Towards optimizing the diagnosis of Lewy body dementia: Lessons from the NACC

Abstract

INTRODUCTION

The diverse presentations and co-pathologies of Lewy body dementia (LBD) present a diagnostic challenge. Utilizing the National Alzheimer's Coordinating Center (NACC) dataset, this study aimed to evaluate the concordance of premorbid diagnoses of LBD in individuals with autopsy-confirmed neocortical Lewy body disease.

METHODS

Demographics, clinical and neuropsychological presentations, Lewy body pathology, and Alzheimer's disease (AD) co-pathology were related to clinical diagnosis.

RESULTS

Diagnosis of LBD had high specificity but low sensitivity, with fewer than half of autopsy-confirmed cases having received an LBD diagnosis. AD was the most common misdiagnosis. Participants diagnosed with AD were more likely to be female, to have a more amnestic phenotype, and to harbor a higher burden of AD co-pathology, and were less likely to have documented clinical features characteristic of LBD.

DISCUSSION

These results highlight the need to improve LBD diagnosis in research and clinical settings, a need that emerging biomarkers may help address.

Highlights

• Less than half of cases with neocortical Lewy bodies were diagnosed with Lewy body dementia (LBD)
• Alzheimer's disease (AD) was the most common misdiagnosis
• Cases misdiagnosed were more likely to be female
• Cases diagnosed with AD presented with a more amnestic phenotype
• Cases diagnosed with AD had more AD co-pathology