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Williams Syndrome (What is WS? (Personality profile (Social disinhibition,…

- - - - Perhaps ToM etc. are intact/relatively unimpaired in WS, but performance on measures is diminished for non-specific reasons
      - Face processing is only superficially intact, but task performance relies on alternative, atypical strategies
    - - When event understanding and cause-and-effect-reasoning controlled, only a small sub-group of WS show ToM deficits
      - Studies of anticipatory looking lacking, but "partial deletion' patients show normal looking even when they fail verbal tasks (opposite to ASD)
    - - Three main types of face processing
        
        holistic/global/gestalt - face is recognised as a whole
        
        feature/local/analytical - recognition is driven by individual features (eyes, nose, mouth, etc)
        
        configural - recognition is driven by the arrangement of the features in the face (spatial relationship between features)
      - The contribution each processes to face recognition change gradually with development
      - Configural processing is the last to emerge
  - - - Everyday face processing unimpaired, experimental face processing performance unimpaired but cog processes underlying performance are atypical
      - True modularity implies that if a module is intact, it should function normally in every way
      - Is the face processing module intact in WS if atypical processes underlie performance?
- - - - Evolutionary advantage because damage to one component would not lead to failure of whole system
      - Developmental disorders that involve selective cognitive impairments are examples of intact and impaired modules
      - Assumes modules are innate and brain is modular from the start
      - E.g. SLI, ASD, WS
      - Logic of cog neuropsychology can be applied to children
      - Double dissociation
        
        Between lang and spatial ability: WS and SLI
        
        Social and spatial ability: WS and ASD
- - - - Stinton et al. (2008) compared 15 individuals with WS (M = age = 22:09) to 15 TD children (M age = 6:04)
      - Groups matched for non-verbal intelligence using Raven's Coloured Progressive Matrices (tests of non-verbal reasoning)
      - Raven's Items
        
        WS sig poorer than TD
        
        Performance was worse than expected on the basis of overall non-verbal intelligence (and age)
        
        Mental rotation task involved presenting pairs of shapes and asking whether they were the same of different
    - - Bertrand et al. (1997): drawings of 18 children w/ WS (M age 9;11) and two TD comparison groups, one matched on age and one matched on mental age
      - Standardised tasks
        
        Object drawing subtest of the Boston Diagnostic Aphasia Examination - drawing then copying a flower, house, and elephant
        
        Short form of the developmental test of visual motor integration (VMI) - set of 24 geometric figures of varying complexity, which have to be copied
      - Results
        
        Object drawing subtest of BDAE
        
        Compared to CA-matched controls, children with WS produced significantly: fewer recognisable drawings, fewer major parts, more disorganised drawings
        
        Children with WS produced significantly fewer recognisable parts than MA-matched controls
        
        Visuomotor integration test
        
        Individuals with WS scored significantly lower and produced significantly fewer recognisable drawings than either control group
  - - - Fodor (1983) argued that lang as a whole is modular
      - According to Anderson (1998), WS represents "a classic case in which [the lang acquisition] module has been spared from the general brain damage caused by the genetic disorder"
      - Chomsky (1980) argued that "computational" aspects of lang (phonology and grammar) are modular
    - - Laing et al. (2002) assessed 15 individuals with WS (M age 15;1) and 15 TD control children (M age 6;9), matched on verbal mental age and reading age
      - Battery of 11 phonological awareness/abilities tasks including:
        
        Rhyme detection - indicate which of three words rhyme with a stimulus word
        
        Rhyme production - supply words/nonwords that rhyme with stimulus words
        
        Phoneme deletion - remove the initial phoneme from a single-syllable word (e.g. what would "meat" sound like without the / m/?)
      - No significant difference between the AS group and control group on 10/11 tasks
      - However, the WS group performed sig less well on the phoneme delection tasks
      - Thus, overall, phonology was commensurate with other verbal abilities (but not chronological age)
    - - If true, grammar should be a performance peak and age-appropriate, but this is not what the evidence shows
      - One of the most widely used tests is the Test for the reception of grammar (TROG)
      - Syntax and morphology have been described as "perfect" WS
      - Studies show that performance of the TROG and other receptive grammar tests is
        
        No better than expected on the basis of non-verbal or overall mental age (therefore not age appropriate)
        
        Significantly poorer than receptive vocabulary
      - Thus, grammar neither a relative strength in language profile, nor age-appropriate in WS
    - - Receptive vocabulary was significantly better amongst adolescents with WS than would be predicted by age or non-verbal intelligence
      - Receptive vocabulary genuinely does appear to be intact - even a strength later in development
      - Children with WS reliably show a performance peak on tests of receptive vocabulary
    - - Vocabulary (lexical semantics) is "intact ater in development, but acquisition is delayed (how does "delay" sit in the modularity framework?)
      - Little evidence for an intact lang module, but lang is mental-age appropriate in WS
      - Neither phonology or grammar are age-appropriate, but both are strengths relative to other domains of cognition