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The Effect of Genetic Differences and Ovarian Failure: Intact Cognitive Function in Adult Women with Premature Ovarian Failure Versus Turner Syndrome

Department of Pediatrics (J.L.R.), Thomas Jefferson University, Philadelphia, Pennsylvania 19107; MossRehab Research Institute (G.A.S.), Albert Einstein Medical Center, Thomas Jefferson University, Philadelphia, Pennsylvania 19141; Biomedical Computer Research Institute (H.K.), Philadelphia, Pennsylvania 19115; Developmental Endocrinology Branch (C.B., L.N.), National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892; Eugene McDermott Center for Human Growth and Development and Department of Internal Medicine (A.Z.), The University of Texas Southwestern Medical School, Dallas Texas 75390; and Department of Medicine (Neurology) (D.R.), Pennsylvania State College of Medicine, Hershey, Pennsylvania 17033

Address all correspondence and requests for reprints to: Judith L. Ross, M.D., Thomas Jefferson University, Department of Pediatrics, 1025 Walnut Street, Philadelphia, Pennsylvania 19107. E-mail: judith.ross{at}mail.tju.edu.

Premature ovarian failure (POF) is generally defined as amenorrhea, hypoestrogenism, and elevated gonadotropins occurring in a woman before the age of 40 yr. Usually, the etiology is unknown. Turner syndrome (TS, monosomy X), also associated with ovarian failure, has a characteristic neurocognitive profile. TS females, as a group, have specific deficits in visual-spatial abilities, visual-perceptual abilities, motor function, nonverbal memory, executive function, and attentional abilities. Observed deficits in TS could be due to endocrine (estrogen deficiency) or genetic factors. If early estrogen deficiency contributes to the cognitive deficits in TS, women with POF would also be at risk for similar findings.

The objective of this work was to examine the specific cognitive profile in women with POF and compare it with women with TS and normal female controls. We compared two unique populations (women with POF vs. TS), both with earlier estrogen deficiency. The TS group only had a major genetic deficiency, absence of all or part of one X chromosome.

We evaluated the cognitive performance of estrogen-repleted women with POF (n = 89), compared with verbal IQ- and socioeconomic status-matched females with TS (n = 94) and controls (n = 96).

Performance by the POF population was similar to that of controls and differed from the TS population. In contrast, TS adults had relative difficulty with measures of spatial/perceptual skills, visual-motor integration, affect recognition, visual memory, attention, and executive function. These deficits are apparent in TS women, despite apparently adequate estrogen treatment.

The cognitive phenotypes of women with POF and normal controls are similar and differ from women with TS, indicating that prior estrogen deficiency does not have a major impact on cognitive function in adult females. The genetic deficiencies of women with TS most likely account for their specific cognitive phenotype.

This work was supported, in part, by NIH Grant NS42777 (to J.L.R.).

Abbreviations: ANCOVA, Analysis of covariance; ERT, estrogen replacement therapy; IQ, intelligence quotient; PANES, Physical and Neurological Examination of Soft Signs; POF, premature ovarian failure; SES, socioeconomic status; TS, Turner syndrome; WAIS-R, Wechsler Adult Intelligence Scale-Revised; WMS-R, Wechlser Memory Scale-Revised; WRAT-III, Wide Range Achievement Test-III.

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