title: Familial aggregation of dementia with Lewy bodies.
abstract: BACKGROUND
  Familial aggregation of dementia with Lewy bodies (DLB) remains unclear.
  
  
  OBJECTIVE
  To determine the degree of family aggregation of DLB by comparing DLB risk between siblings of probands with clinically diagnosed DLB and siblings of probands with clinically diagnosed Alzheimer disease in a cohort of Caribbean Hispanic families and to explore the degree of aggregation of specific clinical manifestations (ie, cognitive fluctuations, visual hallucinations, and parkinsonism) in DLB.
  
  
  METHODS
  Familial cohort study.
  
  
  METHODS
  Academic research.
  
  
  METHODS
  We separately compared risks of possible DLB, probable DLB, and clinical core features of DLB (cognitive fluctuations, visual hallucinations, and parkinsonism) between siblings of probands with clinically diagnosed DLB (n = 344) and siblings of probands with clinically diagnosed Alzheimer disease (n = 280) in 214 Caribbean Hispanic families with extended neurologic and neuropsychological assessment.
  
  
  METHODS
  We applied general estimating equations to adjust for clustering within families. In these models, age and proband disease status were independent variables, and disease status of siblings was the measure of disease risk and the dependent variable.
  
  
  RESULTS
  Compared with siblings of probands having clinically diagnosed Alzheimer disease, siblings of probands having clinically diagnosed DLB had higher risks of probable DLB (odds ratio [OR], 2.29; 95% confidence interval [CI], 1.04-5.04) and visual hallucinations (2.32; 1.16-4.64). They also had increased risks of possible DLB (OR, 1.51; 95% CI, 0.97-2.34) and cognitive fluctuations (1.55; 0.95-2.53).
  
  
  CONCLUSIONS
  Dementia with Lewy bodies and core features of DLB aggregate in families. Compared with siblings of probands having clinically diagnosed AD, siblings of probands having clinically diagnosed DLB are at increased risks of DLB and visual hallucinations. These findings are an important step in elucidating the genetic risk factors underlying DLB and in delineating DLB from other neurodegenerative diseases, such as Alzheimer disease.
authors.affiliation: Gertrude H. Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Nervi
authors.forename: Christiane
authors.initials: C
authors.lastname: Reitz
authors.forename: Ming-Xin
authors.initials: MX
authors.lastname: Tang
authors.forename: Vincent
authors.initials: V
authors.lastname: Santana
authors.forename: Angel
authors.initials: A
authors.lastname: Piriz
authors.forename: Dolly
authors.initials: D
authors.lastname: Reyes
authors.forename: Rafael
authors.initials: R
authors.lastname: Lantigua
authors.forename: Martin
authors.initials: M
authors.lastname: Medrano
authors.forename: Ivonne Z
authors.initials: IZ
authors.lastname: Jiménez-Velázquez
authors.forename: Joseph H
authors.initials: JH
authors.lastname: Lee
authors.forename: Richard
authors.initials: R
authors.lastname: Mayeux
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000235:genetics / Q000523:psychology; D017691:Caribbean Region; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006630:Hispanic or Latino / Q000235:genetics; D006801:Humans; D016631:Lewy Bodies / Q000235:genetics*; D020961:Lewy Body Disease / Q000235:genetics* / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests