Dementia With Lewy Bodies: A Clinicopathologic Series of False-positive Cases

Area of Science:

• Neuropathology
• Clinical Neurology
• Neurodegenerative Diseases

Background:

• Dementia with Lewy bodies (DLB) diagnosis is challenging due to heterogeneous and non-specific clinical presentations.
• Accurate diagnosis is crucial for appropriate patient management and research.

Purpose of the Study:

• To analyze a series of clinicopathologic cases misdiagnosed as probable dementia with Lewy bodies (DLB).
• To identify alternative pathologies and clinical features associated with false-positive DLB diagnoses.

Main Methods:

• Retrospective analysis of cases from the Netherlands Brain Bank meeting probable DLB clinical criteria.
• Pathologic examination to determine the definitive diagnosis in cases initially suspected of DLB.
• Selection of 22 false-positive DLB cases with confirmed non-DLB or non-Parkinson's disease dementia pathologies.

Main Results:

• Alzheimer disease, with or without co-pathology, was the most frequent (64%) underlying diagnosis in false-positive DLB cases.
• Other identified pathologies included frontotemporal dementia, multiple-system atrophy, Creutzfeldt-Jakob disease, and autoimmune encephalitis.
• Nearly half of the false-positive cases exhibited atypical clinical signs that should prompt consideration of alternative diagnoses.

Conclusions:

• Atypical clinical presentations in suspected DLB cases warrant consideration of other neurodegenerative or autoimmune conditions.
• Implementing additional diagnostic examinations and providing feedback on pathologic diagnoses can improve diagnostic accuracy.
• Developing a distinct set of clinical features indicative of alternative conditions is essential to reduce false-positive DLB diagnoses.