Developmental and behavioural disturbances in 13 boys with fragile X syndrome

Developmental and behavioural aspects were studied in 13 boys aged 2.6-12.5 years from three families with the fragile X syndrome. The following observations were made. Moderate to severe retardation was present in all boys; non-verbal IQs ranged between 25 and 67 (mean 46 +/- 14); IQ and age were n...

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Bibliographic Details
Published in:European journal of pediatrics 1985-03, Vol.143 (4), p.269-275
Main Authors: LARGO, R. H, SCHINZEL, A
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Child Behavior Disorders - etiology
Child, Preschool
Chromosome fragility (bloom syndrome, ataxia telangiectasia, fanconi anemia, x-linked mental retardation...)
Developmental Disabilities - etiology
Fragile X Syndrome - complications
Fragile X Syndrome - diagnosis
Fragile X Syndrome - psychology
Humans
Intelligence
Intelligence Tests
Language Development
Male
Medical genetics
Medical sciences
Motor Skills
Pedigree
Play and Playthings
Sex Chromosome Aberrations - complications
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Summary:Developmental and behavioural aspects were studied in 13 boys aged 2.6-12.5 years from three families with the fragile X syndrome. The following observations were made. Moderate to severe retardation was present in all boys; non-verbal IQs ranged between 25 and 67 (mean 46 +/- 14); IQ and age were negatively correlated (P less than 0.01). Language development was grossly delayed in all boys; most had severe articulation problems. Imitative and symbolic play (e.g. doll play) were strikingly retarded as compared to abstract play (e.g. block design). Autistic features such as no use of eye contact, stereotyped movements and echolalia were found in 9/13 boys; the same number showed aggressive behaviour. General activity was reduced during the 1st year of life; most boys became very hyperactive during the second year; and short attention span and increased distractability were observed in all. Motor development was mildly delayed; all boys were clumsy and moderately hypotonic. The fragile X syndrome ought to be considered in retarded boys with a dissociated developmental pattern, in particular a striking delay in language and play development, and autistic features.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF00442299