Phenotypic plasticity and the perception-action-cognition-environment paradigm in neurodevelopmental genetic disorders

Dev Med Child Neurol. 2015 Apr:57 Suppl 2:52-4. doi: 10.1111/dmcn.12694.

Abstract

Careful study of the phenotype can have implications at several levels, namely clinical diagnosis, pathophysiological reasoning, management planning, and outcome measurement. Behavioural phenotypes involve cognition, communication, social skills, and motor control. They can be documented in a host of neurodevelopmental conditions and approached with the recently refined perception-action-cognition-environment (PACE) paradigm, which focuses on the neurodevelopmental processes that underlie learning and adaption to the environment through perception, action, and cognitive processing. Although this paradigm was originally developed in the context of cerebral palsy, it can be applied along developmental trajectories in several neurogenetic conditions, including Down syndrome, fragile X syndrome, Rett syndrome, Angelman syndrome, and Williams syndrome, to name but a few. It must be recognized, however, that relevant, valid tools for assessment and management strategies still need to be developed.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Child
  • Child Behavior / physiology*
  • Cognition / physiology*
  • Environment*
  • Humans
  • Intellectual Disability / genetics
  • Intellectual Disability / physiopathology*
  • Perception / physiology*
  • Phenotype*