Behavioral profiles of children with infantile nephropathic cystinosis.

Children with infantile nephropathic cystinosis have evidence of visuospatial and arithmetic deficits on a background of normal intellectual and verbal skills. This study aimed to define further their behavioral phenotype. The Achenbach Child Behavior Checklist was completed by parents of: 64 children and adolescents with cystinosis (33 females, 31 males; mean age 8 y 8 mo, range 4 to 16y, SD 2 y 11 mo); 101 healthy controls (47 females, 54 males; mean age 8 y 4 mo, range 4 to 16 y, SD 2 y 11 mo); 21 children and adolescents with cystic fibrosis (CF), termed chronic-disease controls (9 females, 12 males; mean age 11 y 3 mo, age range 4 to 17 y, SD 3 y 5 mo). Compared with healthy controls, individuals with cystinosis had evidence of a significantly higher incidence of behavioral problems, including social problems, somatic complaints, and attention problems. Compared with the chronic-disease control group, the cystinosis group differed only on the Social Problems scale, with 22% of participants with cystinosis scoring in the 'at risk' range whereas no participant with CF received an elevated score on this scale. We conclude that children and adolescents with cystinosis have evidence of a significant incidence of social difficulties compared with individuals with another chronic illness and healthy participants. The combination of visuospatial problems, difficulty with arithmetic, attention problems, and social difficulties seen in the cystinosis group constitutes a behavioral phenotype of this genetic disorder. This cluster of cognitive and behavioral symptoms is also seen in the nonverbal learning disabilities syndrome, and suggests a possible early difference in brain development in children with cystinosis compared with children who do not share this genetic disorder.

Verbal learning and memory after childhood stroke.

Verbal learning and memory (VLM) following pediatric stroke was characterized in a cross-sectional neuropsychological and neuroimaging study of 26 subjects, aged 5 to 17, with a history of pediatric stroke and 26 age, SES, and gender matched orthopedic controls. Further comparisons were made between the VLM profiles of stroke subjects with right versus left hemisphere lesions and early (> 12 months) versus late (12 months) strokes. Overall, stroke subjects scored significantly lower than control subjects on several VLM indices (California Verbal Learning Test-Children; CVLT-C), as well as on measures of intellectual functioning (IQ) and auditory attention/working memory (Digit Span). Subgroup analyses of the stroke population found no significant differences in VLM, Digit Span, Verbal IQ or Performance IQ when left-hemisphere lesion subjects were compared to right-hemisphere lesion subjects. In contrast, early strokes were associated with significantly fewer words recalled after delay, reduced discriminability (fewer correct hits relative to false positive errors on recognition testing), and relatively worse auditory attention/working memory scores (Digit Span). These findings indicate that pediatric stroke subjects demonstrated more VLM impairment than control subjects, and early strokes were associated with greater recall and recognition deficits. In stark contrast with adult-onset stroke, both left- and right-hemisphere lesions during childhood resulted in similar VLM performance.

Sustained attention in children and adolescents after traumatic brain injury: relation to severity of injury, adaptive functioning, ADHD and social background.

PRIMARY OBJECTIVE: To examine the relationship of child and family psychosocial variables and traumatic brain injury (TBI) severity as it relates to sustained attention (the Paediatric Assessment of Cognitive Efficiency, PACE). RESEARCH DESIGN: Forty-two children and adolescents were recruited and participated in a 2 year longitudinal study to evaluate sustained attention using the computerized testing metric, PACE. More specifically, errors of omission (inattention) and commission (impulsiveness) were measured. MAIN OUTCOMES AND RESULTS: Significant improvement on inattention and impulsiveness were observed over time. High pre-injury psychosocial adversity and low pre-injury adaptive functioning significantly predicted a greater number of inattention errors. Severity of injury predicted the reduction of impulsiveness. Moreover, omission errors immediately after TBI predicted later secondary attention-deficit/hyperactivity disorder (SADHD, ADHD that emerges after TBI). CONCLUSIONS: Based on these findings, it is important to consider pre-injury child and family psychosocial characteristics in addition to severity of injury when predicting outcome of TBI in children.

Attention deficit hyperactivity disorder and neurocognitive correlates after childhood stroke.

We investigated the frequency and neurocognitive correlates of attention deficit hyperactivity disorder and traits of this disorder (ADHD/Traits) after childhood stroke and orthopedic diagnosis in medical controls. Twenty-nine children with focal stroke lesions and individually matched children with clubfoot or scoliosis were studied with standardized psychiatric, intellectual, academic, adaptive, executive, and motivation function assessments. Lifetime ADHD/Traits were significantly more common in stroke participants with no prestroke ADHD than in orthopedic controls (16/28 vs. 7/29; Fisher's Exact p < .02). Lifetime ADHD/Traits in the orthopedic controls occurred exclusively in males with clubfoot (7/13; 54%). Participants with current ADHD/Traits functioned significantly worse (p < .005) than participants without current ADHD/Traits on all outcome measures. Within the stroke group, current ADHD/Traits was associated with significantly lower verbal IQ and arithmetic achievement (p < .04), more nonperseverative errors (p < .005), and lower motivation (p < .004). A principal components analysis of selected outcome variables significantly associated with current ADHD/Traits revealed "impaired neurocognition" and "inattention-apathy" factors. The latter factor was a more consistent predictor of current ADHD/Traits in regression analyses. These findings suggest that inattention and apathy are core features of ADHD/Traits after childhood stroke. This association may provide clues towards the understanding of mechanisms underlying the syndrome.