Cognitive profile of Turner syndrome

Developmental Disabilities Research Reviews

Volumn 15, Issue 4, 2009, Pages 270-278

  Hong, David ^a   Kent, Jamie Scaletta ^a   Kesler, Shelli ^a  

^a STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Cognitive; Executive function; Social cognition; Turner syndrome; Visual spatial; X monosomy

Indexed keywords

COGNITION; COGNITIVE DEFECT; FRAGILE X SYNDROME; HUMAN; INTELLIGENCE QUOTIENT; MONOSOMY; PHENOTYPE; PRIORITY JOURNAL; REVIEW; SOCIAL COMPETENCE; TURNER SYNDROME; WORKING MEMORY; X CHROMOSOME;

EID: 73849148675     PISSN: 19405510     EISSN: 10982779     Source Type: Journal    
DOI: 10.1002/ddrr.79     Document Type: Review

References (99)

1. Adolphs R. 2009. The social brain: neural basis of social knowledge. Ann Rev Psychol 60: 693-716.
2. Alexander D, Ehrhardt AA, Money J. 1966. Defective figure drawing, geometric and human, in Turner's syndrome. J Nerv Ment Dis 142:161-167.
3. Annaz D, Karmiloff-Smith A, Johnson MH, et al. 2009. A cross-syndrome study of the development of holistic face recognition in children with autism. Down syndrome, and Williams syndrome. J Exp Child Psychol 102:456-486.
4. Bender B, Puck M, Salbenblatt J, et al. 1984. Cognitive development of unselected girls with complete and partial X monosomy. Pediatrics 73:175-182.
5. Bender BG, Linden MG, Robinson A. 1993. Neuropsychological impairment in 42 adolescents with sex chromosome abnormalities. Am J Med Genet 48:169-173.
6. Bergamaschi R, Bergonzoni C, Mazzanti L, et al. 2008. Hearing loss in Turner syndrome: results of a multicentric study. J Endocrinol Invest 31:779-783.
7. Bishop DV, Canning E, Elgar K, et al. 2000. Distinctive patterns of memory function in subgroups of females with Turner syndrome: evidence for imprinted loci on the X-chromosome affecting neurodevelopment. Neuropsychologia 38:712-721.
8. Brown WE, Kesler SR, Eliez S, et al. 2004. A volumetric study of parietal lobe subregions in Turner syndrome. Dev Med Child Neurol 46:607-609.
9. Bruandet M, Molko N, Cohen L, et al. 2004. A cognitive characterization of dyscalculia in Turner syndrome. Neuropsychologia 42: 288-298.
10. Buchanan L, Pavlovic J, Rovet J. 1998. The contribution of visuospatial working memory to impairments in facial processing and arithmetic in Turner syndrome. Brain Cogn 37:72-75.
11. Carel JC, Elie C, Ecosse E, et al. 2006. Selfesteem and social adjustment in young women with Turner syndrome - influence of pubertal management and sexuality: population-based cohort study. J Clin Endocrinol Metab 91:2972-2979.
12. Collaer ML, Geffner ME, Kaufman FR, et al. 2002. Cognitive and behavioral characteristics of turner syndrome: exploring a role for ovarian hormones in female sexual differentiation. Horm Behav 41:139-155.
13. Cornoldi C, Marconi F, Vecchi T. 2001. Visuospatial working memory in Turner's syndrome. Brain Cogn 46:90-94.
14. Cutter WJ, Daly EM, Robertson DM, et al. 2006. Influence of X chromosome and hormones on human brain development: a magnetic resonance imaging and proton magnetic resonance spectroscopy study of Turner syndrome. Biol Psychiatry 59:273-283.
15. Downey J, Ehrhardt AA, Gruen R, et al. 1989. Psychopathology and social functioning in women with Turner syndrome. J Nerv Ment Dis 177:191-201.
16. Downey J, Elkin EJ, Ehrhardt AA, et al. 1991. Cognitive ability and everyday functioning in women with Turner syndrome. J Learn Disabil 24:32-39.
17. Elgar K, Campbell R, Skuse D. 2002. Are you looking at me? Accuracy in processing line of-sight in Turner syndrome. Proc R Soc Lond B Biol Sci 269:2415-2422.
18. Everhart DE, Shucard JL, Quatrin T, et al. 2004. Tone probe event-related potential differences during a face recognition task in prepubertal children and Turner Syndrome girls. Psychoneuroendocrinology 29:1260-1271.
19. Garron DC. 1977. Intelligence among persons with Turner's syndrome. Behav Genet 7: 105-127.
20. Good CD, Lawrence K, Thomas NS, et al. 2003. Dosage-sensitive X-linked locus influences the development of amygdala and orbitofrontal cortex, and fear recognition in humans. Brain J Neurol 126 (Part 11): 2431-2446.
21. Hart SJ, Davenport ML, Hooper SR, et al. 2006. Visuospatial executive function in Turner syndrome: functional MRI and neurocognitive findings. Brain 129(Part 5): 1125-1136.
22. Hepworth SL, Rovet JF. 2000. Visual integration difficulties in a 9-year-old girl with Turner syndrome: parallel verbal disabilities? Child Neuropsychol 6:262-273.
23. Holzapfel M, Barnea-Goraly N, Eckert MA, et al. 2006. Selective alterations of white matter associated with visuospatial and sensorimotor dysfunction in turner syndrome. J Neurosci 26:7007-7013.
24. Hultcrantz M, Sylven L. 1997. Turner's syndrome and hearing disorders in women aged 16-34. Hear Res 103:69-74.
25. Inozemtseva O, Matute E, Zarabozo D, et al. 2002. Syntactic processing in Turner's syndrome. J Child Neurol 17:668-672.
26. Itier RJ, Batty M. 2009. Neural bases of eye and gaze processing: the core of social cognition. Neurosci Biobehav Rev 33:843-863.
27. Joseph RM, Tanaka J. 2003. Holistic and part-based face recognition in children with autism. J Child Psychol Psychiatry 44:529-542.
28. Kesler SR, Haberecht MF, Menon V, et al. 2004. Functional neuroanatomy of spatial orientation processing in Turner syndrome. Cereb Cortex 14:174-180.
29. Kesler SR, Menon V, Reiss AL. 2006. Neurofunctional differences associated with arithmetic processing in Turner syndrome. Cereb Cortex 16:849-856.
30. Kirk JW, Mazzocco MM, Kover ST. 2005. Assessing executive dysfunction in girls with fragile X or Turner syndrome using the Contingency Naming Test (CNT). Dev Neuropsychol 28:755-777.
31. Lagrou K, Froidecoeur C, Verlinde F, et al. 2006. Pyschosocial functioning, self-perception and body image and their auxologic correlates in growth hormone and oestrogen-treated young adult women with Turner syndrome. Horm Res 66:277-284.
32. LaHood BJ, Bacon GE. 1985. Cognitive abilities of adolescent Turner's syndrome patients. J Adolesc Health Care 6:358-364.
33. Lawrence K, Campbell R, Swettenham J, et al. 2003. Interpreting gaze in Turner syndrome: impaired sensitivity to intention and emotion, but preservation of social cueing. Neuropsychologia 41:894-905.
34. Lawrence K, Jones A, Oreland L, et al. 2007. The development of mental state attributions in women with X-monosomy, and the role of monoamine oxidase B in the sociocognitive phenotype. Cognition 102:84-100.
35. Lawrence K, Kuntsi J, Coleman M, et al. 2003. Face and emotion recognition deficits in Turner syndrome: a possible role for X-linked genes in amygdala development. Neuropsychology 17:39-49.
36. Loesch DZ, Bui QM, Kelso W, et al. 2005. Effect of Turner's syndrome and X-linked imprinting on cognitive status: analysis based on pedigree data. Brain Dev 27:494-503.
37. Mazzocco MM. 1998. A process approach to describing mathematics difficulties in girls with Turner syndrome. Pediatrics 102(2, Part 3):492-496.
38. Mazzocco MM. 2001. Math learning disability and math LD subtypes: evidence from studies of Turner syndrome, fragile X syndrome, and neurofibromatosis type 1. J Learn Disabil 34:520-533.
39. Mazzocco MM, Singh Bhatia N, Lesniak-Karpiak K. 2006a. Visuospatial skills and their association with math performance in girls with fragile X or Turner syndrome. Child Neuropsychol 12:87-110.
40. Mazzocco MM, Thompson L, Sudhalter V, et al. 2006b. Language use in females with fragile X or Turner syndrome during brief initial social interactions. J Dev Behav Pediatr 27:319-328.
41. Mazzola F, Seigal A, MacAskill A, et al. 2006. Eye tracking and fear recognition deficits in Turner syndrome. Soc Neurosci 1:259-269.
42. McCauley E, Feuillan P, Kushner H, et al. 2001. Psychosocial development in adolescents with Turner syndrome. J Dev Behav Pediat 22:360-365.
43. McCauley E, Kay T, Ito J, et al. 1987. The Turner syndrome: cognitive deficits, affective discrimination, and behavior problems. Child Dev 58:464-473.
44. McCauley E, Sybert VP, Ehrhardt AA. 1986. Psychosocial adjustment of adult women with Turner syndrome. Clin Genet 29:284-290.
45. Messina MF, Zirilli G, Civa R, et al. 2007. Neurocognitive profile in Turner's syndrome is not affected by growth impairment. Disabil Rehabil Endocrinol Metab 20:677-684.
46. Murphy DG, Allen G, Haxby JV, et al. 1994. The effects of sex steroids, and the X chromosome, on female brain function: a study of the neuropsychology of adult Turner syndrome. Neuropsychologia 32:1309-1323.
47. Murphy DG, DeCarli C, Daly E, et al. 1993. Xchromosome effects on female brain: a magnetic resonance imaging study of Turner's syndrome. Lancet 342:1197-1200.
48. Murphy MM, Mazzocco MM. 2008. Mathematics learning disabilities in girls with fragile X or Turner syndrome during late elementary school. J Learn Disabil 41:29-46.
49. Nijhuis-van der Sanden RW, Smits-Engelsman BC, Eling PA. 2000. Motor performance in girls with Turner syndrome. Dev Med Child Neurol 42:685-690.
50. Pavlidis K, McCauley E, Sybert VP. 1995. Psychosocial and sexual functioning in women with Turner syndrome. Clin Genet 47:85-89.
51. Pennington BF, Bender B, Puck M, et al. 1982. Learning disabilities in children with sex chromosome anomalies. Child Dev 53: 1182-1192.
52. Pennington BF, Heaton RK, Karzmark P, et al. 1985. The neuropsychological phenotype in Turner syndrome. Cortex 21:391-404.
53. Rae C, Joy P, Harasty J, et al. 2004. Enlarged temporal lobes in Turner syndrome: an Xchromosome effect? Cereb Cortex 14:156-164.
54. Reiss AL, Freund L, Plotnick L, et al. 1993. The effects of X monosomy on brain development: monozygotic twins discordant for Turner's syndrome. Ann Neurol 34:95-107.
55. Reske-Nielsen E, Christensen AL, Nielsen J. 1982. A neuropathological and neuropsychological study of Turner's syndrome. Cortex 18:181-190.
56. Romans SM, Roeltgen DP, Kushner H, et al. 1996. Executive function in females with Turner syndrome. Arch Clin Neuropsych 11: 442.
57. Romans SM, Stefanatos G, Roeltgen DP, et al. 1998. Transition to young adulthood in Ullrich-Turner syndrome: neurodevelopmental changes. Am J Med Genet 79:140-147.
58. Ross J, Roeltgen D, Zinn A. 2006. Cognition and the sex chromosomes: studies in Turner syndrome. Horm Res 65:47-56.
59. Ross J, Zinn A, McCauley E. 2000. Neurodevelopmental and psychosocial aspects of Turner syndrome. Ment Retard Dev Disabil Res Rev 6:135-141.
60. Ross JL, Feuillan P, Kushner H, et al. 1997. Absence of growth hormone effects on cognitive function in girls with Turner syndrome. J Clin Endocrinol Metab 82:1814-1817.
61. Ross JL, Kushner H, Roeltgen DP. 1996a. Developmental changes in motor function in girls with Turner syndrome. Pediatr Neurol 15: 317-322.
62. Ross JL, Kushner H, Zinn AR. 1997. Discriminant analysis of the Ullrich-Turner syndrome neurocognitive profile. Am J Med Genet 72:275-280.
63. Ross JL, McCauley E, Roeltgen D, et al. 1996b. Self-concept and behavior in adolescent girls with Turner syndrome: potential estrogen effects. J Clin Endocrinol Metab 81:926-931.
64. Ross JL, 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 83:3198-3204.
65. Ross JL, Roeltgen D, Stefanatos GA, et al. 2003. Androgen-responsive aspects of cognition in girls with Turner syndrome. J Clin Endocrinol Metab 88:292-296.
66. Ross JL, Stefanatos GA, Kushner H, et al. 2004. 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 89:1817-1822.
67. Ross JL, Stefanatos GA, Kushner H, et al. 2002. Persistent cognitive deficits in adult women with Turner syndrome. Neurology 58:218-225.
68. Rovet JF. 1993a. The psychoeducational characteristics of children with Turner syndrome. J Learn Disabil 26:333-341.
69. Rovet J, Ireland L. 1994. Behavioral phenotype in children with Turner syndrome. J Pediatr Psychol 19:779-790.
70. Rovet J, Netley C. 1980. The mental rotation task performance of Turner syndrome subjects. Behav Genet 10:437-443.
71. Rovet J, Netley C. 1982. Processing deficits in Turner's syndrome. Dev Psychol 18:77-94.
72. Rovet J, Szekely C, Hockenberry MN. 1994. Specific arithmetic calculation deficits in children with Turner syndrome. J Clin Exp Neuropsychol 16:820-839.
73. Shaffer JW. 1962. A specific cognitive deficit observed in gonadal aplasia (Turner's syndrome). J Clin Psychol 18:403-406.
74. Sheaffer AT, Lange E, Bondy CA. 2008. Sexual function in women with Turner syndrome. J Womens Health (Larchmt) 17:27-33.
75. Siegel PT, Clopper R, Stabler B. 1998. The psychological consequences of Turner syndrome and review of the National Cooperative Growth Study psychological substudy. Pediatrics 102(2, Part 3):488-491.
76. Silbert A, Wolff PH, Lilienthal J. 1977. Spatial and temporal processing in patients with Turner's syndrome. Behav Genet 7:11-21.
77. Simon TJ, 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.
78. Skuse D, Elgar K, Morris E. 1999. Quality of life in Turner syndrome is related to chromosomal constitution: implications for genetic counselling and management. Acta Paediatr Suppl 88:110-113.
79. Skuse D, Lawrence K, Tang J. 2005a. Measuring social-cognitive functions in children with somatotropic axis dysfunction. Horm Res 64(Suppl 3):73-82.
80. Skuse DH, James RS, Bishop DV, et al. 1997. Evidence from Turner's syndrome of an imprinted X-linked locus affecting cognitive function. Nature 387:705-708.
81. Skuse DH, Morris JS, Dolan RJ. 2005b. Functional dissociation of amygdala-modulated arousal and cognitive appraisal, in Turner syndrome. Brain 128(Part 9):2084-2096.
82. Swillen A, Fryns JP, Kleczkowska A, et al. 1993. Intelligence, behaviour and psychosocial development in Turner syndrome. A crosssectional study of 50 pre-adolescent and adolescent girls (4-20 years). Genet Couns 4:7-18.
83. Temple CM, Carney R, Mullarkey S. 1996. Frontal lobe function and executive skills in children with Turner's syndrome. Dev Neuropsychol 12:343-363.
84. Temple CM. 2002. Oral fluency and narrative production in children with Turner's syndrome. Neuropsychologia 40:1419-1427.
85. Temple CM, Carney R. 1996. Reading skills in children with Turner's syndrome: an analysis of hyperplexia. Cortex 32:335-345.
86. Temple CM, Carney RA. 1993. Intellectual functioning of children with Turner syndrome: a comparison of behavioural phenotypes. Dev Med Child Neurol 35:691-698.
87. Temple CM, Carney RA. 1995. Patterns of spatial functioning in Turner's syndrome. Cortex 31:109-118.
88. Temple CM, Marriott AJ. 1998. Arithmetical ability and disability in Turner's syndrome: a cognitive neuropsychological analysis. Dev Neuropsychol 14:47-67.
89. van Pareren YK, Duivenvoorden HJ, Slijper FM, et al. 2005. Psychosocial functioning after discontinuation of long-term growth hormone treatment in girls with Turner syndrome. Horm Res 63:238-244.
90. Waber DP. 1979. Neuropsychological aspects of Turner's syndrome. Dev Med Child Neurol 21:58-70.
91. Watanabe K, Ogino T, Nakano K, et al. 2005. The Rey-Osterrieth complex figure as a measure of executive function in childhood. Brain Dev 27:564-569.
92. Wechsler D. 1949. Wechsler Intelligence Scale for Children. San Antonio, TX: Harcourt Assessments.
93. Wechsler D. 1955. Wechsler Adult Intelligence Scale. San Antonio, TX: Harcourt Assessments.
94. Wechsler D. 1967. Wechsler Preschool and Primary Scale of Intelligence. San Antonio, TX: Harcourt Assessments.
95. Wechsler D. 1974. Wechsler Intelligence Scale for Children-Revised. San Antonio, TX: Harcourt Assessments.
96. Wechsler D. 1981. Wechsler Adult Intelligence Scale-Revised. San Antonio, TX: Harcourt Assessments.
97. Wechsler D. 1989. Wechsler Preschool and Primary Scale of Intelligence-Revised. San Antonio, TX: Harcourt Assessments.
98. Wechsler D. 2003. Wechsler Intelligence Scale for Children, 4th ed. San Antonio: The Psychological Corporation.
99. Zinn AR, Roeltgen D, Stefanatos G, et al. 2007. A Turner syndrome neurocognitive phenotype maps to Xp22.3. Behav Brain Funct 3:24.