Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Supplemental data
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Ross, J. L.
Right arrow Articles by Roeltgen, D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Ross, J. L.
Right arrow Articles by Roeltgen, D.
Related Collections
Right arrow All Neuropsychology/Behavior
Right arrow All Cognitive Disorders/Dementia
Right arrow All Genetics
Neurology 2002;58:218-225
© 2002 American Academy of Neurology

Persistent cognitive deficits in adult women with Turner syndrome

J. L. Ross, MD, G. A. Stefanatos, DPhil, H. Kushner, PhD, A. Zinn, MD PhD;, C. Bondy, MD and D. Roeltgen, MD

From the Department of Pediatrics (Dr. Ross), Thomas Jefferson University, AI Dupont Hospital for Children, and MossRehab Research Institute (Dr. Stefanatos), Albert Einstein Medical Center, and Biomedical Computer Research Institute (Dr. Kushner), Philadelphia, PA; Eugene McDermott Center for Human Growth and Development and Department of Internal Medicine (Dr. Zinn), The University of Texas Southwestern Medical School, Dallas TX; Developmental Endocrinology Branch (Dr. Bondy), National Institute of Child Health and Human Development, [National Institutes of Health], Bethesda, MD; and Department of Medicine (Neurology) (Dr. Roeltgen), Pennsylvania State College of Medicine, Hershey, PA.

Address correspondence and reprint requests to Dr. Judith L. Ross, Thomas Jefferson University, Department of Pediatrics, 1025 Walnut Street, Philadelphia, PA 19107; e-mail: Judith.Ross{at}mail.tju.edu

Background: Turner syndrome (TS) has a characteristic neurocognitive profile. Verbal abilities are, in general, normal; however, women with TS, as a group, have specific deficits in visual-spatial abilities, visual-perceptual abilities, motor function, nonverbal memory, executive function, and attentional abilities. Observed deficits could be caused by genetic or endocrine factors.

Objective: To evaluate the specific cognitive deficits that appear to persist in adulthood, are not estrogen-responsive, and may be genetically determined.

Methods: The cognitive performance of adult women with TS (n = 71) who were estrogen repleted was compared with verbal IQ– and socioeconomic status–matched female controls (n = 50). Sixty-one women with TS had ovarian failure and received estrogen replacement and 10 had preserved endogenous ovarian function and were not receiving estrogen replacement at the time of evaluation.

Results: Similar to children and adolescents with TS, adults with TS have normal verbal IQ but have relative difficulty on measures of spatial/perceptual skills, visual-motor integration, affect recognition, visual memory, attention, and executive function despite estrogen replacement. These deficits are apparent in women with TS despite apparently adequate estrogen effect, either endogenous or by hormone replacement.

Conclusion: The cognitive phenotypes of adults with TS, with or without ovarian failure, are similar, indicating that estrogen replacement does not have a major impact on the cognitive deficits of adults with TS.




This article has been cited by other articles:


Home page
 J Child NeurolHome page
R. C. Knickmeyer and S. Baron-Cohen
Topical Review: Fetal Testosterone and Sex Differences in Typical Social Development and in Autism
J Child Neurol, October 1, 2006; 21(10): 825 - 845.
[Abstract] [PDF]

Home page
 Cereb CortexHome page
S. R. Kesler, V. Menon, and A. L. Reiss
Neurofunctional Differences Associated with Arithmetic Processing in Turner Syndrome
Cereb Cortex, June 1, 2006; 16(6): 849 - 856.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
D. H. Skuse, J. S. Morris, and R. J. Dolan
Functional dissociation of amygdala-modulated arousal and cognitive appraisal, in Turner syndrome
Brain, September 1, 2005; 128(9): 2084 - 2096.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
V. P. Sybert and E. McCauley
Turner's Syndrome
N. Engl. J. Med., September 16, 2004; 351(12): 1227 - 1238.
[Full Text] [PDF]

Home page
 Cereb CortexHome page
N. Molko, A. Cachia, D. Riviere, J.F. Mangin, M. Bruandet, D. LeBihan, L. Cohen, and S. Dehaene
Brain Anatomy in Turner Syndrome: Evidence for Impaired Social and Spatial-Numerical Networks
Cereb Cortex, August 1, 2004; 14(8): 840 - 850.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Endocrinol. Metab.Home page
J. L. Ross, G. A. Stefanatos, H. Kushner, C. Bondy, L. Nelson, A. Zinn, and D. Roeltgen
The Effect of Genetic Differences and Ovarian Failure: Intact Cognitive Function in Adult Women with Premature Ovarian Failure Versus Turner Syndrome
J. Clin. Endocrinol. Metab., April 1, 2004; 89(4): 1817 - 1822.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
C. D. Good, K. Lawrence, N. S. Thomas, C. J. Price, J. Ashburner, K. J. Friston, R. S. J. Frackowiak, L. Oreland, and D. H. Skuse
Dosage-sensitive X-linked locus influences the development of amygdala and orbitofrontal cortex, and fear recognition in humans
Brain, November 1, 2003; 126(11): 2431 - 2446.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2002 by AAN Enterprises, Inc.