A new account of the neurocognitive foundations of impairments in space, time and number processing in children with chromosome 22q11.2 deletion syndrome

Dev Disabil Res Rev. 2008;14(1):52-8. doi: 10.1002/ddrr.8.


In this article, I present an updated account that attempts to explain, in cognitive processing and neural terms, the nonverbal intellectual impairments experienced by most children with deletions of chromosome 22q11.2. Specifically, I propose that this genetic syndrome leads to early developmental changes in the structure and function of clearly delineated neural circuits for basic spatiotemporal cognition. This dysfunction then cascades into impairments in basic magnitude and then numerical processes, because of the central role that representations of space and time play in their construction. I propose that this takes the form of "spatiotemporal hypergranularity"; the increase in grain size and thus reduced resolution of mental representations of spatial and temporal information. The result is that spatiotemporal processes develop atypically and thereby produce the characteristic impairments in nonverbal cognitive domains that are a hallmark feature of chromosome 22q11.2 deletion syndrome. If this hypothesis driven account is supported by future research, the results will create a neurocognitive explanation of spatiotemporal and numerical impairments in the syndrome that is specific enough to be directly translated into the development of targeted therapeutic interventions.

Keywords: Attention; Brain; Cognition; Number; Space; Time.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Brain / abnormalities
  • Brain / pathology
  • Child
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 22 / genetics*
  • Cognition Disorders / diagnosis*
  • Cognition Disorders / genetics*
  • Cognition Disorders / psychology
  • DiGeorge Syndrome / diagnosis*
  • DiGeorge Syndrome / genetics*
  • DiGeorge Syndrome / psychology
  • Humans
  • Learning Disabilities / diagnosis*
  • Learning Disabilities / genetics*
  • Learning Disabilities / psychology
  • Magnetic Resonance Imaging
  • Mathematics*
  • Neuropsychological Tests
  • Perceptual Disorders / diagnosis*
  • Perceptual Disorders / genetics*
  • Perceptual Disorders / psychology
  • Problem Solving
  • Space Perception*
  • Time Perception*