Research Archive

Visuo-spatial cognition >> More

Are all aspects of visuo-spatial cognition equally impaired in Williams Syndrome?

PhD Studentship: Emily Farran

Supervisors: Dr. Chris Jarrold and Prof. Sue Gathercole

Previous research has shown that individuals with Williams Syndrome perform significantly better at verbal tasks than visual and spatial tasks. This is shown when measuring IQ using the Weschler Intelligence Scale for Children (WISC; Weschler, 1981). This test battery is divided into measurements of Verbal IQ and Performance IQ (which is a composite of mainly visuo-spatial subtests). Most individuals with WS have a significantly higher Verbal IQ than Performance IQ.

Within the domain of Performance IQ, the typical scores achieved by these individuals vary considerably between tasks, indicating that there are strengths and weaknesses within visuo-spatial cognition. It is this variation in visuo-spatial ability that I am investigating. Within the WISC, Williams Syndrome individuals usually do better on the Picture Completion subtest (this involves the participant identifying which part is missing from a familiar item) and the Object Assembly subtest ( the participant is required to assemble an object presented in jig-saw form) and less well on the Block Design subtest ( the participant uses coloured blocks to construct a pattern shown to them as a 2D image) and the Coding subtest ( a series of numbers are given corresponding symbols and the participant must draw the symbols alongside a set of numbers as quickly as possible). Beyond the subtests used in test batteries, WS individuals also show variations in other areas of visuo-spatial ability i.e. they are not so good at drawing, but their ability to recognise faces corresponds to that of a typical individual.

It is not clear why there is such an uneven profile in the performance of WS individuals on visuo-spatial tasks. It could be linked to the type of processing that an individual uses to complete a task. Local processing involves looking at the parts of an item, whilst global processing refers to the individual looking at the whole item. It is possible that WS individuals have a heavy reliance on local processing at the expense of processing items globally. This has been argued by a number of researchers, although there is also evidence that in some circumstances WS individuals are more influenced by global than local aspects of an image which is more typical of the normal adult population. A second reason for the discrepancy in performance on different visuo-spatial tasks, could relate to two types of intelligence. "Fluid intelligence" is the type of thinking needed for reasoning and problem solving tasks and is a purer measure of intelligence. This is dependent on the structure of neurons in the brain and so is largely heritable. "Crystallised ability" is a culturally influenced type of intelligence which develops through experience. It is dependent on factors such as schooling and familial and cultural background. It could be that more abstract tasks such as the Block Design and Coding subtests of the WISC rely on fluid intelligence and that Picture Completion and Object Assembly subtests require crystallised intelligence. This might explain why WS individuals perform better at the latter than the former of these subtest groups and would lead to the proposal that WS individuals have a more developed crystallised than fluid thinking style.

A number of researchers have previously administered the WISC, but direct comparisons between the individual subtests have not been made. Consequently , I will start my research by administering the performance subtests of the WISC to a group of WS individuals in order to directly compare the scores achieved on each subtest. Through the three years of my PhD I intend to develop an understanding as to what underlying factors are involved in visuo-spatial cognition and the effect that each factor has on the WS individual when faced with problems that require the use of visuo-spatial abilities.

Considering that this is the area that WS individuals find most difficult, this is an important area to investigate as not enough research has been done in this area so far. By discovering which areas of visuo-spatial ability are the most difficult for individuals with WS we might be able to advise on methods of teaching and training. Visuo-spatial difficulties may then be minimised thus helping the individuals' development.

For more information, or any queries please contact me at:

8, Woodland Road

Bristol

BS8 1TN

England

Tel: 0117 928 9084

Email: E.K.Farran@bris.ac.uk

WSF Studentship >> 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.

Jon registered as a postgraduate research student at the University of Warwick in January 1999. The regulations require that he register in the first instance for the degree of MSc, with the expectation that, subject to a satisfactory level of work, he will upgrade to PhD around the end of his first year.

Supervision and other substantive support

Jon's major supervisor is Jill Boucher and his minor supervisor is Glyn Collis, who is also Director of Postgraduate Studies in the department. Prearranged supervisions were initially held about once a week, but this has now reduced to approximatly one per fortnight. However, a considerable amount of time is spent in the department, or during joint visits out of the department (e.g. to Sheffield University - see below) in informal work-related discussions.

Jon's studentship is linked to a studentship held by Vesna Stojanovik in the Department of Human Communication Sciences at the University of Sheffield, supervised by Dr Mick Perkins and Dr Sara Howard. Jon and Jill have made two visits to Sheffield over the last five months for extended discussions of the two students' work, and Jon and Vesna have spent additional time in discussion during these visits. Vesna has visited Warwick recently for further ideas sharing with Jon. At the last meeting of the whole Warwick-Sheffield group, it was decided that whilst both students will focus on people with Williams syndrome as their main experimental group, Jon will assess individuals with autism as his main comparison group whereas Vesna will assess individuals with specific language impairments as her comparison group. The approach taken by the two students will also differ, with Jon collecting data on quite large groups of individual (as outlined in the original application for a studentship) whereas Vesna will collect linguistic data from a small number of individuals and analyse the data in detail. Thus Jon will have access to some detailed linguistic data on a subset of the group of people with Williams syndrome who participate in his studies, and Vesna will have the results of psychological assessments on the Williams syndrome individuals whose language she studies. This sharing of data should enrich both students' research.

Jon's proposed area of work has connections with other research ongoing in the department, notably with work on time perception and representation being carried out by Dr Teresa McCormack with Professor Gordon Brown, and Jon can consult with these and other members of staff at any time. Jon has also made work links with postgraduate students and senior researchers in the department who are investigating categorisation abilities, and who are interested in the possibility of looking at categorisation abilities in children with Williams syndrome or with autism. Some preliminary pilot studies in this area are planned, with Jill Boucher participating.

Reading

Jon has spent much of his first five months 'reading himself in' to his broadly identified areas of investigation, namely Williams syndrome (also autism), temporal processing, and hyperacusis. The aims of this reading have been (i) to become generally well informed about research and theories in these areas; and (ii) to identify the precise research questions to be investigated, at least in initial studies. Jon is currently focussing on temporal binding as his narrow research topic. Temporal binding refers to the linkage of different facets of experiences together in time, and related to this, how different areas of the brain integrate with each other. The focus on temporal binding has taken him into readings on neuropsychology, particularly with regard to the cerebellum. Jon recently wrote an outline review of literature covered so far, leading into the issue of temporal binding and its possible relevance for understanding WS and autism. - There have been some problems in making progress on the hyperacusis reading, since there were inexplicable difficulties in obtaining Josephine Marriage's PhD these on "Hyperacusis in WS", which is the logical starting point for such reading. However, the thesis has recentlly arrived, and Jon is currently reading it. We plan to meet and talk with Josephine Marriage at the Stoke-on-Trent convention. - A representative list of references to reading carried out in the first five months is attached.

Contacting schools and families

Jon's immediate aim is to identify at least a small group of WS participants, and to have completed ethical procedures satisfactorily with the families in the near future. His selection of comparison participants with autism, which depends on the ages and developmental abilities of the people with WS who are available to take part, can then proceed.. He plans to carry out some preliminary studies with these participant groups in the Autumn.

With this in mind, Jon has contacted the three nearest WSF co-ordinators (Northern & Yorkshire, Birmingham and West Midlands, and East Midlands). Both Vesna and Jon will attend the next meeting of the N & Y branch in Southport on June 6th, and will meet the other co-ordinators at the Stoke convention. Jon has asked them for lists of names and ages of individuals with WS in their area, and for advice concerning which families and individuals might be interested to take part in his and Vesna's research. He has had a reply from the Farrow Johnsons, of the East Midlands group, and it appears that they have a useful number of older children known to them in their region. In addition, Jon has contacted all the special schools in Warwickshire to ask whether or not they have any children with WS or autism on their registers. The response from schools was slow and patchy. However, follow-up phone calls have produced some positive responses which we are now following up with visits. Finally, in order to familiarise himself firsthand with WS, Jon has spent time with one local family who have a grown up son with WS, as well as visiting the Training Centre where this young man spends much of his time. He has also visited a local special school to observe a young girl with WS.

The next 6 months

The plan is that by the end of the school term, Jon will have identified at least a small group of individuals with WS, and a comparison group of individuals with autism, and have obtained provisional permission from families and from schools to work with these children/young people. We have not yet decided the selection criteria for comparison groups, despite a considerable amount of discussion of this issue with the Sheffield researchers. Over the Summer Jon will make decisions about the preliminary studies he will carry out with his groups of participants, and will also prepare materials and procedures. Early in the Autumn term he will, if necessary, pilot his proposed tests with young typically developing children, and also select a second comparison group of young typically developing children. He will then run his initial set of studies of the remainder of the Autumn term and into the Spring term, with the aim of having completed these studies by the end of his first year. His report of these studies will provide the basis for consideration of whether or not he can upgrade his registration from MSc toPhD.

Professor Jill Boucher, Dr Glyn Collis and Jon Brock

Research Projects >> More

WSF Convention, Stoke-on-Trent, July 1999 - Reports on Current Medical Research Projects

Research by the Warwick University-Sheffield University Research Group

Personnel

At Warwick University:

Jill Boucher (Psychologist and former speech and language therapist) Jon Brock (PhD student in Psychology, supported by a WSF studentship)

At Sheffield University:

Mick Perkins (Linguist and former teacher) Sara Howard (Linguist and former speech and language therapist) Vesna Stojanovik (PhD student in Linguistics) Mark Blades - Psychologist


  1. Research Questions:


  2. Do people with WS have a super-efficient sense of time? (though perhaps only for short periods of time, such as milliseconds, seconds, and minutes) (Jon Brock/Jill Boucher)




  3. Might a super-efficient time sense operating over these short time scales be related to the good ability of people with WS to learn language?(Vesna Stojanovik/Mick Perkins/Sara Howard)




  4. Might a poor sense of space make learning about spaces and places difficult for people with WS? (Jill Boucher/Mark Blades)


Rationale

We live in a universe which is made up of things which exist in SPACE and in TIME. The brains of people - and also of animals - have therefore evolved so as to give us an intuitive 'sense of space' and 'sense of time'. These are important aspects of what we call 'intelligence'.


  • A sense of time is important for:


  • Hearing e.g. music, speech/language




  • Seeing things which move and change e.g. faces, people; animals; rippling, flickering, waving




  • Moving our own bodies, carrying out actions e.g.talking, gesturing; running,dancing




  • Linking together things we see, hear, feel: e.g. faces and voices; sounds and feelings




  • Remembering the past e.g., what we've just heard someone say; what happened when




  • Planning the future: e.g., what to say next; what to do when




  • Knowing 'how long', 'how long ago'




  • A sense of space is important for:


  • Learning about objects, manipulating objects; putting things together and taking them apart.




  • Learning about spaces, thinking about spaces. Knowing where things are.




  • Learning about places, knowing where you are, knowing the way from A to B


Research to be Carried Out

Jon Brock, supervised by Jill Boucher, will begin his PhD research by directly assessing the sensitivity of school-age children with WS to the temporal (time-related) characteristics of things they experience. So, for example, one experiment involves playing two tones which are only fractionally different in length, and asking children to judge which tone lasted longer. Another experiment involves playing two tones with such a short gap between them that it may be difficult to tell whether there was one tone or two. We expect children with WS to do rather well on both these experiments, and on the other similar experiments which will assess their sensitivity to fine-grained time. Jon will plan later research on the basis of what he finds in this first phase of investigation. So we will update you, as his work proceeds.

Vesna Stojanovik, supervised by Mick Perkins and Sara Howard, plans to audio-record conversations with a small number of school-age children with WS. She will transcribe these conversations, and then analyse certain aspects of the language (especially the grammar) which the children spontaneously use. If we are correct in suspecting that the unexpectedly good speech and language which most people with WS have derives from their good sense of time, then those aspects of the children's grammar which are systematically related to timing will have developed well. By contrast, those aspects of grammar which are more related to fully understanding what is going on around one will have developed somewhat less well. Vesna's work might therefore help to explain why speech and language development in people with WS though generally good is nevertheless uneven.

Jill Boucher and Mark Blades planned to investigate route following by school-age children with WS, and the ability to use maps and models of places. This would be done in realistic surroundings, for example, going for a snack two minutes walk away from the department of Psychology at the University of Warwick, and then asking the child to show an adult the way back to the department. Conversations with some of the adults with WS and with families at the Stoke convention suggest that route finding is very good in some people with WS, but very poor in others. Those who have 'cracked' the problem of route finding seem to make very good use of landmarks, and this could suggest a way of helping others to crack the same problem. Since the convention, it has been suggested that Jill and Mark's proposed studies might fit in well with work planned by Janette Atkinson's group in London. Again, we will update WSF members when we have had further discussions about this.

JB 20.7.99

Emotional and Behavioural Difficulties >> 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.

The findings of this study have been published in two booklets ("Guidelines for Families and Professionals", "Guidelines for Employers and Supervisors"), and also as articles in professional journals. We recently published a paper in the British Journal of Psychiatry on the social, emotional and behavioural functioning of our sample of adults with William's Syndrome. The main findings from this paper are summarised below.

Parents reported that many of the adults with Williams Syndrome, who were aged 19 to 39 years, possessed good verbal abilities that enabled them to initiate social contact with others. At the same time most expressed concern about the adults' tendency to be over friendly and too trusting of others, which could leave them vulnerable to exploitation and even abuse. Behaviours such as touching, hugging and kissing other people in affection or excitement were commonly reported, and these can give out the wrong signals to people. Many parents/carers also reported that the adults experienced great difficulty in maintaining friendships with people their own age, and many did not have any friends of their own.

Over a third of the adults in our study had a relationship with a member of the opposite sex, and one person was married. In some cases, though, the relationship was limited to sharing activities at college, day centre or residence. Occasionally adults with Williams Syndrome focused their affection on 'pop- stars' or television personalities, or familiar people such as neighbours, which can cause difficulties where the emotional attachment develops into an all encompassing infatuation or obsession.

People with Williams Syndrome can be prone to emotional and psychological disorders. Many of the adults in our study had high levels of anxiety, and a few were on medication for this. The triggers for anxiety may appear trivial to other people, but they include anticipation of new events or changes in routine, and also situations where excessive demands are placed on the individual. The superior speech and language abilities of adults with Williams Syndrome tend to disguise specific cognitive difficulties, and often give the impression that the individuals are much more capable than they actually are. As a result, adults may be placed in residential or work situations which are too demanding, and with too little support. We came across a few cases where people reacted to such pressure with anxiety, depression and/or behaviour difficulties. Even relatively minor changes in the environment, or in staff, can prove very upsetting, while major life events such as the death of a family member or a move to new residential accommodation can in some cases result in periods of severe anxiety and/or depression. Additional psychological support at such times to prepare individuals for change and to help them cope with unforeseen events may prevent long term psychological difficulties.

Fears are common in Williams Syndrome. In a few cases the fears may be sufficiently intense to restrict activities, for example a fear of balloons or of thunder (originating perhaps from their hypersensitivity to noise) may prevent the individual from going out. About one quarter of our sample were said to be excessively concerned about their health; they worried a great deal about minor aches and pains, made frequent visits to their General Practitioner and required considerable reassurance about their health.

Preoccupations and obsessions were also highly characteristic of the adults in our study, and these typically centred on fascinations and obsessive interests with cars, electrical appliances, animals, future events such as birthdays, disasters and violence on the news, or foreign places. While interests and hobbies are to be encouraged, in some cases these preoccupations significantly intruded into daily life, for example where an individual spent many hours dismantling electrical appliances.

Parents/carers reported that a small number of the adults had had one or more episodes of depression or marked mood swings. These are not common, but when present require the involvement of mental health professionals. Aggressive outbursts were more characteristic, very often as a result of frustration or anxiety. Stereotyped motor movements like rocking or hand rubbing were described in many of the adults, and also skin picking, particularly when the individual is angry or anxious.

Emotional and behavioural difficulties such as those described above can cause considerable distress to the individuals concerned and also to their families. We were concerned to find that less than one quarter of the adults in our sample had been referred to a mental health professional for help and advice for themselves and their families. Where carers have concerns about emotional or behavioural difficulties in people with Williams Syndrome, we would urge them to discuss these with their GP or Social Worker and, if they wish, request referral to a local Psychology or Psychiatry Service for adults with learning difficulties. Our advice booklets and guidelines for families and other carers (available from the Williams Syndrome Foundation) may also provide useful information and advice.

Dr. Orlee Udwin

Consultant Clinical Psychologist (Child Health)

405 Kennington Road

London SE11 4QW

Visual and Visuo-spatial development >> More

Visual and Visuo-spatial development in children with Williams Syndrome

Summary of a talk given at the Williams Syndrome Foundation Convention, July 1999, Stoke-on Trent.

Given by Chris Newman, Graduate student, Visual Development Unit, London.(e-mail - christopher.newman@ucl.ac.uk)

Several years ago, Professors Janette Atkinson and Oliver Braddick initiated a three stage project, which was designed to investigate visual and visuo-spatial development in children with WS.

The investigation has included three stages:

Stage 1. Questionnaire on visual problems.



Stage 2. Full visual assessment, selected visuospatial and cognitive tests.



Stage 3. Further visual assessment. dorsal/ventral stream and frontal tests, motor and visuospatial motor planning tests.

The overall aims of this project were to:


  1. Carry out a systematic in-depth analysis of all aspects of visual development in children with WS (sensory, perceptual, motor and cognitive).




  2. To examine correlations and dissociations of development in visual, linguistic and motor modalities.




  3. To develop neurobiological models of brain structure and function in children with WS.




  4. Using these findings, to develop training strategies for Ws children. At Stoke on Trent conference, I presented the aims and rationale for part of the third stage of the project only.


The aims of this part of the project were:




  1. To look at visuo-spatial motor planning ability in a range of tasks of varying difficulty.




  2. To understand which parts of brain development are different in WS children.


  3. And to devise strategies to help children with WS overcome their spatial difficulties on similar motor tasks in everyday life.



Post-box task

The first task we used to investigate motor planning ability in children with WS was the post-box task. This task compares the functioning of two visual pathways in the brain. One of these pathways, the ventral stream or ('what' stream), deals with recognition of familiar faces, identifying objects and comparing objects with each other. The other pathway, the dorsal stream or ('how' stream) takes the visual formation we get about the world and uses it to guide our actions - it helps us get our hands to the right place when we are reaching out for something and makes sure we don't knock things over or fumble them when we try to pick things up.



In the post-box task, children have to either post a letter into a post-box, or they have to match the orientation of the letter with that of the slot, without actually posting it. What we find here is that like some adult patients with brain problems, children with WS can do the matching part of the task relatively well, but have ifficulty with posting the letter smoothly and accurately. This implies that the dorsal stream may not have developed (or be developing) normally in children with WS. One thing that we noticed when doing this task was that children with WS seemed to have problems planning how to carry out the posting action. They would often, for instance, end up with their hands in very awkward positions because they had rotated the card in the wrong direction to start with.



Motor planning tasks

We have therefore devised a set of tests which help us to look in more detail at the abilities of children with WS to plan simple reaching movements. This ability to plan ahead is what makes our movements more efficient and fluent, so one movement flows into the next. We use it all the time in a whole range of everyday tasks, such as turning on a tap, picking up a glass from the table , or putting on a jumper the right way round. This planning depends on connections from visual and motor areas to the frontal lobes of the brain.



One of the tasks we have been using to test this ability involves what looks like a large clock-face. The children are asked to turn a handle, so that an arrow points to one of a set of different coloured mice around the clock-face. If the arrow is pointing to the mouse at 12 o'clock, and you are asked to turn the handle round to 6 o'clock, this requires motor planning. If you start by grasping the handle with your thumb pointing upwards, your arm will be in an awkward position by the time you have turned it round to reach the mouse at 6 o'clock. We ask children to turn the handle to mice at different positions, and observe whether their grasp at the start of the movement takes into account what their hand position will be at the end. Typically developing children show evidence of planning ahead in this task by the age of 5-6 years (Smyth and Mason,1997). A majority of children with WS, however, tended not to plan ahead but to grip the handle in the most obvious way at the start of the movement, so their arms often ended up in an awkward position at the end.



Movement kinematics in children with WS

Another test which taps the function of the dorsal 'how' stream of visual processing in the brain simply involves observing children with WS as they pick up objects of different sizes. Our movements are generally very smooth and automatic, so we do not realise how complicated a problem is solved in the brain every time we move accurately. For example, when we reach out to pick up an object, we have to get our hand accurately to the right place, turn it to the correct angle, open our hand to the right size, and at just the right time, so that we are ready to close the fingers and grasp the object smoothly.



In order to study the 'online' control of action in the children who visit our unit, we are taking very detailed measurements of the movements of their hands when they pick things up. We put small reflective pads onto the children's hands, and a computer tracks the position of these markers as the children reaches out to pick up a square block. We can then look in detail at whether the movement is smooth or jerky, and whether the opening between the fingers is matched accurately to the size of the object. Using this task, we hope to see whether the movements of children with WS are organised differently from those of typically developing children, and to analyse this in terms of the underlying organisation of the brain.



Practical considerations

In terms of practical considerations for children with WS, they hould be encouraged to:




  • Stop and think before acting - not to act impulsively.




  • Talk through how they are going to do an action before performing it.




  • Break down a task such as getting dressed into single steps.




  • Get children to verbally prompt themselves, at first with the help of a parent/carer or teacher, before carrying out each step.



Jon Fowler, Administrative Secretary,

Visual Development Unit



Tel: 0171 380 7574

Fax: 0171 380 7576



Visual Development Unit

Department of Psychology

University College London

Gower Street

London WC1E 6BT

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