Brain Advance Access originally published online on January 9, 2006
Brain 2006 129(3):729-735; doi:10.1093/brain/awh725
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What best differentiates Lewy body from Alzheimer's disease in early-stage dementia?
1 Dipartimento di Scienze Neurologiche, Ospedale Niguarda Ca' Granda, Milano, Italy, 2 Department of Neurosciences, University of California, San Diego, La Jolla and 3 Neurology Service, VA San Diego Healthcare System, San Diego, CA, USA
Correspondence to: Jody Corey-Bloom, MD, Neurology Service (9127), San Diego VA Medical Center, 3350 La Jolla Village Drive, San Diego, CA 92161-3064, USA E-mail: jcoreybl{at}vapop.ucsd.edu
To determine which clinical feature(s) [among visual hallucinations (VH), extrapyramidal signs (EPS) and visuospatial impairment] in the earliest stages of disease best predicted a diagnosis of dementia with Lewy bodies (DLB) at autopsy, first-visit data of 23 pathologically proven DLB and 94 Alzheimer's disease cases were compared. There were no group differences with regard to age, gender, education or global severity of dementia at presentation (mean Mini-Mental State Examination: 24.0 versus 25.0, mean Dementia Rating Scale: 123.6 versus 125.7). DLB patients at initial presentation displayed an increased frequency of VH (P = 0.001), but not EPS (P = 0.3), compared to Alzheimer's disease patients. However, only a minority of DLB cases had either VH (22%), EPS (26%) or both (13%). In contrast, although not a core feature, visuospatial/constructional impairment was observed in most of the DLB cases (74%). Among clinical variables, presence/recent history of VH was the most specific to DLB (99%), and visuospatial impairment was the most sensitive (74%). As a result, VH at presentation were the best positive predictor of DLB at autopsy (positive predictive value: 83% versus 32% or less for all other variables), while lack of visuospatial impairment was the best negative predictor (negative predictive value: 90%). We conclude that the best model for differentiating DLB from Alzheimer's disease in the earliest stages of disease includes VH and visuospatial/constructional dysfunction, but not spontaneous EPS, as predictors. This suggests that clinical history plus a brief assessment of visuospatial function may be of the greatest value in correctly identifying DLB early during the course of disease.
Key Words: Alzheimer's disease; dementia with Lewy bodies; core clinical features; diagnostic accuracy
Abbreviations: DLB = dementia with Lewy bodies; DRS = Dementia Rating Scale; DRS-C = DRS construction subscale; EPS = extrapyramidal signs; MMSE = Mini-Mental State Examination; NPV = negative predictive value; PPV = positive predictive value; VH = visual hallucinations
Received October 7, 2005. Revised November 18, 2005. Accepted November 25, 2005.
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