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Ursula Hohl, MD; Pietro Tiraboschi, MD; Lawrence A. Hansen, MD; Leon J. Thal, MD; Jody Corey-Bloom, MD, PhD

Arch Neurol. 2000;57:347-351.

Background  Diagnostic criteria for dementia with Lewy bodies (DLB) are still evolving. No data exist on prospective differentiation of DLB and Alzheimer disease (AD).

Objective  To examine the clinician's diagnostic accuracy for DLB and analyze factors contributing to false-positive DLB diagnoses.

Methods  A prospective series of 10 patients with clinically diagnosed DLB who came to autopsy was compared with 32 autopsy-confirmed cases of DLB (27 Lewy body variant, 5 diffuse Lewy body disease) and 20 autopsy-confirmed cases of AD (matched on age, sex, education, and initial Mini-Mental State Examination score) with regard to distinguishing and/or confounding clinical features.

Results  The clinical diagnostic accuracy for DLB was 50%, with 5 of the 10 patients clinically presumed to have DLB confirmed at autopsy. Of the 5 misdiagnosed cases, 4 had AD and 1 had progressive supranuclear palsy. The misdiagnosed DLB cases who had pure AD had fewer hallucinations (25%) than those with Lewy body variant (63%) or diffuse Lewy body disease (100%) (P = .048); however, an equal amount of spontaneous (in the absence of neuroleptics) extrapyramidal signs was found. There were no differences among groups with regard to daily fluctuations in cognition or falls. Compared with the AD control group, the misdiagnosed DLB cases with pure AD showed significantly more spontaneous extrapyramidal signs (P.02).

Conclusions  The clinician's diagnostic accuracy for DLB was poor. Early spontaneous extrapyramidal signs in AD were associated with false-positive clinical diagnoses of DLB. The distinction between DLB and AD may be improved by greater emphasis on hallucinations.

From the Department of Neurosciences, University of California, San Diego, La Jolla, Calif (Drs Hohl, Hansen, Thal, and Corey-Bloom); Neurology Service, Veterans Affairs Medical Center, San Diego, Calif (Drs Hohl, Thal, and Corey-Bloom); and Neurologia Prima, Ospedali Riuniti, Bergamo, Italy (Dr Tiraboschi).

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