Journal of the International Neuropsychological Society



CRITICAL REVIEW

A model of neurocognitive function in spina bifida over the life span


MAUREEN  DENNIS  a1 c1 , SUSAN H.  LANDRY  a2 , MARCIA  BARNES  a3 and JACK M.  FLETCHER  a4
a1 Brain and Behavior Program, The Hospital for Sick Children and the University of Toronto, Ontario, Canada
a2 Department of Pediatrics, University of Texas Health Science Center, Houston, Texas
a3 Department of Psychology, University of Guelph, Guelph, Ontario, Canada
a4 Department of Psychology, University of Houston, Houston, Texas

Article author query
dennis m   [PubMed][Google Scholar] 
landry sh   [PubMed][Google Scholar] 
barnes m   [PubMed][Google Scholar] 
fletcher jm   [PubMed][Google Scholar] 

Abstract

Spina bifida myelomeningocele (SBM), a neural tube defect that is the product of a complex pattern of gene-environment interactions, is associated with naturally occurring, systematic variability in the neural phenotype and in environmental factors that lead to systematic variability in the cognitive phenotype. We characterize the basis for variability in the cognitive phenotype of children with SBM with reference to a model of key biological, cognitive, and environmental events unfolding over the course of development from infancy to middle age. The cognitive phenotype is not domain-specific, but represents manifestations of unobservable constructs involving associative and assembled processing, the latter directly reflecting the impact of the neural phenotype on core deficits involving movement, timing, and attention orienting. The expression of the cognitive phenotype is variable, being moderated by features of the neural phenotype involving secondary CNS insults (such as hydrocephalus) that impair assembled processing, as well as by environmental factors (such as poverty, parenting, and education) that impair associative processing. The preservation of strengths in associative processing depends in part on the severity of the CNS deficits in SBM and the impact of the environment. (JINS, 2006, 12, 285–296.)

(Received August 4 2005)
(Revised November 7 2005)
(Accepted November 10 2005)


Key Words: Neural tube defects; Cerebellum; Midbrain; Perception; Attention; Language; Mathematics.

Correspondence:
c1 Reprint requests to: Maureen Dennis, Ph.D., Brain and Behavior Program, Department of Psychology, The Hospital for Sick Children, 555 University Avenue, Room 6278A, Toronto, Ontario M5G 1X8, Canada. E-mail: maureen.dennis@sickkids.ca


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