3: WS & DS

Question 1

For a very long time, people believed that there were only ToM deficits in ASD, whereas now we know that there are ToM deficits in most children with neurodevelopmental disabilities.

Tassebehji, 2003

Answer 1

For a very long time, people believed that there were only ToM deficits in ASD, whereas now we know that there are ToM deficits in most children with neurodevelopmental disabilities. These cross-comparisons have really enriched our understanding, not only of DS and WS but also in ASD too. We know the genetic basis is of WS and DS. DS can be diagnosed prior to birth and WS can be diagnosed with a blood test. Williams Syndrome: Deletion of around 28 genes on chromosome 7q.11.23 (Tassebehji, 2003), Confirmed with FISH test, Deletion of Elastin gene, Prevalence: there are 1 in 7,500 to 1 in 20,000 live births with WS, this seems like quite a rare disorder, but the prevalence might increase as we become more aware of the disorder (as happened with ASD). In Down syndrome the prevalence is 1 in 800 live births, this is quite a common NDD. Addition of an extra copy of chromosome 21 (trisomy 21), Diagnosis can occur before birth, using amninocentesis, or can be confirmed with FISH.

Question 2

Both WS and DS show atypical brain development.

Reiss et al., 2000

Fidler & Nadal, 2007

Answer 2

Both WS and DS show atypical brain development. There is atypical brain development in WS, there is relative preservation of some aspects of brain development and abnormalities in others. Total brain volume decreased by around 13% in WS compared to controls, cerebral volume decreased more than cerebellar volume, significantly reduced brainstem tissue, Relative preservation of grey matter and reduction of white matter (Reiss et al., 2000). In DS there are a lot of differences in the early development of the brain. Brain weight and proportional size of lobes ‘normal’ at birth
by 3-5 months, reduction in growth of frontal lobes, diminished brain stem and cerebellum. Preserved amygdala but reduced hippocampal volume (Fidler & Nadal, 2007).

Question 3

There are really atypical behaviours compared to TD. Children with WS are extremely charming and they really seem to like people. The quality in which people with WS react to people has led them to being very over estimated in terms of their social skills. Over-friendliness Indiscriminate approach to familiar and unfamiliar faces.

Answer 3

There are really atypical behaviours compared to TD. Children with WS are extremely charming and they really seem to like people. The quality in which people with WS react to people has led them to being very over estimated in terms of their social skills. Over-friendliness Indiscriminate approach to familiar and unfamiliar faces. WS children behave as though they have never met a stranger, they don’t have this concept (charming but not safe). WS show quite a prolonged gaze, in that they stare. TD people modulate gaze (fine grained action), but social gaze seems to be a problem in most neurodevelopmental disorders. WS show repetitive behaviours and have a reliance on routine. Many people write about WS as anti ASD, they are the opposite, but this is analysing the behaviours in an extremely superficial way because at a deep level there a lot of similarities. One thing that is problematic is that WS suffer from appalling fear and anxiety, they are very anxious people. A previous Goldsmiths student, found that people with WS who were actively engaged with music, tend to be less anxious. There are some therapeutic approaches that can be used to reduce anxious. Its thought that if we give someone a diagnosis that they are going to be exactly the same as one another but they never are, there is always a lot of variability. But research is based on data of group means.

Question 4

With Down Syndrome on they are quite quick to abandon difficult tasks as cognitive tasks are very difficult and its difficult to get them to do experimental studies as they find it very difficult to do experimental paradigms.

Answer 4

With Down Syndrome on they are quite quick to abandon difficult tasks as cognitive tasks are very difficult and its difficult to get them to do experimental studies as they find it very difficult to do experimental paradigms. People with DS over-reliant on social strategies as distraction and are much more interested in the experimenter (people) than anything else and will often try and distract from the very boring data collection by chatting and getting very friendly with lots of over visual engagement, increased smiling and engagement behaviours in infancy which facilitates bonding through eliciting like a TD child does.

Question 5

In Perceptual Processing weak central coherence predicted they would show a local processing bias.

Saldarna

Answer 5

In Perceptual Processing weak central coherence predicted they would show a local processing bias. When people with ASD do the block design test it has been hypothesised that they can do it because they are local processors. But as there are more children diagnosed, there are less children showing this local bias. Saldarna showed in a review article that the embedded figures task is not a reliable marker for ASD as an awful lot of child aren’t good at this task. However, there are a group that are incredible at local processing, they can do tasks upside down, draw impossible figures. Therefore, it is a marker of ASD but is not a marker of everyone with ASD. In a free drawing task (a house), in WS, the drawing of the house had no outer walls yet the child with DS draw a good picture in terms if the global aspect of the drawing (walls, roof windows etc.), but very few internal features in comparison to the child with WS who has a lot of intern features.

Question 6

The block design task, from the Weshler tests.

Answer 6

The block design task, from the Weshler tests. The child is shown a picture and given blocks to match the picture/deign which is timed. In WS, their spatial processing is so impaired, they can’t even make a square with the four blocks. The DS have made the global shape of the square but they have been very insensitive to the internal features. In the Navon task (D made of lots of little Y’s), a child with WS is good at doing the y’s/the local features but there are really bad at doing the global features. However in DS, there is only global features. Therefore, there is a really different pattern of perceptual processing here. Is the featural processing bias like what is seen n ASD? A child with ASD can do the block design test and its been hypothesised that its because they are local processers. But to get a score on WASI block design, they have to make it and do it in time, ASD always get the blocks back together again in time. Therefore, it can be argued that they do well because they have a local bias but they don’t have any problems putting it back together again because if they did they wouldn’t get a good score on this test. Thus they display a global ability? The local bias in WS compared to DS is qualitatively quite different.

Question 7

In work by Bellugi et al., (1994) who started doing most of the work with WS.

Answer 7

In work by Bellugi et al., (1994) who started doing most of the work with WS. It cannot be explained in a simple way in what causes the profound problems (e.g. difficult walking through doors because of a lack spatial awareness problems). Focus on parts not wholes. WS show a LOCAL bias in visual perception. Similarities with ASD?

Question 8

Multifactorial Explanation for what causes these difficulties in WS. Farran & Jarrold, (2005) found that 11–33 yr old individuals with WS are

Farran et al, 2001

Answer 8

Multifactorial Explanation for what causes these difficulties in WS. Farran & Jarrold, (2005) found that 11–33 yr old individuals with WS are poor at encoding spatial relations between local elements (they may have the local elements but working out how they are put together is very difficult). WS is rare so researchers use all they can get. But how different is memory from 11 years of age to 33? If matched on IQ, what aspects of IQ. People with WS, do not use typical mental rotation strategies (Farran et al, 2001), this has implications for the block design task as it has different sides with different patterns. Instead, they rely on manual manipulation of blocks resulting in slow performance.

Question 9

Executive problems: if a person does not have normal perceptual or spatial processing, this will really impact on the ability to plan.

Hoffman et al., 2003; Hudson & Farran, 2011

Answer 9

Executive problems: if a person does not have normal perceptual or spatial processing, this will really impact on the ability to plan. A person needs to understand what they are dealing with perceptually before they know what to do with it. You don’t see really abnormal planning in WS when it’s relatively straight forward or simple stimuli as the stimuli become more complex, the planning problems become more problematic. Performance on simple block construction and drawing tasks appear to recruit typical executive planning skills in WS. Atypical planning strategies are observed when tasks become more complex (Hoffman et al., 2003; Hudson & Farran, 2011). A person with ASD who often very good perception, people would say its a planning problem but you can’t attribute it in WS because there are all these other lower level difficulties happening.

Question 10

The methodological issues are particularly important to the theme of this course. When an IQ test is done there are lots of subtests (citing, vocabulary, visual spatial ability etc.) and the can be plotted on a graph.

Answer 10

The methodological issues are particularly important to the theme of this course. When an IQ test is done there are lots of subtests (citing, vocabulary, visual spatial ability etc.) and the can be plotted on a graph. The problem is that all of the sub scales are used to derive a fun scale IQ measure. A child with NDD may have a hugely spikily profile which matching to an CA matched control won’t work (e.g. a child with full scale IQ of 100 but a vocabulary of 10 will have a difficulty in understanding the instructions of the experiment in comparison to a CA matched child with an IQ of 100 with a regular profile). There will be some subtest that the child with the spiky profile will be extraordinary good at. Therefore, matching this child on a global IQ will not control for anything. Verbal and non-verbal IQ could be controlled for with two control groups but it still would not be perfect and would still be problematic. A child with developmental delay who has a CA of 10 but a verbal mental age of 7. This child can be matched with a control group of TD 7 year olds but then the problem is that these children have had 3 years more life experience. Thus it’s important to think carefully on how NDD’s are matched.

Question 11

In a study by Bertrand et al. (1997) who compared children in study 1 with WS (9-10yrs) to chronological aged (CA) matched and mental ages (MA) matched controls on object drawing task.

Answer 11

In a study by Bertrand et al. (1997) who compared children in study 1 with WS (9-10yrs) to chronological aged (CA) matched and mental ages (MA) matched controls on object drawing task. In a second study, they compared TD children of 4yrs, 5yrs, 6ys, 7yrs on an object drawing task. They were looking at the development of TD children of being able to draw objects. In the first study they found that children WS produced fewer recognisable drawings the CA and MA age matched children. WS included fewer major parts of each object in their drawings than CA and MA matched, WS drawings were more disorganised than CA matched but not significantly different to MA matched. Therefore WS are not developing completely normally on these components but on this component they are looking like children that have the same level of language or have the same MA. They also found that typical 4-yr-olds produced fewer recognisable drawings, with fewer major parts and higher levels of disorganisation than older age groups. This is suggesting that what is seen in WS is quite characteristic of what is seen in younger children. Typically developing children go through the local processing before global processing. If children are compared with MA controls and they are performing in a similar way, then these children are ‘developmentally delayed’ if they are much worse than MA matched children then they have a deficit. There needs to be a distinction between delays and deficits. In study 2: Children with WS show similarities to TD 4 yr olds thus a DEVELOPMENTAL DELAY, but children with WS show more serious disorganisation of drawings than TD 4 yr olds thus a ATYPICAL TRAJECTORY (deficit in global processing abnormalities in WS).

Question 12

People have questioned the neuro-substrate of this. It can be considered as a problem in Dorsal Stream difficulties which is guiding action, visual control, visuospatial processing in WS.

Milner & Goodale, 1995

Answer 12

People have questioned the neuro-substrate of this. It can be considered as a problem in Dorsal Stream difficulties which is guiding action, visual control, visuospatial processing in WS. Or os it ventral stream and only to do with perception (Milner & Goodale, 1995). To investigate this, in the Postbox Task (Milner & Goodale, 1995) in Task 1: What is the orientation of the slot? This is the VENTRAL TASK and then in Task 2: Post the letter through that slot in the dorsal task. The results showed that those with WS had a greater problem with the dorsal task (they approached the slot at the wrong angle to post the letter and use trail and error to get the letter into the slot). Similar to the mental rotation difficulty of the block design.

Question 13

(Atkinson et al., 1997) On the Postbox task

Answer 13

(Atkinson et al., 1997) On the Postbox task individuals with WS showed relatively less difficulty with the ventral than with DORSAL task. Approached the slot at wrong angle to post the letter. Used trial and error to get the letter into slot.

Question 14

Individuals with WS display impairments on other ‘dorsal stream’ tasks. Mobbs et al. (2007) Meyer-Lindenberg et al. (2004)

Answer 14

Individuals with WS display impairments on other ‘dorsal stream’ tasks. Mobbs et al. (2007) showed a reduced activation in occipital / parietal cortex and Meyer-Lindenberg et al. (2004) showed a reduced activation in parietal cortex. Therefore these findings seem fairly consistent.

Question 15

Perceptual Processing task in DS, there are loads of problems with matching across the studies, the review by Yang et al., 2014 constantly addresses all the way through about how people have matched and just how difficult it is to do work with these groups.

(Lanfranchi et al, 2012).

Answer 15

Perceptual Processing task in DS, there are loads of problems with matching across the studies, the review by Yang et al., 2014 constantly addresses all the way through about how people have matched and just how difficult it is to do work with these groups. Anecdotally people think that people with DS have good visual spatial skills and they compare that to their language skills which are much more problematic. However, the evidence does not support the idea of good visual spatial skills, they can be seen in ASD but not in DS. The kind of evidence that we have is based on quite methodologically faulty studies where the matching is quite problematic across studies which use individuals with a massive age span. They found that block design performance is fairly consistent with general cognitive ability level (in a full scale IQ, they would score around the same expected level). There will be variability within the group because people with DS have a range of IQ scores, often some can be lower but some can have an IQ of much higher. There is a larger variability in Block Design performance in DS compared with TD (Lanfranchi et al, 2012).

Question 16

In a longitudinal study over 17 yrs of 200 individuals with DS showed the largest within-group variance on pattern analysis tasks.

Couzens, Cuskelly, & Haynes, 2011

Answer 16

In a longitudinal study over 17 yrs of 200 individuals with DS showed the largest within-group variance on pattern analysis tasks. Compared with things like (more variable than) vocabulary, quantitative bead memory, memory for sequences, memory for sentences subtests etc. The sore were more stable across the DS group compared with these pattern recognition (Couzens, Cuskelly, & Haynes, 2011). By doing an experimental design, it is possible to get by chance a lot of people at the upper end on pattern design but it very variable within this group.

Question 17

Its important to think about trajectories in NDD. Couzens et al., (2011) Block Design performance in DS is qualitatively different to that of TD people.

Gibson et al., (1988)

Answer 17

Its important to think about trajectories in NDD. Couzens et al., (2011) Block Design performance in DS is qualitatively different to that of TD people. DS showed a rapid improvement from 4 to about 8 yrs then gradually increases until around 30 yrs. TD performance declines in early adulthood. Gibson et al., (1988) suggested rapid decline in BD performance during 4th decade in DS but not in Intellectual Disability. In ASD trajectory, there seems to be a decline in memory for words in ASD in older people, the control group was carefully matched on a 1-1 basis with the ASD people which worked because they were HFA and had easy profiles. She found that in the TD group memory increased in comparison to the sharp decline of the ASD group.

Question 18

Perceptual Processing in DS have found very mixed findings on figure copying skills in DS but there are differences in matching procedures and image complexity varies over studies which has a big impact.

Answer 18

Perceptual Processing in DS have found very mixed findings on figure copying skills in DS but there are differences in matching procedures and image complexity varies over studies which has a big impact. If a simple image was presented, there wouldn’t be the same level of abnormality in reproduction as we’d see if we gave a more complex task. Therefore, there is a problem in shining about how we can make sense of these studies with such different matching procedures, its also important to make sense of studies that have used really different types of stimuli.

Question 19

We do know that studies comparing groups with DS and WS all show that figure copying is superior

(Klein & Mervis, 1999; Vicari et al., 2004; Wang, Dougherty,Rourke, & Bellugi, 1995).

Answer 19

We do know that studies comparing groups with DS and WS all show that figure copying is superior in DS who are nothing like as impaired as children with WS (Klein & Mervis, 1999; Vicari et al., 2004; Wang, Dougherty,Rourke, & Bellugi, 1995).

Question 20

In the block design there are problems seen in mental rotation. Therefore, in a perceptual processing in DS Mental Rotation, Hinnell & Virji-Babul, (2004) and Vicari et al., (2006) matched groups with DS with MA matched TD controls and showed similar levels of performance.

Answer 20

In the block design there are problems seen in mental rotation. Therefore, in a perceptual processing in DS Mental Rotation, Hinnell & Virji-Babul, (2004) and Vicari et al., (2006) matched groups with DS with MA matched TD controls and showed similar levels of performance. In these experimental paradigms, there are changes in performance depending on how rotated it is, then we can look at reaction times in terms of how rotated something is. This gives a good indication of the structure of how somebody is doing the task. The more its rotated the slower someone becomes at making a judgement. DS showed increased RT with increased angle of rotation. Implies similar structure to the skill. Therefore, this suggests that they are showing a developmental delay as the structure of performance is quite similar. Of course they are going to do worse in regards to scoring on the stimuli, but by looking at how they are solving the problem, it looks normal. This explains why DS are doing much better than WS on many of these tasks. If doing a BD task with a person with DS, another problem is that they have motor manual difficulties. Therefore, its important to factor that in when they are trying to manipulate blocks at speed, they will find it difficult.

Question 21

Executive Functions: In a study by Constanzo (2013) who matched compared WS and DS with TD children on Executive Function Tasks.

Answer 21

Executive Functions: In a study by Constanzo (2013) who matched compared WS and DS with TD children on Executive Function Tasks. They compared 15 participants with WS (CA = 10–35), 15 participants with DS (CA 8–21) and 15 TD children (CA = 7.4). As there are very few people with WS they take any age. Furthermore, they are also matched on mental age. They looked at attention, memory, planning, categorization, shifting and inhibition. When a person is participating in the Wilcokson Card task for example, they have to change ‘set shift’ quickly. People with executive problems will continue sorting by previous rule for quite a while before they can re-update the rule.

Question 22

Costanzo (2013) found that both groups showed impairments in

Answer 22

Costanzo (2013) found that both groups showed impairments in auditory sustained attention, visual selective attention, visual categorization (visual fixation, poorly modulated eye gaze) and working memory. They found that DS were poor in shifting and verbal aspects of memory (they are not good at set-shifting and they have problems with language). The WS were specifically poor in planning. WS were stronger than DS in visual sustained attention, verbal categorization and verbal inhibition. There WS do well on things, it often has a verbal component, which is a relative strength for them in comparison to their visual spatial ability. This is not see in DS.

Question 23

Rather than having a group with a massive age range and comparing them with a group of little children, Carney, Brown & Henry (2013) used regression techniques rather than relying on MA (much younger) matched control group.

Answer 23

Rather than having a group with a massive age range and comparing them with a group of little children, Carney, Brown & Henry (2013) used regression techniques rather than relying on MA (much younger) matched control group. They looked at EF and focussing on areas that we know are particular problematic both disorders: Visuospatial (WS weakness) and verbal (DS weakness) tasks used to probe executive- loaded working memory (ELWM), inhibition, fluency and set-shifting. They saw executive functioning deficits (compared to TD participants) were observed in both groups but they say a level of syndrome specificity. Children with neurodevelopmental disorders do not show the same patterns on test of executive functions. There were WS-visuo spatial difficulties, on tests of Executive loaded working memory and fluency, thus we can see where its impacting. In DS, there were relative verbal difficulties in the domain of set-shifting (delayed language processing system will affect card sorting etc.). But results also suggested that its not incredibly clear how it pans out with executive functions, there is not a constant manifestation of visual-spatial processing across executive functions task and equally the same for DS.

Question 24

Language in Williams Syndrome: Early accounts of WS proposed that language is preserved (Bellugi et al, 1988, 1997).

Answer 24

Language in Williams Syndrome: Early accounts of WS proposed that language is preserved (Bellugi et al, 1988, 1997). Recently there has been quite a big change in paradigm before that everyone was excited about modules in the brain (e.g. Chomsky: language modules, etc.). People believed the WS had a completely preserved language module, although they are really damaged globally intellectually.

Question 25

There was a study which asked WS to describe animals/creatures and the children were coming up with very obscure animals which resulted in people believing they had wide verbal categories etc.

Answer 25

There was a study which asked WS to describe animals/creatures and the children were coming up with very obscure animals which resulted in people believing they had wide verbal categories etc. Once tested in larger groups with proper standardised language testing they found that its not the case at all. In a standardised language test of hundreds of children, 85% of TD 5 year olds will know this word. Therefore, we are using these measures that are really well developed. Later research revealed there is a delayed development of vocabulary and comprehension, a delayed & atypical grammatical skills (use of past tense and irregular verbs), atypical development of categorisation and delayed development of phonological segmentation (being able to segment words quite well is a real building block of development of language).

Question 26

A study done by Dan Levantine of WS who found that they could tell different vacuum cleaners

Answer 26

A study done by Dan Levantine of WS who found that they could tell different vacuum cleaners from each other from recordings on a tape. The real difference between WS and ASD was that a child with WS asked to come back to the house and see the vacuum cleaners.

Question 27

An infant and a parent talking to each other is dyadic (in ASD, you would be grateful for a dyadic interaction with a child).

Answer 27

An infant and a parent talking to each other is dyadic (in ASD, you would be grateful for a dyadic interaction with a child). The triadic interaction is really important for language, the child can look at an object and share attention with the caregiver, by referencing an object at the same time enables a mother to name an object.

Question 28

Laing et al., (2002) Investigated joint- attention (pre-language skill) in play tasks (Toys to request, Tickle game, Turn-taking, Posters on the wall).

Answer 28

Laing et al., (2002) Investigated joint- attention (pre-language skill) in play tasks (Toys to request, Tickle game, Turn-taking, Posters on the wall). For example, in the posters on the wall task (ADOS task), a person points to a poster and if the child looks at you (dyadic) but if the child looks at the poster and then at you its a triadic interaction by developing joint attention skills (mental representation of other peoples thoughts). Therefore, in dyadic, language will develop slower but also a delayed ToM. Laing et al., (2002) results found that TD better at Initiating, requesting and responding to joint attention. From 12-18months there is a massive amount of this kind of behaviour. There is a lot of dyadic social interaction behaviours in WS because they like other people but there will be less triadic things going on. Interestingly, the children with WS who are showing more tryadic behaviours are showing better language skills. WS better at dyadic social interaction behaviours. Strong relationship between joint attention and language skills (comprehension & production) in both groups. Therefore joint attention and triadic interactions are serving very similar functions for these children. They are delayed but there is a correlation. For the different types of social behaviours are really linked to eat other. TD participants there were significant correlations between social interaction & joint attention and social interaction & initiating & requesting. There are different behaviours but they will correlate and are very linked in TD children.

Question 29

However, this is not seen in WS, in that social interaction behaviours less integrated in WS.

Answer 29

However, this is not seen in WS, in that social interaction behaviours less integrated in WS. Behaviour mostly dyadic rather than triadic, WS are less interested in objects, more interested in faces. Therefore of course there is a bias towards dyadic interactions. Whereas a TD child is interested in people and objects, therefore the attention mechanisms will develop and enable the child to process those at the same time.

Question 30

In a review article, on language in DS, which suggest that there is an effect of hearing loss and hyperaccusis (sensitivity to sound in ASD, DS, WS) and thus a disruption to the hearing processes more frank in DS and oral-motor problems (some children have a large tongue which makes articulation quite difficult).

Answer 30

In a review article, on language in DS, which suggest that there is an effect of hearing loss and hyperaccusis (sensitivity to sound in ASD, DS, WS) and thus a disruption to the hearing processes more frank in DS and oral-motor problems (some children have a large tongue which makes articulation quite difficult). Furthermore, some children DS do not speak at 3 years and thus show a similar kind of delay as children with ASD. Delayed vocabulary development is linked to joint attention (parent and child look at something and share attention of an object, parent names object). Therefore we can speculate that delayed vocabulary development is a problem in joint attention. Delayed pragmatic (social e.g. gestures) use of language (paralinguistic things that we do) to communicate. This is very delayed but is in line with mental age. A group of DS children who are 7/10-15 year olds might get MA matched with TD 4 year olds, to look at developmental delay. They have problems with reciprocal eye contract or more intense (Sticky fixation: eye contact is fixed). Joint attention needs massive amounts of flexibility, a 3 year can flit between the object and the person thats its sharing joint attention with. Sticky fixation interferes. Children with DS use more gestures more often than TD children, they are very expressive but they are less integrated than can be witnessed in a TD child. In TD, there is a very seamless development of linguistic and paralinguistic skills. Children as young as 2 can use pragmatic gestures. There is more gesture in DS but they are unlikely to be as well integrated as in TD children.

Question 31

In a more naturalistic study, Zampini, Salvi & D’Odorio (2015) conducted an semi-structured study and assessed attention skills in 18, 24-month-old children with DS during semi-structured free-play sessions with mothers.

Answer 31

In a more naturalistic study, Zampini, Salvi & D’Odorio (2015) conducted an semi-structured study and assessed attention skills in 18, 24-month-old children with DS during semi-structured free-play sessions with mothers. This was an observational study that used video coding. Results showed that children with DS spent a large part of the interactive play session in joint attention situations. A mother of a child with ASD, helped develop him into a pHd graduate by doing floor play with him, trying to work out how he could learn. The same would be for DS, the more it is worked out what the child needs to work on and focus on reinforcing and building on those areas, there will be a lot of development. Interestingly, children that initiate joint attention (e,g, reference something) is predictive vocabulary comprehension skills when assessed 6 months later. This is the same pattern in TD that joint attention is really associated with language. Therefore, this looks like an more of a developmental delay than a deficit.

Question 32

People with ASD do not have the propensity to engage with other people on the whole as a group mean. Some people with ASD look like people with WS who are very social but they are just inappropriate. However, in DS & WS both considered to be highly sociable and they look at faces a lot (sticky fixation).

Answer 32

People with ASD do not have the propensity to engage with other people on the whole as a group mean. Some people with ASD look like people with WS who are very social but they are just inappropriate. However, in DS & WS both considered to be highly sociable and they look at faces a lot (sticky fixation). They have the ability to process faces in a TD expertise way. But there is an issue that is not to do with social behaviour, in that their perception is really bizarre. The normal balance between global and local processing that is seen in typical development where there are changes from more more local to more global or integration, there is a developmental trajectory on how we use global or local information. Therefore that looks like a deficit, even if its developmental delay it will still impact on facial processing because faces are a social stimuli, we still treat them in a similar way to how we treat other complex, meaningful information. In WS, there was an abnormality in global processing and difficulties connecting local features that Fraons study showed. Does atypical local/global processing extend to faces? Similarly with DS, who are very global processors going on the basis on this. Facial processing needs global and local (eyes and mouths are very distinctive features used to recognise other people).

Question 33

In the early account of WS suggested that face processing is preserved in WS and provides evidence for discrete face processing module.

Answer 33

In the early account of WS suggested that face processing is preserved in WS and provides evidence for discrete face processing module. There are very mixed findings from studies. The extent that face processing looks typical/atypical may depend on when the participants are tested. Thinking very much about developmental trajectories in these childen.

Question 34

D’Souza et al., (2015) Studied infants with WS (28m), DS (30m) and TD (14m) (all with MA 14m – Bayley scale of infant development which looks at many different aspects of development).

Answer 34

D’Souza et al., (2015) Studied infants with WS (28m), DS (30m) and TD (14m) (all with MA 14m – Bayley scale of infant development which looks at many different aspects of development). Infants were familiarized with a schematic (cartoon) face, after which they saw a novel face in which either the features (eye shape) or the configuration of the features were changed. Even by this stage of development, the TD infants sensitive to featural and configural change (specialised face processing here), WS only sensitive to featural change (visual spatial problems seemed to be impacting at this stage) and the DS infants looked at both types of stimuli at levels that didn’t differ from chance (the experimental paradigm could not elicit anything from the DS because they were not showing any kind of patten that a could be measured). This suggests syndrome specific differences in early face processing between TD and WS and TD and DS and DS and WS.

Question 35

Annaz et al., (2009) (look at this paper because she uses a different kind of methodology) talks about modularisation, in that, its a developmental process to become modularised.

Answer 35

Annaz et al., (2009) (look at this paper because she uses a different kind of methodology) talks about modularisation, in that, its a developmental process to become modularised. In this study they used cross-sectional trajectory analyses to test part-whole face perception in groups of children (around 8 years) with WS, DS, HFA, LFA & TD. Assessed changes in part-whole face perception that relate to age, mental age, face face recognition ability etc within different diagnostic groups. All children completed tests of receptive vocabulary, pattern construction and facial recognition. When a TD child has got these skills on face perception and pattern construction this is what their face processing is going to be like. Therefore, you can look across these different tests to see if there is something quite strange going on in terms of the links between cognitive mechanisms.
The Experimental Paradigm (Annaz et al., 2009). There is a Whole-face condition and a Part-face condition. There are upright, 90”orientation and inverted trials that can upside down. The question asks, Which of the lower stimuli matches the upper (target) face? The decision is made on the basis of the features or the global configuration. This is the Benton face recognition task with frontal and different orientations yet the WS performed as well as TD controls (the study from when they are two in which they are showing abnormal facial processing, by 8 there are showing normal facial recognition as TD), HFA, LFA & DS performed more poorly than TD controls, HFA performed at higher levels than LFA (SS) and DS and the DS and LFA did not differ. The experimental results of the HFA group (on some trials they need to make a judgement on just the eye or the whole face and the face were in different rotations and were inverted), therefore, its the pattern of performance across these different conditions thats going to be important. The HFA group did not have a problem when the faces were turned upside down as do TD, and that they are much more interested in features, weak central coherence and enhanced perceptual functioning theories. Normal part/whole effect but performance did not reliably decrease on inverted stimuli conditions Results across tasks suggested that good levels of face recognition (on the Benton test) could be delivered by a system that is insensitive to the orientation of faces (on the part–whole task). If the object recognition system has greater feature-based sensitivity in ASD than In TD, it may be able to support an adequate level of the within-category discrimination that is required for face recognition. Therefore, they are using different mechanisms, this is true for language in ASD where the trajectory is qualitatively quite different. In WS performing as well as TD children on Benton test face recognition and are looking normal. But they showed really different underlying processes: WS group did not show an emerging inversion effect for whole faces although they did show an emerging inversion effect for part-face discrimination. Therefore the WS group are relying on different components of the faces in be able to achieve the task, in the same with that the ASD people are although they are not compensating as well as people with WS. The DS group performed as poorly as LFA on face recognition task (Benton) and they differed from TD and other groups in discriminating features better when they were presented in whole faces than when they were presented in isolation (drawing of a house was all outline/ navon figure was a large letter/ global). Therefore the pattern is the sam in processing faces as has been found when processing non-social stimuli in DS. Results suggested poor featural processing in DS. Global style may reflect poor processing of local elements.

Question 36

WS is characterised by ‘hypersociability’ (never met a stranger) “an exaggerated interest to engage in social encounters with both familiar and unfamiliar people” (Jawaid et al., 2012).

Answer 36

WS is characterised by ‘hypersociability’ (never met a stranger) “an exaggerated interest to engage in social encounters with both familiar and unfamiliar people” (Jawaid et al., 2012). Therefore in WS, there is a lot of disinhibition in social behaviour. If a person has ‘never seen a stranger’ they are probably quite insensitive to facial cues. WS seem to ignore facial cues a lot of the time. Some of the first studies thought that their social behaviour is qualitatively quite similar to that seen in TD children.

Question 37

Tager-Flusberg & Sullivan (2000) investigated social-emotional development in age and IQ matched children with Williams Syndrome, Prader-Willi syndrome & Intellectually handicap using the 1) False belief tasks, 2) Explanation of actions task and 3) Recognition of emotional expressions.

Answer 37

Tager-Flusberg & Sullivan (2000) investigated social-emotional development in age and IQ matched children with Williams Syndrome, Prader-Willi syndrome & Intellectually handicap using the 1) False belief tasks, 2) Explanation of actions task and 3) Recognition of emotional expressions. They found that WS (groups) were impaired on all tasks, they do not have good theory of mind. Therefore the desire to engage with others is not because they understand others deeply, it’s driven by something else. They also found that WS like IQ matched controls were impaired in emotion recognition and found negative emotions, particularly hard to distinguish. Therefore there are basic problems in WS with social perception, understanding emotions on faces and social cognition (ToM).

Question 38

Plesa-Skwerer, Faja, Schofield, Verbalis & Tager Flusberg (2006) tested adolescents and adults with WS, age and IQ matched controls with intellectual impairment and age matched typical controls

Answer 38

Plesa-Skwerer, Faja, Schofield, Verbalis & Tager Flusberg (2006) tested adolescents and adults with WS, age and IQ matched controls with intellectual impairment and age matched typical controls on the faces and paralanguage subtests of the Diagnostic Analysis of Nonverbal Accuracy Scale. WS participants (like IQ matched controls) were impaired on emotion recognition and found negative emotions particularly difficult to distinguish.

Question 39

In a study by Lacroix, Guidetti, Roge & Reilly (2009) that directly and closely compared WS and ASD and verbal mental age (VMA) the two groups.

Answer 39

In a study by Lacroix, Guidetti, Roge & Reilly (2009) that directly and closely compared WS and ASD and verbal mental age (VMA) the two groups. This study into identification of emotional facial expression, compared 12 participants with WS (6–15 years) with 12 participants with Autism (4.9 – 8 years) (VMA matched). The results showed that recognition of emotional facial expressions was poorer in the WS group than in the Autism group. All the production were that they would be better as they do more social approach but they actually were more impaired than children with ASD.

Question 40

This was an important study, LINCOLN, SEARCY, JONES & LORD (2007) compared social behaviour profiles of children with WS and Autism.

Answer 40

This was an important study, LINCOLN, SEARCY, JONES & LORD (2007) compared social behaviour profiles of children with WS and Autism. Method: Twenty children with WS (27-58 months) and 26 age- and IQ-equivalent children with AD were administered the Autism Diagnostic Observation Schedule (ADOS). This study was specifically looking for the diagnostic criteria for ASD. The results found that two children with WS met DSM-IV criteria for AD, one of whom was also classified as having AD by the ADOS algorithm. Discriminant analysis of ADOS behaviours indicated that gesture, showing, and quality of social overtures (more appropriate in WS) best discriminated the groups. Some are as impaired globally in social communication skills but the quality is really quite different. Although some children with WS demonstrated some ASD behaviours, and a minority of children with WS had coexisting AD, the symptom profile in WS was different from AD.
Despite some deficits in communication behaviors, showing, and initiating joint attention, children with WS made social overtures and efforts to engage others, whereas children with AD tended not to do so.

Question 41

Riby, Hancock, Jones & Hanley (2013) in an experimental study, compared 16 participants with WS (aged 8–28 years) and 22 participants with ASD (aged 8–17 years).

Answer 41

Riby, Hancock, Jones & Hanley (2013) in an experimental study, compared 16 participants with WS (aged 8–28 years) and 22 participants with ASD (aged 8–17 years). WS and ASD participants individually matched to TD controls on the basis of non-verbal intelligence (not relying on language skills). Very good eye tracking study (worth reading). Participants viewed actors looking at different target objects (e.g. cups, glasses case) in different settings (e.g. office, kitchen, sitting room). In the eye tracking: Condition 1 (uncued), she tracked their spontaneous looking patterns (allocation of attention). In cued Condition 2 (cued) “detect and name what the actor is looking at”. Both groups showed atypical interpretation of the socio-cognitive cues (not very good at working out whats going on) but their patterns of performance were very different. In the uncued condition (watching to see what thy will naturally look at), the WS over-attended to faces (sticky fixation) and the opposite pattern in ASD under-attended to faces. In cued condition (where they have to look so they can answer the question). WS increased gaze towards correct and incorrect target objects but maintained high engagement with actors face and eyes so were less accurate in identifying objects than controls. ASD appeared to understand that they needed to follow the actors eye gaze but they did not seem able to do this and looked at the incorrect object. Klein did a study where they had people coming into a room where there were three paintings. A person would say “thats a really interesting picture” and naturalistically the person with ASD would have no idea what picture the person was looking at whereas the NT would know because they would be tracking their eye gaze the whole time. Therefore, there are problem even in really high functioning people. In WS, its a different patterns of performance but its causing similar kinds of problems.

Question 42

Social Behaviour in WS is impaired ToM & emotion recognition in WS associated with an exaggerated interest in familiar and unfamiliar people (Jawaid et al., 2012).

Answer 42

Social Behaviour in WS is impaired ToM & emotion recognition in WS associated with an exaggerated interest in familiar and unfamiliar people (Jawaid et al., 2012). Does if reflect an impaired ability to recognise threat that is related to the Amygdala? Does it reflect difficulties inhibiting strong impulses towards social interactions related to Frontal Lobes? Furthermore, does it reflect difficulties in inhibiting strong impulses towards social interactions. We know in EF there is a delay thus they can’t inhibit the prepotent response.

Question 43

Therefore, Hariri et al. (2002) presented threatening scenes and facial expressions to participants with WS and TD and they reported greater activation for faces than scenes in right amygdala in controls.

Answer 43

Therefore, Hariri et al. (2002) presented threatening scenes and facial expressions to participants with WS and TD and they reported greater activation for faces than scenes in right amygdala in controls. The Opposite pattern was observed in WS. Hypoactivation in WS.

Question 44

Thinking about the frontal lobe hypothesis, we also see individuals with WS have significantly increased grey matter volumes in the frontal lobes which is correlated with inattention.

Reviews in Meyer-Lindenberg et al. (2006); Jawaid et al. (2012).

Answer 44

Thinking about the frontal lobe hypothesis, we also see individuals with WS have significantly increased grey matter volumes in the frontal lobes which is correlated with inattention. Is this related to problems with response inhibition and difficulties switching attention (particularly away from faces… Reviews in Meyer-Lindenberg et al. (2006); Jawaid et al. (2012). Considering causal links between atypical behaviours and brain abnormalities.

Question 45

Social behaviours in Down Syndrome. Like individuals with WS those with DS are often very friendly and can show indiscriminate approach (never seen a stranger). Porter, Coltheart & Langdon (2007) investigated different explanations for atypical social approach in DS and WS.

Answer 45

Social behaviours in Down Syndrome. Like individuals with WS those with DS are often very friendly and can show indiscriminate approach (never seen a stranger). Porter, Coltheart & Langdon (2007) investigated different explanations for atypical social approach in DS and WS. Indiscriminate approach seen in DS and WS is also seen in people with acquired amygdala damage and acquired frontal lobe impairments (we have to be very careful in extrapolating from clinical case studies of people with adult brain damage to developmental disorders). Hypothesis 1: Atypical social approach in WS & DS may be de to poor emotion recognition reflecting abnormal amygdala functioning. Hypothesis 2: Atypical social approach in WS & DS may reflect poor control of behaviour reflecting a frontal lobe abnormality. Hypothesis 3: Atypical social approach in WS & DS may a heightened salience for social stimuli. In this particular study, there are 20 participants with WS CA: 5.33 – 43.67: MA: 3.58 – 6.41 (general cognitive ability). There are 20 participants with DS CA: 5.75 – 40.75: MA: 4.00 – 6.16 and 40 TD controls matched on gender, CA and MA to DW and WS participants (at least they have some older people in their study too). They looked at emotion recognition abilities and the relationship with social approach. They found that William syndrome abilities, were the same as the MA matched controls, so they had developmental delay. Looked at emotion recognition abilities and their relationship with social approach: WS abilities commensurate (corresponding in size or degree) with MA, DS below mental age (e.g. VMA came out at 4, they were worse than an average 4 year olds). They also showed a tendency to report negative emotions as positive. DS particularly marked difficulties recognising sad emotions in general and were significantly poorer than WS participants in recognising sad paralanguage. Domain general problem with those negative emotions. Other studies of emotion recognition in children with DS found impairments in recognition of anger (Kasari, Freeman and Hughes, 2001; Porter, Coltheart & Langdon, 2007) and impairments in the recognition of fear (Kasari et al., 2001; Porter et al, 2007; Williams et al., 2005; Wishart et al., 2007).

Question 46

Hippolyte et al., (2009) Studied emotion recognition and emotion attribution in adults with DS and tested adults with mean age around 34 years and non-verbal intelligence scores (ravens matrices RM) of around 60.

Answer 46

Hippolyte et al., (2009) Studied emotion recognition and emotion attribution in adults with DS and tested adults with mean age around 34 years and non-verbal intelligence scores (ravens matrices RM) of around 60. TD controls (mean age around 6 years) matched on receptive vocabulary scores. They found that DS very impaired on expression matching tasks with specific difficulties on tests of Expression identification & Facial discrimination. Poor performance on neutral conditions and assessed emotions as more positive than they were (consistent with the studies from children with DS). Receptive vocabulary, selective attention and non-verbal reasoning important for some emotions. Hippolyte et al., (2009) did a second experiment in which they used a pictures test in which protagonists with masked faces experienced emotions in every day situations e.g. Joy (e.g. opening Christmas present), Sadness (e.g. seeing wounded animal), Anger (e.g. getting splashed by a car) or Fear (e.g. encounter with a scary dog). Participants shown pictures of faces depicting neutral, joy, sadness, anger and fear and told to chose the picture that the protagonist would be feeling in response to the situation described (e.g. meeting scary dog). Therefore, approaching emotional recognition in this way is quite complex and it appears they are conceptually understanding better than presumed. DS participants performance was only worse than controls on Sadness. They sometimes substituted Joy for Sadness. All the studies are showing a fairly similar kind of pattern in these negative emotions.

Question 47

ZELAZO et al (1996) tested TOM in adults with Down Syndrome (mean mental age: 5.1 yrs) and non-handicapped MA matched pre-school children.

Answer 47

ZELAZO et al (1996) tested TOM in adults with Down Syndrome (mean mental age: 5.1 yrs) and non-handicapped MA matched pre-school children. DS participants showed poorer performance on several standard TOM tasks. He also looked at whether or not they were showing corresponding deficits on an EF task: colour-shape card-sorting task (EF) in which subjects were required to switch between two incompatible sets of rules. There appeared to be a kind of ink between difficulties on these EF task that seemed to be related to difficulties in ToM. Intuitively social behaviour feels very different to other types of cognitive behaviour but this is suggesting that they are very linked.