Archive

Articles on Medical Research funded by the Williams Syndrome Foundation:

Reports on current medical research projects by Jill Boucher. more >>

Visual and Visuo-spatial development in children with Williams Syndrome. more >>

Progress report on WSF studentship awarded to Jill Boucher and held by Jon Brock in the Department of Psychology at the University of Warwick. more >>

Research Update: Emotional and Behavioural Difficulties and Needs of Adults with Williams Syndrome - By Dr Orlee Udwin
In 1993 the Williams Syndrome Foundation awarded a research grant to Dr Orlee Udwin and Professor Pat Howlin to undertake a study into the adjustment difficulties and needs of adults with Williams Syndrome, and over the following two years Mark Davies travelled across the country in order to interview the parents/carers, tutors and supervisors of a sample of 70 adults with the syndrome. Most of the adults themselves were also interviewed. The interviews sought information on their living arrangements and daily living skills, their daytime activities, social and personal relationships, and behavioural and emotional difficulties.more >>

Are all aspects of visuo-spatial cognition equally impaired in Williams Syndrome? - by Emily Farran - PhD Studentship
The weakest area of cognition in WS is the area of visuo-spatial processing. Relatively little research has focused on this area. I aim to investigate whether all aspects of visuo-spatial cognition are equally impaired in WS. Previous research suggests that this is not the case and that the profile of performance shown by WS individuals on different visuo-spatial tasks shows a number of strengths and weaknesses. Through research I intend to make suggestions as to the potential causes for this unusual pattern of abilities which may have implications for intervention strategies and teaching methods. more >>

The Williams Syndrome Foundation PhD Studentship
The Williams Syndrome Foundation awarded a 3-year PhD studentship to Professor Annette Karmiloff-Smith at the newly formed Neurocognitive Development Unit at the Institute of Child Health, starting October 1999. It will be supervised byProfessor Karmiloff-Smith and Dr Emma Laing, another specialist in cognitive development. The area of study will be the numerical processing of infants, children and adults with Williams Syndrome.

A recent PhD supervised by Professor Karmiloff-Smith and financed by the Down's Syndrome Association had as part of its focus the relationship between number skills and language in Down's syndrome and Williams syndrome. This provided some clear evidence of the difficulties which people with Williams Syndrome experience with number. The new PhD will build on the results of this initial research.

Further in-depth studies are required to fully understand the underlying processing skills involved and how such skills develop from early infancy to adulthood. An important aim of the project is to examine whether it is the absence of certain number-specific predispositions prior to learning or problems with the learning processes themselves which lead to subsequent difficulties with number. It is hoped that by focusing on very early atypical development we will, in the future, be in a position to adopt remedial strategies at the time when the neocortical circuits of the brain are in their period of greatest plasticity.

It is proposed that the PhD student will study first infants and toddlers and later, older children and young adults. The previous work focused on toddlers from 24 to 36 months. In the present PhD the aim is to see infants in the first 6 to 24 months of life, as more infants are now being diagnosed early on. Traditional infancy techniques will be employed (e.g. habituation and preference paradigms). These techniques have been used to examine early predispositions to number processing in typically developing infants as young as 3 months of age.

The study will also use non-invasive brain imaging techniques in collaboration with the Event Related Potential (ERP) laboratory at Birkbeck College. The aim of this approach is to determine whether subsequent brain circuitry specialisation for number takes place or not. Infants will be tested at 6 month intervals to gather both longitudinal and cross-sectional data. Reuslts will be compared to 2 groups of normal controls, mental age matched, and chronological aged matched. The student will be given full training in infancy and brain imaging techniques and in the administration of standardised clinical tests.

Experiments will also be carried out with older children and adults to examine the number processing skills of individuals with Williams Syndrome who have reached the end state. An important aim will be to ascertain which number principles participants abide by, (e.g. one-to-one mapping, ordinality and cardinality principles) and which they do not. The relationship between number skills and reading may also be explored, since Dr. Laing is an expert in the field of reading. In this way, it is hoped to tease apart domain-specific impairments from domain-general ones.

The 3 year studentship should provide the opportunity to further our understanding of the number skills of individuals with WS and allow us to consider what the most appropriate remedial strategies might be. The Institute of Child Health is an excellent research environment and provides PhD students with the most up-to-date training facilities.
The Neurocognitive Development Unit's website is at: http://www.ich.ucl.ac.uk/units/ncdu/NDU_homepage.htm