Family History is Associated with Phenotype in Dementia with Lewy Bodies

J Alzheimers Dis. 2020;73(1):269-275. doi: 10.3233/JAD-190825.


It is currently unknown whether patients with dementia with Lewy bodies (DLB) with relatives with dementia or Parkinson's disease (familial DLB patients) have a different phenotype than sporadic DLB patients. In this study, we aimed to examine disease onset, rate of cognitive decline, survival, and Alzheimer's disease (AD) biomarkers in patients with familial DLB (n = 154) and sporadic DLB (n = 137), using linear mixed model analysis and Cox regression analysis, among others. Familial patients had a shorter survival (8.0 years) and more often elevated cerebrospinal fluid AD biomarkers (47%) than sporadic patients (9.0 years; p≤0.001; 30%, p = 0.037). Our findings suggest that genetic factors are important in DLB and that the identification of new genetic factors will probably improve the prediction of prognosis.

Keywords: Dementia with Lewy bodies; family history; phenotype; survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Alzheimer Disease / cerebrospinal fluid
  • Biomarkers / cerebrospinal fluid
  • Cognitive Dysfunction
  • Family
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Lewy Body Disease / diagnostic imaging*
  • Lewy Body Disease / epidemiology
  • Male
  • Middle Aged
  • Phenotype
  • Prognosis
  • Retrospective Studies
  • Survival Analysis


  • Biomarkers