Twin pairs discordant for neuropathologically confirmed Lewy body dementia.


Journal Article

AIM: Little is known about the concordance rate in twins for dementia with Lewy bodies (DLB). The rate of agreement between clinical and pathological diagnoses for DLB is typically low, necessitating confirmation of the diagnosis neuropathologically. METHODS: Participants were 17 twin pairs enrolled in the Duke Twins Study of Memory in Aging in which at least one member of the pair had an autopsy confirmed diagnosis of DLB, Alzheimer's disease (AD) with Lewy bodies or frontotemporal dementia with Lewy bodies. The characteristics of those with dementia were assessed and rates of concordance for pathological confirmed dementia were examined. RESULTS: Four monozygotic twin pairs had a proband with neuropathologically confirmed pure DLB; all remained discordant for dementia for periods up to 16 years or more. Five of 13 pairs in which the proband had AD plus DLB were concordant for dementia but only one pair was concordant for AD plus DLB, while the co-twins in the other four pairs had other types of dementia. CONCLUSIONS: The present study indicates that even among twins, a diagnosis of DLB in one twin does not predict the same diagnosis in the other twin. Neuropathological discordance in type of dementia among monozygotic pairs hints at environmental or epigenetic factors playing a role in Lewy body pathology.

Full Text

Duke Authors

Cited Authors

  • Wang, CS-M; Burke, JR; Steffens, DC; Hulette, CM; Breitner, JCS; Plassman, BL

Published Date

  • May 2009

Published In

Volume / Issue

  • 80 / 5

Start / End Page

  • 562 - 565

PubMed ID

  • 19372291

Pubmed Central ID

  • 19372291

Electronic International Standard Serial Number (EISSN)

  • 1468-330X

Digital Object Identifier (DOI)

  • 10.1136/jnnp.2008.151654


  • eng

Conference Location

  • England