Neuropsychological functioning in end-stage renal disease.

AIMS: To compare the neuropsychological functioning and behaviour of children with non-syndromic end-stage renal disease (ESRD) and sibling controls. METHODS: The study was carried out at two tertiary care paediatric teaching hospitals, in Halifax and Vancouver, Canada. Children with ESRD were on a renal transplant waiting list and either pending dialysis or on dialysis therapy. Twenty two patient-sibling pairs were evaluated. Neuropsychological assessments consisting of measures of intelligence, academic achievement, memory, and motor abilities were carried out. Maternal ratings of behaviour and self-report rating of self-esteem were collected. RESULTS: The Verbal, Performance, and Full Scale IQs of patients with ESRD were significantly lower than the IQs of the sibling controls. The mean differences were 8.6, 11.7, and 10.9 points, respectively. ESRD patients also had significantly more difficulty on measures of fine motor coordination and ability to copy geometric designs than sibling controls. There were no differences between groups on measures of academic achievement, memory, behaviour, or self-esteem. CONCLUSIONS: Although children with ESRD exhibited mild deficits on measures of intelligence and some measures of motor abilities, their neuropsychological outcome was more favourable than earlier reports indicated.

ADHD in preschool children: parent-rated psychosocial correlates.

Twenty-five children (21 males, 4 females; mean age 4.8 years) with attention-deficit-hyperactivity disorder (ADHD) participated in this study in addition to 25 typically developing children (21 males, 4 females; mean age 4.9 years). Parental ratings of the preschool children and preschool child self-ratings were examined within the framework of three domains: behavioral disturbance, social competence, and familial environment. Compared to their typically developing peers, preschool children classified as having ADHD were rated by their parents as significantly more aggressive, more demanding of parental time, less socially skilled, less adaptable to change in routine, and as exhibiting more noncompliance. In contrast to these parental ratings, preschool children with ADHD perceived themselves as equally competent, and as socially accepted as their peers. Parents of preschool children with ADHD rated themselves as less competent parents, and as experiencing a restricted parenting role. Although parenting a preschool child with ADHD was viewed as stressful, the parents did not rate general family functioning to be adversely affected.

Preschoolers classified as having attention-deficit hyperactivity disorder (ADHD): DSM-IV symptom endorsement pattern.

In the present study, 50 preschoolers were formally and independently classified using both the Diagnostic and Statistical Manual of Mental Disorders, fourth edition (DSM-IV) and third edition-revised (DSM-III-R) criteria for attention-deficit hyperactivity disorder (ADHD). The sample consisted of 25 preschoolers classified as having ADHD and 25 typically developing preschoolers for comparison; the sample was matched on gender, age, and socioeconomic status. All 50 preschoolers were without neurologic or neurodevelopmental disorders, oppositional defiant disorder, or language delay. There were four key findings: first, of the 25 preschoolers with ADHD, DSM-IV classification was as follows: hyperactive-impulsivity type (68%), combined type (28%), and inattentive type (4%). Second, the DSM-IV profiles showed that several symptoms were either infrequently endorsed by parents, reflecting limited applicability to preschoolers with ADHD, or frequently endorsed by parents of typically developing preschoolers, thus reducing their diagnostic value. Third, of the 25 preschoolers classified as having ADHD using DSM-IV criteria, 16% would not have been classified as having ADHD using the DSM-III-R criteria. The DSM-IV criteria therefore appear to be more lenient than the DSM-III-R criteria for this age group. Fourth, two symptoms that were not included in the DSM-IV, but were part of the DSM-III-R, were found to have clinical value for differentiating preschoolers with ADHD from their typically developing peers.

The cognitive and behavioural effects of clobazam and standard monotherapy are comparable. Canadian Study Group for Childhood Epilepsy.

PURPOSE: To compare the cognitive and behavioural effects of clobazam versus standard monotherapy in the treatment of childhood epilepsy. METHODS: A randomized, double-blind, prospective design was carried out at three Canadian pediatric epilepsy centres. This study was part of a larger multi-centre study on the efficacy of clobazam. Children with newly diagnosed epilepsy were assigned randomly to receive clobazam or carbamazepine. Children who had failed previous treatment with carbamazepine were assigned randomly to clobazam or phenytoin. Children who had failed on any other antiepileptic drug were assigned randomly to receive clobazam or carbamazepine. In a subset of patients neuropsychological assessments were carried out at 6 weeks and 12 months after initiation of medication. Intelligence, memory, attention, psychomotor speed, and impulsivity were assessed. RESULTS: There were no differences between the clobazam and standard monotherapy groups on any of the neuropsychological measures obtained at 6 weeks or 12 months. There was no evidence for a deterioration in performance for those children who remained on clobazam for the entire 12-month study period. CONCLUSION: The cognitive and behavioural effects of clobazam appear to be similar to those of standard monotherapy.

Early clinical assessment of attention.

Fifty preschoolers participated in this study. Twenty-five preschoolers classified as ADHD were matched with 25 typically developing preschoolers, and assessed using three tests of attention (two vigilance tests, one visual-search test). Their behavior exhibited during these attention tests was also assessed. Compared to their peers, preschoolers classified as ADHD exhibited significantly more omission and commission errors on the visual attention test. On the visual-search attention test, preschoolers classified as ADHD exhibited significantly more commission errors, and they took significantly longer to complete it. They did not exhibit significantly more omission or commission errors on the auditory attention test. The preschoolers classified as ADHD were also more vocal, more often off-task and out-of-seat, and they required more adult redirectives to return to task. Discussion is focused on the clinical value of developmentally appropriate attention tests and behavioral observation systems in the early clinical assessment of attention in very young children.

Peer relationship problems in children with Tourette's disorder or diabetes mellitus.

Peer relationships, social skills, self-esteem, parental psychopathology, and family functioning of children with Tourette's disorder and a chronic disease control group of children with diabetes mellitus were compared. Children with Tourette's disorder had poorer peer relationships than their classmates and were more likely to have extreme scores reflecting increased risk for peer relationship problems than children with diabetes mellitus, but did not report self-esteem problems or social skills deficits. Measures of peer relationships were not related to severity or duration of tics. Children with Tourette's disorder and Attention Deficit Hyperactivity Disorder were at increased risk for poor peer relationships. The psychosocial problems of children with Tourette's disorder do not appear to be the generic result of having a chronic disease.

Clinical assessment of psychopharmacological treatment of preschoolers with ADHD.

Sixteen preschoolers, (8 with Attention-Deficit/Hyperactivity Disorder [ADHD], 8 matched controls) were assessed twice, 5 months apart. Preschoolers with ADHD were rated by their parents as significantly more inattentive, exhibited more behavior problems, fewer age-appropriate social skills, made more errors of omission on both the visual and auditory attention tests, and more errors of commission on both the visual attention and the visual-search cancellation tests. Preschoolers with ADHD were then treated with stimulant medication and exhibited improved behavior as well as significantly reduced errors of omission on visual and auditory preschool vigilance tests, and fewer errors of commission on the visual-search preschool cancellation test. Developmentally appropriate direct measures of attention, in conjunction with parental ratings of child behavior, can be used to assess the efficacy of pharmacological treatment of preschoolers with ADHD.

Treatment of children with Williams syndrome with methylphenidate.

Children with Williams syndrome frequently present with symptoms of attention deficit hyperactivity disorder (ADHD), but there is little information that stimulant medication is useful in this population. A series of double-blind, placebo-controlled case studies was used to evaluate the cognitive and behavioral effects of methylphenidate on four children with Williams syndrome. Teachers and mothers completed behavioral rating scales and cognitive tests of attention, learning and memory, and academic productivity and accuracy in mathematics in each medication condition. Two of the children responded favorably in terms of decreased impulsivity, decreased irritability, and lower activity level as well as improved ability to pay attention. Methylphenidate is a useful adjunct in the treatment of some children with Williams syndrome.

MRI and nonverbal cognitive deficits in children with neurofibromatosis 1.

Magnetic resonance imaging brain scans and neuropsychological assessments of 17 children who met the NIH consensus diagnostic criteria for neurofibromatosis Type 1 were carried out in order to determine if there is a relationship between presence of high intensity signal abnormalities on MRI scans and nonverbal cognitive deficits. Cranial MRI scans in 10 patients (58.8%) demonstrated high intensity signal abnormalities, most frequently in the cerebral peduncles. Fifteen patients had nonverbal cognitive deficits (88.2%), including difficulty judging the orientation of lines, matching complex visual stimulus configurations, recalling pictures of faces, as well as copying and drawing from memory a complex geometric figure. There was not a significant association between nonverbal neuropsychological deficits and presence of high intensity signal abnormalities on MRI scans, possibly because the location of these hyperintense abnormalities was typically below the level of the basal ganglia. These findings suggest that the high intensity signal lesions seen on the MRI scans of children with neurofibromatosis Type 1 do not predict or explain their nonverbal cognitive deficits.

Valproic acid treatment of learning disorder and severely epileptiform EEG without clinical seizures.

Using a single-patient (n = 1) clinical trial, we studied a 7-year-old boy who presented with unsatisfactory progress in school and whose electroencephalogram had very active independent frontal spike discharges. He had not had clinical seizures. The patient was randomized to receive valproic acid, 125 mg twice daily, (four periods) or matching placebo (four periods) over 8 weeks. Primary endpoints were the Wechsler Intelligence Scale for Children-Revised Coding sub-test and a handwriting sample. Behavior was monitored using teacher's and parent's Conners questionnaires. His score on the Wechsler Intelligence Scale for Children-Revised Coding subtest was significantly improved while he was taking valproic acid (P = .03). His electroencephalogram improved from a pretreatment recording of 28 spike discharges per minute to a normal recording while on valproic acid. We conclude that in this patient, valproic acid had a cognitive enhancing effect, probably by reducing epileptiform discharges.

CT and MR of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency.

Siblings with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency were evaluated with CT and MR. Both imaging studies demonstrated a leukodystrophy with preferential involvement of the deeper arcuate fibers.

Reading disabilities and aggression: a critical review.

Several authors have suggested that there is a strong association between specific learning disabilities and aggression, antisocial behavior, and juvenile delinquency. Claims that learning disabilities cause aggressive behavior and delinquency are increasingly common in the popular press, and a variety of theories concerning this purported causal relationship have been proposed. This research is flawed by a lack of specificity in the definition of learning disabilities, with studies often examining heterogeneous groups of children with learning problems. The present review examines the relationship between specific reading disabilities (the most frequently diagnosed learning disability) and aggressive behavior. The data suggest that there is not enough evidence to conclude that reading disability causes aggressive or delinquent behavior, although limited evidence does suggest that reading disability may worsen preexisting aggressive behavior.

The effects of head injury in children on neuropsychological and behavioural functioning.

In a prospective study 76 children were divided into three groups on the basis of severity of head injury as defined by the Glasgow Coma Scale and duration of increased intracranial pressure. The children were administered a neuropsychological test battery and behavioural ratings were made by parents and teachers at three intervals: time of hospital discharge and 3 and 9 months post-initial testing. There were cognitive deficits related to severity of injury with the greatest difference in abilities observed between the severe and the other two groups. The greatest differences in skills were on the Performance IQ and timed tests of visual-motor speed and co-ordination. The greatest improvement in skills occurred in the first 3 months post-injury. Several children in coma for up to 4 weeks were able to obtain normal IQ scores. In the mild and moderate injury groups very few had behavioural change while in the severe group approximately 90% had one learning or adjustment difficulty and 40% had three or more problems.

Peer problems in Tourette's disorder.

To explore the social adjustment and peer relationships of children with Tourette's disorder, 29 patients with mild to moderate Tourette's disorder were studied. Children underwent neuropsychological testing. The patients completed self-esteem scales and their parents and teachers completed behavior rating scales. Peer relationships were examined with the Pupil Evaluation Inventory, which is a sociometric questionnaire completed by the child's classmates and provides measures of aggression, withdrawal, and likability. As a group, Tourette's disorder patients were significantly more withdrawn, more aggressive, and less popular than their classmates. Thirty-five percent of the children with Tourette's disorder received the lowest rating in the class on one or more of the Pupil Evaluation Inventory factors. These social problems were not predicted by the frequency or duration of tics. A clinical diagnosis of attention-deficit hyperactivity disorder and teachers' ratings on the summary scale of the Child Behavior Checklist and the Pupil Evaluation Inventory did predict poor adjustment. It is concluded that social adjustment is a major difficulty for many children with Tourette's disorder, irrespective of tic severity.

Anxiety and life events in childhood migraine.

The assumption that anxiety and stressful life events are major precipitants of childhood migraine was examined by comparing a group of children referred for evaluation of headaches with their headache-free best friends. Before assessment, 39 children (average age 11 years, 20 girls) and their parents completed standard anxiety, personality, and life events scales. The same scales were administered to the control children and their parents. All subjects met Prensky's criteria for migraine, and all reviewed an audiovisual program on migraine and were given the same instruction about analgesic medications. History of headache averaged 35 months (1 to 132 months). No statistically significant differences were found between patients and controls or between the two groups of parents on any of the anxiety or life events scales. Children's anxiety scores were not related to parents' anxiety scores. Personality profiles of patients were similar to controls. Headache diaries were used to assess headache severity and frequency during a 4-month follow-up period. Although all patients had anxiety scores within the normal range, those with higher self-rated anxiety scores at initial assessment had significantly more frequent and severe headaches during the follow-up period (P less than .001). We conclude that children with migraines are not more anxious or stressed than their friends. Normal amounts of stress and anxiety appear to lead to the expression of migraine; however, more anxious children with migraines have more frequent and severe headaches.

Speeded performance following head injury in children.

Fifty-one children who has sustained head injuries were divided into mildly, moderately and severely injured groups according to neurological criteria. The groups were matched for age, sex, and injury-test interval. Approximately 1 year after their injuries, patients were tested on speeded and nonspeeded measures of motor, visual-motor, and visual-spatial functioning as well as on the WISC-R. The performance of the mildly and moderately injured groups was similar, with both groups performing significantly faster than the severely injured group on measures of speeded performance. There were few significant differences between groups on measures requiring little speed. In contrast to the results for the other two groups, the severely head-injured group performed significantly worse on the highly speeded tests than on the low speed tests. The findings are discussed in relation to the literature on the cognitive sequelae of head injuries.

Neuropsychological deficits following head injury in children.

Children who had sustained head injuries were divided into three groups on the basis of severity of injury defined according to neurological criteria, and their performance on a battery of neuropsychological tests was compared. The groups were matched for age and sex. Performance IQ and timed tests of motor speed, fine-motor coordination, tactual-spatial functions, and verbal fluency showed significantly greater deficits for severely injured patients than for those who were mildly or moderately injured. Relations between neurologic indices of severity of injury and psychological outcome measures were investigated. Glasgow Coma Scale severity scores and the duration of altered consciousness were related to performance on several major neuropsychological functions. Acute intracranial pressure measures were unrelated to outcome. Comparisons of neuropsychological profiles indicated that the pattern of deficit after severe head injury is more distinctive than are those after mild and moderate injuries. Deficit patterns were least similar for the mild and severe groups, and were most similar for the mild and moderate groups. Results were discussed in terms of their prognostic utility, and suggestions were made for the future study of neurologic-psychologic relations after head injury.