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.

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.

The relationship between phonological awareness and reading and spelling achievement eleven years later.

This study reports follow-up data from 24 teenagers (mean age = 17 years; 11 boys and 13 girls) who had participated in a study of phonological analysis and reading and spelling abilities 11 years earlier, when they were enrolled in kindergarten. The results indicated that phonological awareness ability assessed during kindergarten (via the Auditory Analysis Test) was a significant predictor of word identification and spelling skills 11 years later, when both socioeconomic status and vocabulary development were controlled. In contrast, socioeconomic status, vocabulary development, word recognition, and spelling achievement assessed at kindergarten were not significantly correlated with reading and spelling achievement 11 years later. Results are discussed regarding the relative stability of phonological awareness and the importance of this awareness as a precursor to the development of word identification and spelling skills.

Williams syndrome: a neuropsychological profile.

Seven children with Williams syndrome were seen for neuropsychological assessment. Their performances were compared with those obtained from a clinical control group matched for age, sex, and Peabody Picture Vocabulary Test Standard Score. The results support the view that children with Williams syndrome suffer from a severe impairment in visual-motor integration. No child in the Experimental Group outperformed his/her matched control on either subtest assessing visual-motor integration skills, while no significant differences were noted between the groups on tests for simple motor skills (e.g., finger oscillation). Significant differences were also found on the subtests of the Wide Range Achievement Test. No significant differences between the groups were noted on tests for general language skills. The remedial educational, and neuropsychological implications of these findings are discussed.

Neuropsychological studies on adolescents with phenylketonuria returned to phenylalanine-restricted diets.

Nine adolescents with phenylketonuria (PKU), who had been on unrestricted diets for 2 to 11 years, underwent serial neuropsychological testing over two consecutive 4- to 5-week periods during which each was maintained on a low-phe diet supplemented in a triple-blind fashion either with L-phe (high phe) or L-alanine (low phe). Assignment to the initial condition was done randomly, and the alternate condition was substituted at the end of the first 4- to 5-week period. In 6 of 7 subjects with PKU, baseline median choice reaction times (RTs) were slower than those of controls matched for age, sex, handedness, and Full-Scale IQ (WISC-R). A highly significant improvement occurred during the low-phe phases of the study. Results suggest that adolescents with PKU on unrestricted diets have a neuropsychological deficit that is out of proportion to their overall intellectual handicap. Moreover, this deficit appears to be at least partly reversible by return to dietary phe restriction despite years of hyperphenylalaninemia.

Experience with adolescents with phenylketonuria returned to phenylalanine-restricted diets.

In order to test the feasibility of returning older children with classical phenylketonuria (PKU) to therapeutically and nutritionally adequate phenylalanine-restricted diets after 8 to 13 years of unrestricted diets, 7 adolescents (13 to 19 years old) of normal or near-normal intelligence with classical PKU were returned to phenylalanine-limited diets for periods of 8 to 10 weeks. During a 4- to 5-week period when the phenylalanine-restricted diet was supplemented with added L-phenylalanine to mimic pretreatment conditions, plasma phenylalanine levels were 1,327 +/- 282 microM on total phenylalanine intakes of 2,794 +/- 248 mg/day (55.3 +/- 11.5 mg/kg/day). During a similar period of dietary phenylalanine restriction, plasma phenylalanine levels were successfully maintained at 713 +/- 266 microM on dietary intakes of 655 +/- 210 mg/day (12.6 +/- 4.3 mg/kg/day). On the basis of 3-day diet records, the intakes of total protein, energy, calcium, phosphorus, iron, vitamins D, E, and A, ascorbic acid, thiamin, riboflavin, niacin, folacin, and vitamin B-12 over the entire 8- to 10-week study period were adequate by the standards of the Recommended Nutrient Intakes (RNI) for Canada, where the study was undertaken. Intakes of magnesium and zinc were significantly lower than the RNI. Although the return to nutritionally and therapeutically adequate dietary phenylalanine restriction was judged to be successful in each case, the amount and intensity of re-education and reinforcement required to maintain compliance was much greater than anticipated.

Comparison of cognitive ability, personality profile, and school success in epileptic children with pure right versus left temporal lobe EEG foci.

By reviewing electroencephalographic reports compiled over five years and checking clinical details, we identified and studied 27 children with apparently pure left or pure right temporal lobe epilepsy. The children underwent neurological examination and neuropsychological testing (Halstead-Reitan Battery, Wechsler Intelligence Scale for Children [WISC] and Wide Range Achievement Test), and parents completed the Personality Inventory for Children (PIC). Of 15 boys and 12 girls with a mean age of 12.7 years, 14 had right and 13 left temporal lobe epilepsy. Seizure control was excellent in 17. Five (2 with left, 3 with right temporal lobe epilepsy) had received professional emotional help, 6 (3 with left, 3 with right focus) received special help at school, and 5 (3 with left, 2 with right focus) had failed a grade in school. No significant left-right differences were seen in WISC full scale, verbal, or performance IQ scores, neuropsychological test battery findings, or clinical scale scores on the PIC. Most of the children with unilateral temporal lobe epilepsy had no measurable cognitive or emotional difficulties. Cognitive, personality, and school problems were equally distributed between left and right temporal lobe epilepsy groups. When the two groups were combined, however, 10 patients (5 with left, 5 with right focus) were identified by the PIC as maladjusted, and as a group showed significantly lower neuropsychological test functioning than did the normally adjusted children.

Behavioral treatment for common childhood problems.

Parents often consult family physicians about child rearing, child development, and school-related problems. Behavioral treatment is one method of dealing with such concerns. It involves identifying problems with a child's behavior, working to resolve them by rewarding desirable behavior and withholding rewards for undesirable behavior, and evaluating the outcome. Before treatment begins, it is necessary to establish that the parents feel the child's behavior is a problem; that the child can voluntarily control the behavior; that at least one parent or primary caretaker can benefit from instruction in how to modify behavior, and that the behavior to be changed is not just one facet of a larger family problem. Both parents and physicians may find self-help books and printed handouts very useful. Referral to specialized services may be appropriate for complex or recalcitrant problems.