Use of estrogen in young girls with Turner syndrome - Effects on memory

Background: The Turner syndrome (TS) phenotype is characterized by a specif ic neurocognitive profile of normal verbal skills, impaired visual-spatial and visual-perceptual abilities, and impaired nonverbal more than verbal me mory. We compared verbal and nonverbal memory in estrogen- and placebo-trea ted girls with TS (ages 7 to 9 years) and age-matched female controls. Meth ods: Children received either estrogen (ethinyl estradiol, 25 ng/kg/d) or p lacebo for 1 to 3 years (mean, 2.1 +/- 0.9 years) in a randomized, double-b lind study. Memory and language tasks administered included the Wechsler in telligence Scale for Children-Revised, Digit Span (forward and backward), t he Children's Word List, the Denman Paragraph, the Peabody Picture Vocabula ry Test, Boston Naming, immediate and delayed Recall of the Rey Complex Fig ure, Nonword Reading, Wide Range Achievement Test-Revised reading subtest, Verbal fluency, and the Token Test. Results: The estrogen-treated TS group performed better than the placebo-treated TS group for the Children's Ward List immediate and delayed recall and the Digit Span backwards test (p < 0. 01 to 0.04), although the results were not significant after adjusting for multiple comparisons. The placebo-treated TS group performed less well than the controls for recall of Digit Span backward (p < 0.0001; placebo-treate d, 2.8 +/- 1.3; estrogen-treated, 3.4 +/- 1.2; and controls, 4.2 +/- 1.3) a nd immediate and delayed recall of the Children's Word List (delayed recall , p < 0.0001; placebo-treated, 6.2 +/- 3.1; estrogen-treated, 8.0 +/- 2.9; and controls, 9.0 +/- 2.9). Performance for these measures was similar for the estrogen-treated TS group and the control group. Conclusions: Estrogen replacement therapy in young girls with Turner Syndrome is associated with improved verbal and nonverbal memory. The optimal patient age, dose, and du ration of estrogen replacement require further study.