Williams
Terms
undefined, object
copy deck
- Criteria
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rare genetic
microdeletion
supravalvuraorticstenosis
hypercalcaemia
Hyper acusisFacial features
Eating probs
Sleeping
developmental - Udwin & Yule 91
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Outgoing personality
Empathic to distress - Karmiloff Smith 95
- Good TOM
- Good at
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Language
Social
Face processing - Cognitive profile
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low IQ
VIQ> PIQ
good vocab
Block design - Reilly 90
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MLU>MA
WS)DS grammar
WS> DS empathy - Bellugi 88
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Vocab higher than mental age and DS> CA
Preserved grammar
VMA not appropriate
VIQ not near 100 (normal) - Other cog abilities
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low spatial
low drawing (elephant!)
Block design (Mervis 99)
spatial memory - Jarold 2001
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Language better than other abilities
Lang better than CA
Lang good but different - Neville 94
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ERP
Coffee,sugar , radiator
WS- diff brain activity therefore diff lang process - Singer Harris 97
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lang devel delayed?
Lang produ better than comprehen
pre-verbal problems:
-Joint attention
-social ref
-pointing - laing 2002
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low instrumental and declarative
low pointing comprehension - Word learning
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TD good at exhaustive sorting and fast mapping (use odd word to map to only object don't know) before vocab spurt
WS opposite
WS vocab b4 point
more depend on phenological mem - Karmiloff smith 97
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French words : Grammar agreement
WS good grammar rules for non-sense words difficulty with grammar across sentence elements (nouns & pronouns): good at local info (one wrd and ending) bad in sentence context
some rules ok some not able to generalize - Pinker 99
- Brains atypical from outset, some cog modules "damaged"
- Patterson 2001
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Innate modules
WS vs DS toddlers
WS spatial lang > Number
Spatial & DS number > lang - Preferential looking
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look at car -WS & DS bad
lang poor infancy gets better in development
double dissociation?
cannot count on phenotype outcome of intact modules - Wang & Bellugi 94
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Pheno memory
WS> DS
WS and lang = pheno memory
pheno growth doesn't = semantic markers
Increase in pheno encoding - Deurelle 99
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Featural processing Out of step w visuo-spatial cognition
- looked at inversion fx for houses, faces and shapes
-no inversion fx ; can't be floor fx as same as MA controls
- no age trend pattern - Modularity?
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lang not spared to TD level
Lang not dissociated ( nonverbal low as well as verbal)
syntax not ok - Tessabehjui
- Genetic Claims been challenged
- Tessabehjui 99
- Genetic Claims been challenged
- Karmil 98
- Uneven cog profile
- Bellugi & Wang 94
- Lang and face processing spared
- Karmiloff smith 98
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Newborn is precursor to later behavior
Equations mean that simple concepts increase in
Abstractness and complexity after equation of operation
Lower level representations converted to higher level representation - Karmiloff Smith 2003
- WS can't generalise to global processing "sticky fixation" to parts of the face - can't disengage
- Karmiloff smith, thomas 2003
- Genetic work ignores process of ontogenic development
- Cappelletti 2001
- Trauma= behavioral deficit
- Coltheart 2002
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Cognitive modules
agrammatisim
double diss
syntaz fluent
focal damage rest of system ok
Cognitive scores in normal range - Karmiloff Smith, Halit 2001
- ok on behavioral but not cog task
- Karmiloff Smith & Thomas 2003
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Is it a) widespread damage & normal plasticity
b) focal damage and atypical plasticity
c)* widespread damage and atypical plasticity
d)focal damage with normal plasticity - Karmiloff Smith Thomas 2003
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Genetic widespread across system
Tiny imparement in infancy develops over time - Johnson & Morton 91
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Child normal brains grows up in atypical environment
embryo/ drugs
"neurocomputational constraints" vs environment or constrained environment vs neurobiology
therefore look at constraints - Karmiloff Smit, Scerif & Thomas 2002
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Genetic mutations
no specific domain
why doesn't plasticity compensate for genetic? - Grice 2001
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Physicality, behaviour, abnormalities
Chemical, structural, chemical and functional - Morris, thomas & greenburg
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MZ twins
and parent to child transmittions - Ewart 93
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Genetic markers
- hemozygous elastin w partents w ws - Perez-Juralo 96
- Micro deletion w elastin gene
- Peoples Franke 2000
- Large repeats lending themselves to misalignment
- Frangiskakis 96
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Lim Kinase 1 gene expressed
deleted in all patients - Proschel 95
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Mouse models
LIMK1 expressed in CNS in embryos - Broder 99
- High blood pressure
- Jones 2000
- WS kids compensate with language to meet a social need Acquisition restricted by difficulty in extracting sense and context.
- Gosch & Pankau 97
- Lacking social judgement skills
- Tager Flusberg
- High anxiety about new situations
- Mervis 99
- Lang more advanced than downs
- Tagerflusberg & Karmiloff smith in press a review
- Studies in germany, italy, france and spain
- Good verbal?
- 25yr WS may have verbal MA of 8 but mastered syntax... not so great!
- Howlin 98
- Probs w comprehend non literal lang
- Temple 2002
- WS has low freq items in language
- Clansen & Almazan 2001
- probs w irregular words
- Temple and clasen press
- Normal modular system w selective deficits
- Ansari & Karmiloff Smith 2002
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Small samples used
Replicated; failed to get result- irregualr word learning - Pinker 94
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Aphasia & WS : both word inflection probs
Ws has parallels with depmentia - Bates & Roe 2001
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Kids w unilateral brain damage on lang development
Probs w 1st lang prod
“getting language off the ground”
Esp if left hemisphere posterior damage
If damaged before 5-7yrs plastic reorginisation happens - Rourke & Tsatsanis 96
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Dissociation btw performance and
Structural lang than pragmatics - Rourke 87
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White matter hypothesis
White matter has prolongued development
Is vunerable to disruption
R hemisphere w.matter used for developing and maintaining skills
L hemisph for developing not maintaining - white matter hyp
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Damage to white matter
Impared right –visuospacial pragmatics
Specific impare to left hemisp-productive
and expressive language and auditory procc.
Explains uneven profile of cog abilities. - Causal model
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Genes linked to behavior
But is through many to many one to one mappings
Between pheno and geno - Pennington 2001
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Genetic mutations limited in 3 ways
1.Brain size no of neurons
2.Neuronal migration
3. Neurotransmitters - Huttenlocher 2002
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Timing of gene expression
Long development and neurocomputational
Constraints = environment - Genie
- Neglect Lost capacity for languageAtropy of brain MRI
- Jarrold Philips Baddeley Grand and KS 2001
- Developmental framework Need spatial encoding to understand relational information i.e. darker than DoesnÂ’t explain why lang in WS is delayed
- Kellog
- Donald – picked up squeeks and
- Kendal 93
- Deficits & Processing
- Laing Butterworth 2001
- WS good at dyadic interaction with caregiver MA scores sometimes exceeded as persistently fixated on face WS can’t switch attention from adult –
- Majataka 2000
- Poverty in 2-3 yr old WS to use gestural information
- Merris 99
- Phonological memory strong in childhood and adulthoodWS
- Mervis & Bertrand 97
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Productive lang > than pointing
name spurt b4 fast mapping WS,
same time in TD
Naming and exhaustive mapping also not together (naming 1st ^ may use semantic info to name) - Nazzi Patterson & Karmiloff Smith 2003
- Fluent speech stream delayed
- Nazzi, Karmiloff Smith 2002
- 2yrs WS less able to use verbal cues to constrain categories
- Neuroconstructivist
- -Karmiloff-Smith Modules Plastic
- Patterson 2000
- Modular continuity hypothesis
- Stevens and Karmiloff Smith 97
- w WS used atypical constraints to label novel words
- Bellugi , Wang & Jerrigan 94
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Spatial cognition
Number
Problem solving - Rossen 95
- We can find in the wS pop that normal face processing is at floor performance on spatial tasks; Intact and impared mental abilities; linguistic functioning preserved while prob solv and visuo spatial impared
- Clahsen & Almazan 98
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Can't do irregular past tense
Statistical conclusion validity impared: small sample (4p's) - Clahzen & Almazan 98
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But small sample
not lang-level matched
Thomas 2001 - couldn't repeat - Thomas 2001
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21WS
42 TD
Controlled VMA- no deficit irreg
WS<TD irreg wrds only
But not fair if say irreg wrse(Clahzen & Almazan) than reg : development follows this way
usedlarge comparison grp-sample
- variability in WS ability w irreg and reg: can see prob w clahzen's wrk - Laing 2001
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WS - reading for concrete and abstract wrds
Controls- concrete easier to read, WS otherway round - Grant 97
- Vocab test - WS 4-5yrold ability at 8yrs - relied on phonology not semantics - therefore semantics unlike TD, it plays a less constraining role on phonology with WS
- Volterra 2001
- Italian Speaking WS kids: gender a prob
- Mervis 93
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pointing after language
high interest in faces over toys
config processing bad
featural processing good - Mills 2000
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18 p's
ERP study
abnormality in early wave forms -not found in controls - Grice 2000
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•Decrease in right lateralization
•ERP study
•18 p's
•waveform differences for faces
•no specialisation of faces
•no inversion fx
•still atypical
•Behav and cog evidence not same
•Same cognitive processes but overlap in overall development - Patterson, Brown, Johnson & K-S 2000
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Vocab > number:
sensitivity to numerical display of objects
65 kids
horse pic
MA & CA controls WS had lower deficit in sensitivity
DS impared
WS good as infants tho kids and adults no so good
But could be due to face processing: horse picture used - Patterson, Brown, Johnson, Karmiloff Smith 1999
- Diff trajectories not modular
- Laing (in press)
- pointing and JA reduced in WS toddlers vs MA and CA controls
- de Haan, Johnson 2001
- 3m preferred morphed face of already seen to one already seen: don't learn exemplars but prototypical representation of configuration of processed faces
- Brown 2000- pilot
- WS no prototype fx - de Hann's study