Developmental and behavioural disturbances in 13 boys with fragile X syndrome

✍ Scribed by R. H. Largo; A. Schinzel

• Publisher: Springer
• Year: 1985
• Tongue: English
• Weight: 601 KB
• Volume: 143
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00442299

✦ Synopsis

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. (1) 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 < 0.01). ( 2) Language development was grossly delayed in all boys; most had severe articulation problems. (3) Imitative and symbolic play (e.g. doll play) were strikingly retarded as compared to abstract play (e.g. block design). ( 4) 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. ( 5) 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. (6) 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.