Parental follow-through of neuropsychological recommendations for childhood-cancer survivors.

In the past 40 years, outcomes for children with cancer have changed considerably. The survival rate has increased to approximately 80%. With success and survival come detriments that often occur over time called late effects of cancer treatment. When the central nervous system is treated with radiation or chemotherapy, we often see impairment to the senses, cognition, and learning. For children who receive central nervous system treatment, follow-up with a neuropsychological evaluation is an excellent tool to evaluate learning and behavior in relationship to a child's brain. The authors' research examined neuropsychological evaluations for common themes related to diagnosis, age, sex, and/or treatment received, and the authors investigated whether the families implemented recommendations suggested in the neuropsychological evaluation. Less than 50% of recommendations from evaluations were implemented. The authors found that families need ongoing support and knowledge to implement the neuropsychological testing recommendations. Families need assistance navigating the school system and advocating for their child's needs. Continued surveillance of the child's academic needs by both the psychology and oncology teams is essential for long-term success.

Intermediate neurodevelopmental outcome after repair of ventricular septal defect.

BACKGROUND: Children with congenital heart disease are at risk for impaired neurodevelopmental outcome. Despite the high prevalence of ventricular septal defects relative to other cardiac defects, few studies have specifically examined this population. The purpose of this study was to provide data on the intermediate neurodevelopmental outcome for children after repair of a ventricular septal defect. METHODS: Neurodevelopmental outcome was assessed for 31 children using standardized tests of IQ, memory, visual-motor ability, and academic achievement. Standardized parent and teacher reports of behavioral and psychosocial functioning were also examined. Patients were grouped into younger (less than 6 years old; n = 18) and older (6 years and older; n = 13) cohorts. RESULTS: Full-scale IQ scores were within the normal range. Although most other domains of neurodevelopmental and behavioral functioning were comparable to normative data, patient functioning was lower than population norms in the areas of performance IQ for the younger cohort (90.6 ± 16.1; p = 0.023), visual memory for the older cohort (84.5 ± 15.3; p = 0.003), and visual-motor ability (87.9 ± 14.2; p < 0.001) and adaptive behavior (94.3 ± 15.3; p = 0.050) for the entire cohort. CONCLUSIONS: After repair of ventricular septal defects, children function within the normal range in most areas. Three of the four domains in which lower functioning was observed tap into visual cognitive abilities rather than verbal abilities. Lower mean scores in these areas were, however, generally within one standard deviation of the population mean, suggesting mild weaknesses rather than significant impairments.

Adaptive skills, cognitive, and behavioral characteristics of Costello syndrome.

Costello syndrome is a rare congenital disorder whose diagnosis is based on clinical findings. The underlying genetic cause has not been identified. Common characteristics include failure to thrive, feeding problems, short stature, coarsening of facial features, developmental delay, mental retardation, skeletal problems, cardiac complications, and increased risk for solid tumors. Given its rarity, existing literature is limited regarding its natural course. Developmental milestones and intellectual functioning have never been systematically studied in this population, therefore this study focused on defining developmental and intellectual attributes. Data was obtained through solicited participation at the 3rd International Costello Syndrome Meeting. A total of 18 children (age 3-20 years) were administered the Leiter-R brief-IQ and the Peabody picture vocabulary test (PPVT)-III to assess nonverbal cognitive functioning and receptive vocabulary. Parents/guardians completed the child behavior checklist (CBCL) and participated in an interview process to complete the survey form of the Vineland adaptive behavior scales to assess emotional/behavioral issues and adaptive behavior. Results indicated that nonverbal cognitive functioning ranged from 1.5 to 4.3 SD below the age mean. Receptive vocabulary skills ranged from average functioning to 4 SD below the age mean. Adaptive behavior composites were all in the low range (2.0 to >5.0 SD below age mean), however there was significant variability in the range of domain scores. CBCL results ranged from normal to clinically significant across various clinical parameters. These data support a generalized descriptive pattern of developmental delay and mental retardation, with noted variability in receptive language, level of adaptive behavior functioning, and emotional/behavioral aspects. Skills and behavior are compared to existing literature of children with various syndromes.