Mary C. Kral

Neuropsychological Aspects of Pediatric Sickle Cell Disease

Sickle cell disease (SCD), a class of genetic disorders characterized by abnormal, sickled red blood cells, is a chronic illness that results in progressive cerebrovascular disease. Neurocognitive sequelae of clinically apparent cerebrovascular accidents in children with SCD are characterized by pervasive impairments, including decrements in general intellectual functioning, language and verbal abilities, visual-motor and visual-spatial processing, memory, academic achievement, and processing of subtle prosodic information. In contrast, subtle neurocognitive deficits in the areas of attention and concentration, executive function, and visual-motor speed and coordination appear to be associated with silent infarcts that are not necessarily detected on physical examination. Investigation of the disease course and associated neurocognitive sequelae suggest a disease-specific model of neuropsychological impairment. Recommendations are made for clinical and research efforts in the field of pediatric neuropsychology.

The Role of Neuropsychological Evaluation in Pediatric Sickle Cell Disease

Neuropsychological impairment occurs in children and adolescents with sickle cell disease. While the neuropsychological sequelae associated with cerebrovascular disease in these children have been identified, a well-informed clinical approach to neuropsychological evaluation for these vulnerable children has not been delineated. Moreover, issues related to standard of care for children and adolescents with sickle cell disease and transition to adulthood remain understudied despite the relevance to long-term medical, psychosocial, and neuropsychological outcomes. We provide recommendations for an informed approach to neuropsychological evaluation of youth with sickle cell disease that is determined by pertinent illness-related factors, consideration of general developmental level and timing of evaluation (i.e., baseline vs follow-up evaluation), relationship to medical treatment, and transition to adulthood. Although the neuropsychological methods presented in this manuscript are specific to sickle cell disease, this empirically informed approach may serve as a model for other pediatric populations.

The health education for lupus study: a randomized controlled cognitive-behavioral intervention targeting psychosocial adjustment and quality of life in adolescent females with systemic lupus erythematosus.

Introduction:To examine in a randomize controlled feasibility clinical trial the efficacy of a cognitive-behavioral intervention designed to manage pain, enhance disease adjustment and adaptation and improve quality of life among female adolescents with systemic lupus erythematosus. Methods:Female adolescents (n = 53) ranging in age from 12 to 18 years were randomly assigned to 1 of 3 groups including a cognitive-behavioral intervention, an education-only arm and a no-contact control group. Participants were assessed at baseline, postintervention and at 3- and 6-month intervals after completion of the intervention. Results:No significant differences were revealed among the 3 treatment arms for any of the dependent measures at any of the assessment points. For the mediator variables, a posthoc secondary analysis did reveal increases in coping skills from baseline to postintervention among the participants in the cognitive-behavioral intervention group compared with both the no-contact control group and the education-only group. Conclusion:Although no differences were detected in the primary outcome, a possible effect on coping of female adolescents with systemic lupus erythematosus was detected in this feasibility study. Whether the impact of training in the area of coping was of sufficient magnitude to generalize to other areas of functioning, such as adjustment and adaptation, is unclear. Future phase III randomized trials will be needed to assess additional coping models and to evaluate the dose of training and its influence on pain management, adjustment and health-related quality of life.

Ameliorating attention problems in children with sickle cell disease: a pilot study of methylphenidate.

Objective: This pilot study examined whether methylphenidate (MPH) was effective in enhancing cognitive performance and attention for children with sickle cell disease (SCD) with cerebrovascular complications who evidence attention problems. Methods: In this multisite, pilot study, we evaluated 2 separate double-blind controlled clinical trials, including a laboratory trial of the short-term efficacy of MPH, with the second study a 3-week home/school crossover trial evaluating the efficacy of MPH. The laboratory trial included 14 participants between the age of 7 and 16 years. Assessments included measures of sustained attention, reaction time, executive functions, and verbal memory. The home/school trial included 20 participants. The outcome measures were parent and teacher ratings of attention. The first study compared MPH with placebo, while the second trial compared placebo, low-dose (LD) MPH, and moderate-dose MPH. Results: In the laboratory trial, significant effects were revealed for measures of memory and inhibitory control. Parent and teacher reports from the home/school trial indicate that moderate-dose MPH produced superior improvement in attention relative to the placebo and LD MPH. Conclusions: Stimulant medication positively impacted select measures of memory and inhibitory control in some children with SCD. Attention, as rated by parent and teachers, was improved for a greater number of children and adolescents on higher doses of MPH relative to LD MPH and placebo. Stimulant medication may provide an effective intervention for some children with SCD and cerebrovascular complications who demonstrate attention problems.

Identification of ADHD in youth with epilepsy

PURPOSE To further characterize Attention-Deficit/Hyperactivity Disorder (ADHD) in a clinic-referred sample of youth with epilepsy. METHODS Children and adolescents with epilepsy who were referred for comprehensive neuropsychological evaluation comprised the sample. Data were obtained via archival record review. Youth with epilepsy were grouped according to ADHD diagnosis (no ADHD, ADHD - Combined Type, and ADHD - Predominantly Inattentive Type) and compared for a number of neurocognitive variables, seizure-related variables, and parent and teacher completed behavior rating scale variables. Data was examined via analysis of variance and logistic regression models with post hoc pairwise comparisons, employing Tukey procedure where appropriate. RESULTS Youth in this clinical case series (N = 204) demonstrated a high rate of ADHD diagnosis. ADHD - Combined Type was identified with slightly greater frequency than ADHD - Predominantly Inattentive Type. Age of seizure onset, seizure classification, and seizure frequency did not differ significantly between the groups. The groups also did not differ on most cognitive variables examined. Significant group differences were found for parent and teacher completed behavior rating scales assessing inattention, hyperactivity, and executive dysfunction. CONCLUSION Screening for ADHD in youth with epilepsy is important as these symptoms can significantly interfere with rehabilitative efforts. Behavior rating scales may be the most efficient and cost effective way to identify ADHD in youth with epilepsy.

Not All Generic Concerta Is Created Equal Comparison of OROS Versus Non-OROS for the Treatment of ADHD

Treatment effectiveness between equivalent doses of non-OROS (osmotic controlled release oral delivery system) methylphenidate ER and OROS methylphenidate ER (brand name Concerta) was examined in a clinical case series of children and adolescents followed for treatment of attention-deficit/hyperactivity disorder (ADHD). The Conners-Third Edition: Parent Rating Scale was used to compare ADHD symptoms when patients were taking non-OROS versus OROS at follow-up visits. A repeated-measures mixed-model approach was used to compare treatment effectiveness. The entire sample (N = 14) demonstrated a reduction in the mean score on the Inattention Scale from clinically significant (T-score > 65) to not clinically significant (T-score < 65) when patients were changed from non-OROS to OROS at the same dosage (mean T-score reduction = 23, p < .0001). The reduction in mean T-score after changing from non-OROS to OROS at the same dosage is indicative of improvement in symptoms of ADHD. Results provide empirical support for US Food and Drug Administration concerns regarding the therapeutic equivalence of non-OROS versus OROS for the treatment of ADHD.

Bullying and ostracism in youth with and without ADHD: implications for risk and resilience

ABSTRACT Objective: This study compared bullying experiences, psychosocial adjustment, and resiliency in youth with and without ADHD. Method: Participants included 73 youth ages 12–17 with and without ADHD. Participants and their caregivers completed the Bullying and Ostracism Screening Scale, Pediatric Symptom Checklist, and Fitness and Flourish Scale. Results: There were no significant group differences in youth self-report for rates of bullying experiences or psychosocial adjustment. In contrast, caregivers reported significantly higher levels of victimization experiences (bullying and ostracism) and psychosocial maladjustment for youth with ADHD. Youth who reported increased victimization experiences also reported significantly more internalizing symptoms and lower levels of resilience, irrespective of ADHD diagnosis. Conclusion. The diagnosis of ADHD did not confer increased risk for bullying or ostracism according to youth self-report. In contrast, caregivers of youth with ADHD reported increased risk for victimization and higher rates of psychosocial maladjustment. Potential reasons for these inter-rater differences are discussed.