The aim of this study was to analyse the refractive state of four different groups of children: those with spastic cerebral palsy (CP), aged between 7 and 81 months(N=50); psychomotor retardation, aged between 19 and 70 months (N=16); other neuromotor dysfunctions, aged between 12 and 75 months (N=37); and without psychomotor retardation, aged between 9 and 73 months (N=181). Refractive errors were determined using cycloplegic retinoscopy and non-cycloplegic retinoscopy (Mohindra's technique). We found higher percentages of hyperopia, tendency toward hyperopia, and other refractive anomalies in all the pathological groups of children than in the non-pathological control groups. Children from both the non-CP pathological control group and the group with psychomotor retardation had similar or even higher levels of hyperopia than children from the group with spastic CP. Our results in different age groups indicate a less effective normal emmetropization course in all the pathological groups of children studied. The correction of refractive errors is needed in these children before the end of the neural plasticity period.

This study examines the prevalence of cerebral palsy (CP) in two north-east Italian provinces, Padua and Rovigo (overall population 1030000). Six-hundred and ten children with a diagnosis of CP (330 male, 280 female), born between 1965 and 1989, were studied. The prevalence of CP progressively increased from the 1960s to the mid-1980s, and then decreased in the 5-year period, 1985 to 1989. These quantitative changes were associated with qualitative ones. For example, the number of low-birthweight (LBW) infants progressively increased, similar to results of epidemiological studies from other European countries. The clinical features of the types of CP in this study (hemiplegia, diplegia, ataxic diplegia, quadriplegia, pure ataxia, dyskinesia) generally correspond with those described in other studies. However, the prevalence of quadriplegia in this study is higher. The prevalence of types of CP related to preterm birth, such as diplegia, increased over the years, while those associated with term babies, such as dyskinesia, decreased. This study suggests that prenatal factors are associated with some types of CP, while in others, such as diplegia, quadriplegia, and dyskinesia, the perinatal factors are notable. Perinatal factors were associated with LBW children, while prenatal factors were greater for normal-birthweight infants. The variation in the prevalence of CP over the years, with two peaks corresponding with the introduction of neonatal intensive care units in Padua and Rovigo, suggests that changes in neonatal care could influence the levels of CP, independent of the original presence of predisposing prenatal factors.

The present study examines the coordination of fingertip forces during object manipulation in both the involved and non-involved hand of 14 children with hemiplegic cerebral palsy (CP) aged between 8 and 14 years. While no impairment could be observed in the non-involved hand, subtle deficits in the sequencing of the grip–lift movement were observed in all children tested in this hand. However, they were able to scale the fingertip force output of the non-involved hand in advance (use anticipatory control) based on the object's weight. In the second experiment in this paper, we tested whether the anticipatory control can be generalized across hands. The results indicate that sensory information from the non-involved hand can be used for anticipatory scaling of isometric force increase during subsequent lifts with the contralateral involved hand. These findings suggest that the initial lack of anticipatory control usually observed in the involved hand of children with hemiplegic CP is likely to be based on disturbed sensory input.

It was hypothesized that children clinically identified with sensory-modulation disruptions (SMD) would have atypical physiological responses to sensation, and that such responses would predict parent-reported behavioral responses to sensation. Nineteen children with clinically identified disruptions, aged 3 to 9 years, mean 6.0 years, and 19 age- and sex-matched healthy (control) children, aged 3 to 9 years, mean 6.6 years, were examined. The subjects were presented with five stimuli. Ten trials were conducted for each stimulus and the electrodermal activity of the child was recorded. Four children with SMD did not show electrodermal responses (EDR) to stimulation; all control children responded. Excluding non-responders, children with SMD showed more and larger EDR than control children. Participants with disruptions habituated more slowly to repeated stimulation, as measured by the number of responses to stimuli and proportion of stimuli that evoked responses. Children with atypical EDR had more parent-reported abnormal behavioral responses to sensation. Children with clinically identified SMD respond physiologically differently to sensory stimuli than typically developing children; these differences have ramifications for functional behavior.

This study sought to determine whether neonatal cranial ultrasound abnormalities are related to motor and cognitive outcomes in low-birthweight (LBW, <2000 g) children without major motor or cognitive disability. The relation of neonatal cranial ultrasound abnormalities to motor performance and general cognitive ability at ages 2, 6, and 9 years was examined in a prospectively followed regional cohort of LBW children, excluding those with major disability, and controlling for other risk factors. Compared with children without ultrasound abnormalities, children with abnormalities indicative of ischemic white-matter injury had poorer motor performance at all three ages and, at age 2, lower general cognitive ability. The latter finding was not seen when motor performance was also controlled for. It was concluded that in LBW children without major motor or cognitive disability, ischemic white-matter injury indicated by neonatal cranial ultrasound abnormalities adversely affected motor performance at ages 2, 6, and 9 years, but not general cognitive ability.

The aim of this study was to compare the linear growth of children with cerebral palsy (CP) with that of children without CP. The segmental lengths (humerus, ulna, femur, tibia, and spine), recumbent length, body weight, and bone age of 62 children with CP (age range 2.25 to 14 years, mean 7.13 years) were measured and compared with 68 children without CP (age range 1.50 to 12.67 years, mean 6.73 years). The results show that bone-age delay is common in children with CP (68% with a delay of more than 1 year). The linear growth of children with CP is similar to that of children without CP when bone age is used instead of chronological age. There is a strong correlation between segmental lengths and body height. Hence, this study favours using segmental lengths for estimating height in children with CP and using the comparison group as a reference for this purpose. Nutritional and non-nutritional factors on bone-age delay are discussed.

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.

Eighteen adolescents who had survived Reye syndrome (RS) in early childhood were assessed on cognitive, emotional, and behavioural variables in a second follow-up study tracking this group. Siblings were used as controls. The entire group with RS had survived with no obvious neurological damage at the first follow-up study. Indeed, current findings suggested that long-term cognitive, emotional, and behavioural functioning was comparable to siblings in approximately half of the group with RS. However, two factors were associated with a less favourable outcome. Cognitive, emotional, and behavioural functioning were significantly poorer in the subgroup of survivors whose illness had occurred in the first year of life. In addition, loss of consciousness, although the association with poor outcome was not as noticeable, was also associated with relative deficits on some scales of cognitive ability. Many of these deficits had not been obvious at the first follow-up and the importance of neurodevelopmental factors are considered. Finally, the implications of these findings for research and interventions in RS and other such encephalopathies are discussed.

The repeatability of energy-expenditure measurements were studied in five children and four adults without disabilities using the Cosmed K4 (high technology). The ability to detect change in measurements was compared between this instrument and the Physiological Cost Index (PCI; low technology). The results of repeatability (95% range) for oxygen cost were 13.1% in children and 13% in adults. In contrast, the SD of PCI was 6 to 72% of the mean in adults and wider in children (91%; 95% range). The validity of PCI as an outcome measure was questioned. In addition, 177 children with motor disability were prospectively studied using the Cosmed K4. Previous experience with the Cosmed K2 (intermediate technology) helped to develop a practical and repeatable protocol for testing children with disability using the Cosmed K4. The protocol commenced with 5 minutes of rest to achieve baseline values of heart rate and oxygen consumption, followed by 10 minutes of continuous walking at a self-selected speed on a 10-metre level oval walking track. The test concluded with 5 minutes of rest to monitor the return to baseline values. Ninety-one percent of the children with disability quickly reached a steady-state of oxygen consumption and carbon-dioxide production. The carbon-dioxide sensor in the Cosmed K4 has enabled a new group of severely involved children with cerebral palsy (9%) to be defined. These children have been termed ‘physiologically marginal ambulators’.

This study aimed to investigate the validity of disintegrative psychosis (DP) as defined in the ICD-9. The history of epilepsy in 13 subjects with DP was compared with that of 39 subjects with infantile autism (IA) who were matched for sex, age, IQ, and socioeconomic status (SES). The average follow-up time was 22 and 23 years (range 11 to 33 years). A significant difference was found between the DP and IA groups in terms of incidence of epilepsy, 77% versus 33% respectively. The peak period of onset of epilepsy occurred before puberty in both groups. Different types of epilepsy were seen, but the psychomotor variant accounted for 50% in the DP group, while 46% of the IA group had the psychomotor and 62% had the grand mal variant. The types are not mutually exclusive. Individuals without epilepsy had significantly higher IQ scores than those with epilepsy, but only within the IA group. The increased risk of developing epilepsy in the DP group is most likely a reflection of an underlying early brain pathology probably present in most individuals with DP. On the whole our findings can be seen as a contribution to the validation of DP as separate from IA, as these two conditions could be distinguished in terms of the way they develop with reference to epilepsy.

The common synaptic drive shared between two groups of motor neurones synchronizes the timing of discharges between the motor-neurone groups. Recordings were made of motor-unit discharges during cocontraction of ipsilateral pairs of thumb muscles in eight subjects with cerebral palsy (CP) aged 4 to 13 years and eight neurologically healthy subjects aged 4 to 12 years, and in pairs of lower-limb muscles in 21 subjects with CP and 21 control subjects, both aged 3 to 15 years. Common synaptic drive, likely to be derived at least partly from activity in branched corticospinal-tract neurones, produced motor-unit synchronization between pairs of thumb muscles in control subjects but was absent in all subjects with CP. Motor unit synchronization was not found between lower-limb antagonist muscles that cocontract abnormally in CP, nor was synchronization present in more widely separated muscle pairs. Therefore, abnormal patterns of muscle activation and more widespread muscle reflex responses do not result from an abnormal distribution of common synaptic drive in CP.

Developmental coordination disorder (DCD) occurs in a small but significant proportion of children who present with impaired body–eye coordination and show poor acquisition of motor skills. This study investigated the visual–proprioceptive mapping ability of children with DCD from a small selected group, with particular reference to the use of vision in matching tasks. The children with DCD in this study were significantly poorer than control children on all matching tasks. They seemed to have particular difficulty in cross-modal judgements that required the use of visual information to guide proprioceptive judgements of limb position. A distinction is drawn between tasks that can be achieved purely through sensory matching and those that require body-centred spatial judgements, suggesting that it is the latter that posits a particular difficulty for children with DCD.

Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.

Current and comprehensive information about the medical issues affecting children with Down syndrome (DS) is of value in counselling parents who are considering prenatal diagnosis and in planning services for people with DS as they age, especially given the continued improvements in their survival. Parents of school-aged children (mean age 11.37 years, 57.3% male, 42.7% female) with DS were identified by linking registers from the Disability Services Commission and the Birth Defects Registry. Less than half the children had cardiac and bowel conditions. More than half had ear conditions and more than three quarters had eye conditions. Ear, nose, and throat professionals were the specialists seen most often and the rate of tympanostomy tube insertion was nearly 17 times that of the general childhood population. Children with DS were over five times more likely to wear glasses than other children. These findings suggest that chronic, non life-threatening conditions impose a burden on families but do not threaten quality of life.

This study compares bone mass in a national sample of girls with Rett syndrome (RS) with a sample of control children. The Australian RS Database was the source of cases for this population-based study. Hand radiographs were available from 101 of 137 subjects (74% of the known Australian population of girls with RS aged [les ]20 years). Control radiographs matched for age, sex, and laterality were obtained from hospital radiology departments. A measure of cortical thickness was made from the difference between the outer diameter and the medullary space in the second metacarpal bone. A mean z-score value for cortical thickness and percentage cortical area for each individual was calculated. The mean cortical thickness (z score) for girls with RS was −1.94 compared with −0.38 for control children (P<0.001). In girls with RS, the mean cortical thickness decreased with age (P<0.001). In girls who were taking epilepsy medication it was −2.21 compared with −1.23 in those not taking epilepsy medication (P<0.001). There was no evidence of a beneficial effect of increased calcium intake on cortical thickness. A similar pattern was obtained when percentage cortical area was estimated. In multivariate analysis, increasing age and use of anticonvulsant medication were associated with decreased cortical thickness and only use of anticonvulsant medication with decreased percentage cortical area. Fractures had occurred in one-third of cases and it was estimated that just over 40% of girls would sustain a fracture by the age of 15 years. Girls with RS may be at increased risk of fractures and their bone quality compromised as determined by cortical thickness and percentage cortical area measurements from the second metacarpal.

A 4-year follow-up study of academic achievement in children aged between 11 and 17 years with epilepsy or asthma was carried out to identify differences between the two samples and to identify change in achievement over time. Differences based on sex and seizure severity also were explored. There were 98 subjects in the group with epilepsy and 96 subjects in the group with asthma. Academic achievement in five areas (Composite, Reading, Mathematics, Language, and Vocabulary) was measured using school-administered group test scores. To explore change over time in condition severity, each child was categorized as having a low or high condition severity at baseline (time I) and again 4 years later, resulting in four groups: low–low, low–high, high–low, and high–high. There were too few cases in the low–high group to be included in the analyses. Data were processed using analysis of covariance (ANCOVA), intraclass correlation coefficients, and paired t tests. At follow-up the children with epilepsy continued to perform significantly worse in all five achievement areas than the children with asthma. Children with either inactive or low-severity epilepsy had mean scores comparable to national norms; those with high seizure severity had mean scores ranging from 3 to 5 points below national norms. No changes were found in academic achievement over time for either sample, even among those whose conditions improved. Although boys with high-severity epilepsy continued to have the lowest achievement scores, there was no trend for them to decline in achievement over time.

The reliability and responsiveness of the Barry–Albright Dystonia (BAD) Scale, a 5-point ordinal severity scale for secondary dystonia, was assessed. For interrater reliability, 13 raters scored 10 videotaped patients; for intrarater reliability, two raters rated the videotape again. For test–retest reliability, patients were rated on two occasions. Four inexperienced raters scored patients, received training, then scored additional patients. To assess responsiveness, we compared patient and physician global ratings of change (better, same, and worse) with BAD Scale score changes for 18 patients on intrathecal baclofen (ITB) trials. We assessed reliability with the intraclass correlation coefficient (ICC). The mean ICC for total BAD Scale scores were as follows: interrater reliability 0.866, intrarater reliability 0.967 and 0.978, test–retest reliability 0.978 (before training) and 0.967 (after training). We found the BAD Scale responsive to change, with most improved scores in patients rated by the patient, family, and neurosurgeon as ‘better’. The total scores were reliable for experienced raters. We recommend training for clinicians interested in using the scale.

Forty-seven children with non-organic failure to thrive (NOFT) were identified from a whole-population survey of children's growth and development. A significant proportion (N=17) of these 47 children were found to have oral-motor dysfunction (OMD) identified using a previously validated assessment tool. NOFT children with OMD and those with normal oral-motor function (N=30) were compared in order to ascertain whether there were any neurodevelopmental differences which might explain this finding. We hypothesized that children with OMD might have a subtle neurodevelopmental disorder. Few psychosocial variables discriminated the two groups. However, cognitive stimulation within the home and cognitive-growth fostering during mealtimes was much poorer for children with OMD. Some evidence has suggested that NOFT children with OMD may be ‘biologically’ more vulnerable from birth. We suggest that the continued use of the term ‘non-organic’ to describe failure to thrive in such children is questionable and requires redefining.

As part of a wider survey of parents of children with autistic spectrum disorders in the UK, the diagnostic experiences of 614 parents of children with autism and 156 with Asperger syndrome were compared. Although the ages of the children in the two groups were very similar at the time of the survey, parents of children given a diagnosis of Asperger syndrome had experienced significantly longer delays and greater frustration in obtaining a diagnosis than those with a child with autism. In the ‘autism group’ the average age when diagnosis was confirmed was around 5.5 years; in the ‘Asperger group’ it was 11 years. Parents of children with a diagnosis of autism were generally aware of problems in their child's development by 18 months of age; in the Asperger group concerns emerged later, at around 30 months of age. Initial worries in both groups centred around abnormal social development but parents of children with Asperger syndrome were less likely to have noted communication problems. Stereotyped or repetitive behaviours were not prominent in the early years in either group. Despite the problems inherent in data collected by postal survey, many of the findings of this study are supported by other research. The practical implications of delayed diagnosis, especially in the case of more able children with Asperger syndrome are discussed.

As infants with bronchopulmonary dysplasia (BPD) have difficulty maintaining adequate levels of oxygenation during rest, it was decided to investigate how the additional respiratory demands associated with nutritive feeding disrupt their breathing rates. The sucking and breathing patterns of six (three male, three female) preterm infants (between 23 and 29 weeks gestational age at birth), classified as having BPD were individually compared with the patterns observed in 12 (six male, six female) healthy term (control) infants ([ges ]38 weeks gestational age at birth) with no known respiratory ailments. All infants were recruited from the neonatal unit at Simpson's Maternity Pavilion, Edinburgh, Scotland. In general, the breathing patterns recorded for the infants with BPD during the pause periods of intermittent feeding lacked the striking regularity observed in the term infants. It was found that the severity of the BPD affected breathing rates by significantly reducing the duration and the regularity of a breath (P<0.05) while sucking during the intermittent phase of feeding.