DOI: 10.1111/j.1399-0004.1987.tb03351.x. Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients

Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients

10.1111/j.1399-0004.1987.tb03351.x

Crossref journal-article

Wiley

Clinical Genetics (311)

Abstract

In this study a further analysis of the psychological profile in the prepubertal fragile (X) (fra(X)) male was performed. The results of the psycho‐diagnostic examination of 23 fra(X) boys were compared to a control group of 17 males of the same age with ‘non‐specific’ mental retardation. A number of important quantitative and qualitative differences were observed between the two groups. In the preschool age group the majority of fra(X) boys was mildly mentally retarded. In the school‐age group, however, most boys were moderately to severely mentally retarded. This indication of a decline in intellectual performance with age in the fra(X) syndrome was confirmed by a longitudinal individual follow‐up of seven fra(X) boys in this age group. In contrast to intellectual performance, appearance of the attention deficit disorder (or hyperkinesis), with its attendent overactivity and impulsiveness, decreases with age, and is independent of the intellectual level. Autistic behaviour was more frequently observed in the youngest fra(X) males, and was more pronounced in the moderately mentally retarded. In more than 50% of the boys of preschool age the association of hyperkinesis and autistic features was found. Language and speech development in the fra(X) syndrome is both symptomatic and specific. Beside a severe, global speech retardation, there are some distinct speech characteristics in the young fra(X) males such as rapid speech rhythm, speech impulsiveness and perseverative speech.

Bibliography

Borghgraef, M., Fryns, J. P., Dlelkens, A., Pyck, K., & Berghe, H. V. D. (1987). Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients. Clinical Genetics, 32(3), 179â186. Portico.

Authors 5

M. Borghgraef (first)
J. P. Fryns (additional)
A. Dlelkens (additional)
K. Pyck (additional)
H. Van Den Berghe (additional)

References 37 Referenced 71

10.1111/j.1399-0004.1982.tb00965.x
{'key': 'e_1_2_1_3_1', 'first-page': '5', 'article-title': 'A chromosomal survey of an institution for mentally retarded', 'volume': '30', 'author': 'Brøndum‐Nielsen K.', 'year': '1983', 'journal-title': 'Dan. Med. Bull.'} / Dan. Med. Bull. / A chromosomal survey of an institution for mentally retarded by Brøndum‐Nielsen K. (1983)
{'key': 'e_1_2_1_4_1', 'first-page': '399', 'volume-title': 'Cyto‐genetics of the Mammalian X Chromosome', 'author': 'Carpenter N. J.', 'year': '1983'} / Cyto‐genetics of the Mammalian X Chromosome by Carpenter N. J. (1983)
10.1001/archpedi.1982.03970410010002
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{'key': 'e_1_2_1_7_1', 'first-page': '1247', 'article-title': 'X‐gebonden mentale retarda‐tie en fragiel X‐chromosoom', 'volume': '39', 'author': 'Fryns J. P.', 'year': '1983', 'journal-title': 'Tijdschr. Geneeskd.'} / Tijdschr. Geneeskd. / X‐gebonden mentale retarda‐tie en fragiel X‐chromosoom by Fryns J. P. (1983)
10.1111/j.1399-0004.1984.tb01099.x
10.1111/j.1399-0004.1984.tb00474.x
{'key': 'e_1_2_1_10_1', 'first-page': '33', 'volume-title': 'Aspects of Human Genetics', 'author': 'Fryns J. P.', 'year': '1984'} / Aspects of Human Genetics by Fryns J. P. (1984)
{'key': 'e_1_2_1_11_1', 'first-page': '415', 'volume-title': 'Cytogenetics of the Mammalian Chromosome', 'author': 'Glover T.', 'year': '1983'} / Cytogenetics of the Mammalian Chromosome by Glover T. (1983)
{'key': 'e_1_2_1_12_1', 'first-page': '674', 'article-title': 'Learning disabilities and attentionai problems in boys with the fragile X syndrome', 'volume': '139', 'author': 'Hagerman R.', 'year': '1985', 'journal-title': 'Am. J. Dis. Child.'} / Am. J. Dis. Child. / Learning disabilities and attentionai problems in boys with the fragile X syndrome by Hagerman R. (1985)
{'key': 'e_1_2_1_13_1', 'first-page': '17', 'volume-title': 'The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention', 'author': 'Hagerman R.', 'year': '1983'} / The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention by Hagerman R. (1983)
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10.1002/ajmg.1320070407
{'key': 'e_1_2_1_16_1', 'first-page': '15', 'volume-title': 'W.P.P.S.I. profile form', 'author': 'Hobby K. L.', 'year': '1976'} / W.P.P.S.I. profile form by Hobby K. L. (1976)
{'key': 'e_1_2_1_17_1', 'first-page': '431', 'volume-title': 'Cytogenetics of the Mammalian X Chromosome', 'author': 'Howard‐Peebles P. N.', 'year': '1983'} / Cytogenetics of the Mammalian X Chromosome by Howard‐Peebles P. N. (1983)
{'key': 'e_1_2_1_18_1', 'first-page': '172', 'article-title': 'Psychologische aspecten van het fragiel X‐syndroom', 'volume': '7', 'author': 'Jacobs J.', 'year': '1983', 'journal-title': 'Tijdschr. Orthoped. Kinderpsych.'} / Tijdschr. Orthoped. Kinderpsych. / Psychologische aspecten van het fragiel X‐syndroom by Jacobs J. (1983)
10.1002/ajmg.1320070408
{'key': 'e_1_2_1_20_1', 'first-page': '5', 'volume-title': 'De Utrechtse taalniveau Test voor 4–7 jarigen', 'author': 'Kohnstamm G. A.', 'year': '1971'} / De Utrechtse taalniveau Test voor 4–7 jarigen by Kohnstamm G. A. (1971)
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{'key': 'e_1_2_1_23_1', 'first-page': '153', 'volume-title': 'The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention', 'author': 'Levitas A.', 'year': '1983'} / The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention by Levitas A. (1983)
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{'key': 'e_1_2_1_25_1', 'first-page': '175', 'volume-title': 'The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention', 'author': 'Newell K.', 'year': '1983'} / The Fragile X Syndrome. Diagnosis, Biochemistry and Intervention by Newell K. (1983)
{'issue': '1', 'key': 'e_1_2_1_26_1', 'article-title': 'X‐Linked mental retardation 2', 'volume': '23', 'author': 'Opitz J. M.', 'year': '1986', 'journal-title': 'Am. J. Med. Genet.'} / Am. J. Med. Genet. / X‐Linked mental retardation 2 by Opitz J. M. (1986)
10.1002/ajmg.1320170111
{'key': 'e_1_2_1_28_1', 'volume-title': 'Reynell Developmental Language Scales (revised)', 'author': 'Reynell J.', 'year': '1977'} / Reynell Developmental Language Scales (revised) by Reynell J. (1977)
10.1002/ajmg.1320170113
{'key': 'e_1_2_1_30_1', 'volume-title': 'Stutsmann Intelligenti‐etest Voor Kleuters', 'author': 'Smulders F. J. M.', 'year': '1981'} / Stutsmann Intelligenti‐etest Voor Kleuters by Smulders F. J. M. (1981)
{'key': 'e_1_2_1_31_1', 'volume-title': 'Snijders‐Oomen Niet‐Verbale Intelligentie‐schaal. S.O.N. 2 2/17', 'author': 'Snijders J. Th.', 'year': '1975'} / Snijders‐Oomen Niet‐Verbale Intelligentie‐schaal. S.O.N. 2 2/17 by Snijders J. Th. (1975)
{'key': 'e_1_2_1_32_1', 'first-page': '158', 'volume-title': 'W.P.P.S.I.: Wechsler Preschool and Primary Scale of Intelligence. Handleiding bij de Vlaam‐se aanpassing', 'author': 'Stinissen J.', 'year': '1981'} / W.P.P.S.I.: Wechsler Preschool and Primary Scale of Intelligence. Handleiding bij de Vlaam‐se aanpassing by Stinissen J. (1981)
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10.1007/BF00273283
{'key': 'e_1_2_1_35_1', 'first-page': '24', 'volume-title': 'Auti‐schaal. Ten behoeve van de onderkenning van vroegkinderlijk autisme', 'author': 'Berckelaer‐Onnes I. A.', 'year': '1981'} / Auti‐schaal. Ten behoeve van de onderkenning van vroegkinderlijk autisme by Berckelaer‐Onnes I. A. (1981)
{'key': 'e_1_2_1_36_1', 'first-page': '183', 'volume-title': 'Baylen Ontwikkelingsschalen. BOS 2–30. Handleiding', 'author': 'Meulen B. F.', 'year': '1983'} / Baylen Ontwikkelingsschalen. BOS 2–30. Handleiding by Meulen B. F. (1983)
{'key': 'e_1_2_1_37_1', 'volume-title': 'Wechsler Intelligence Scale for Children. Revised. Nederlandstalige Uitgave. Handleiding voor Instructies en Scoring', 'author': 'Haasen P.', 'year': '1985'} / Wechsler Intelligence Scale for Children. Revised. Nederlandstalige Uitgave. Handleiding voor Instructies en Scoring by Haasen P. (1985)
{'key': 'e_1_2_1_38_1', 'first-page': '947', 'article-title': 'Cytogenetic abnormalities including the marker X chromosome in patients with severe mental retardation', 'volume': '62', 'author': 'Venter P. A.', 'year': '1982', 'journal-title': 'S. African Med. J.'} / S. African Med. J. / Cytogenetic abnormalities including the marker X chromosome in patients with severe mental retardation by Venter P. A. (1982)

Dates

Type	When
Created	15 years, 1 month ago (July 19, 2010, 12:25 a.m.)
Deposited	1 year, 10 months ago (Oct. 21, 2023, 1:11 p.m.)
Indexed	2 days, 6 hours ago (Sept. 4, 2025, 9:25 a.m.)
Issued	38 years ago (Sept. 1, 1987)
Published	38 years ago (Sept. 1, 1987)
Published Online	17 years, 2 months ago (June 28, 2008)
Published Print	38 years ago (Sept. 1, 1987)

Funders 0

None

BibTeX

@article{Borghgraef_1987, title={Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients}, volume={32}, ISSN={1399-0004}, url={http://dx.doi.org/10.1111/j.1399-0004.1987.tb03351.x}, DOI={10.1111/j.1399-0004.1987.tb03351.x}, number={3}, journal={Clinical Genetics}, publisher={Wiley}, author={Borghgraef, M. and Fryns, J. P. and Dlelkens, A. and Pyck, K. and Berghe, H. Van Den}, year={1987}, month=sep, pages={179–186} }

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