Neonatal cerebral white matter echolucencies predict visual resolution acuity deficits in very-low-birthweight (VLBW) infants. We examined maternal sociodemographic, lifestyle, intrapartum, infant birth/perinatal, and ocular motor/refractive characteristics to determine whether they accounted for this association in infants who were tested once between postnatal age 25 and 56 weeks (corrected for gestational age at birth). Cranial ultrasound scans were read by consensus to identify echolucency in a population of VLBW infants with no known ocular abnormalities. Visual resolution acuity was measured with the Acuity Card Procedure (ACP) in 14 infants with echolucency and compared with that of 81 VLBW infants born in the same hospitals with normal ultrasound scans. In time-oriented logistic regression models, echolucency remained a consistent predictor of abnormal visual resolution acuity after adjustment for covariates in three developmental periods (pre-, peri-, and postnatal). Odds ratios ranged from 19.3 (95% confidence interval, 4.5 to 82.2; p=0.001) to 10.4 (95% confidence interval, 1.3 to 81.9; p=0.03). Reduced visual resolution acuity in VLBW infants appears to be due to cerebral white matter damage.

To assess the prevalence of macrocephaly (head circumference [ges ]1.88 standard deviations above normative data for age and sex or >97th centile) in autism and other pervasive developmental disorders, 41 children with autism, and a comparison group of 21 children with tuberous sclerosis complex (TSC) or an unspecified seizure disorder were studied. Familiality of head circumference was also assessed from measurements of 133 first-degree relatives. Significantly higher rates of macrocephaly were found in probands with autism (12.2%) and their first-degree relatives (15.5%) when compared against a published normative sample. The incidence of macrocephaly in the comparison group of probands with TSC and seizure disorder (9.5%) and their first-degree relatives (8.3%) was higher than normative data as well, although the relation between macrocephaly and autism was more pronounced. Head circumference and extreme scores reflecting macrocephaly were moderately heritable in the present sample (H2=0.47). The increased prevalence of macrocephaly in relatives of children with autism compared with control children suggests that this characteristic may be a familial risk factor in the pathogenesis of autism.

The Kinaesthetic Sensitivity Test (KST) was used to measure the development of kinaesthetic acuity in adolescent boys. Thirty boys were tested longitudinally, at intervals of 6 months, between the ages of 11½ and 14 years. A second group of 20 boys was tested at the ages of 14 and 16½ years. The findings were compared with existing normative data on 5- to 12-year-old children and young adults, and they indicated improvement in kinaesthetic acuity with age. Although the age effect is statistically significant only in the older group, confidence intervals show that the rate of improvement in both groups is comparable to improvement between the ages of 5 and 12 years. The reliability of the test is rather poor. The conclusion is that kinaesthetic development continues throughout adolescence. Further, development is quite robust and detectable even with a fairly unreliable measurement instrument. However, individual assessments should be interpreted with caution.

The aim of this study was to apply the International Classification of Impairments, Disabilities and Handicap (ICIDH; WHO 1980) parallel to the Gross Motor Function Classification System (GMFCS; Palisano et al. 1997) in a population-based series of children with cerebral palsy (CP). Of the 116 children studied, birth characteristics, data on gross motor function, and level of handicap at 5 to 6 years of age, were retrospectively collected from medical records and documentation made by rehabilitation team members. Low handicap scores and mild levels of gross motor disability were present in children with hemiplegic CP, moderate scores in children with diplegic CP, simple ataxia, and athetotic CP, and high scores in children with dystonic CP and tetraplegic CP. A significant correlation was found between high handicap scores as well as high levels on the GMFCS and the presence of learning disability, epilepsy, and obvious aetiology of CP. A strong correlation was found between the handicap code and the GMFCS, the strongest concerning the dimension of mobility (r=0.95, p<0.0001). A striking similarity in the grading of disability was present between the ICIDH handicap code and the GMFCS. The GMFCS is considerably less time-consuming and can be evaluated retrospectively. The handicap code requires more detailed information and is more useful for a comprehensive profile of the child.

To test the hypothesis that children with suboptimal fetal growth have significantly poorer mental health outcomes than those with optimal growth, a population random sample survey of children aged 4 to 16 years in Western Australia in 1993 was conducted. The Child Behavior Checklist (Achenbach 1991a) and the Teacher Report Form (Achenbach 1991b) were used to define mental health morbidity. Survey data for 1775 children aged 4 to 13 years were available for linkage with original birth information. The percentage of expected birthweight (PEBW) was used as the measure of fetal growth. Children below the 2nd centile of PEBW who had achieved only 57% to 72% of their expected birthweight given their gestation at delivery were at significant risk of a mental health morbidity (OR 2.9, 95% CI 1.18, 7.12). In addition, they were more likely to be rated as academically impaired (OR 6.0, 95% CI 2.25, 16.06) and to have poor general health (OR 5.1, 95% CI 1.69, 15.52).

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (19 participants) and occipital spikes (three participants). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen 2 to 4 times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike–waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy and in most cases this probably had a direct relation with the paroxysmal EEG activity.

A parental questionnaire was used to investigate the outcome for children who had had ischaemic stroke, who were seen at Great Ormond Street Hospital, London between 1990 and 1996. The results of functional assessments carried out by a physiotherapist and an occupational therapist, and of quantitative evaluations carried out by a neuropsychologist were used for validation where possible. The relationship between clinical and radiological factors and outcome were examined. The children were aged between 3 months and 15 years at the time of stroke (median age 5 years) and the period of follow-up ranged from 3 months to 13 years (median duration 3 years). Of the 90 children for whom data were obtained, 13 (14%) had no residual impairments. Outcome was good in 37 children (40%) and poor in 53 (60%) (defined according to whether impairments interfered with daily life). Agreement, as measured by Cohen's kappa, was good or very good between the parents' responses and the qualitative measures provided by the medical professionals and the therapists, but only fair to moderate for the quantitative measures provided by the neuropsychologists. This may reflect different parental perceptions of the physical and cognitive aspects of outcome. Younger age at time of the stroke was the only significant predictor of adverse outcome.

Visual–perceptual abilities were assessed in 5-year-old children with the following neonatal neurological conditions: born preterm with normal ultrasound scan (NL, n=17); born preterm with ultrasound diagnosis of intraventricular haemorrhage (IVH, n=17); born preterm with ultrasound diagnosis of periventricular leukomalacia (PVL, n=12); born term with hypoxic–ischaemic encephalopathy (HIE, n=11). Visual–perceptual ability was evaluated with the L94: eight visual–perceptual tasks designed to evaluate different aspects of visual perception at the preschool level in children with multiple disabilities. Impairment was established in comparison to the performance age obtained on non-verbal intelligence subtests, instead of chronological age. Frequency of L94 impairment was highest in children with PVL, while children with IVH did not differ from the NL control group. Impairment rates were increased also in children with transient periventricular echodensities, and in children with HIE. Impairments were only moderately related to the delay of visual acuity maturation in infancy.

Respiratory sinus arrhythmia (RSA), a non-invasive indicator of vagal regulation of the heart, and heart period (HP) were monitored before, during, and after oral or gastric-tube bolus feedings in 32 preterm infants. Group 1 infants (n=15) were ≤30 weeks gestational age (GA) at birth (mean 28.3 weeks) and group 2 infants (n=17) were ≥31 weeks GA at birth (mean=33.2 weeks). Mean postmenstrual ages at the time of study were 33.5 ± 2.3 (SD) weeks in group 1 and 33.9 ± 1.6 (SD) weeks in group 2. RSA and HP decreased in both groups during feeding. However, postfeeding RSA and HP increased toward prefeed levels only for group 2 infants. In addition, RSA and HP changes during feeding were correlated only for group 2 infants. The results suggest that the preterm infant may experience a maturational lag in vagal function and in the influence of vagal activity on metabolic mechanisms (i.e. heart rate) related to ingestive needs. This maturational lag may contribute to continued feeding difficulties and may be a measurable marker of subtle neurodevelopmental problems.

The aim of this prospective follow-up study was to evaluate the accuracy of a parent-completed questionnaire compared with professionally detected developmental delay. Parents of 108 very-low-birthweight (VLBW) infants and parents of 279 term control infants completed the German version of the Revised Prescreening Developmental Questionnaire (R-PDQ) at the corrected age of 12 months. Simultaneously, infants underwent developmental examination using the Griffiths Developmental Scale. Sixty-nine VLBW infants were classified as not delayed, 16 as delayed by both methods (conegativity 76% and copositivity 94%), as compared to 240 and six term control infants (conegativity 88%, copositivity 94%). The questionnaire suggested delay in 22 VLBW infants and 32 control infants, which was not substantiated by professional examination (P=0.006). In contrast, examination-diagnosed delay was missed by the questionnaire in one infant in each group. The R-PDQ is a reliable monitoring instrument for both VLBW and term infants at the age of 12 months. Parents of VLBW infants tend to underestimate their infants' development.

This study aimed to analyse the relationship between supratentorial irradiation dose and the intellectual outcome in 36 children (aged between 5 and 15 years) treated for medulloblastoma. The supratentorial radiation dose was reduced to 25Gy in 23 children and given at the standard dose, 35Gy, in 13 other children. Neuropsychological evaluation was performed at a mean of 4.3 years (SD 4.7 years) after radiotherapy. The supratentorial radiation dose was the principal risk factor associated with impaired intellectual outcome. Verbal fluency, immediate word list recall, block design, and fine motricity of the dominant hand were significantly lower in children irradiated at the standard doses than in those irradiated at reduced doses. These findings suggest that the dose of radiotherapy applied to the brain strongly influences later verbal and non-verbal skills in children with medulloblastoma. This should be taken into account in treatment planning and in rehabilitation programs.

Little is known about long-term physical sequelae, cognitive functioning, and quality of life in children who have experienced ischemic stroke. Thirty-seven patients under 16 years of age were studied; the median interval after stroke was 7 years. CT-scans were reassessed to determine the type of infarction at baseline. Occurrences of death, of new cardiovascular events, and of seizures during follow-up were recorded. Surviving patients were invited for a follow-up examination, including physical check-up, global screening of cognition, and an inventory of subjective health perception. Only two patients were lost to follow-up. During follow-up four died, nine developed seizures, eight had transient ischemic attacks, and two experienced a recurrent ischemic stroke. None of the patients had cardiac complications during follow-up. In 11 of 27, no functional impairment was found, in 15 there was a hemiparesis of varying severity, and in one a paraplegia. There was a significant shift in cognitive functioning towards lower levels, especially in children with epilepsy. Remedial teaching was frequently needed. Many of the parents perceived their child's behavior to be very changeable. Three-quarters of the children considered themselves as healthy as other children, and almost all of them as happy. The physical and functional prognosis after ischemic stroke in childhood is relatively good, particularly in children with no serious causative illness, but special education is often needed and social changes occur.

This study aimed to determine the frequency and outcome of possible cerebral herniation in relation to lumbar puncture (LP) in postneonatal infants and children with pyogenic meningitis in the tropics. Children with meningitis aged between 6 weeks and 15 years (mean age 4.07 years; n=123) were recruited consecutively over 3 ½ years at the University of Maiduguri Teaching Hospital, Nigeria. The frequency of possible herniation was determined by clinical evaluation in relation to the severity of illness (high versus low risk) on presentation and performance of LP. Previously described scoring schemes were used. Eighteen (15%) patients had evidence of herniation on presentation. The relative risk (RR) of herniation in high- versus low-risk patients was 66.6 (9.3 to 477.1, 95% CI), p<0.0001 while the RR of death or neurological sequelae in high- versus low-risk patients was 2.6 (1.8 to 3.7, 95% CI), p<0.0001. In 99 patients with known outcomes who had LP on presentation, 21 of 81 without herniation pre- or post-LP, four of four with herniation pre- and post-LP, seven of eight with herniation post-LP only, and five of six with herniation pre-LP only died or recovered with sequelae (χ2 = 25.24, df = 3, p<0.0001). It is concluded that outcome depends on the severity of illness and the presence and timing of herniation. A policy of selective rather routine LPs may improve the outcome in meningitis in developing countries.

Although cerebral palsy (CP) is the most common cause of motor deficiency in young children, it occurs in only 2 to 3 per 1000 live births. In order to monitor prevalence rates, especially within subgroups (birthweight, clinical type), it is necessary to study large populations. A network of CP surveys and registers was formed in 14 centres in eight countries across Europe. Differences in prevalence rates of CP in the centres prior to any work on harmonization of data are reported. The subsequent process to standardize the definition of CP, inclusion/exclusion criteria, classification, and description of children with CP is outlined. The consensus that was reached on these issues will make it possible to monitor trends in CP rate, to provide a framework for collaborative research, and a basis for services planning among European countries.

Somatosensory evoked potentials (SSEPs) are a very sensitive measure of the functional integrity of the neuroaxis, including peripheral and central structures. When used in diagnostic mode they can provide additional information regarding the probable areas of dysfunction. SSEPs were recorded from 44 children (64 feet with congenital talipes equinovarus, CTEV), between the ages of 2 to 15 years, who had structural CTEV deformity previously treated by surgery, with no clinical evidence of neurological deficit. SSEPs were elicited after sequential and bilateral stimulation (0.1ms/5Hz/10 to 20mA) of the posterior tibial nerve and the common peroneal nerve and were recorded cortically (P40). In half the children, additional recordings were conducted at the knee (N5), the first lumbar spinous process (N14), and the seventh cervical spinous process (N20). Eighteen children had abnormal responses, four children had non-reproducible responses, and 22 children had normal responses. Analysis of the data at different levels of the nervous system showed that eight children had abnormality at the spinal level. The surgical outcome was influenced by the neurological abnormality, with an excellent or good outcome in 34 of 36 feet with normal neurology and 19 of 28 feet where a deficit was present (p<0.05). These findings support the neurological theory as an etiological factor in CTEV deformity.

Whether antireflux surgery should be routinely performed at the time of gastrostomy in children with neurological disorders is debatable because of the risk of gastroesophageal reflux. Some argue that these children should be screened for occult gastroesophageal reflux as this will determine the need for fundoplication. This study retrospectively examines outcome in 29 children with neurological disorders who underwent percutaneous endoscopic gastrostomy (PEG) without concomitant fundoplication. Children were included if they had no clinical evidence of severe gastroesophageal reflux before PEG insertion. The median age of children at PEG insertion was 5.6 years (range 1.1 to 18.0). The children were followed for a median of 2.6 years (range 0.4 to 4.9). Insertion of PEG was technically impossible in two children; and an asymptomatic gastrocolic fistula in another child led to subsequent tube removal. Fourteen of the 26 remaining children developed symptomatic gastroesophageal reflux after PEG; five of these showed no reflux on pH monitoring prePEG. Control of symptoms was achieved by medical intervention in 12, but two required fundoplication. Our findings indicate that in the child with neurological disabilities without symptoms indicating severe gastroesophageal reflux, fundoplication is unlikely to be necessary as a consequence of PEG insertion. We conclude that routine investigation for gastroesophageal reflux in the child without severe vomiting can be avoided and the number of antireflux procedures reduced.

A newly established tertiary epilepsy clinic at Guy's Hospital, London was evaluated using a structured questionnaire. Fifty parents of children with epilepsy completed this questionnaire, with some contribution from the children. While a high degree of satisfaction was obtained by the parents and where appropriate the children, specific questions about additional resources implied a large number of unmet needs. This raises methodological issues about the value of open-ended questions in audit questionnaires. In addition, concerns were expressed about many areas, some of which had direct medical implications that were not always raised with the doctors. It is concluded that the service has benefited from both clinical and neuropsychological support. The audit has prompted a number of improvements, including establishing a discussion group for adolescents.

The aim of this study was to estimate the prevalence and severity of feeding and nutritional problems in children with neurological impairment within a defined geographical area. In a cross-sectional study, a validated questionnaire was sent to 377 parents of children (aged 4 to 13 years) on the Oxford Register of Early Childhood Impairments with oromotor dysfunction. The return rate was 72%. Of these, 93% had cerebral palsy; 47% were unable to walk; 78% had speech difficulty; and 28% continuous drooling of saliva. Gastrointestinal problems were commonly encountered: 59% were constipated; 22% had significant problems with vomiting, and 31% had suffered at least one chest infection in the previous 6 months. Feeding problems were prevalent: 89% needed help with feeding and 56% choked with food; 20% of parents described feeding as stressful and unenjoyable. Prolonged feeding times (3h/day) were reported by 28%. Only 8% of participants received caloric supplements and 8% were fed via gastrostomy tube. Even though 38% of respondents considered their child to be underweight, 64% of children had never had their feeding and nutrition assessed. The results highlight that feeding problems in children with neurological impairment are common and severe, causing parental concern. Many of these children would benefit from nutritional assessment and management as part of their overall care.

This study investigated the association between family relationships and compliance in the treatment of childhood epilepsy. It was a prospective study of 21 families with a child who had epilepsy attending Central Middlesex Hospital, London. There were 13 boys, eight girls, with a mean age of 12.0 years (SD 2.9) at initial assessment. Mothers were interviewed for assessment of expressed emotion as a measure of parent–child relationships. Assessment of the mothers' adjustment using the General Health Questionnaire (Goldberg 1978), and psychological adjustment of the children using the Rutter Scales (Rutter et al. 1970a), were completed by mothers and teachers. Reassessment was 3 to 4 years after initial contact, including paediatric case-note review to assess clinic attendance and overall treatment compliance. Significantly more of the group who had a good level of compliance had recovered from epilepsy at follow-up. Good treatment compliance was found to be associated with less maternal hostility and criticism. Children and mothers in the good compliance group had fewer psychiatric symptoms. Poor treatment compliance and the associated psychological disturbances suggest assertive paediatric and psychosocial intervention may be needed for some children with epilepsy.

To evaluate the presence of electrophysiological abnormalities in the visual function of young persons with diabetes, visual evoked potentials were recorded, in basal conditions and after photostress, in 30 patients with newly diagnosed insulin-dependent diabetes mellitus. Their mean age was 17.6 years (3.6 SD), and their glycosated haemoglobin (HbA1c) was 9.4% (1.6 SD). Thirty healthy age- and sex-matched individuals were evaluated as the control group. This study showed that the P100 latency was significantly delayed in patients with diabetes compared with the control group (p<0.01), while the N75 to P100 amplitude was similar in both groups. These measurements were repeated after 6 months, when all participants with diabetes had achieved good metabolic control (HbA1c 7.2% [1.5 SD]). At this second evaluation a complete normalisation of all parameters was observed. These findings suggest that early functional abnormalities of the optic nerve can be detected at onset of diabetes, and that glycaemic control reverses these abnormalities.