Literature DB >> 18612330

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

Tony J Simon1.   

Abstract

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.

Entities:  

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

Mesh:

Year:  2008        PMID: 18612330      PMCID: PMC2442464          DOI: 10.1002/ddrr.8

Source DB:  PubMed          Journal:  Dev Disabil Res Rev        ISSN: 1940-5529


  61 in total

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4.  Domain specific attentional impairments in children with chromosome 22q11.2 deletion syndrome.

Authors:  Joel P Bish; Renee Chiodo; Victoria Mattei; Tony J Simon
Journal:  Brain Cogn       Date:  2007-05-11       Impact factor: 2.310

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Journal:  J Opt Soc Am       Date:  1979-10

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Authors:  W R Kates; C P Burnette; E W Jabs; J Rutberg; A M Murphy; M Grados; M Geraghty; W E Kaufmann; G D Pearlson
Journal:  Biol Psychiatry       Date:  2001-04-15       Impact factor: 13.382

7.  Brain and behaviour in children with 22q11.2 deletion syndrome: a volumetric and voxel-based morphometry MRI study.

Authors:  Linda E Campbell; Eileen Daly; Fiona Toal; Angela Stevens; Rayna Azuma; Marco Catani; Virginia Ng; Therese van Amelsvoort; Xavier Chitnis; William Cutter; Declan G M Murphy; Kieran C Murphy
Journal:  Brain       Date:  2006-03-28       Impact factor: 13.501

8.  Vernier acuity, crowding and cortical magnification.

Authors:  D M Levi; S A Klein; A P Aitsebaomo
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9.  A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: velo-cardio-facial syndrome.

Authors:  R J Shprintzen; R B Goldberg; M L Lewin; E J Sidoti; M D Berkman; R V Argamaso; D Young
Journal:  Cleft Palate J       Date:  1978-01

10.  Atypical cortical connectivity and visuospatial cognitive impairments are related in children with chromosome 22q11.2 deletion syndrome.

Authors:  Tony J Simon; Zhongle Wu; Brian Avants; Hui Zhang; James C Gee; Glenn T Stebbins
Journal:  Behav Brain Funct       Date:  2008-06-17       Impact factor: 3.759

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  38 in total

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Review 2.  The importance of considering all attributes of memory in behavioral endophenotyping of mouse models of genetic disease.

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Journal:  Behav Neurosci       Date:  2012-06       Impact factor: 1.912

3.  Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation.

Authors:  Michael R Hunsaker; Naomi J Goodrich-Hunsaker; Rob Willemsen; Robert F Berman
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4.  The 1000 Genomes Project: deep genomic sequencing waiting for deep psychiatric phenotyping.

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Journal:  J Psychiatry Neurosci       Date:  2011-05       Impact factor: 6.186

5.  Adaptation of the Arizona Cognitive Task Battery for use with the Ts65Dn mouse model (Mus musculus) of Down syndrome.

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Review 6.  Comprehensive neurocognitive endophenotyping strategies for mouse models of genetic disorders.

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Journal:  Prog Neurobiol       Date:  2012-01-13       Impact factor: 11.685

7.  Female CGG knock-in mice modeling the fragile X premutation are impaired on a skilled forelimb reaching task.

Authors:  Amanda A Diep; Michael R Hunsaker; Richard Kwock; Kyoungmi Kim; Rob Willemsen; Robert F Berman
Journal:  Neurobiol Learn Mem       Date:  2011-12-21       Impact factor: 2.877

8.  Social impairments in chromosome 22q11.2 deletion syndrome (22q11.2DS): autism spectrum disorder or a different endophenotype?

Authors:  Kathleen Angkustsiri; Beth Goodlin-Jones; Lesley Deprey; Khyati Brahmbhatt; Susan Harris; Tony J Simon
Journal:  J Autism Dev Disord       Date:  2014-04

9.  Progressive spatial processing deficits in a mouse model of the fragile X premutation.

Authors:  Michael R Hunsaker; H Jürgen Wenzel; Rob Willemsen; Robert F Berman
Journal:  Behav Neurosci       Date:  2009-12       Impact factor: 1.912

Review 10.  Mathematical learning disabilities in special populations: phenotypic variation and cross-disorder comparisons.

Authors:  Maureen Dennis; Daniel B Berch; Michèle M M Mazzocco
Journal:  Dev Disabil Res Rev       Date:  2009
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