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.

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doi.org/10.1097/wad.0000000000000308, hdl.handle.net/1765/128726
Alzheimer Disease and Associated Disorders
Department of Neurology

Vergouw, L.J.M., Marler, L.P., van de Berg, W.D.J., Rozemuller, AJ, & de Jong, F.J. (2020). Dementia With Lewy Bodies A Clinicopathologic Series of False-positive Cases. Alzheimer Disease and Associated Disorders, 34(2), 178–182. doi:10.1097/wad.0000000000000308