Differences in age-related effects on brain volume in Down syndrome as compared to Williams syndrome and typical development

Mary Ellen I. Koran, Timothy J. Hohman, Courtney M. Edwards, Jennifer N. Vega, Jennifer Pryweller, Laura E. Slosky, Genea Crockett, Lynette Villa De Rey, Shashwath A. Meda, Nathan Dankner, Suzanne N. Avery, Jennifer U. Blackford, Elisabeth M. Dykens, Tricia A. Thornton-Wells

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Abstract

Background: Individuals with Down Syndrome (DS) are reported to experience early onset of brain aging. However, it is not well understood how pre-existing neurodevelopmental effects versus neurodegenerative processes might be contributing to the observed pattern of brain atrophy in younger adults with DS. The aims of the current study were to: (1) to confirm previous findings of age-related changes in DS compared to adults with typical development (TD), (2) to test for an effect of these age-related changes in a second neurodevelopmental disorder, Williams syndrome (WS), and (3) to identify a pattern of regional age-related effects that are unique to DS. Methods: High-resolution T1-weighted MRI of the brains of subjects with DS, WS, and TD controls were segmented, and estimates of regional brain volume were derived using FreeSurfer. A general linear model was employed to test for age-related effects on volume between groups. Secondary analyses in the DS group explored the relationship between brain volume and neuropsychological tests and APOE. Results: Consistent with previous findings, the DS group showed significantly greater age-related effects relative to TD controls in total gray matter and in regions of the orbitofrontal cortex and the parietal cortex. Individuals with DS also showed significantly greater age-related effects on volume of the left and right inferior lateral ventricles (LILV and RILV, respectively). There were no significant differences in age-related effects on volume when comparing the WS and TD groups. In the DS group, cognitive tests scores measuring signs of dementia and APOE ε4 carrier status were associated with LILV and RILV volume. Conclusions: Individuals with DS demonstrated a unique pattern of age-related effects on gray matter and ventricular volume, the latter of which was associated with dementia rating scores in the DS group. Results may indicate that early onset of brain aging in DS is primarily due to DS-specific neurodegenerative processes, as opposed to general atypical neurodevelopment.

Original languageEnglish (US)
Article number8
JournalJournal of Neurodevelopmental Disorders
Volume6
Issue number1
DOIs
StatePublished - Apr 9 2014

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Williams Syndrome
Down Syndrome
Brain
Dementia
Parietal Lobe
Neuropsychological Tests
Lateral Ventricles
Prefrontal Cortex

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cognitive Neuroscience

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Differences in age-related effects on brain volume in Down syndrome as compared to Williams syndrome and typical development. / Koran, Mary Ellen I.; Hohman, Timothy J.; Edwards, Courtney M.; Vega, Jennifer N.; Pryweller, Jennifer; Slosky, Laura E.; Crockett, Genea; De Rey, Lynette Villa; Meda, Shashwath A.; Dankner, Nathan; Avery, Suzanne N.; Blackford, Jennifer U.; Dykens, Elisabeth M.; Thornton-Wells, Tricia A.

In: Journal of Neurodevelopmental Disorders, Vol. 6, No. 1, 8, 09.04.2014.

Research output: Contribution to journalArticle

Koran, MEI, Hohman, TJ, Edwards, CM, Vega, JN, Pryweller, J, Slosky, LE, Crockett, G, De Rey, LV, Meda, SA, Dankner, N, Avery, SN, Blackford, JU, Dykens, EM & Thornton-Wells, TA 2014, 'Differences in age-related effects on brain volume in Down syndrome as compared to Williams syndrome and typical development', Journal of Neurodevelopmental Disorders, vol. 6, no. 1, 8. https://doi.org/10.1186/1866-1955-6-8
Koran, Mary Ellen I. ; Hohman, Timothy J. ; Edwards, Courtney M. ; Vega, Jennifer N. ; Pryweller, Jennifer ; Slosky, Laura E. ; Crockett, Genea ; De Rey, Lynette Villa ; Meda, Shashwath A. ; Dankner, Nathan ; Avery, Suzanne N. ; Blackford, Jennifer U. ; Dykens, Elisabeth M. ; Thornton-Wells, Tricia A. / Differences in age-related effects on brain volume in Down syndrome as compared to Williams syndrome and typical development. In: Journal of Neurodevelopmental Disorders. 2014 ; Vol. 6, No. 1.
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abstract = "Background: Individuals with Down Syndrome (DS) are reported to experience early onset of brain aging. However, it is not well understood how pre-existing neurodevelopmental effects versus neurodegenerative processes might be contributing to the observed pattern of brain atrophy in younger adults with DS. The aims of the current study were to: (1) to confirm previous findings of age-related changes in DS compared to adults with typical development (TD), (2) to test for an effect of these age-related changes in a second neurodevelopmental disorder, Williams syndrome (WS), and (3) to identify a pattern of regional age-related effects that are unique to DS. Methods: High-resolution T1-weighted MRI of the brains of subjects with DS, WS, and TD controls were segmented, and estimates of regional brain volume were derived using FreeSurfer. A general linear model was employed to test for age-related effects on volume between groups. Secondary analyses in the DS group explored the relationship between brain volume and neuropsychological tests and APOE. Results: Consistent with previous findings, the DS group showed significantly greater age-related effects relative to TD controls in total gray matter and in regions of the orbitofrontal cortex and the parietal cortex. Individuals with DS also showed significantly greater age-related effects on volume of the left and right inferior lateral ventricles (LILV and RILV, respectively). There were no significant differences in age-related effects on volume when comparing the WS and TD groups. In the DS group, cognitive tests scores measuring signs of dementia and APOE ε4 carrier status were associated with LILV and RILV volume. Conclusions: Individuals with DS demonstrated a unique pattern of age-related effects on gray matter and ventricular volume, the latter of which was associated with dementia rating scores in the DS group. Results may indicate that early onset of brain aging in DS is primarily due to DS-specific neurodegenerative processes, as opposed to general atypical neurodevelopment.",
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T1 - Differences in age-related effects on brain volume in Down syndrome as compared to Williams syndrome and typical development

AU - Koran, Mary Ellen I.

AU - Hohman, Timothy J.

AU - Edwards, Courtney M.

AU - Vega, Jennifer N.

AU - Pryweller, Jennifer

AU - Slosky, Laura E.

AU - Crockett, Genea

AU - De Rey, Lynette Villa

AU - Meda, Shashwath A.

AU - Dankner, Nathan

AU - Avery, Suzanne N.

AU - Blackford, Jennifer U.

AU - Dykens, Elisabeth M.

AU - Thornton-Wells, Tricia A.

PY - 2014/4/9

Y1 - 2014/4/9

N2 - Background: Individuals with Down Syndrome (DS) are reported to experience early onset of brain aging. However, it is not well understood how pre-existing neurodevelopmental effects versus neurodegenerative processes might be contributing to the observed pattern of brain atrophy in younger adults with DS. The aims of the current study were to: (1) to confirm previous findings of age-related changes in DS compared to adults with typical development (TD), (2) to test for an effect of these age-related changes in a second neurodevelopmental disorder, Williams syndrome (WS), and (3) to identify a pattern of regional age-related effects that are unique to DS. Methods: High-resolution T1-weighted MRI of the brains of subjects with DS, WS, and TD controls were segmented, and estimates of regional brain volume were derived using FreeSurfer. A general linear model was employed to test for age-related effects on volume between groups. Secondary analyses in the DS group explored the relationship between brain volume and neuropsychological tests and APOE. Results: Consistent with previous findings, the DS group showed significantly greater age-related effects relative to TD controls in total gray matter and in regions of the orbitofrontal cortex and the parietal cortex. Individuals with DS also showed significantly greater age-related effects on volume of the left and right inferior lateral ventricles (LILV and RILV, respectively). There were no significant differences in age-related effects on volume when comparing the WS and TD groups. In the DS group, cognitive tests scores measuring signs of dementia and APOE ε4 carrier status were associated with LILV and RILV volume. Conclusions: Individuals with DS demonstrated a unique pattern of age-related effects on gray matter and ventricular volume, the latter of which was associated with dementia rating scores in the DS group. Results may indicate that early onset of brain aging in DS is primarily due to DS-specific neurodegenerative processes, as opposed to general atypical neurodevelopment.

AB - Background: Individuals with Down Syndrome (DS) are reported to experience early onset of brain aging. However, it is not well understood how pre-existing neurodevelopmental effects versus neurodegenerative processes might be contributing to the observed pattern of brain atrophy in younger adults with DS. The aims of the current study were to: (1) to confirm previous findings of age-related changes in DS compared to adults with typical development (TD), (2) to test for an effect of these age-related changes in a second neurodevelopmental disorder, Williams syndrome (WS), and (3) to identify a pattern of regional age-related effects that are unique to DS. Methods: High-resolution T1-weighted MRI of the brains of subjects with DS, WS, and TD controls were segmented, and estimates of regional brain volume were derived using FreeSurfer. A general linear model was employed to test for age-related effects on volume between groups. Secondary analyses in the DS group explored the relationship between brain volume and neuropsychological tests and APOE. Results: Consistent with previous findings, the DS group showed significantly greater age-related effects relative to TD controls in total gray matter and in regions of the orbitofrontal cortex and the parietal cortex. Individuals with DS also showed significantly greater age-related effects on volume of the left and right inferior lateral ventricles (LILV and RILV, respectively). There were no significant differences in age-related effects on volume when comparing the WS and TD groups. In the DS group, cognitive tests scores measuring signs of dementia and APOE ε4 carrier status were associated with LILV and RILV volume. Conclusions: Individuals with DS demonstrated a unique pattern of age-related effects on gray matter and ventricular volume, the latter of which was associated with dementia rating scores in the DS group. Results may indicate that early onset of brain aging in DS is primarily due to DS-specific neurodegenerative processes, as opposed to general atypical neurodevelopment.

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