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Original Investigation
March 2017

Duration and Pathologic Correlates of Lewy Body Disease

Author Affiliations
  • 1Department of Neurology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Health Sciences Research, Mayo Clinic and Foundation, Rochester, Minnesota
  • 3Department of Psychiatry and Psychology, Mayo Clinic, Jacksonville, Florida
  • 4Department of Radiology, Mayo Clinic, Jacksonville, Florida
  • 5Department of Pathology and Laboratory Medicine, Mayo Clinic, Jacksonville, Florida
  • 6National Alzheimer's Coordinating Center, Department of Epidemiology, University of Washington, Seattle
JAMA Neurol. 2017;74(3):310-315. doi:10.1001/jamaneurol.2016.4926
Key Points

Question  What are the pathologic correlates of disease duration in individuals with Lewy body disease?

Findings  In this observational study of 807 individuals, diffuse Lewy body disease was associated with shorter disease duration compared with transitional Lewy body disease, whereas Braak neurofibrillary tangle stage and extent of neuritic plaque were not associated with disease duration.

Meaning  From onset of cognitive symptoms, patients with diffuse Lewy body disease have a shorter disease duration.

Abstract

Importance  Although patients with dementia with Lewy bodies (DLB) have shorter disease duration than patients with Alzheimer disease dementia, little is known about which factors influence disease duration among patients with DLB.

Objective  To identify pathologic correlates of disease duration in participants with Lewy body disease (LBD).

Design, Setting, and Participants  This observational study, performed from September 1, 2005, to June 1, 2015, using the National Alzheimer’s Coordinating Center database included 807 participants with transitional or diffuse LBD.

Main Outcomes and Measures  The study used Braak neurofibrillary tangle (NFT) stage, frequency of neuritic plaques, and LBD stage to determine whether pathologic variables are associated with disease duration.

Results  This study included 807 participants with transitional or diffuse LBD (mean [SD] age, 70.0 [9.9] at the onset of cognitive decline and 79.2 [9.8] years at death; 509 male [63.1%]). Shorter disease duration from cognitive symptom onset to death was observed in men (β, −0.73; 95% CI, −1.33 to −0.14; P = .02) and in those with a later age at onset (β, −0.11; 95% CI, −0.14 to −0.08; P < .001). Diffuse (neocortical) LBD was associated with shorter disease duration compared with transitional LBD (β, −1.52; 95% CI, −2.11 to −0.93; P < .001). Braak NFT stage and the presence of neuritic plaques were not significantly associated with differences in disease duration.

Conclusions and Relevance  Diffuse LBD was associated with shorter disease duration compared with transitional LBD, and this effect is independent of Braak NFT stage or extent of neuritic plaque disease. Identifying antemortem biomarkers of LBD stage may provide important prognostic information to patients with DLB.

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