Executive functioning and psychological adjustment in children and youth with spina bifida.

Children and adolescents with spina bifida are at risk for poor neuropsychological functioning and psychological outcomes. The relationship between executive functioning and psychological adjustment is an area worthy of investigation in this population. The current study assessed executive functioning and psychological outcomes in a group of children and adolescents with spina bifida (SBM) (n = 51) and nondisabled controls (n = 45). A mediation model was hypothesized, such that Metacognition, as measured by the Behavior Rating Inventory of Executive Function (BRIEF), mediated the relationship between group status (spina bifida versus nondisabled controls) and psychological outcomes. Results indicated that metacognitive skills fully explained the relationship between group and internalizing and depressive symptoms as reported by mothers. In particular, specific components of the BRIEF Metacognition composite were most responsible for this relationship, including Initiate, Working Memory, and Plan/Organize. The study limitations include its cross-sectional nature that precludes drawing conclusions about causality. The results have implications for treatment interventions for children and adolescents with spina bifida and typically developing individuals.

Factors of biological risk and reserve associated with executive behaviors in children and adolescents with spina bifida myelomeningocele.

This study examined differences between healthy children (n = 35) and those with spina bifida myelomeningocele (SBM; n = 42) on the Behavior Rating Inventory of Executive Function (BRIEF), a measure of executive function behaviors. It also examined whether aspects of biological risk associated with SBM and reserve factors within the family could account for variability in BRIEF scores for children and adolescents with SBM. Patients in the SBM group exhibited more problems than both published norms and a local comparison group of healthy children in metacognition but not behavior regulation. Behavior regulation problems in children with SBM were predicted by parent psychological distress. More shunt-related surgeries and history of seizures predicted poorer metacognitive abilities.

Taxonicity of nonverbal learning disabilities in spina bifida.

As currently defined, it is not clear whether Nonverbal Learning Disabilities (NLD) should be considered a matter of kind or magnitude (Meehl, 1995). The taxonicity of NLD, or the degree to which it is best construed as discrete versus continuous, has not been investigated using methods devised for this purpose. Latent Class Analysis (LCA) is a method for finding subtypes of latent classes from multivariate categorical data. This study represents an application of LCA on a sample of children and adolescents with spina bifida myelomeningocele (SBM) (N = 44), those presenting with features of NLD (N = 28) but no medical condition, and control volunteers (N = 44). The two-class solution provided evidence for the presence of a taxon with an estimated base-rate in the SBM group of .57. Indicator validities (the conditional probabilities of indicator endorsement in each latent class) suggest a somewhat different priority for defining NLD than is typically used by researchers investigating this disorder. A high degree of correspondence between LCA classifications and those based on a more conventional algorithm provided evidence for the validity of this approach.

Abnormal physiological responses to touch among children with persistent feeding difficulties.

Pediatric feeding/swallowing specialists commonly observe infants and toddlers who present with gagging or significant behavior state changes as a result of touch to oral and body regions. To date, this behavior has not been fully characterized or documented experimentally. This paper describes an exploratory study aimed at filling in these lacunae and providing a possible explanation. The study compares the responses to firm tactile pressure of 40 children between the ages of 3 and 18 months. The experimental group consisted of 20 children (12 males, 8 females) with heterogeneous medical diagnoses, at least 2 weeks of alternative feedings during the first 3 months of life, and a common history of persistent feeding difficulties. The comparison group consisted of 20 children with an unremarkable medical history matched by age and sex to the experimental group. Firm, tactile pressure was applied in a predetermined sequence following dermatome regions from legs to mouth. Response was recorded in terms of presence/absence of gagging and/or behavior state changes. Fifteen of the 20 children in the experimental group showed gagging (14) or inconsolable crying (1) in response to touch. None of the matched comparison group demonstrated either gagging or behavior state changes. The experimental and comparison groups showed significant differences in response to tactile input. Further, the source of these abnormal responses must be sought in similarity of experience across heterogeneous medical diagnoses and interventions. One possible explanation is a history of oral deprivation due to alternative feedings.

Valproate embryopathy: clinical and cognitive profile in 5 siblings.

Valproate embryopathy is a well recognized syndrome caused by prenatal exposure to the anticonvulsant valproic acid (Depakote). We report five half-siblings with the same mother (four different fathers) who all have valproate embryopathy. Valproic acid was the sole anticonvulsant in all five pregnancies, with doses ranging from 500 to 2,000 mg per day. All children were examined by a clinical geneticist and developmental pediatrician, and had formal developmental testing. Mean birth weight at term was 2,900 g (range: 2,400-3,400 g). Common features in the five children included: flat, broad nasal bridge (5/5), hypoplastic midface (4/5), apparent hypertelorism/telecanthus (4/5), smooth philtrum (4/5), thin upper lip (5/5), long thin tapering fingers (4/5), hypoplastic 5th toenails (2/5), and irregularly placed toes (2/5). Less frequent features were micro/brachycephaly (2/5), cleft palate (1/5), duplication cyst of small intestine (1/5), and hemangioma (1/5). None had neural tube defect. Neuropsychologic testing of the three children older than 4 years of age showed cognitive ability in the low normal or borderline range (mean IQ = 83; range: 75-86), with significantly lower scores in adaptive behavior and motor skills. Study of this family offers insight into the potentially high risk of valproate embryopathy in exposed pregnancies, and affords a unique opportunity to study the variability of expression and cognitive profile of the syndrome within one family.