This group of studies examines how dysmorphism in the cerebellum and midbrain is related to behavioral dysfunction in children with spina bifida meningomyelocele and hydrocephalus (SBH). SBH is associated with defining dysmorphologies of the cerebellum and midbrain, as well as with characteristic cognitive deficits in coordinate movement (movements are erratic and poorly timed), visuo-spatial analysis (difficult with visual attention, spatial visualization, and spatial orientation), and automatization. Although SBH disrupts both brain structure and cognitive function, the prototypical dysmorphisms of the cerebellum and midbrain have not been quantified; the signature functional deficits in coordinate movement, visuo-spatial analysis and automatization have been measured but not analyzed with respect to core processes; and the relations between dysmorphology and dysfunction have not been explained. We have three specific aims:
AIM1 is to measure brain dysmorphism in both volume and shape in the cerebellum and midbrain.
AIM2 is to describe dysfunction for: clinical markers (signs of dysfunction in the cerebellum and midbrain), signature psychometric test deficits in coordinate movement, visuo-spatial analysis, and automatization, and core processed deemed on the basis of cognitive theory to underlie each signature test deficit.
AIM 3 is to examine correlations between functional outcome and MRI indices of brain dysmorphism.
These aims will be pursued in a sample of 335 children with SBH and 96 controls, all 8-15 years of age. The large sample and the proposed analysis of brain structure-cognitive function relations should provide a unique window into the source of variability in motor and attention deficits in children with SBH in relation to their prototypical cerebellum and midbrain dysmorphologies.

Project Start
1998-03-20
Project End
1999-02-28
Budget Start
1997-10-01
Budget End
1998-09-30
Support Year
1
Fiscal Year
1998
Total Cost
Indirect Cost
City
Houston
State
TX
Country
United States
Zip Code
77225
Ware, Ashley L; Kulesz, Paulina A; Juranek, Jenifer et al. (2017) Cognitive control and associated neural correlates in adults with spina bifida myelomeningocele. Neuropsychology 31:411-423
Raghubar, Kimberly P; Barnes, Marcia A (2017) Early numeracy skills in preschool-aged children: a review of neurocognitive findings and implications for assessment and intervention. Clin Neuropsychol 31:329-351
Ware, Ashley L; Kulesz, Paulina A; Williams, Victoria J et al. (2016) Gray matter integrity within regions of the dorsolateral prefrontal cortical-subcortical network predicts executive function and fine motor dexterity in spina bifida. Neuropsychology 30:492-501
Dennis, Maureen; Cirino, Paul T; Simic, Nevena et al. (2016) White and grey matter relations to simple, choice, and cognitive reaction time in spina bifida. Brain Imaging Behav 10:238-51
Bradley, Kailyn A; Juranek, Jenifer; Romanowska-Pawliczek, Anna et al. (2016) Plasticity of Interhemispheric Temporal Lobe White Matter Pathways Due to Early Disruption of Corpus Callosum Development in Spina Bifida. Brain Connect 6:238-48
Arrington, C Nikki; Ware, Ashley L; Ahmed, Yusra et al. (2016) Are Shunt Revisions Associated with IQ in Congenital Hydrocephalus? A Meta -Analysis. Neuropsychol Rev 26:329-339
Williams, Victoria J; Juranek, Jenifer; Stuebing, Karla K et al. (2015) Postshunt lateral ventricular volume, white matter integrity, and intellectual outcomes in spina bifida and hydrocephalus. J Neurosurg Pediatr 15:410-9
Ruggiero, Jaclyn E; Northrup, Hope; Au, Kit Sing (2015) Association of facilitated glucose transporter 2 gene variants with the myelomeningocele phenotype. Birth Defects Res A Clin Mol Teratol 103:479-87
Treble-Barna, Amery; Juranek, Jenifer; Stuebing, Karla K et al. (2015) Prospective and episodic memory in relation to hippocampal volume in adults with spina bifida myelomeningocele. Neuropsychology 29:92-101
Kulesz, Paulina A; Tian, Siva; Juranek, Jenifer et al. (2015) Relations between volumetric measures of brain structure and attentional function in spina bifida: utilization of robust statistical approaches. Neuropsychology 29:212-25

Showing the most recent 10 out of 128 publications