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- Title
Clinical Manifestations of Early-Onset Dementia With Lewy Bodies Compared With Late-Onset Dementia With Lewy Bodies and Early-Onset Alzheimer Disease.
- Authors
Sim, Jingwei; Li, Huihua; Hameed, Shahul; Ting, Simon Kang Seng
- Abstract
This case-control study investigates clinical features that distinguish early-onset dementia with Lewy bodies from late-onset dementia with Lewy bodies and from early-onset Alzheimer disease (AD) dementia. Key Points: Question: Do patients who develop dementia with Lewy bodies (DLB) younger than 65 years have unique clinical features? Findings: In this case-control study of 542 individuals, early-onset DLB was often misdiagnosed; certain motor features and, to a lesser extent, neuropsychiatric features were associated with a diagnosis of early-onset DLB over early-onset Alzheimer disease dementia. Late-onset DLB had more prominent amnestic features but lower rates of depression than early-onset DLB. Meaning: In evaluation of suspected early-onset DLB, one should assess for motor signs, apathy, depression, and determine if motor deterioration predated cognitive and behavioral changes; the amnestic features seen in late-onset DLB may be associated with Alzheimer disease copathology. Importance: Early-onset dementia, presenting in individuals younger than 65 years, is a diagnosis with significant social and financial implications. The early-onset form of dementia with Lewy bodies (DLB) is poorly understood. Objective: To investigate clinical features that distinguish early-onset DLB (onset and diagnosis at age <65 years) from late-onset DLB (onset at age ≥65 years) and from early-onset Alzheimer disease (AD) dementia. Design, Setting, and Participants: This is a retrospective case-control study on patients with pathologically confirmed DLB or AD enrolled in the National Alzheimer's Coordinating Center database from January 2005 to July 2017. The National Alzheimer's Coordinating Center Uniform Data Set comprised deidentified data collected by Alzheimer disease centers in the United States. Of patients fulfilling criteria for all-cause dementia at enrollment (n = 1152), those who at post mortem received a pathological diagnosis of either AD (n = 848) or Lewy body disease (n = 218) were selected. Excluding 52 patients owing to missing data and 12 diagnosed with Parkinson disease dementia, remaining patients were classified by age of symptom onset into early-onset AD, early-onset DLB, and late-onset DLB subgroups. Data were analyzed from June to December 2018 and from November to December 2021. Exposures: Demographics, cognitive, behavioral, and motor features recorded at first clinic visit and neuropathological characteristics at autopsy were analyzed by disease subgroup. Main Outcomes and Measures: Concordance between initial etiologic diagnosis of dementia and final pathological diagnosis was assessed, as was time to death. Results: A total of 542 individuals were categorized as having early-onset AD (n = 363; mean [SD] age, 53.0 [5.8] years; 208 [57.3%] male), early-onset DLB (n = 32; mean [SD] age, 57.9 [3.2] years; 23 [71.9%] male), and late-onset DLB (n = 147; mean [SD] age, 73.5 [5.5] years; 103 [70.1%] male). Early-onset DLB was clinically misdiagnosed in 16 individuals (50%). Features that predicted a diagnosis of early-onset DLB over early-onset AD included visual hallucinations (15 [46.9%] vs 42 [11.6%]), slowness (23 [71.9%] vs 95 [26.2%]), apathy (23 [71.9%] vs 189 [52.1%]), and motor deterioration that preceded cognitive and behavioral symptoms (7 [21.9%] vs 6 [1.7%]). Late-onset DLB had more amnestic features, but this was accounted for by a higher proportion of neocortical neuritic plaques and diffuse plaques (frequent in 79 [53.7%] vs 8 [25%]) than seen in early-onset DLB. Conclusions and Relevance: This study found that early-onset DLB has clinical features that distinguish it from early-onset AD, whereas features of late-onset DLB are associated with a higher burden of AD copathology.
- Publication
JAMA Neurology, 2022, Vol 79, Issue 7, p702
- ISSN
2168-6149
- Publication type
Article
- DOI
10.1001/jamaneurol.2022.1133