Family History Is Associated with Phenotype in Dementia with Lewy Bodies

Leonie J. M. Vergouw, Brechje Bosman, Marleen van de Beek, Mariet Salomé, Susanne E. Hoogers, Inger van Steenoven, Gerwin Roks, Vincenzo Bonifati, John C. van Swieten, Afina W. Lemstra, Frank Jan de Jong

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Abstract

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.

Original languageEnglish
Pages (from-to)269-275
Number of pages7
JournalJournal of Alzheimer's Disease
Volume73
Issue number1
Early online date19 Nov 2019
DOIs
Publication statusPublished - 2020

Keywords

  • Dementia with Lewy bodies
  • family history
  • phenotype
  • survival

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