Orlee Udwin

Cognitive Functioning in Adults with Williams Syndrome

This paper reports the results of cognitive, linguistic, and academic assessments in a representative sample of 62 adults with Williams syndrome. The average age of the group was 26 years and their mean full scale IQ was 61. Differences between Verbal and Performance IQ, and between receptive and expressive language skills, were smaller than generally found in studies of children with this condition. However, an examination of subtest scores revealed an almost identical cognitive profile to that found in children. Skills in other areas, such as reading, spelling, arithmetic, and social adaptation remained at a low level, with functioning generally being around a 6-8-year age equivalent. The consistency of reports on intellectual abilities in both child and adult studies of individuals with Williams syndrome lends increased support to the notion of a syndrome specific pattern of cognitive, linguistic, and adaptive functioning.

A LONGITUDINAL STUDY OF COGNITIVE ABILITIES AND EDUCATIONAL ATTAINMENT IN WILLIAMS SYNDROME

The paper reports on changes in IQ scores and in reading, spelling and arithmetic skills over time in 23 young adults with Williams syndrome. They were first assessed in their early to mid‐teens and followed up 8 to 9 years later, at an average age of 21 years 9 months. Cognitive assessments indicated increases in Full Scale, Verbal and Performance IQ scores. These increases allow us to conclude that in the case of Williams syndrome (unlike some other conditions) there does not appear to be a decline in the rate of cognitive development over time. Comparisons of Reading. Spelling and Arithmetic scores attained at first and second testing periods revealed only modest increases in reading accuracy and spelling scores, a slight decline in reading comprehension scores, and little change in arithmetic test scores. Differences in the tests used at the two assessment periods do not allow for definitive conclusions to be drawn, but the findings suggest that individuals with Williams syndrome make little progress in their educational skills beyond their early teenage years.

Augmentative communication systems taught to cerebral palsied children-a longitudinal study. I. The acquisition of signs and symbols, and syntactic aspects of their use over time

A longitudinal study of augmentative communication training with 40 language-impaired, cerebral palsied children examined the acquisition of Blissymbols and Makaton Vocabulary signs and syntactic aspects of their use over an 18-month period. The children made measurable gains in these areas over time, but progress was very slow and they continued to show severe limitations in the repertoires of signs/symbols they acquired and in the average number, length and complexity of utterances they produced in semi-structured conversational settings. Possible reasons for these findings are discussed with reference to subject and system characteristics, and the teaching practices adopted in the schools. Comparisons between the Bliss and signing groups suggest that neither augmentative system facilitated greater progress in sign/symbol acquisition and use than the other. However, there was wide variability in performance among the children within each group.

Outcomes in neurodevelopmental and genetic disorders

Preface Patricia Howlin 1. Attention deficit hyperactivity disorder Jody Warner-Rogers 2. Developmental language disorders Nancy J. Cohen 3. Reading and other specific learning disorders Arlene R. Young and Joseph H. Beitchman 4. Metabolic disorders Anupam Chakrapani and John Walter 5. Hemiplegic cerebral palsy Robert Goodman 6. Autistic disorders Patricia Howlin 7. Down syndrome Janet Carr 8. Fragile X syndrome Randi J. Hagerman and Paul J. Hagerman 9. Prader-Willi syndrome and Angelman syndromes: from childhood to adult life A. J. Holland and J. Butler 10. Rett disorder Alison Kerr 11. Tuberous sclerosis Petrus J. de Vries and Patrick F. Bolton 12. Williams syndrome and Smith Magenis syndrome Orlee Udwin Index.

Augmentative communication systems taught to cerebral-palsied children-a longitudinal study. II. Pragmatic features of sign and symbol use

Pragmatic analysis of the sign and symbol utterances produced by 40 language-impaired, cerebral-palsied children in semi-structured conversational settings revealed severe restrictions in the range of communicative functions that were used. The children were able to communicate certain conversational acts effectively, but over 80% of all utterances expressed just four communicative functions. There were also some significant gaps, with certain functions not being used at all. The childrens progress was followed up at 6-month intervals over a period of 1 1/2 years. Few changes were found in the range and relative frequencies of communicative functions expressed over time. There were few significant differences between the symbol and sign users on these measures, which suggests that overall neither augmentative mode facilitated greater communicative use than the other.

Augmentative communication systems taught to cerebral-palsied children-a longitudinal study. III. Teaching practices and exposure to sign and symbol use in schools and homes

A longitudinal study of augmentative communication training with 40 language-impaired, cerebral-palsied children examined the teaching practices adopted in schools, and the extent to which teachers and parents used the augmentative systems with the children. The study found limited exposure to sign and symbol training in formal teaching sessions and in other school settings, and teachers made relatively few attempts to foster spontaneous use of the systems and generalisation outside of formal training settings. Exposure to augmentative communication in the childrens homes was equally limited, and a significant number of parents expressed reservations about the value of the systems for their children. These findings may at least partly account for the childrens limited progress in sign and symbol acquisition and use over an 18-month period, which was documented in two earlier reports.