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Learning Disabilities and Attentional Problems in Boys With the Fragile X Syndrome FREE

Randi Hagerman, MD; Melinda Kemper, MA; Michael Hudson, MD
[+] Author Affiliations

Reprint requests to Child Development Unit, The Children's Hospital, 1056 E 19th Ave, Denver, CO 80218 (Dr Hagerman).


Am J Dis Child. 1985;139(7):674-678. doi:10.1001/archpedi.1985.02140090036021.
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• The fragile X syndrome is a relatively common form of mental retardation that tends to affect boys more severely than girls. The syndrome Is associated with a fragile site at q27 on the X chromosome and with physical features including large or prominent ears and macro-orchidism. Four boys had physical and cytogenetic features of the fragile X syndrome. However, the IQ scores of these patients extended into the normal range. All four patients demonstrated similar learning difficulties that Included hyperactivity, vlsuomotor incoordination, language deficits, and academic delays In mathematics. The fragile X syndrome should be considered in the differential diagnosis of learning disabled children.

(AJDC 1985;139:674-678)

REFERENCES

Turner G, Jacobs P:  Marker (X)-linked mental retardation . Adv Hum Genet 1983;;13:83-112.
Turner G, Daniel A, Frost M:  X-linked mental retardation, macro-orchidism and the Xq27 fragile site . J Pediatr 1980;;96:836-841.
Opitz JM, Sutherland GR:  Conference report: International workshop on the fragile X and X-linked mental retardation . Am J Med Genet 1984;;17:5-94.
Opitz JM, Westphal JM, Daniel A:  Discovery of a connective tissue dysplasia in the Martin-Bell syndrome . Am J Med Genet 1984;; 17:101-109.
Hagerman RJ, Van Housen K, Smith ACM, et al:  Consideration of connective tissue dysfunction in the fragile X syndrome . Am J Med Genet 1984;;17:111-121.
Hagerman RJ, Smith ACM, Mariner R:  Clinical features of the fragile X syndrome , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 17-53.
Brown WT, Friedman E, Jenkins EC, et al:  Association of fragile X syndrome with autism . Lancet 1982;;1:100.
Levitas A, Hagerman RJ, Braden M, et al:  Autism and the fragile X syndrome . JDBP 1983;; 4:151-158.
Meryash DL, Szymanski LS, Gerald PS:  Infantile autism associated with the fragile X syndrome . J Autism Dev Disord 1982;;12:295-301.
Daker MG, Chidiac P, Fear CN, et al:  Fragile X in a normal male: A cautionary tale . Lancet 1981;;2:780.
Webb GC, Rogers JG, Pitt DB, et al:  Transmission of fragile (X) (q27) site from a male. Lancet 1981;;2:1231-1232.
Terman LM, Merrill MA: Stanford-Binet Intelligence Scale , 1972 norms ed. Boston, Houghton Mifflin Co, 1973;.
Wechsler D: Wechsler Intelligence Scale for Children-Revised. New York, The Psychological Corp, 1974;.
Kaufman AS, Kaufman NS: Kaufman Assessment Battery for Children. Circle Pines, Minn, American Guidance Services, 1983;.
Kirk SA, Kirk WD:  On defining learning diabilities . J Learn Disabil 1983;;16:20-21.
Hagerman RJ:  Pediatric assessment of the learning disabled child . JDBP 1984;;5:274-284.
Hagerman RJ, Smith ACM:  The heterozygous female , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 83-94.
Van Roy BC, de Smedt MC, Raes RA, et al:  Fragile X trait in a large kindred: Transmission also through normal males . J Med Genet 1983;; 20:286-289.
Howard-Peebles PN, Friedman JM: Transmission of the fragile X syndrome through unaffected carrier males. Presented to the American Society of Human Genetics, Toronto, Oct 31, 1984.
Camerino G, Mattei MG, Mattei JF, et al:  Close linkage of fragile X-mental retardation syndrome to haemophilia B and transmission through a normal male . Nature 1983;;306:71-704.
Fryns JP, van den Berghe H:  Transmission of fragile (X) (q27) from normal males . Hum Genet 1982;;61:262-263.
Nielsen KB, Tommerup N, Poulsen H, et al:  X-linked mental retardation with fragile X: A pedigree showing transmission by apparently unaffected males and partial expression in female carriers . Hum Genet 1981;;59:23-25.
Sherman SL, Morton NE, Jacobs PA, et al:  The marker (X) syndrome: A cytogenetic and genetic analysis . Ann Hum Genet 1984;;48:21-37.
McGavran L, Maxwell F:  Appendix A: Expression of the fragile X, media requirements, processing and analysis , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 229-234.

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References

Turner G, Jacobs P:  Marker (X)-linked mental retardation . Adv Hum Genet 1983;;13:83-112.
Turner G, Daniel A, Frost M:  X-linked mental retardation, macro-orchidism and the Xq27 fragile site . J Pediatr 1980;;96:836-841.
Opitz JM, Sutherland GR:  Conference report: International workshop on the fragile X and X-linked mental retardation . Am J Med Genet 1984;;17:5-94.
Opitz JM, Westphal JM, Daniel A:  Discovery of a connective tissue dysplasia in the Martin-Bell syndrome . Am J Med Genet 1984;; 17:101-109.
Hagerman RJ, Van Housen K, Smith ACM, et al:  Consideration of connective tissue dysfunction in the fragile X syndrome . Am J Med Genet 1984;;17:111-121.
Hagerman RJ, Smith ACM, Mariner R:  Clinical features of the fragile X syndrome , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 17-53.
Brown WT, Friedman E, Jenkins EC, et al:  Association of fragile X syndrome with autism . Lancet 1982;;1:100.
Levitas A, Hagerman RJ, Braden M, et al:  Autism and the fragile X syndrome . JDBP 1983;; 4:151-158.
Meryash DL, Szymanski LS, Gerald PS:  Infantile autism associated with the fragile X syndrome . J Autism Dev Disord 1982;;12:295-301.
Daker MG, Chidiac P, Fear CN, et al:  Fragile X in a normal male: A cautionary tale . Lancet 1981;;2:780.
Webb GC, Rogers JG, Pitt DB, et al:  Transmission of fragile (X) (q27) site from a male. Lancet 1981;;2:1231-1232.
Terman LM, Merrill MA: Stanford-Binet Intelligence Scale , 1972 norms ed. Boston, Houghton Mifflin Co, 1973;.
Wechsler D: Wechsler Intelligence Scale for Children-Revised. New York, The Psychological Corp, 1974;.
Kaufman AS, Kaufman NS: Kaufman Assessment Battery for Children. Circle Pines, Minn, American Guidance Services, 1983;.
Kirk SA, Kirk WD:  On defining learning diabilities . J Learn Disabil 1983;;16:20-21.
Hagerman RJ:  Pediatric assessment of the learning disabled child . JDBP 1984;;5:274-284.
Hagerman RJ, Smith ACM:  The heterozygous female , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 83-94.
Van Roy BC, de Smedt MC, Raes RA, et al:  Fragile X trait in a large kindred: Transmission also through normal males . J Med Genet 1983;; 20:286-289.
Howard-Peebles PN, Friedman JM: Transmission of the fragile X syndrome through unaffected carrier males. Presented to the American Society of Human Genetics, Toronto, Oct 31, 1984.
Camerino G, Mattei MG, Mattei JF, et al:  Close linkage of fragile X-mental retardation syndrome to haemophilia B and transmission through a normal male . Nature 1983;;306:71-704.
Fryns JP, van den Berghe H:  Transmission of fragile (X) (q27) from normal males . Hum Genet 1982;;61:262-263.
Nielsen KB, Tommerup N, Poulsen H, et al:  X-linked mental retardation with fragile X: A pedigree showing transmission by apparently unaffected males and partial expression in female carriers . Hum Genet 1981;;59:23-25.
Sherman SL, Morton NE, Jacobs PA, et al:  The marker (X) syndrome: A cytogenetic and genetic analysis . Ann Hum Genet 1984;;48:21-37.
McGavran L, Maxwell F:  Appendix A: Expression of the fragile X, media requirements, processing and analysis , in Hagerman RJ, McBogg PM (eds): The Fragile X Syndrome: Diagnosis, Biochemistry and Intervention. Dillon, Colo, Spectra Publishing Co Inc, 1983;, pp 229-234.

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