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From the Neurology Service (Dr. Gómez-Isla, W.B. Growdon, M. McNamara, and Drs. Newell, Gómez-Tortosa, Hedley-Whyte, and Hyman) and Pathology Service (Drs. Newell and Hedley-Whyte), Massachusetts General Hospital, Boston, MA.
Address correspondence and reprint requests to Dr. Teresa Gómez-Isla, Department of Neurology, Mayo Building, Box 295, 420 Delaware Street, Minneapolis, MN 55455.
OBJECTIVE: To address the relationship between dementia and neuropathologic findings in dementia with Lewy bodies (DLB) in comparison with AD.
METHODS: We evaluated the clinical presentation of autopsy-confirmed DLB in comparison with AD according to new Consortium on DLB criteria and compared the two conditions using quantitative neuropathologic techniques. This clinicopathologic series included 81 individuals with AD, 20 with DLB (7 "pure" DLB and 13 "DLB/AD"), and 33 controls. We counted number of LB, neurons, senile plaques (SP), and neurofibrillary tangles (NFT) in a high order association cortex, the superior temporal sulcus (STS), using stereologic counting techniques.
RESULTS: The sensitivity and specificity of Consortium on DLB clinical criteria in this series for dementia, hallucinations, and parkinsonism are 53% and 83%, respectively, at the patient’s initial visit and 90% and 68%, respectively, if data from all clinic visits are considered. In pathologically confirmed DLB brains, LB formation in an association cortical area does not significantly correlate with duration of illness, neuronal loss, or concomitant AD-type pathology. Unlike AD, there is no significant neuronal loss in the STS of DLB brains unless there is concomitant AD pathology (neuritic SP and NFT).
CONCLUSIONS: The evaluation of new Consortium on DLB criteria in this series highlights their utility and applicability in clinicopathologic studies but suggests that sensitivity and specificity, especially at the time of the first clinical evaluation, are modest. The lack of a relationship of LB formation to the amount of Alzheimer-type changes in this series suggests that DLB is a distinct pathology rather than a variant of AD.
Key words: Superior temporal sulcus—Stereology—Lewy bodies—Neuronal loss—Neurofibrillary tangles—Amyloid deposition—Dementia with Lewy bodies.
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