Frontal white matter integrity in adults with Down syndrome with and without dementia

David Powell, Allison Caban-Holt, Gregory Jicha, William Robertson, Roberta Davis, Brian T. Gold, Frederick A. Schmitt, Elizabeth Head

Research output: Contribution to journalArticlepeer-review

59 Scopus citations


Adults with Down syndrome (DS) are at high risk for developing Alzheimer's disease after the age of 40years. To detect white matter (WM) changes in the brain linked to dementia, fractional anisotropy (FA) from diffusion tensor imaging was used. We hypothesized that adults with DS without dementia (DSn=10), DS with dementia (DSAD n= 10) and age matched non-DS subjects (CTL n= 10) would show differential levels of FA and an association with scores from the Brief Praxis Test and the Severe Impairment Battery. WM integrity differences in DS compared with CTL were found predominantly in the frontal lobes. Across all DS adults, poorer Brief Praxis Test performance correlated with reduced FA in the corpus callosum as well as several association tracts, primarily within frontoparietal regions. Our results demonstrate significantly lower WM integrity in DS compared with controls, particularly in the frontal tracts. DS-related WM integrity reductions in a number of tracts were associated with poorer cognition. These preliminary results suggest that late myelinating frontal pathways may be vulnerable to aging in DS.

Original languageEnglish
Pages (from-to)1562-1569
Number of pages8
JournalNeurobiology of Aging
Issue number7
StatePublished - Jul 2014

Bibliographical note

Funding Information:
The authors are grateful to the participants and their families for enrolling in the study. We appreciate assistance in the study provided by Drs Erin Abner and Richard Kryscio for database support and Dr Brandon Davis and Dr Amy Dowling at the University of Kentucky for assistance with the study. Editing assistance was graciously provided by Ms Paula Thomason at the Sanders-Brown Center on Aging. The authors also would like to thank the Down syndrome Association of Louisville Inc and the Down syndrome Association of Greater Cincinnati for helping with recruitment and outreach. Further, Jose de Leon, MD, Director, UK Research Office; Director, Residency Education, Eastern State Hospital, Allen Brenzel, MD Medical Director, Kentucky Department for Behavioral Health, Developmental and Intellectual Disabilities, Edward N. Maxwell, MD, UK Outpatient Child & Adolescent Psychiatrist, and Harold Kleinert, Ed.D, Professor of Rehabilitation Sciences, Executive Director of the Human Development Institute provided assistance in the early phase of our study. Guidance regarding recruitment and cognitive testing was provided by Dr Ira Lott and Eric Doran at the University of California, Irvine. Research reported in this manuscript was supported by Eunice Kennedy Shriver National Institute of Child Health and Development of the National Institutes of Health under award number R01HD064993 . The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.


  • Aging
  • BPT
  • Brief praxis test
  • DMR
  • Dementia questionnaire for persons with mental retardation
  • Diffusion tensor imaging
  • Fractional anisotropy
  • SIB
  • Severe impairment battery
  • Trisomy 21

ASJC Scopus subject areas

  • Neuroscience (all)
  • Aging
  • Clinical Neurology
  • Developmental Biology
  • Geriatrics and Gerontology


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