Duration and pathologic correlates of Lewy Body Disease

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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 stagemay provide important prognostic information to patients with DLB.

Original languageEnglish (US)
Pages (from-to)310-315
Number of pages6
JournalJAMA Neurology
Volume74
Issue number3
DOIs
StatePublished - Mar 1 2017

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Lewy Body Disease
Neurofibrillary Tangles
Amyloid Plaques
Alzheimer Disease
Neurobehavioral Manifestations
Age of Onset
Observational Studies

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

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title = "Duration and pathologic correlates of Lewy Body Disease",
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 stagemay provide important prognostic information to patients with DLB.",
author = "Jonathan Graff-Radford and Jeremiah Aakre and Rodolfo Savica and Boeve, {Bradley F} and Kremers, {Walter K} and Ferman, {Tanis Jill} and Jones, {David T} and Kantarci, {Kejal M} and Knopman, {David S} and Dickson, {Dennis W} and Kukull, {Walter A.} and Petersen, {Ronald Carl}",
year = "2017",
month = "3",
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doi = "10.1001/jamaneurol.2016.4926",
language = "English (US)",
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pages = "310--315",
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T1 - Duration and pathologic correlates of Lewy Body Disease

AU - Graff-Radford, Jonathan

AU - Aakre, Jeremiah

AU - Savica, Rodolfo

AU - Boeve, Bradley F

AU - Kremers, Walter K

AU - Ferman, Tanis Jill

AU - Jones, David T

AU - Kantarci, Kejal M

AU - Knopman, David S

AU - Dickson, Dennis W

AU - Kukull, Walter A.

AU - Petersen, Ronald Carl

PY - 2017/3/1

Y1 - 2017/3/1

N2 - 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 stagemay provide important prognostic information to patients with DLB.

AB - 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 stagemay provide important prognostic information to patients with DLB.

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