Abstract

Estrogen and androgen appear to influence brain function in females at puberty. Environmental and cultural factors interact with the biological effects of estrogen and androgen on he brain an consequently on cognition and behavior. Women with Turner syndrome have dysgenetic ovaries that do not produce estrogen or androgen, before or at puberty. Therefore, Turner syndrome represents a unique, sex hormone-deficient, model in which to study the biological effects of androgen on cognition and behavior. The overall goal of this project is to study design. we will study (1) two Turner syndrome groups: (A) androgen and (B) no androgen and (2) an age-matched, normal female control group.
The specific aims of this project are to: 1) examine the effects of androgen on cognitive and social function in adolescent (11-13 years), growth hormone-treated girls with Turner syndrome, and 2) documented the differences and similarities in cognitive and behavioral function between adolescent Turner syndrome girls (treated or not treated wit androgen) and age-and VIQ-girls treated with androgen, or not androgen-treated: (1) Turner syndrome girls treated with androgen versus no androgen will perform better on the tests of visual-spatial ability and visual-motor ability, (2) Turner syndrome girls treated with androgen versus no androgen will not perform differently on tests of attention, executive function, social function, and affective competence, (3) Turner girls treated with androgen for 2 years will demonstrate the greatest treatment effects, compared to girls treated for 1 year, and (4) androgen treatment will significantly reduce the differences between Turner syndrome and normal controls girls on tests executive function, social function, and affective competence. This investigation of adolescent cognitive and social development is an important step in understanding normal brain development. In addition, these data will determine how to optimize cognitive function in Turner syndrome, and will extend knowledge of the underlying mechanisms of sexual dimorphism.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
5R01NS032531-02
Application #
2270795
Study Section
Human Development and Aging Subcommittee 3 (HUD)
Project Start
1994-01-01
Project End
1998-12-31
Budget Start
1995-03-02
Budget End
1995-12-31
Support Year
2
Fiscal Year
1995
Total Cost
Indirect Cost

  Institution

Name
Thomas Jefferson University
Department
Pediatrics
Type
Schools of Medicine
DUNS #
061197161
City
Philadelphia
State
PA
Country
United States
Zip Code
19107

  Publications

Lepage, J-F; Hong, D S; Raman, M et al. (2014) Brain morphology in children with 47, XYY syndrome: a voxel- and surface-based morphometric study. Genes Brain Behav 13:127-34
Beaton, Elliott A; Stoddard, Joel; Lai, Song et al. (2010) Atypical functional brain activation during a multiple object tracking task in girls with Turner syndrome: neurocorrelates of reduced spatiotemporal resolution. Am J Intellect Dev Disabil 115:140-56
Ross, Judith L; Mazzocco, Michele M M; Kushner, Harvey et al. (2009) Effects of treatment with oxandrolone for 4 years on the frequency of severe arithmetic learning disability in girls with Turner syndrome. J Pediatr 155:714-20
Zinn, Andrew R; Kushner, Harvey; Ross, Judith L (2008) EFHC2 SNP rs7055196 is not associated with fear recognition in 45,X Turner syndrome. Am J Med Genet B Neuropsychiatr Genet 147B:507-9
Simon, T J; Takarae, Y; DeBoer, T et al. (2008) Overlapping numerical cognition impairments in children with chromosome 22q11.2 deletion or Turner syndromes. Neuropsychologia 46:82-94
Russell, Heather F; Wallis, Deeann; Mazzocco, Michele M M et al. (2006) Increased prevalence of ADHD in Turner syndrome with no evidence of imprinting effects. J Pediatr Psychol 31:945-55
Mazzocco, Michele M M; Thompson, Laurie; Sudhalter, Vicki et al. (2006) Language use in females with fragile X or Turner syndrome during brief initial social interactions. J Dev Behav Pediatr 27:319-28
Ross, Judith L (2005) Effects of growth hormone on cognitive function. Horm Res 64 Suppl 3:89-94
Wilson, Courtnay A; Heinrichs, Claudine; Larmore, Kimberly A et al. (2003) Estradiol levels in girls with Turner's syndrome compared to normal prepubertal girls as determined by an ultrasensitive assay. J Pediatr Endocrinol Metab 16:91-6
Ross, J L; Roeltgen, D; Stefanatos, G A et al. (2003) Androgen-responsive aspects of cognition in girls with Turner syndrome. J Clin Endocrinol Metab 88:292-6

Showing the most recent 10 out of 18 publications