Ware AL, Kulesz PA, Juranek J, Cirino PT, Fletcher JM. (2017) Cognitive control and associated neural correlates in adults with spina bifida myelomeningocele. Neuropsychology. 2017 May;31(4):411-423. doi: 10.1037/neu0000350. Epub 2017 Feb 16. PMID: 28206781

 

Abstract

OBJECTIVE:

Accelerated aging can occur in adult survivors of neurodevelopmental disorders, but has been narrowly studied in spina bifida myelomeningocele (SBM). Since discrete aspects of cognitive control and related neural network macrostructure deteriorate in normal aging, the specificity and trajectory of cognition and neuropathology incurred across adulthood in SBM were examined.

METHOD:

Adults (N = 120) with and without SBM completed working memory span and manipulation tasks, and an inhibitory control task. A subset (n = 53) underwent structural MRI. Effects of group, age, and their interaction on performance and select gray matter volumes were examined.

RESULTS:

Adults with SBM had significantly poorer working memory accuracy and overall inhibitory control performance than typical peers. Age negatively predicted inhibitory control. Group × Age significantly interacted on span accuracy; advanced age related to diminished performance in typical adults, but not in adults with SBM. SBM related to disproportionately enlarged cortical and putamen and reduced hippocampus volumes. Group × Age significantly interacted on cortical, but not subcortical gray matter volumes. Dorsolateral prefrontal, hippocampus, and putamen volumes negatively correlated with cognitive performance.

CONCLUSIONS:

Supporting previous literature, current findings elucidated a profile of executive impairment in SBM that was maintained in a parallel maturational trajectory to typical aging. Accelerated aging in cognitive control or subcortical gray matter was not supported in SBM. However, reductions in anterior and posterior cortical regions were exacerbated in older adults with SBM compared with typical peers. Overall results supported persistent anomalous neurodevelopmental maturation across the life span in SBM that related to diminished cognitive control. (PsycINFO Database Record

(c) 2017 APA, all rights reserved).

Goal Management Training improves everyday executive functioning for persons with spina bifida: Self-and informant reports six months post-training.

Stubberud J, Langenbahn D, Levine B, Stanghelle J, Schanke AK. (2013) Goal Management Training improves everyday executive functioning for persons with spina bifida: Self-and informant reports six months post-training. Neuropsycholical Rehabilitation. 2013 Oct 30

Executive dysfunction accounts for significant disability for many patients with spina bifida (SB), thus indicating the need for effective interventions aimed at improving executive functioning in this population. Goal Management Training™ (GMT) is a cognitive rehabilitation approach that targets disorganised behaviour resulting from executive dysfunction, and has received empirical support in studies of other patient groups. The purpose of this study was to determine if GMT would lead to perceived improved executive functioning in the daily lives of patients with SB, as evidenced by reduced report of dysexecutive problems in daily life on self- and informant questionnaires. Thirty-eight adults with SB were included in this randomised controlled trial (RCT). Inclusion was based upon the presence of executive functioning complaints. Experimental subjects (n = 24) received 21 hours of GMT, with efficacy of GMT being compared to results of subjects in a wait-list condition (n = 14). All subjects were assessed at baseline, post-intervention, and at six-month follow-up. Self-report measures indicated that the GMT group's everyday executive functioning improved significantly after training, lasting at least 6 months post-treatment.

There were no significant effects on informant-report questionnaires. Overall, these findings indicate that executive difficulties in everyday life can be ameliorated for individuals with congenital brain dysfunction.

doi: 10.1080/09602011.2013.847847

Age-related Differences in Executive Function Among Children with Spina Bifida/Hydrocephalus Based on Parent Behavior Ratings

Tarazi RA, Zabel TA, Mahone EM. Age-related Differences in Executive Function Among Children with Spina Bifida/Hydrocephalus Based on Parent Behavior Ratings.
Clinical Neuropsychology. 2007 Aug 18;:1-18

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence. Parents of 36 individuals with MMH and 35 typically developing peers, ages 10 to 18 years, completed the Behavior Rating Inventory of Executive Function (BRIEF). The BRIEF is organized into eight scales and two primary indices - Metacognition (MCI) and Behavioral Regulation (BRI). As a whole, the children with MMH had significantly higher BRIEF T-scores, as well as a higher prevalence of clinically significant T-scores across subscales, particularly those representing cognitive control. Effects of group, age, and age-by-group interactions on the mean raw scores of the MCI and BRI were examined using regression analyses. There were significant group effects (p < .05) for both the BRI and MCI, with the controls having significantly lower mean ratings than the MMH group. There was also a significant contribution of age-by-group interaction on the BRI (p < .05). Although mean raw scores on the BRI for the MMH group remained stable across ages, mean raw scores in the control group decreased as age increased. Thus, healthy children have age-related improvements in executive control behaviors across adolescence, particularly behavioral control, while children with MMH demonstrate no age-related improvements in parent reported executive behaviors across adolescence. Therefore, children with MMH may continue to require targeted interventions and modifications to address executive dysfunction into young adulthood in order to promote functional independence.

PMID: 17853154

Neuropsychological profile of young adults with spina bifida with or without hydrocephalus.

Iddon JL, Morgan DJ, Loveday C, Sahakian BJ, Pickard JD.
Neuropsychological profile of young adults with spina bifida with or without hydrocephalus. Journal of Neurology, Neurosurgery, and Psychiatry. 2004 Aug;75(8):1112-8

OBJECTIVES: To determine the relative impact of hydrocephalus and spinal dysraphism in young adults on intellectual and cognitive functioning. Sub-groups of patients with congenital hydrocephalus and/or spina bifida were assessed between 1995 and 2003. The entry criteria were that individuals should have (i) intact global function, (ii) average verbal intelligence (or above), and (iii) should not have clinical depression. There were three sub-groups: patients with hydrocephalus and spina bifida, patients with hydrocephalus without spina bifida, and patients with spina bifida without hydrocephalus.

METHODS: Patients were neuropsychologically assessed as part of their normal clinical assessment during their annual medical review. Each individual completed a screening battery assessing global functioning, verbal intelligence, and mood. In addition they completed additional tests including measures of emotional intelligence, memory, attention, and executive function. Results were analysed to compare the performance of the patient sub-groups and to compare them to a healthy control group.

RESULTS: Patients with hydrocephalus (with or without spina bifida) were significantly impaired on the vast majority of all test scores as compared to patients with spina bifida and healthy controls. They were particularly poor on measures assessing executive function. By contrast for patients with spina bifida with no associated hydrocephalus, the significant majority of all test scores fell within the average range or above.

CONCLUSIONS: The neuropsychological profile of patients with hydrocephalus is one of relative impairment and this is so whether or not spina bifida is present. In spina bifida alone, in the absence of hydrocephalus, cognitive function is relatively spared.

Attention and Executive Functions in Adolescents with Spina Bifida.

BM, Holmbeck GN.
Attention and Executive Functions in Adolescents with Spina Bifida. Journal of Pediatric Psychology. 2007 Jun 7

OBJECTIVE: This study was designed to examine attention processes and executive functioning in adolescents with spina bifida, and to explore whether impairment in these domains contributes to problems with social adjustment.

METHODS: A sample of adolescents with spina bifida (n = 68) and a matched comparison group (n = 68) and their families were followed longitudinally. All participants completed questionnaires, and the adolescent participants underwent neurocognitive testing.

RESULTS: The spina bifida sample showed greater impairment on objective and subjective measures of attention and executive functioning, even when differences in intellectual functioning were controlled. Additionally, attention and executive deficits were found to be predictive of social adjustment difficulties. A mediational analysis suggested the neurocognitive deficits mediate associations between spina bifida status and social adjustment difficulties.

CONCLUSIONS: Adolescents with spina bifida appear to exhibit clear impairment in attention and executive functioning and this impairment may contribute to their well-established social difficulties.