Environmental and cultural factors interact with estrogen to influence the brain, cognition, and behavior in females before, during, and shortly after puberty. Women with Turner syndrome (TS) lack estrogen as a result of dysgenetic ovaries. Thus, TS women offer a special estrogen-deficient model to study biological effects of estrogen on cognition and behavior. This proposal is a continuation, expansion, and enhancement of a prior award to evaluate the impact of estrogen replacement therapy in 8 - and 12-year old girls. Incorporating both cross-sectional and longitudinal design elements with the addition of a 16-year old group, the proposal will evaluate long-term effects of hormones upon visuo-spatial, social, and affective competencies in TS women.
Specific aims i nclude: 1) examine the effects of continuous estrogen replacement in early childhood on cognitive and social function in a unique, randomized, double-blind, placebo-controlled, treatment trial; and 2) document further the cognitive differences between TS girls at ages 8, 12, and 16 years and age-matched control girls. Specifically, the investigators hypothesize that estrogen treatment will be associated with improved performance on certain tasks (motor, visuospatial, visual-motor, and social) in TS girls, and that cumulative estrogen treatment (4 yrs +) in TS girls will be associated with improved psychological well being at age 16 versus 12, and at age 12 versus 8. In addition, the investigators hypothesize that the cognitive differences between estrogen-treated TS subjects and controls will be similar at the follow-up evaluations (12 - and 16-year old phases) in comparison to the initial (8- and 12-year olds) evaluation, suggesting neurocognitive deficiencies are not completely reversible under estrogen therapy and despite pubertal onset. The investigators also hypothesize that TS cognitive abilities reflect problems with """"""""encoding and transforming of visual spatial information"""""""" associated with atypical functioning of the left hemisphere. The proposed continuing line of research offers to enhance understanding of normal brain development, help to optimize cognitive functioning in TS girls, and generally extend knowledge of the mechanisms of sexual dimorphism.
| Ross, J L; Stefanatos, G A; Kushner, H et al. (2002) Persistent cognitive deficits in adult women with Turner syndrome. Neurology 58:218-25 |
| McCauley, E; Feuillan, P; Kushner, H et al. (2001) Psychosocial development in adolescents with Turner syndrome. J Dev Behav Pediatr 22:360-5 |
| Ross, J; Zinn, A; McCauley, E (2000) Neurodevelopmental and psychosocial aspects of Turner syndrome. Ment Retard Dev Disabil Res Rev 6:135-41 |
| Ross, J L; Roeltgen, D; Feuillan, P et al. (2000) Use of estrogen in young girls with Turner syndrome: effects on memory. Neurology 54:164-70 |
| Ross, J L; Roeltgen, D; Feuillan, P et al. (1998) Effects of estrogen on nonverbal processing speed and motor function in girls with Turner's syndrome. J Clin Endocrinol Metab 83:3198-204 |
| Romans, S M; Stefanatos, G; Roeltgen, D P et al. (1998) Transition to young adulthood in Ullrich-Turner syndrome: neurodevelopmental changes. Am J Med Genet 79:140-7 |
| Ross, J L; Feuillan, P; Kushner, H et al. (1997) Absence of growth hormone effects on cognitive function in girls with Turner syndrome. J Clin Endocrinol Metab 82:1814-7 |
| Ross, J L; Kushner, H; Zinn, A R (1997) Discriminant analysis of the Ullrich-Turner syndrome neurocognitive profile. Am J Med Genet 72:275-80 |
| Ross, J L; Kushner, H; Roeltgen, D P (1996) Developmental changes in motor function in girls with Turner syndrome. Pediatr Neurol 15:317-22 |
| Ross, J L; McCauley, E; Roeltgen, D et al. (1996) Self-concept and behavior in adolescent girls with Turner syndrome: potential estrogen effects. J Clin Endocrinol Metab 81:926-31 |
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