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Observational Study
. 2017 Mar 1;74(3):310-315.
doi: 10.1001/jamaneurol.2016.4926.

Duration and Pathologic Correlates of Lewy Body Disease

Jonathan Graff-Radford  1 , Jeremiah Aakre  2 , Rodolfo Savica  1 , Bradley Boeve  1 , Walter K Kremers  2 , Tanis J Ferman  3 , David T Jones  4 , Kejal Kantarci  5 , David S Knopman  1 , Dennis W Dickson  6 , Walter A Kukull  7 , Ronald C Petersen  1
Affiliations
Observational Study

Duration and Pathologic Correlates of Lewy Body Disease

Jonathan Graff-Radford et al. JAMA Neurol. .

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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Conflict of interest statement

Conflict of Interest Disclosures: Dr Knopman reported serving on a data safety monitoring board for Lund beck Pharmaceuticals and for the DIAN study; working as an investigator in clinical trials sponsored by Tau RX Pharmaceuticals, Lilly Pharmaceuticals, and the Alzheimer's Disease Cooperative Study; and receiving research support from the National Institutes of Health. Dr Petersen reported working as a consultant for Merck, Roche, Genentech, Biogen, and Eli Lilly and Company; receiving royalties from publishing Mild Cognitive Impairment (Oxford University Press, 2003); and receiving research support from the National Institutes of Health. Dr Boeve reported receiving royalties from the publication of Behavioral Neurology of Dementia and receiving research support from Cephalon Inc, Allon Therapeutics, Forum Pharmaceuticals, GE Healthcare, and the National Institute on Aging and the Mangurian Foundation. Dr Ferman reported receiving support from the Mayo Clinic Alzheimer's Disease Research Center funded by the National Institutes of Health. Dr Dickson reported receiving research support from grants at the Mayo Clinic, Jacksonville, Florida. Dr Kantarci reported serving on the data safety monitoring board for Takeda Global Research & Development Center Inc, serving on the data monitoring boards of Pfizer and Janssen Alzheimer Immunotherapy, and being funded by the National Institutes of Health. Dr Graff-Radford reported receiving research support from the National Alzheimer's Coordinating Center. No other disclosures were reported.

Figures

Figure
Figure. Change in Mini-Mental State Examination (MMSE) Scores
Change in MMSE scores between transitional and diffuse Lewy body disease.
See this image and copyright information in PMC

References

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