What can developmental disorders tell us about the neurocomputational constraints that shape development? The case of Williams syndrome.
The uneven cognitive phenotype in the adult outcome of Williams syndrome has led some researchers to make strong claims about the modularity of the brain and the purported genetically determined, innate specification of cognitive modules. Such arguments have particularly been marshaled with respect to language. We challenge this direct generalization from adult phenotypic outcomes to genetic specification and consider instead how genetic disorders provide clues to the constraints on plasticity that shape the outcome of development. We specifically examine behavioral studies, brain imaging, and computational modeling of language in Williams syndrome but contend that our theoretical arguments apply equally to other cognitive domains and other developmental disorders. While acknowledging that selective deficits in normal adult patients might justify claims about cognitive modularity, we question whether similar, seemingly selective deficits found in genetic disorders can be used to argue that such cognitive modules are prespecified in infant brains. Cognitive modules are, in our view, the outcome of development, not its starting point. We note that most work on genetic disorders ignores one vital factor, the actual process of ontogenetic development, and argue that it is vital to view genetic disorders as proceeding under different neurocomputational constraints, not as demonstrations of static modularity.
Neurocognitive Development Unit, Institute of Child Health, London, United Kingdom. a.karmiloff-smith@ich.ucl.ac.uk
Annette
A
Karmiloff-Smith
Michael
M
Thomas
D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004268:DNA-Binding Proteins / Q000235:genetics; D005838:Genotype; D006801:Humans; D054461:Lim Kinases; D009473:Neuronal Plasticity / Q000502:physiology; D010641:Phenotype; D017354:Point Mutation / Q000235:genetics; D011494:Protein Kinases; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D018980:Williams Syndrome / Q000150:complications* / Q000235:genetics*
D004268:DNA-Binding Proteins; D011494:Protein Kinases; C515892:LIMK1 protein, human; D054461:Lim Kinases; D017346:Protein Serine-Threonine Kinases