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.

路易体（DLB）诊断痴呆症具有挑战性，因为症状异质且并非特定于该疾病。在这里，我们介绍一系列假阳性DLB病例的临床病理。当患者符合可能的DLB的临床标准但具有DLB或帕金森氏病痴呆以外的病理诊断时，从荷兰脑库回顾性入组。选择了22例假阳性病例。伴有或不伴有病理学的阿尔茨海默病是最常见的病理诊断（64％）。还遇到其他病理学诊断，例如额颞叶痴呆，多系统萎缩，克雅氏病和自身免疫性脑炎。非典型临床体征几乎一半的病例中出现了DLB征兆，可能会触发考虑DLB以外的其他诊断。额外的诊断检查，病理诊断的反馈以及一系列指示其他情况的临床特征的创建，可以减少假阳性DLB病例的数量。