Frank Zelko

Children's consensual knowledge about the experiential determinants of emotion.

Kindergarten, third-, and sixth-grade children were given vignettes describing experiences that were likely to produce emotional states, and their consensus about the probable affective reaction was determined. A sample of eight social and personal (private) experiences was utilized in the vignettes: success, failure, dishonesty (caught or not caught), experiencing nurturance or aggression, and experiencing justified or unjustified punishment. The potential affective reactions that children were asked to choose among included happiness, sadness, anger, fear, and neutral affect. There were no sex differences. Children of all ages agreed that relatively simple experiences such as success and nurturance would elicit a happy reaction. For other categories of experience, multiple consensuses appeared for more than one affective reaction. There were developmental differences in the affective reactions anticipated to five of the eight experience categories. Results are discussed in terms of cognitive and social learning determinants of knowledge about the experimental antecedents of emotion for oneself and others.

Age at Onset of Epilepsy, Pharmacoresistance, and Cognitive Outcomes: A Prospective Cohort Study.

Objectives: Increasing evidence suggests that uncontrolled seizures have deleterious effects on cognition and behavior, particularly in the developing brain. Methods: In a community-based cohort, 198 children, aged <8 years with new-onset epilepsy were followed prospectively and reassessed with the Wechsler Intelligence Scales for Children, Third Edition (WISC-III) 8–9 years later. Linear regression analyses with interactions between age at onset (age) and pharmacoresistance (PR) were used to test whether earlier onset conveyed greater vulnerability to the effects of uncontrolled seizures. Full-scale IQ (FSIQ) and the 4 subdomain scores were examined. Adjustment for adaptive behavior scores in a subset was performed. A dichotomous indicator for IQ <80 or ≥80 was used to permit inclusion of children who were not tested, particularly those who were untestable. Results: FSIQ was not correlated with age. PR was associated with an 11.4 point lower FSIQ (p = 0.002) and similar decrements in each WISC-III domain. There were substantial age-PR interactions for FSIQ (p = 0.003) and 3 domain scores, indicating a lessening impact of PR with increasing age. The dichotomous IQ indicator was strongly correlated with age at onset in the pharmacoresistant group (p < 0.0001) and not in the non-pharmacoresistant group (p = 0.61). Adjustment for adaptive behavior measured near onset did not alter the conclusions. Conclusions: Uncontrolled seizures impair cognitive function with effects being most severe in infancy and lessening with increasing age at onset. These findings further emphasize the need for early aggressive treatment and seizure control in infants and young children.

Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability.

OBJECTIVES. Children with primary extrahepatic portal vein thrombosis (EHPVT) have portal-systemic shunting, which may lead to disturbed neurocognitive function similar to portal-systemic encephalopathy (PSE) seen with chronic liver disease and cirrhosis. The functions most affected are those involving fluid cognitive ability, which comprise neurocognitive domains such as attention, processing speed, and short-term memory, that are particularly vulnerable to systemic illness or diffuse neurologic insult. We determined the fluid cognitive ability of children with EHPVT and whether surgically restoring portal blood flow by mesenteric left portal vein bypass (MLPVB) improved it. DESIGN. Twelve children with EHPVT and no overt PSE underwent comprehensive neurocognitive testing before and 1 year after undergoing surgery with intent to perform MLPVB. The evaluations sampled 4 functional domains at both time points: (1) neurobehavioral (behavior, emotional, executive functioning); (2) broad cognitive (intelligence, achievement); (3) fluid ability (attention, mental speed, working memory, memory encoding); and (4) visual motor (drawing, fine motor). Tasks in the fluid-ability and visual-motor domains were expected to be especially sensitive to adverse effects of EHPVT and to be most likely to show improvement with MLPVB. The test group consisted of 8 subjects who underwent successful MLPVB, and the comparison group was composed of 3 patients who received distal splenorenal shunts and one whose MLPVB failed. RESULTS. Both groups demonstrated similar fluid cognitive ability at initial evaluation. Successful MLPVB resulted in significantly improved fluid cognitive function: in the fluid cognitive domain, significant improvements were seen for the hit reaction time variability in the Conners Continuous Performance Test, the attention scale of the Cognitive Assessment System, and immediate verbal memory in the Childrens Memory Scale. In the visual-motor domain, z scores on the Grooved Pegboard Test improved. No improvement was observed in the comparison group. DISCUSSION. The results show that surgically restoring portal flow to the liver in children with primary EHPVT results in improved fluid cognitive ability. Subjects showed some neurocognitive abnormalities involving mainly fluid cognitive ability consistent with minimal PSE seen in adults with chronic liver disease. Cognitive defects in patients with minimal PSE seem to relate primarily to attention and fine motor skill, and although affected patients can function in everyday life, they are at risk for performance deficits in educational and vocational situations requiring the ability to pay close attention and react quickly (eg, driving, employment in manufacturing). The tests we administered in these domains are pediatric equivalents to measures used to detect minimal PSE in adults and should detect abnormalities in the same functional domains. Our results suggest that a narrow battery of tests could be used to detect minimal PSE in children in a manner similar to the 5-test battery used in adults, eliminating the need for the comprehensive and broad testing we performed. Our findings suggest that shunting of portal blood from the liver in primary EHPVT can result in PSE and question whether it is as benign a disease as previously thought. The importance of our findings is twofold. For understanding the pathophysiology of PSE, we have shown that restoring blood flow to the liver improves cognitive function in children with EHPVT. For therapy for EHPVT, it becomes clear that MLPVB is an excellent treatment option. It is effective for treating the complications of portal hypertension and provides effective portal blood flow that other medical and surgical therapies do not. The findings provide additional evidence that primary EHPVT should be considered curable by MLPVB. However, comparison of overall risks and benefits of MLPVB with those of other therapeutic options and longer-term outcome studies must be completed before MLPVB can be fully endorsed as the best treatment for EHPVT in children. CONCLUSIONS. Surgical restoration of portal venous flow to the liver in children with primary EHPVT by MLPVB improves fluid cognitive ability. MLPVB should be considered in treating primary EHPVT, because it corrects portal blood flow and could optimize learning potential.

Cognitive and Academic Outcomes after Pediatric Liver Transplantation: Functional Outcomes Group (FOG) Results

This multicenter study examined prevalence of cognitive and academic delays in children following liver transplant (LT). One hundred and forty‐four patients ages 5–7 and 2 years post‐LT were recruited through the SPLIT consortium and administered the Wechsler Preschool and Primary Scale of Intelligence, 3rd Edition (WPPSI‐III), the Bracken Basic Concept Scale, Revised (BBCS‐R), and the Wide Range Achievement Test, 4th edition (WRAT‐4). Parents and teachers completed the Behavior Rating Inventory of Executive Function (BRIEF). Participants performed significantly below test norms on intelligence quotient (IQ) and achievement measures (Mean WPPSI‐III Full Scale IQ = 94.7 ± 13.5; WRAT‐4 Reading = 92.7 ± 17.2; WRAT‐4 Math = 93.1 ± 15.4; p < 0001). Twenty‐six percent of patients (14% expected) had ‘mild to moderate’ IQ delays (Full Scale IQ = 71–85) and 4% (2% expected) had ‘serious’ delays (Full Scale IQ ≤ 70; p < 0.0001). Reading and/or math scores were weaker than IQ in 25%, suggesting learning disability, compared to 7% expected by CDC statistics (p < 0.0001). Executive deficits were noted on the BRIEF, especially by teacher report (Global Executive Composite = 58; p < 0.001). Results suggest a higher prevalence of cognitive and academic delays and learning problems in pediatric LT recipients compared to the normal population.

Validation of the Pediatric Automated Neuropsychological Assessment Metrics in childhood-onset systemic lupus erythematosus.

To evaluate the reproducibility and validity of the Pediatric Automated Neuropsychological Assessment Metrics (Ped‐ANAM) when used in childhood‐onset systemic lupus erythematosus (cSLE).

Congenital central hypoventilation syndrome: Neurocognitive functioning in school age children

Examine indices of neurocognitive functioning in children with PHOX2B mutation‐confirmed neonatal onset congenital central hypoventilation syndrome (CCHS) and relate them to indices of PHOX2B genotype, demographics, and disease severity.

Longitudinal study of cognitive and academic outcomes after pediatric liver transplantation.

OBJECTIVE To determine the evolution of cognitive and academic deficits and risk factors in children after liver transplantation. STUDY DESIGN Patients ≥2 years after liver transplantation were recruited through Studies of Pediatric Liver Transplantation. Participants age 5-6 years at Time 1 completed the Wechsler Preschool and Primary Scale of Intelligence, 3rd edition, Wide Range Achievement Test, 4th edition, and Behavior Rating Inventory of Executive Function (BRIEF). Participants were retested at age 7-9 years, Time 2 (T2), by use of the Wechsler Intelligence Scales for Children, 4th edition, Wide Range Achievement Test, 4th edition, and BRIEF. Medical and demographic variables significant at P ≤ .10 in univariate analysis were fitted to repeated measures modeling predicting Full Scale IQ (FSIQ). RESULTS Of 144 patients tested at time 1, 93 (65%) completed T2; returning patients did not differ on medical or demographic variables. At T2, more participants than expected had below-average FSIQ, Verbal Comprehension, Working Memory, and Math Computation, as well as increased executive deficits on teacher BRIEF. Processing Speed approached significance. At T2, 29% (14% expected) had FSIQ = 71-85, and 7% (2% expected) had FSIQ ≤70 (P = .0001). A total of 42% received special education. Paired comparisons revealed that, over time, cognitive and math deficits persisted; only reading improved. Modeling identified household status (P < .002), parent education (P < .01), weight z-score at liver transplantation (P < .03), and transfusion volume during liver transplantation (P < .0001) as predictors of FSIQ. CONCLUSIONS More young liver transplantation recipients than expected are at increased risk for lasting cognitive and academic deficits. Pretransplant markers of nutritional status and operative complications predicted intellectual outcome.

A proposed framework to standardize the neurocognitive assessment of patients with pediatric systemic lupus erythematosus.

To develop and propose a standardized battery of neuropsychological tests for the assessment of cognitive functioning of children and adolescents with pediatric systemic lupus erythematosus (SLE).

Special education participation in children with epilepsy: what does it reflect?

Epilepsy is associated with academic and neurocognitive disorders, with the latter often assumed to explain the former. We examined utilization of special education services (SpES) in relation to neurocognitive test scores in a case-matched sibling control study. In a follow-up assessment 8-9 years after entry into a prospective study of childhood-onset epilepsy, cases and siblings participated in an interview and standardized neurocognitive testing. Analyses included 142 pairs in which both had Full Scale IQ ≥ 80 and the case had normal examination and imaging. Sixty-four (45%) cases and 25 (17.6%) controls reported SpES utilization, (matched odds ratio [mOR]=5.3, P<0.0001). Adjustment for neurocognitive test scores resulted in a mOR of 4.6 (P<0.0001). Types and duration of services were similar in cases and controls. Twenty-four percent of school-aged cases were already receiving services at the time of initial epilepsy diagnosis. Young people with epilepsy have academic difficulties that are not explained simply by cognitive test scores.

Multidisciplinary Approach to Childhood Epilepsy: Exploring the Scientific Rationale and Practical Aspects of Implementation

The management of childhood epilepsy requires attention to more than seizure control because children with epilepsy often suffer from comorbidities that lead to an increased frequency of psychiatric disease, learning difficulties, and other problems of psychosocial development. These comorbidities can stem in part from the same genetic traits that determine seizure susceptibility. Thus, mutations affecting potassium, calcium, and sodium channels have been linked with epilepsy syndromes and affective and behavioral abnormalities. It is important to consider the effect of antiepilepsy drugs on comorbid conditions and the effect on seizures of drugs used to treat comorbidities. A number of antiepilepsy drugs are available that have minimal adverse cognitive effects, and some can have positive effects on mood and behavior. Epilepsy in a child is a condition that affects and is affected by the entire family situation. In addition to appropriate neuropsychologic evaluation, optimal management of childhood epilepsy also can require the involvement of the social worker, advanced practice nurse, and educational specialist. Many elements of the multidisciplinary team approach can be instituted by the child neurologist in community practice and at large, specialized epilepsy centers. (J Child Neurol 2004;19:362-378).