentation:
- Standard diagnostic interview
- Neurological exam
- Cognitive functioning:
- CERAD Neuropsychological Battery
- Visual Design Copy Task (Seltzer & Mack, 1981 adaptation of Rey-Osterrieth )
Noncognitive symptoms:
- CERAD Behavioral Rating Scale for Dementia
Functional abilities:
- Cleveland Activities of Daily Living Scale
RESULTS
|
Pure AD
|
LBV
|
|
Clinical History
a
|
|
Memory Loss |
100% |
100% |
|
Language Impairment |
82% |
75% |
|
Personality Changes |
85% |
69% |
|
Delusions/Hallucinations |
6% |
94%*** |
|
Neurological Exam
a
|
|
Masked Facies |
0% |
24% |
|
Stooped Posture |
46% |
100% |
|
Tremor/Rigidity |
36% |
66% |
|
Functional Abilities
b
|
|
Basic ADLs |
20.4(20.6) |
30.1(20.5) |
|
Instrumental ADLs |
27.1(12.8) |
34.1(11.3) |
|
Noncognitive Symptoms
b
|
|
Behavioral Dysregulation |
3.9(3.1) |
4.7(4.2) |
|
Depressive Symptoms |
4.3(5.1) |
2.6(2.4) |
|
Inertia/Apathy |
1.2(.96) |
3.4(.69)** |
|
Irritability/Aggression |
4.6(4.4) |
2.9(3.4) |
Psychotic Symptoms |
1.9(4.0) |
5.5(3.2)*** |
|
Vegetative Symptoms |
1.4(1.2) |
2.9(1.0)* |
|
Cognitive Test
b
|
|
Naming |
8.6(3.6) |
9.7(1.8) |
|
Verbal Fluency |
5.6(3.5) |
5.0(4.1) |
|
Word List Learning Total |
5.7(4.2) |
8.8(2.6) |
|
Word List Recall |
0.3(0.9) |
1.8(1.5)*** |
|
Word List Recognition |
13.2(2.8) |
16.6(2.8)** |
|
Constructional Praxis |
5.8(2.7) |
4.8(2.8) |
|
Visual Design Copy |
22.4(8.1) |
13.1(3.5)* |
|
Trailmaking A |
146.2(96.3) |
286.4(36.7)* |
a
Group differences in % subjects within each group with symptoms were examined
using chi-square analyses
b
Group differences were examined using ANCOVA adjusted for covariates of
duration of dementia, dementia severity, patient age, education, and gender;
unadjusted means are reported in tables;
*p<.05 **p<.01 ***p<.001
Diagnostic Profile for LBV
|
Domain |
Differential Pattern
|
|
Clinical history |
Initial symptom: Hallucinations/delusions |
|
Neurological exam |
Masked facies and stooped posture |
|
Neuropsychological |
Better delayed recall
Better recognition memory
More impaired visuospatial abilities
Equal level of impairment in word list learning, naming, and fluency
|
|
Noncognitive symptoms |
Increased apathy, psychosis, and vegetative symptoms |
|
Functional status |
Equal level of impairment in pure AD and LBV |
CONCLUSIONS
- The assessment of multiple domains, including both cognitive and noncognitive symptoms enhances the clinical differentiation of LBV from pure AD
- Understanding how the presence of Lewy bodies alters the clinical expression of AD may lead to better understanding of the pathogenesis, diagnosis, and treatment of dementia subtypes
Presented at the Annual Meeting of the International Neuropsychological Society, Chicago, IL, February, 2001
(THIS RESEARCH WAS SUPPORTED BY NIA ADRC GRANT P50 AG08012)