VISION, COGNITION AND DEVELOPMENTAL CHARACTERISTICS OF GIRLS AND WOMEN WITH RETT-SYNDROME

Forty-two females with Rett syndrome, aged 2.5 to 47 years, were asses sed with the Teller Acuity Cards and a new version of the Fagan test f or age 2 years and above, and their parents were interviewed about the children's communication skills. Thr visual function of the subjects indicated arrested development, and they scored significantly lower on the Fagan test than a normal comparison group. Their visual processin g and memory deteriorated somewhat with age, while those of the compar ison group showed a slight increase. Both age at onset of Rett syndrom e symptomatology and speech measures were inversely correlated with vi sual processing and memory, indicating that age at recession may have differential consequences for different functions. Among the subjects, persistent looking was associated with low cognitive function. The re sults have implications for intervention, and demonstrate that the par adigm of preferential looking may be useful in cognitive assessment of females with Rett syndrome.