ASCS09: Proceedings of the 9th Conference of the Australasian Society for Cognitive Science
Williams Syndrome: Dissociation and Mental Structure
Mitch Parsell ([email protected])
Human Sciences, Macquarie University
Sydney NSW 2109 Australia
Abstract
Williams syndrome (WS) is a genetic disorder that, because
of its unique cognitive profile, has been marshalled as
evidence for the modularity of both language and social
skills. But emerging evidence suggests the claims of
modularity based on WS have been premature. This paper
offers an examination of the recent literature on WS. It argues
the literature gives little (if any) support for mental
modularity. Rather than being rigidly modular, the WS brain
is an extremely flexible organ that that co-opts available
neural resource in a highly dynamic manner to cope in the
world.
Keywords: William syndrome; modularity; social cognition.
Introduction
If you aim to understand the structure of the human mind
you face a substantial methodological hurdle at the very
outset: namely, mental processes are not directly observable.
This brings difficulties at the most fundamental level. It
means, for example, that even individuating mental
processes is extremely demanding. One way to attack the
problem of individuation is with the logic of dissociation.1 If
you can show that some intervention impacts cognitive
ability A1, but not A2 you seem to have reason for supposing
A1 and A2 are independent abilities. Different domains of
cognitive
science
favour
different
interventions.
Neuropsychologists tend to favour impairments. They look
to atypical brains to provide information about the
associations between cognitive deficits and the dissociations
between cognitive skills.
For neuropsychological impairment, the basic notion of
dissociation is straightforward: if ability A1 is impaired
while other abilities (A2-N) remain intact, A1 is said to
dissociate for A2-N. For example, if some lesion is found to
impact face recognition only, face recognition is said to
dissociate from other cognitive abilities. Notice it is
essential that A1 is selectively impaired. If all (or a
substantial range of) abilities are impaired, then we have no
dissociation. Thus dissociative evidence relies not merely on
the presence of deficits, but the co-existence of deficits with
preserved abilities. It relies on patterns of spared and
impaired abilities. When two patterns mirror each other, we
1
Of course, the inability to directly observe the critical
phenomena is not unique to psychology/cognitive science. Indeed,
such a situation is typical of much of science with different
sciences developing different methods and protocols for dealing
with the challenge. What is (some what) unique to
psychological/cognitive theorising a heavy relies on dissociative
evidence.
Article DOI: 10.5096/ASCS200942
have a double dissociation. Specifically, if A1 can be
impaired independent of A2 and A2 can be impaired
independent of A1, then A1 and A2 form a double
dissociation. If, for example, face recognition can be
impaired without impacting object recognition and object
recognition can be impaired independent of a deficit to face
recognition, then face recognition and object recognition
form a double dissociation.
Double dissociations can hold between individuals or
between entire atypical populations. The literature on
semantic processing is rich with individuals who form
double dissociation pairs for particular concepts. See, for
example, Samson and Pillon (2003) who examine individual
patients that appear to demonstrate double dissociations for
the concept “animal” versus the concept “fruit and
vegetable.” The literature on atypical populations also has
abundant claims of double dissociations. The population of
individuals with Williams syndrome (WS) has attracted
increasing attention in this regard.
WS is a genetic disorder that results from a disruption to a
contiguous section of approximately 26 genes in the
7q11.23 region on chromosome 7 (see Francke, 1999;
Peoples et al. 2000). It is extremely rare, reported to occur
in between 1 per 5 500 and 1 per 50 000 live births (see
Wang et al. 1997; Strømme, Bjørnstad & Ramstad, 2002).
The disorder has well-documented behavioural and
cognitive effects. People with WS have general intelligence
in the range of 50-65 IQ points, specific difficulties with
arithmetic, spatial reasoning, planning and problem solving,
while auditory and music skills, language, face recognition
and social abilities appear to remain relatively spared. This
unique pattern of apparently spared and impaired abilities
has seen WS recruited as evidence for mental modularity.
Apparently preserved language coupled with general
intellectual impairment seems to many especially significant
(e.g. Bellugi et al. 2000). This has seen WS recruited as
evidence for the modularity of language and especially
syntactic processing (e.g. Pinker, 1994; 1999). Pinker relies
heavily on the apparent double dissociation with individuals
with Specific Language Impairment (SLI): ‘the genetic
double dissociation is striking . . . . The genes of one group
of children [SLI individuals] impair their grammar while
sparing their intelligence; the genes of another group of
children [WS] impair their intelligence while sparing their
grammar ‘(Pinker, 1999, p. 262). For others, the pattern of
impaired versus “spared” abilities recommends WS as
evidence for a social reasoning module (e.g. TagerFlusberg, Boshart & Baron-Cohen, 1998; Baron-Cohen,
1998). Still others have argued that preserved musical
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abilities in WS individuals support the existence of an
independent music module (Levitin & Bellugi, 1998).
But recent research has questioned whether it is legitimate
to characterise language as preserved in WS. Language
abilities once thought to be intact are now thought to be
either significantly delayed or to follow atypical
developmental trajectories (see Brock, 2007). On the one
hand, comprehension, phrase repetition, mean utterance
length and object categorization appear to be delayed. On
the other hand, phonological processing and morphology
appear to follow an atypical developmental trajectory (see
Martens et al. 2008, pp. 580-582). More complex
grammatical skills (such as gender agreement, pragmatics
and semantic fluency) seem to be both delayed and
developmentally atypical (see Martens et al. 2008, p. 582).
And if language is delayed or developmentally atypical (or
both), then the argument from the WS population to mental
modularity is jeopardised. And it is not just skills in the
language domain that seem to have been previously
overstated for the WS population. In all four domains once
thought preserved, the latest research is suggesting the real
story is more nuanced and complex than simple skill
preservation.
In this paper, I look at the recent literature on WS. I argue
the literature gives little (if any) support for mental
modularity. The argument takes place over four sections that
examine each of the cognitive abilities often claimed to be
preserved in the WS population: namely, music, face
recognition, language and social skills. I argue in each case
there are significant challenges to using the WS cognitive
profile to argue for independent cognitive modules. There is
strong empirical evidence that the preserved music and face
recognition abilities of the WS population are served by
different cognitive resources than in the typically
developmental (TD) brain. This means the necessary
contrast with the TD brain cannot be made and the WS
population cannot be used as one side of a double
dissociation. Thus WS does not provide evidence of mental
modularity. Recent evidence for language and social skills
demonstrates that these abilities are not actually preserved.
Obviously, if these skills are no preserved, then they cannot
be used to ground dissociative claims and the arguments to
mental modularity are again jeopardized.2 While my main
purpose is negative I will offer a very broad-brush sketch of
a positive account of mental architecture in WS. The sketch
2
It is important at the outset to be clear about the argument
structure. My purpose is primarily negative: specifically, to
counter arguments from WS to mental modularity. This is
achieved by demonstrating that the assumptions required to licence
arguments to modularity do not hold in the case of WS. More
specifically, that in all four cases to be discussed the cognitive
skills are either impaired or atypical. Hence the WS population
does not provide evidence for mental modularity. Nevertheless, the
mind may still be modular; the claim is merely that WS does not
provide any support for modularity. In sum, my primary aim is
neither to develop an alternative to nor arguments against
modularity, but merely to remove one specific line of evidence for
modularity.
Article DOI: 10.5096/ASCS200942
offered shares a great deal with the neuroconstructivist
theory that Annetta Karmiloff-Smith has been advocating
for over a decade (e.g. Karmiloff-Smith, 1992; 1998; 2006;
2009). I argue that when taken seriously the empirical
evidence is suggestive of a developmentally flexible brain
that co-opts available neural resources in a highly dynamic
manner to cope in the world. The positive case is erected on
two pillars: First, significant interaction between the
“preserved” cognitive domains in WS. This establishes the
dynamic nature of the WS brain and is in direct conflict with
a rigidly modular account of mental architecture.3 Second,
the fundamental importance of particular cognitive drives
and biases in the development of the WS cognitive profile.
This shows the WS brain to be flexible and establishes the
need to pay serious attention to developmental trajectory in
explaining the WS cognitive profile.
Music
Individuals with WS have often claimed to have preserved
musical abilities (see Don, Schellenberg & Rourke, 1999;
Levitin & Bellugi, 1998; 1999). Further, they have a
particularly high musical drive: they tend to spend more
time listening to music and demonstrate an elevated
emotional response to music (Don et al. 1999; Levitin &
Bellugi, 1999). Indeed, individuals with WS are often
reported to have hyperacusis: a heightened sensitivity to all
or some specific sounds. Udwin (1990), Klein et al. (1990)
and Don et al. (1999) all report over 90% of WS individuals
surveyed had a history of hyperacusis. More recently,
Levitin et al. (2005) have looked closely at the response of
118 WS individuals to sound. They distinguish 4 types of
abnormal reactions to sound:
(i)
Hyperacusis: lowering of hearing threshold;
(ii) Odynacusis: lowering of pain threshold;
(iii) Phobias (auditory allodynia): aversion to or fear of
certain sounds; and,
(iv) Fascinations: abnormal attraction to certain sounds.
True hyperacusis remains low at only 4.7% of the sample,
whereas both odynacusis and phobias are remarkable high
in the WS sample: 79.8% and 90.6% respectively. The types
of sounds reported to cause aversions in WS were similar to
the other surveyed groups (namely, Down syndrome, autism
and TDs). The most commonly cited sounds were fireworks,
3
Of course, even extreme modularists accept there is interaction
between modules, but the interaction must remain shallow.
Although I do not have time to rehearse the argument in detail, I
have previously argued that modules are characterised by
information encapsulation (see Parsell 2009; also see Fodor 1983,
especially p. 122). Critically, encapsulation entails that any
processing within a module is unavailable to the rest of the mind.
For example, face processing cannot access earlier stages of the
representation(s) delivered to it. Thus interaction between modules
is shallow in that modules can trade outputs, but cannot penetrate
each other to impact processing. The type of dynamic interaction I
will point to in this paper is suggestive of deeper interaction then
the shallow communication allowed between modules.
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ASCS09: Proceedings of the 9th Conference of the Australasian Society for Cognitive Science
engines, thunder and vacuum cleaners. Several unusual
sounds were also reported to cause phobias in WS
individuals including cicadas, cows, spraying of shaving
cream and the saxophonist Kenny G. While fascination
levels in WS individuals are low (9%), they are much higher
than the levels reported by other surveyed groups: only one
non-WS participant reported a fascination. Fascinations
tended to be for broadband noises such as buzzing,
humming and motor noises. Interestingly, the attraction to
sounds felt by people with WS extends to fascinations with
the objects themselves. Levitin et al. (2005) note many
reports of collecting either the objects or depictions of the
objects. One WS individual is reported to own 18 vacuum
cleaners and can name the types from their sound alone.
What is the cause of these unusual sensitivities, phobias
and, to a lesser extent, fascinations? The problem does not
appear to reside in the peripheral auditory system. Levitin et
al. (2005) note that there is no evidence of systematic
auditory abnormalities in WS. They suggest rather an
explanation based on heightened awareness and central
auditory abnormalities. Bellugi et al. (1990) have provided
direct empirical evidence of hyper-excitability at a cortical
level in WS. Further, they suggest that different
neurological pathways may well handle auditory processing
in WS individuals. The WS brain appears to deal with
auditory information differently than the TD brain. The
auditory cell-packing density and neuronal size is abnormal
in WS (Galaburda & Bellugi, 2000; Levitin et al. 2005).
These abnormalities are consistent with hyper-connectivity
(Galaburda & Bellugi, 2000). For Levitin et al. (2005) the
evidence points towards organizational differences at both
the micro and macro levels of the brain that may explain not
merely the relative sparing of music, but the increased
music drive and unusual reactions to sounds. Musical
fascinations—the special emotional meaning associated
with particular sounds and their associated objects—can be
explained by supposing the auditory neurology co-opts or
otherwise significantly interacts with emotional centres.
Levitin & Bellugi (1998) have argued that preserved
musical abilities in WS individuals support the existence of
an independent music module. Such an argument depends
on the musical/auditory abilities of WS individuals being
served by same cognitive resources as in the TD brain. If
this does not hold the argument to modularity breaks down
for it depends on selective impairment/preservation.
Preservation of a skill (behaviour) due to compensation
(alternative cognitive route) undermines selectivity. And
this seems to be the case with the preserved musical abilities
of the WS population. More generally, as Gerran (2003) has
emphasised, alternative routes are an significant open
possibility
for
genetic
deletions
as
the
cognitive/neurological impact of the deletion can ‘be
compensated for in different ways over a protracted
developmental history in which the brain is at its most
plastic. As a consequence, the end state or behavioural
phenotype of someone with a genetic defect may be very
similar to that of a normal subject, but the developmental
Article DOI: 10.5096/ASCS200942
route, cognitively and neurally, may be very different’
(Gerrans 2003, p. 46). If the WS brain is developmentally
atypical then it is illegitimate to characterise the WS brain
as a TD brain with a pattern of specific preservation and
deficits. But without selectivity the argument from
preserved behavioural skills in the WS population to
specific cognitive modules fails. This will become clearer in
the next section where I discuss face recognition which also
provides support for the existence of alternative routes.
Face Recognition
WS individuals have preserved face recognition abilities,
despite having deficits in spatial cognition (see Bellugi et al.
2000). They are relatively good at interpreting socially
significant facial cues. They can judge emotions from facial
expression (Tager-Flusberg et al. 1998) and can infer
intentions from eye-gaze data (Karmiloff-Smith et al. 1995).
In a parallel to auditory input, faces seem to hold a
particular attraction to people with WS. In infancy,
individuals with WS show abnormal intense looking
towards strangers (Mervis et al. 2003) and throughout life
demonstrate abnormally high ratings of approachability for
unfamiliar faces (Bellugi et al. 1999; Frigerio et al. 2006).
Again paralleling auditory input, although face recognition
abilities are preserved in WS, there is substantive evidence
to suggest that the neurological route that delivers these
abilities is different from the route in the TD brain (e.g.
Deruelle et al. 1999; Grice et al. 2003). There are two broad
ways that faces can be recognised: feature by feature
(featurally) or in virtue of their overall pattern of features
(configurally). TDs rely heavily on configural information
to recognise faces. But individuals with WS are known to
have a relative deficit in processing configural information
generally (see Farran, Jarrold & Gathercole, 2002) and their
processing of visual information appears to rely heavily on
featural analysis (Donnai & Karmiloff-Smith, 2000). This
compensation strategy applies equally to face recognition:
individuals with WS predominantly employ featural
information to recognise faces (see Donnai & KarmiloffSmith, 2000). Thus WS individuals have spared face
recognition, but the cognitive-level skills used to support
that ability are different to TD subjects.
How is this compensation achieved? The early brain is
known to be extremely flexible. Thus it is plausible to
suppose that the early brain compensates for a lack of neural
resources responsible for configural processing by
redeploying available resources to significant tasks. We
have already seen that faces hold particular attraction for
WS individuals. Thus we should expect available neural
resources to be co-opted for face recognition. Thus neural
plasticity supports cognitive reorganisation in early
development. There is direct empirical evidence for face
recognition being served by different neural pathways in
individuals with WS. Mills et al. (2000) tested ERP
functioning of WS subjects in tasks requiring the matching
of upright face to inverted face. WS displayed different
pattern of activation to TD subjects in the right hemisphere
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that is responsible for configural processing. Mills et al.
(2000) suggest this is due to abnormal cerebral
specialisation of spared cognitive functions in WS (also see
Grice et al. 2001). Thus we have a case of spared abilities
being served by an alternative neural route that supports
different cognitive-level skills (featural as opposed to
configural). The behavioural output is the same, but the
cognitive level realisation and the neurological instantiation
are substantially different. This makes face recognition an
inappropriate domain to ground modular claims: the ‘data
refutes the idea of an intact module and instead suggests that
people WS may use a general object processes to recognize
faces’ (Donnai & Karmiloff-Smith, 2000).
Language
Individuals with WS are often claimed to have preserved
language abilities (e.g. Bellugi, Lai & Wang, 1997).
Grammatical and syntactic skills (e.g. Pinker 1994; 1999),
receptive vocabulary (e.g. Bellugi et al. 1990) and affective
language (e.g. Reilly, Klima & Bellugi, 1990) have been
cited as particular strengths. But recently the view that
language is preserved has come under escalating assault.
The most prominent attack is perhaps due to Stojanovik.
Stojanovik and colleagues have closely examined three
areas of the language claimed to be preserved in the WS
population: syntactic skills, social communicative abilities
and vocabularies. They argue that it is misleading to
describe WS skills across these areas as intact and, by
extension, that the WS profile does not provide evidence of
a modular basis for language (see, for example, Stojanovik,
Perkins & Howard, 2001; Stojanovik, Perkins & Howard,
2004; Stojanovik, 2006; Stojanovik & van Ewijk, 2008).
The most explicit argument for mental modularity from the
basis of the WS cognitive profile is found in the area of
grammatical and syntactic processing. The argument
proceeds via a posited double dissociation with SLI.
Individuals with SLI have specific problems with language
comprehension and/or production, while all other cognitive
areas appear to be preserved. According to Pinker (1994,
1999) this is in clear contrast to the WS population: ‘the
genetic double dissociation is striking, suggesting that
language is both a specialisation of the brain and that it
depends on generative rules that are visible in the ability to
compute regular forms. The genes of one group of children
impair their grammar while sparing their intelligence; the
genes of another group of children impair their intelligence
while sparing their syntax’ (Pinker 1999, p. 262).
Stojanovik and colleagues question whether this double
dissociation actually holds. Most directly, Stojanovik et al.
(2004) found no significant difference between the WS and
SLI populations on standardised language measures.
Furthermore, that the two populations often overlap in
abilities and on some measures their SLI participants out
performed their WS participants. This tells against the
purported double dissociation and offers a direct challenge
to those who argue for an independently language module
on the basis of the contrast between the WS and SLI
Article DOI: 10.5096/ASCS200942
profiles. The challenge is further strengthened by later work
on the social communicative language skills.
Individuals with WS have a number of documented social
communicative problems. They tend to chatter incessantly,
ask socially inappropriate questions and talk to themselves
(Stojanovik, 2006) and are not sensitive to the needs of their
conversational partner (Udwin & Yule, 1991). Stojanovik
(2006, p. 166) reports they are actually ‘conversationally
less able and less mature’ than both the TD and SLI
populations. They produce significantly fewer of the
continuations that demonstrate a willingness to maintain a
conversation rather then merely respond in conversational
turn. Their linguistic problems tend to be pragmatic
(Stojanovik, 2006; Laws & Bishop, 2004). They provide
insufficient information and fail to interpret either the literal
or inferential meaning of interlocutors’ utterances more than
the TD individuals (Stojanovik, 2006; pp. 166-167). Thus
the linguistic behaviour of individuals with WS either tends
to be parasitic on their partner or relies on their specialised
knowledge in their domains of fascination.
In sum, despite earlier claims to the contrary, it appears
language skills in WS are either significantly delayed or
follow atypical developmental trajectories. The work of
Stojanovik and colleagues suggests there is no evidence of a
double dissociation between the WS and SLI. Rather the
language trajectory in WS is atypical and the WS phenotype
is not a profile of ‘clearly identifiable patterns of strengths
and weaknesses’ (Stojanovik, 2006; p. 167-168). Further,
interaction between language and other preserved domains
is indicative of a highly interactive mind in which ‘language
abilities cannot develop separately from other cognitive
skills’ (Stojanovik, 2006; p. 168). I now move to consider
social cognitive skills that show significant interaction with
the face recognition and language domains.
Social Cognition
Individuals with WS are often described as having
preserved social cognitive abilities. This is an extreme
simplification. They are in fact inappropriately sociable,
hyperactive and socially anxious, overly friendly and
demonstrate abnormal social approach behaviour (see, for
example, Gosch & Pankau, 1994; Bellugi et al. 1999;
Frigerio et al. 2006). These behaviours appear to be the
result of increased social drive. Individuals with WS have
been characterised as having a strong compulsion towards
social engagement (Frigerio et al. 2006). Importantly, this
hyper-sociability is not restricted to familiar people.
Individuals with WS show abnormally high ratings of
approachability for unfamiliar faces (Bellugi et al. 1999;
Frigerio et al. 2006) and are far more likely then non-WS
individuals to approach strangers (Doyle et al. 2004). This is
regardless of the perceived trustworthiness of faces (see
Porter et al. 2007 for more details). That is, whether a face is
rated as trustworthy or not, individuals with WS will rate
the face as more approachable than non-WS individuals.
Thus it is surely illegitimate to characterise the social skills
of individuals with WS as preserved. Thus the WS profile
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does not provide dissociative evidence for the modularity of
social cognition. Moreover, social skills interact in complex
ways with other cognitive domains. Such interaction tells
against rigid modularity.
Recently, Riby, Doherty-Sneddon and Bruce (2008) have
argued that face perception abilities may be especially
significant to the social cognitive abilities displayed by WS
individuals. A rather unusual familiarity effect for face
recognition vividly demonstrates the complex interaction
between these domains. TD subjects rely more on internal
than external cues when recognising familiar faces. Thus
masking internal cues (eyes, nose and mouth) disrupts
recognition abilities for familiar faces more than masking
external cues (hair, chin and ears) (see Young et al. 1985).
But this is not the case for unfamiliar faces (see Bonner &
Burton, 2004). Thus normal subjects show a familiarity
effect: they demonstrate a relative advantage for internal
cues for familiar, but not for unfamiliar faces. Individuals
with WS do not show this familiarity effect. Moreover, WS
individuals can more accurately match unfamiliar faces
using internal cues than TDs (Riby et al. 2008). In fact, WS
subjects match unfamiliar faces to an accuracy only ever
reported in any population for familiar faces (Riby et al.
2008). Riby et al. (2008) suggest this unusual ability to
process unfamiliar faces, results from the atypical social
phenotype of WS: specifically, their hyper-sociability and
drive to gaze at face regardless of familiarity.
TDs are only willing to attend to internal cues for familiar
faces. That is, normal subjects will not attend to eyes, nose
and mouth of people they do not know. Thus the superiority
for internal cues for familiar faces is due to attentional bias.
Individuals with WS, on the other hand, will attend to
internal cues for unfamiliar faces. It is their increased social
drive—hyper-sociability irrespective of familiarity and
decreased levels of physiological arousal when gazing at
unfamiliar faces—that “enables” this attention. Thus their
reliance on external cues is decreased for all faces. Thus
there is no superiority effect for internal cues for familiar
faces, as internal cues are available for processing faces
whether or not they are familiar. This is indicative of a
complex interaction between face processing and social
abilities. It is not that face processing provides the building
blocks or part of the input for social cognition. We have
rather a dynamic system in which social drives and face
processing abilities show a complex two-way interaction.
Such dynamic interaction is indicative of a holistic
“problem solving” system rather than a rigidly specialised
modular mind. Thus we have not merely a counter to the
argument from WS to modularity as evidence by the
impaired social abilities of individuals with WS, but also
positive evidence that at least points towards a cognitive
profile that is non-modular. Such complex interaction can be
found in other areas of social reason. Consider, for example,
an otherwise surprising result on drawing tasks.
Dykens, Rosner and Ly (2001) report no global-local
dissociation on person drawing tasks: individuals with WS
can draw both global and local features of human figures.
Article DOI: 10.5096/ASCS200942
This is in distinction to the drawing of geometrical shapes
where there is a clear for bias to local features. Dykens et al.
(2001) argue this may results from the social phenotype of
WS. Thus due to their high sociability and interest in human
faces, global features of humans can be more easily drawn
than those of geometric figures. This interaction overcomes
a deficit in the WS phenotype by driving attention to
particular stimulus. In the above suggestion by Riby et al.
(2008) a similar attentional drive explains the lack of
familiarity effect for face recognition. In both cases,
increased drive forces attention to a particular stimulus that
in turn explains increased performance in the target domain.
In the drawing task, the drive-to-attention corrects
performance: increased attention leads to performance over
the WS baseline such that the usual global processing deficit
is overcome. In the lack of familiarity effect, increased
attention actually places WS individuals at a relative
advantage over TD individuals in the processing of
unfamiliar faces. This appears to be unique, but the drive-toattention correction of otherwise usual deficits appear to be
widespread. Consider just two cases involving interaction
between language and social cognition. First, the relatively
preserved ability of adolescents to convey affect using both
lexical expressions and vocal prosody appears to result from
increased social drive (Reilly et al. 1990; Losh et al. 2000).
Individuals with WS use affective modifiers more than TDs
(Losh et al. 2000). Both Reilly et al. (1990) and Losh et al.
(2000) suggest that WS individuals use these devices to
keep listeners engaged in the conversation. They argue this
represents a linguistic manifestation of the hyper-sociability.
Second, consider Theory of Mind (ToM) reasoning. A
number of studies suggest ToM abilities are spared (e.g.
Karmiloff-Smith et al. 1995; Tager-Flusberg et al. 1998).
But, more recently, Porter, Coltheart and Langdon (2008)
have found ToM reasoning to be impaired. The difference
between this and earlier studies was the use of a spatial (i.e.
sequencing) false beliefs task. On these non-verbal false
belief tasks the WS group performed significantly below TD
controls. This suggests ToM skills have previously been
overstated because of a verbal biasing: that the apparent
preservation of ToM abilities in previous studies is actually
an artefact of the verbal nature of standard false belief and
other ToM tests. That is, WS individuals are able to rely on
their relatively spared language skills to pass ToM tasks
even though their ToM abilities are impaired.
Before concluding it is worth making two more
speculative points in support of a thoroughly anti-modular,
dynamic account of WS. First, there is emerging evidence to
challenge the syndrome-specific understanding of WS.
Porter and Coltheart (2005) demonstrate that only some WS
individuals display the typical cognitive peaks and valleys,
while others actually display relative strengthens in
nonverbal skills and weakness in verbal abilities. Indeed,
WS seems to be a particularly heterogeneous disorder
(Porter & Coltheart, 2005; 2006). If this is the case, then the
argument from particular spared and impaired abilities in
the WS profile to modularity fails at the very outset. In fact,
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rather than presenting a population that can form one side of
a double dissociation, the most recent evidence is pointing
to double dissociations within the WS population between
two large sub-groups (Porter & Coltheart, 2005). A rigidly
modular account of cognitive architecture would seem to
lack the resources to explain such within population
variation. Second, the present account has the resources to
naturally explain the late onset of some “preserved” abilities
in WS individuals. For example, consider the surprising
results that younger children with WS perform lower than
expected for their chorological age on vocabularies tasks
(Thal, Bates & Bellugi, 1989; Volterra et al.2003) and WS
individuals typically have delayed first words acquisition
(Mervis et al. 2003) despite having essentially preserved
vocabularies in later life. The drive-to-attention correction
has a straightforward explanation of such delayed onset.
Extra time is required for an atypical route to develop due to
the drive-to-attention correction forcing the co-opting of
alternative neural resources. Hence WS individuals should
show slow starts followed by increased performance leading
to “preserved” abilities.
Conclusion
The WS brain is developmentally atypical. As such, it is
wrong to characterise the WS brain as a TD brain with a
pattern of specific preservation and deficits. Thus arguments
from WS to specific modules fail. Rather than speaking to a
system of functionally independent modules, the WS
literature suggests a highly dynamic cognitive architecture.
The story is one of complex interaction over the
developmental trajectory of the WS cognitive profile, a
story far too nuanced and intricate to be accounted for by
positing functionally independent modules.
Acknowledgments
Thanks to Jay Garfield, Cynthia Townley, Kim Sterelny and
members of the audience at ACS09, especially Phil Gerrans.
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Citation details for this article:
Parsell, M. (2010). Williams Syndrome: Dissociation and
Mental Structure. In W. Christensen, E. Schier, and J.
Sutton (Eds.), ASCS09: Proceedings of the 9th Conference
of the Australasian Society for Cognitive Science (pp. 277284). Sydney: Macquarie Centre for Cognitive Science.
DOI: 10.5096/ASCS200942
URL:
http://www.maccs.mq.edu.au/news/conferences/2009/ASCS
2009/html/parsell.html
Article DOI: 10.5096/ASCS200942
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