Early development in Williams syndrome.

BACKGROUND: The aim of the present study was to gain a better understanding of the motor performance in Williams syndrome for early intervention of rehabilitation programs.

METHODS: Eleven Williams syndrome patients were evaluated from the pediatric clinics. Seven patients younger than 42 months were evaluated with the Bayley II Test for mental development index (MDI) and psychomotor development index (PDI). Four patients older than 42 months were evaluated with the Bruininks-Oseretsky Test (short form) of motor profile. The raw scores were measured and converted to the standard scores for comparison.

RESULTS: A significantly mental and psychomotor development delay of 6.1 and 5.7 months individually was found compared to that of the mean age (P > 0.0047 and 0.0053). Juvenile Williams syndrome patients were apparently retarded (<10 per thousand rank) in motor development in comparison with persons of the same age. The results showed that motor performance was severely delayed not only in Williams syndrome children but also in Williams syndrome juveniles.

CONCLUSION: It is important for clinicians to work out a comprehensive plan to help the motor development of patients with Williams syndrome in addition to treating their medical problems, and upper limb dexterity may be the goal for training.