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J Neurol Neurosurg Psychiatry 2009;80:562-565 doi:10.1136/jnnp.2008.151654
  • Short report

Twin pairs discordant for neuropathologically confirmed Lewy body dementia

  1. C Sheei-Meei Wang1,
  2. J R Burke3,5,
  3. D C Steffens2,
  4. C M Hulette4,5,
  5. J C S Breitner6,
  6. B L Plassman2
  1. 1
    Department of Psychiatry, Tainan Hospital, Department of Health, Taiwan
  2. 2
    Department of Psychiatry and Behavioral Sciences, Duke University Medical Center, Durham, North Carolina, USA
  3. 3
    Department of Neurology, Duke University Medical Center, Durham, North Carolina, USA
  4. 4
    Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA
  5. 5
    Joseph and Kathleen Bryan Alzheimer’s Disease Research Center, Duke University Medical Center, Durham, North Carolina, USA
  6. 6
    VA Puget Sound Health Care System and the Department of Psychiatry and Behavioral Sciences, University of Washington School of Medicine, Seattle, Washington, USA
  1. Dr B L Plassman, 905 W Main Street, Box 41, Ste 25-D, Duke University Medical Center, Durham, NC 27701, USA; brenda.plassman{at}duke.edu
  • Received 10 April 2008
  • Revised 20 August 2008
  • Accepted 26 September 2008

Abstract

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.

Footnotes

  • Funding: NIH AG-08549 (JCSB, BLP), American Health Assistance Foundation #95112 (JCSB) and NIH AG-05128 and AG-02837 (CMH).

  • Competing interests: None.

  • Ethics approval: Ethics approval was obtained.

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