A multimethod,multi-informant,and multidimensional perspective on psychosocial adjustment in preadolescents with spina bifida

This study examined the psychosocial adjustment of preadolescents with spina bifida in relation to a comparison sample of able-bodied preadolescents (8- and 9-year-olds; n = 68 in each sample). The study also examined the potential clinical utility of a narrowband multimethod, multi-informant, and multidimensional perspective on the assessment of psychosocial functioning in children and adolescents with pediatric conditions. Findings revealed that children with spina bifida tended to be socially immature and passive, less likely to have social contacts outside of school, more dependent on adults for guidance, less competent scholastically, less physically active, less likely to make independent decisions, and more likely to exhibit attention and concentration difficulties. No group differences were found for externalizing symptoms, affective functioning, or global self-worth, suggesting resilience in these domains for the spina bifida sample. Findings also suggest that low socioeconomic status and the presence of a physical disability may be additive risk factors for certain psychosocial adjustment difficulties.     

Schizophrenic-like neurocognitive deficits in children and adolescents with 22q11 deletion syndrome.

22q11.2 Deletion Syndrome (22q11DS) is the most common genetic microdeletion syndrome affecting humans. The syndrome is associated with general cognitive impairments and specific deficits in visual-spatial ability, non-verbal reasoning, and planning skills. 22q11DS is also associated with behavioral and psychiatric abnormalities, including a markedly elevated risk for schizophrenia. Research findings indicate that people with schizophrenia, as well as those identified as schizoptypic, show specific cognitive deficits in the areas of sustained attention, executive functioning, and verbal working memory. The present study examined such schizophrenic-like cognitive deficits in children and adolescents with 22q11DS (n = 26) and controls (n = 25) using a cross-sectional design. As hypothesized, 22q11DS participants exhibited deficits in intelligence, achievement, sustained attention, executive functioning, and verbal working memory compared to controls. Furthermore, deficits in attention and executive functioning were more pronounced in the 22q11DS sample relative to general cognitive impairment. These findings suggest that the same pattern of neuropsychological impairment seen in patients with schizophrenia is present in non-psychotic children identified as at-risk for the development of schizophrenia based on a known genetic risk marker.     

Maternal, Paternal, and Marital Functioning in Families of Preadolescents with Spina Bifida

Based on a family systems/social-ecological perspective, mothersand fathers of 8-and 9-year-old children with spina bifida (n=55;28male, 27 female) were examined in comparison to a matched groupof parents with 8-and 9-year-old able-bodied children (n=55;29 male, 26 female) across several areas of functioning (individual,parental and marital). Findings suggested that mothers and fathersin the spina bifida sample tended to report more psychosocialstress than their counterparts in the able-bodied sample. Specifically,mothers and fathers in the spina bifida group reported lessparental satisfaction than parents in the able-bodied group.Mothers in the spina bifida group reported less perceived parentalcompetence, more social isolation, and less adaptability tochange; fathers in the spina bifida group reported more psychologicalsymptoms. No differences between the spina bifida and able-bodiedgroups were found with respect to marital satisfaction. Copingpredictors of adjustment tended to vary as a function of parentgender rather than group status.     

Pretreatment predictors of cognitive deficits in early psychosis

BACKGROUND: Predicting cognitive deficits in early psychosis may well be crucial to identify those individuals most in need of receiving intensive intervention. As yet, however, the identification of potential pretreatment predictors for cognitive performance has been hampered by inconsistent findings across studies. We aimed to examine the associations of functional and clinical pretreatment variables with cognitive functioning after a first psychotic episode. METHOD: One hundred and thirty-one patients experiencing first-episode psychosis were assessed for psychopathology, pre-morbid functioning, duration of illness, age of onset, and family history of psychosis and neurocognitive functioning. Multiple regression analyses were conducted for six basic cognitive dimensions known to be affected in this population: verbal learning, verbal memory, verbal comprehensive abilities, executive functioning, motor dexterity and sustained attention. RESULTS: Pre-morbid functioning was the main predictor for five out of the six basic cognitive domains. Pre-morbid social adjustment difficulties were associated with worse performance in executive functioning, motor dexterity and sustained attention. Academic functioning was associated with verbal comprehension, and verbal learning and memory. Gender, age of onset, duration of untreated psychosis, and family history of psychosis had no or limited value as predictors of neurocognitive outcome. CONCLUSIONS: Poor pre-morbid functioning was related to a worse performance in the six basic cognitive dimensions evaluated; however, this accounted for only a small amount of the explained variance. Cognitive impairment is a prominent feature in patients with early psychosis regardless of favorable prognostic features such as short duration of illness, female gender, later age of onset, and non-family history of psychosis.     

Psychosocial aspects of PKU: Hidden disabilities – A review

Phenylketonuria (PKU) is an inborn error of metabolism, and its detrimental effects on neurocognitive functioning have been well studied. Early detection and treatment of PKU prevent the severe consequences of this disorder. However, even early- and well-treated patients experience hidden disabilities, including subtle deficits in executive functioning, mild reductions in mental processing speed, social difficulties, and emotional problems that may remain unnoticed for years. Poor executive function (EF) may impact treatment adherence and may lead to psychosocial deficits that are not always visible. These psychosocial aspects include social difficulties and psychosocial problems, such as forming interpersonal relationships, achieving autonomy, attaining educational goals, and having healthy emotional development. Studies report EF deficits in children and adults with early-treated PKU, which contribute significantly to the hidden disabilities in this population. In adults, hidden disabilities affect job performance and social relationships as a result of residual attention deficits, poor EF (e.g., planning, organizing), and reduced processing speed. An indirect relationship also exists between quality of life and EF impairment. In the absence of overt psychiatric symptoms, low level depressive or anxious symptom may be present. The interaction between the neurocognitive deficits and psychiatric symptoms puts this population of patients at significant risk for experiencing hidden disability. PKU is a disorder in which a less than optimal psychosocial outcome arises from the cumulative impact of relatively mild symptoms. The key to reducing risks associated with PKU is metabolic control throughout life.     

Neurocognitive functioning and magnetic resonance imaging in children with sickle cell disease

OBJECTIVE: To examine neurocognitive functioning in children classified with overt cerebral vascular accidents (CVAs), silent infarcts, or without central nervous system (CNS) pathology on magnetic resonance imaging. METHODS: Participants were 63 children and adolescents with sickle cell disease (SCD). RESULTS: Children with overt CVAs and silent infarcts differed from their peers without CNS pathology on measures of attention and executive functioning. CONCLUSIONS: We consider these deficits the result of the high frequency of frontal lobe deficits incurred by children with SCD. Recommendations include the use of tests designed to measure attention and executive functioning as a way of screening children with SCD for possible CNS pathology. We also suggest that future research examine the mechanism underlying frontal lobe involvement for individuals with SCD.     

Effects of continuous positive airway pressure on cognitition and neuroimaging data in sleep apnea

Obstructive sleep apnea (OSA) has been associated with a broad range of neurocognitive difficulties. The current view is that the neurocognitive impairment in OSA is due to the adverse effects of sleep fragmentation and/or intermittent hypoxia. The overall picture of cognitive deficits in OSA is complex. On balance, there appears to be negative effects of OSA on cognition, most likely in the domains of attention/vigilance, verbal and visual delayed long-term memory, visuospatial/constructional abilities, and executive dysfunction. Continuous positive airway pressure (CPAP) is the most effective and widely used treatment of OSA. In the majority of studies of OSA patients treated with CPAP, attention/vigilance improved, but changes in global functioning, executive functioning, and memory improved in about half of the studies. This may be due, in part, to variability in study design and sampling methodology across studies.     

Observed and perceived dyadic and systemic functioning in families of preadolescents with spina bifida

OBJECTIVE: To examine dyadic and systemic family functioning across several domains (conflict, cohesion, and stress) in families of preadolescents with spina bifida in comparison to families of able-bodied preadolescents (8- and 9-year olds; n = 68 in each sample). METHODS: Mother-, father-, and child-reported questionnaire data and observational ratings of family behavior were employed. RESULTS: Findings revealed significant group and socioeconomic status (SES) differences, particularly for the observational family data. Compared to families of able-bodied children, families in the spina bifida sample were less cohesive and children from this sample were more passive during family interaction tasks. Additional analyses suggested that some of these significant associations between group status and family functioning were mediated by verbal IQ, indicating that a significant portion (42%-55%) of the overall group effect was due to variations in child cognitive functioning. Lower SES families demonstrated higher levels of observed mother-child conflict, less observed and perceived family cohesion, and more life events. Lower SES families from the spina bifida sample appear to be particularly at risk for lower levels of family cohesion. CONCLUSIONS: Findings for the spina bifida sample support a resilience-disruption view (Costigan, Floyd, Harter, & McClintock, 1997) of systemic functioning in families of children with pediatric conditions.     

Impact of executive function deficits and attention-deficit/hyperactivity disorder (ADHD) on academic outcomes in children

The association between executive function deficits (EFDs) and functional outcomes were examined among children and adolescents with attention-deficit/hyperactivity disorder (ADHD). Participants were children and adolescents with (n = 259) and without (n = 222) ADHD, as ascertained from pediatric and psychiatric clinics. The authors defined EFD as at least 2 executive function measures impaired. Significantly more children and adolescents with ADHD had EFDs than did control participants. ADHD with EFDs was associated with an increased risk for grade retention and a decrease in academic achievement relative to (a) ADHD alone, (b) controlled socioeconomic status, (c) learning disabilities, and (d) IQ. No differences were noted in social functioning or psychiatric comorbidity. Children and adolescents with ADHD and EFDs were found to be at high risk for significant impairments in academic functioning. These results support screening children with ADHD for EFDs to prevent academic failure.     

Spinal Lesion Level, Shunt Status, Family Relationships, and Psychosocial Adjustment in Children and Adolescents with Spina Bifida Myelomeningocele

Investigated whether family functioning and child psychosocialadjustment were associated with spinal lesion level and shuntstatus in 65 children and adolescents with spina bifida myelomeningocele(age range = 8–16). Mothers of children with higher lesionlevels (i.e., thoracic level) reported more attachment to theirchildren, less family conflict, and a greater willingness togrant autonomy to their offspring. Such findings support a "marginality"interpretation of the data, insofar as the least physicallyimpaired children with spina bifida exhibited the greatest familydifficulties. Based on maternal report, children with shuntsperformed more poorly in school and exhibited lower levels ofcognitive competence than children without shunts. Findingsare discussed in relation to literatures on neuropsychologicalfunctioning and psychosocial adjustment in children with spinabifida.     

Condition-related knowledge among children with spina bifida: longitudinal changes and predictors

OBJECTIVE: To examine changes in three domains of condition-related knowledge among youth with spina bifida and to examine the utility of youth cognitive ability level and condition severity as predictors of knowledge change. METHODS: Seventy preadolescents with spina bifida completed a 12-item questionnaire assessing knowledge of spina bifida at three time points during middle childhood and early adolescence. Specific domains of knowledge assessed included (a) etiology of spina bifida, (b) functional status, and (c) shunt functioning (completed by participants with shunted hydrocephalus only). RESULTS: Findings revealed gains in accuracy of knowledge on 6 of 12 items; however, neither children's cognitive ability level nor condition severity predicted changes in knowledge over time. Most condition domains were characterized by low-to-moderate levels of knowledge across time. CONCLUSIONS: Although significant gains were evident in children's condition-related knowledge, at Time 3, many participants still failed to understand basic information about the etiology of their condition or major functional issues associated with spina bifida. Additional education about catheterization and shunt malfunction are two domains that may be of particular clinical significance.     

Parent-child problem-solving interactions in families of visually impaired youth

Investigated problem-solving and conflict-resolution strategies, an important aspect of family functioning, in visually impaired adolescents and their parents. Visually impaired adolescents were compared to adolescents with spina bifida and a control group of adolescents without disabilities. Parent-adolescent dyads participated in a problem-solving discussion of topics reflecting family disagreement. Videotapes of these discussions were rated for patterns of interaction using the Marital Interaction Coding System (MICS-III). Examination of positive and negative reciprocal patterns of interaction using sequential analyses and contrasting frequencies of specific behavioral codes revealed no differences between groups for adolescents, mothers, and fathers on the problem-solving discussion. Results are discussed in terms of (a) the impact of visual impairment on family functioning and (b) the need to identify those subgroups of visually impaired and their families that may be at heightened risk for maladjustment.     

Neuropsychological Functioning in Children With Spina Bifida and/or Hydrocephalus

Reviewed research on neuropsychological functioning (IQ verbal, visuospatial, tactile, motor, executive, and academic abilities) in children with spina bifida and/or hydrocephalus (SB/HC). These children usually have average to low-average intelligence but are prone to visuomotor deficits and delayed school progress, especially in arithmetic. Complications of SB/HC, including ventriculitis and seizures, tend to lower IQ scores and achievement in individuals.     

Condition severity and psychosocial functioning in pre-adolescents with spina bifida: disentangling proximal functional status and distal adjustment outcomes

OBJECTIVE: To examine relations between condition severity and psychosocial functioning in 70 8- and 9-year-old pre-adolescents with spina bifida by testing several direct, indirect, and mediated effects models for proximal functional status and distal adjustment outcomes. METHODS: Proximal functional status outcomes (e.g., degree of involvement in activities, scholastic competence, athletic competence, attentional problems) and distal adjustment outcomes (e.g., behavior problems, social competence) were assessed with mother, father, and teacher report. Severity variables included spinal lesion level, spina bifida classification, shunt status, ambulation status, number of shunt surgeries, and two severity composites. RESULTS: Condition severity was associated with the proximal functional status outcomes across parent and teacher report. In contrast, no significant relationships were found between the severity parameters and distal adjustment outcomes. Findings supported a proximal effects model of condition severity as well as an indirect effects model (e.g., presence of a shunt-->less scholastic competence-->less social competence) and were consistent with recent theoretical formulations (e.g., Wallander & Varni, 1995). CONCLUSIONS: Disentangling proximal functional status outcomes and distal adjustment outcomes is critical in studies of condition severity and psychosocial functioning. We discuss clinical implications.     

ADHD,learning, and academic performance in phenylketonuria

Despite having average intellectual abilities, academic difficulties are relatively common in children and adolescents with PKU. These academic difficulties may be a function of attention deficit hyperactivity disorder (ADHD), executive functioning deficits, and processing speed deficits, all of which are known to affect academic performance in non-PKU populations. This review focuses on what is currently known about academic performance in youth with PKU and offers suggestions for future research.     

Memory and executive functioning in obsessive-compulsive disorder: a selective review

BACKGROUND: The neurocognitive deficits that underlie the unique features of obsessive-compulsive disorder (OCD) are not yet completely understood. This paper reviews the main neuropsychological findings in memory and executive functioning in this disorder, and examines a number of challenges facing this area of research. METHOD: A selective review of the neuropsychological literature on OCD was conducted using MEDLINE and drawing on literature known to the authors. RESULTS: The neuropsychological profile of OCD appears to be one of primary executive dysfunction. Although memory functioning may be affected, these deficits appear secondary to an executive failure of organizational strategies during encoding. On tasks of executive functioning patients with OCD demonstrate increased response latencies, perseveration of responses, and difficulties utilizing feedback to adapt to change. LIMITATIONS: A statistical meta-analysis was not performed and only the cognitive domains of memory and executive functioning were examined. CONCLUSIONS: Given the prominence of chronic doubt and indecision in clinical settings, it is surprising that decision making as a cognitive construct as related to OCD has not received greater attention in the neuropsychological literature. On the basis of emerging literature we suggest that it is a potential area of dysfunction and one that warrants further investigation as it may assist in enhancing our understanding of the pathophysiology of OCD.     

Cognitive functioning in a population-based sample of young adults with a history of non-psychotic unipolar depressive disorders without psychiatric comorbidity

BACKGROUND: There is evidence for cognitive dysfunction in unipolar depression among middle-aged and elderly patients, but cognitive functioning among depressed young adults has scarcely been systematically investigated. The aims of the present study were to examine cognitive functioning among depressed young adults identified from the general population and to determine whether cognitive deficits vary as a function of different disorder characteristics, such as severity and age at onset. METHODS: Performance in verbal and visual short-term memory, verbal long-term memory and learning, attention, processing speed, and executive functioning was compared between a population-based sample of 21-35-year-olds with a lifetime history of non-psychotic unipolar depressive disorders without psychiatric comorbidity (n=68) and healthy controls derived from the same population (n=70). RESULTS: Depressed young adults were not found to be impaired in any of the assessed cognitive functions, except for some suggestion of mildly compromised verbal learning. Nevertheless, younger age at depression onset was associated with more impaired executive functioning. LIMITATIONS: The results may slightly underestimate of the true association between depression and cognitive impairments in the young adult population due to possible dropout of participants. Additionally, the problem of multiple testing was not entirely corrected. CONCLUSION: The findings from this study indicate that a lifetime history of non-psychotic unipolar depressive disorders among young adults without psychiatric comorbidity may be associated only with minimal cognitive deficits, even when some residual depressive symptoms are prevalent. However, early-onset depression may represent a more severe form of the disorder, associated with more cognitive dysfunction.     

Comparative meta-analyses of neuropsychological functioning in antisocial schizophrenic persons

To test the hypothesis that antisocial persons with schizophrenia are characterized by a distinct profile of neurocognitive deficits, meta-analyses of 43 studies were conducted to compare the neuropsychological performance of antisocial schizophrenic individuals to non-antisocial schizophrenic individuals, and to antisocial individuals without schizophrenia. Performance was evaluated across several different domains of neuropsychological functioning for both types of comparisons. Results indicated antisocial schizophrenic individuals demonstrated widespread deficits across multiple domains (Full Scale IQ, Verbal and Performance IQ, attention, broadly-defined executive function, and memory) in comparison to their antisocial counterparts. However, in comparison to their schizophrenic counterparts, persons with antisocial schizophrenia were characterized instead by reduced general intellectual functioning and memory dysfunction (as opposed to hypothesized Verbal IQ and executive function deficits). Findings may suggest a biologically distinct subgroup of antisocial schizophrenic individuals, whose study and treatment require differing approaches from those traditionally used in non-antisocial presentations of schizophrenia.     

Performance on the NEPSY among children with spina bifida.

Usefulness of the NEPSY: A Developmental Neuropsychological Assessment (NEPSY) to assess attention/executive function skills was examined in a sample of 30 children with spina bifida and shunted hydrocephalus (SBSH; 20 females/10 males; age range: 6-12 years). Statistically significant moderate-to-strong correlations between the NEPSY and other measures of executive functioning were obtained. The strength of these associations decreased when controlling for intelligence as measured by the WISC-III. The Attention and Executive Function domain appeared more closely associated with tests requiring focused attention and an ability to shift set than those emphasizing more abstract reasoning, memory, or non-verbal problem-solving skills. The NEPSY appears useful in the assessment of specific executive function abilities in young individuals with SBSH. More research into the NEPSY and particularly its Attention and Executive Function domain is needed among individuals with SBSH before its use as a stand-alone tool apart from adjunctive cognitive assessment instruments.     

Brief report: psychosocial adjustment in adolescents with inflammatory bowel disease

BACKGROUND: Inflammatory bowel disease (IBD) is an ideal disease for investigating adolescent adjustment to chronic illness, given its embarrassing, socially limiting, appearance-changing symptoms and adolescent onset. OBJECTIVE: To compare psychosocial adjustment among adolescents with a chronic illness to that of healthy adolescents and examine the role of adolescent disease onset. METHODS: Participants were 50 adolescents with IBD and their parents, and parents of 42 healthy comparison adolescents who completed questionnaires assessing behavioral, emotional, social, and family functioning. RESULTS: Adolescents with IBD were reported to have worse anxious and/or depressed and social problems than healthy adolescents. More adolescents with IBD were reported to have clinically significant social problems. Those diagnosed during adolescence were reported to have significantly worse social competence scores. CONCLUSIONS: Adolescents with a chronic illness such as IBD may be at higher risk for specific psychosocial difficulties than healthy adolescents. Diagnosis of a chronic illness during adolescence may have implications for social functioning.