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In Vivo Modeling of the Morbid Human Genome using Danio rerio
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Cognitive modularity and genetic disorders.

S J Paterson1, J H Brown, M K Gsödl

  • 1Neurocognitive Development Unit, Institute of Child Health, University College, London WC1N 1EH, UK.

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Neuropsychological models may not explain genetic developmental disorders. Infants with Williams syndrome show distinct cognitive development, improving in language but declining in numerosity over time.

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Area of Science:

  • Developmental psychology
  • Neuroscience
  • Genetics

Background:

  • Adult neuropsychological models are often applied to understand genetic developmental disorders.
  • Existing models assume cognitive profiles in childhood or adulthood reflect initial infant states, with modules being either intact or impaired from birth.

Purpose of the Study:

  • To challenge the application of adult neuropsychological models to genetic developmental disorders.
  • To investigate the developmental trajectories of cognitive abilities in infants with Williams syndrome.

Main Methods:

  • Two experiments were conducted with infants diagnosed with Williams syndrome.
  • Cognitive assessments focused on numerosity judgments and language abilities at different developmental stages.

Main Results:

  • A within-syndrome double dissociation was observed in Williams syndrome.
  • Infants performed well on numerosity tasks in infancy but declined in adulthood.
  • Conversely, language abilities were poor in infancy but improved in adulthood.

Conclusions:

  • Developmental trajectories in genetic disorders can be complex and do not necessarily align with adult models.
  • Findings suggest a dynamic interplay of cognitive development, challenging assumptions of static innate modularity.
  • Results may necessitate a re-evaluation of theoretical and clinical approaches to studying genetic disorders.