Brief ReportsDementia With Lewy Bodies A Clinicopathologic Series of False-positive CasesVergouw, Leonie J.M. MD*; Marler, Luca P. MD*; van de Berg, Wilma D.J. PhD‡; Rozemuller, Annemieke J.M. PhD§,∥; de Jong, Frank Jan PhD*; Netherlands Brain Bank,†Author Information *Department of Neurology and Alzheimer Center, Erasmus Medical Center, Rotterdam †Netherlands Institute of Neuroscience ‡Department of Anatomy and Neurosciences, Section Clinical Neuroanatomy, Amsterdam Neuroscience, Amsterdam UMC ∥Department of Pathology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam §Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands L.J.M.V. and L.P.M. contributed equally to this work. The authors declare no conflicts of interest. Reprints: Leonie J.M. Vergouw, MD, Department of Neurology and Alzheimer Center, Erasmus Medical Center, P.O. Box 2040, Rotterdam 3000 CA, The Netherlands (e-mail: firstname.lastname@example.org). Alzheimer Disease & Associated Disorders: April–June 2020 - Volume 34 - Issue 2 - p 178-182 doi: 10.1097/WAD.0000000000000308 Buy Metrics Abstract Diagnosing dementia with Lewy bodies (DLB) is challenging as symptoms are heterogenous and not specific to the disease. Here we present a clinicopathologic series of false-positive DLB cases. Patients were enrolled retrospectively from the Netherlands Brain Bank when they met the clinical criteria of probable DLB, but with a pathologic diagnosis other than DLB or Parkinson’s disease dementia. Twenty-two false-positive cases were selected. Alzheimer disease with or without copathology was the most common (64%) pathologic diagnosis. Other pathologic diagnoses, such as frontotemporal dementia, multiple-system atrophy, Creutzfeldt-Jakob disease, and autoimmune encephalitis, were also encountered. Atypical clinical signs for DLB were present in almost half of the cases and could be a trigger to consider other diagnoses than DLB. Additional diagnostic examinations, feedback of pathologic diagnosis, and the creation of a set of clinical features that are indicative of other conditions, could reduce the amount of false-positive DLB cases. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.