To test the hypothesis that characteristics of perinatal infection are associated with long-term cognitive limitations among preterm infants, we analyzed data from 294 infants (142 females, 152 males) [les ]1500g birthweight and <37 completed weeks of gestation who were examined at age 9 years. We identified 47 children (20 females, 27 males) who had a non-verbal Kaufman Assessment Battery for Children (K-ABC) scale standard value below 70, i.e. more than 2 SDs below the age-adjusted mean. The 247 children (122 females, 125 males) with a score [ges ]70 served as control participants. Maternal nationality and education, and low gestational age were significantly associated with a K-ABC non-verbal standard value <70. Both neonatal brain damage (intraventricular hemorrhage) and long-term sequelae (cerebral palsy [CP], diagnosed at age 6 years) were significantly associated with a below-normal non-verbal K-ABC score. Maternal fever at birth was present in five cases (11%) and eight controls (3%; odds ratio 3.6, 95% confidence interval 1.1 to 11.4). Clinical chorioamnionitis and preterm labor and/or premature rupture of membranes (as opposed to toxemia and other initiators of preterm delivery) were also more common among cases than control participants. When adjusting for potential confounders such as gestational age, maternal education and nationality, and CP, the risk estimate for maternal fever remained unchanged (3.8, 0.97 to 14.6). We conclude that perinatal infection might indeed contribute to an increased risk for long-term cognitive deficits in preterm infants.

Epilepsy has a significant impact on a child's life, the extent to which is based on four factors: epilepsy, cognition, behavioral, and physical/neurologic function. This study evaluates the ability of the 44-item Impact of Childhood Neurologic Disability Scale (ICND) to assess each of these four realms. Parents of children (aged 2 to 18 years) with epilepsy rated their child's overall quality of life and completed the ICND. External validation compared the ICND with (1) neurologists' reports of children's behavior, cognitive abilities, physical/neurologic disability, and epilepsy; and (2) parents, teachers, and children's ratings on six ‘criterion standard’ questionnaires. Families of 68 children with epilepsy only and 29 children with ‘epilepsy-plus’ (additional cognitive, behavioral, or physical/neurologic disability; 39 males, 58 females; mean age at testing 10 years 3 months [SD 4.5] age range 2 to 17 years) participated. Internal consistency was excellent (Cronbach's α=0.92) as was test–retest reliability (intraclass correlation=0.89). Caregivers distinguished the impact of each of the four realms. Scores were negatively related to quality of life (Pearson's r=–0.59). Children with high ICND scores had more difficulties at home and school. Their parents saw them as less rewarding and adaptable and the children saw themselves as less intelligent and less popular with more emotional problems. In addition, children with ‘epilepsy-plus’ had significantly higher total ICND scores as well as markedly elevated scores within each of the four realms when compared with the epilepsy-only group. It is concluded that the ICND is an accurate, quick measurement tool reflecting the impact of behavior, cognitive learning ability, physical/neurologic disability, and epilepsy on children and their families.

Hemispatial neglect is commonly observed in adults following right-hemisphere brain lesions. Given the potential for reorganization in the developing brain, spatial neglect may not be apparent following early unilateral damage. This study aimed to determine whether infants who experienced pre- or perinatal focal brain lesions showed evidence of spatial neglect. Study participants were 33 infants/toddlers (22 males, 11 females; age range 6 to 48 months); 27 preschool children (14 males, 13 females; age range 28 to 75 months) with either left hemisphere (LH) or right hemisphere (RH) damage of pre- or perinatal onset (total 60), and 36 control individuals (15 females, 21 males; age range 28 to 75 months). Participants were assessed using two object-removal preference tasks. Control children showed no lateralized preference for object removal. Those with LH or RH damage preferentially removed objects from the side of the board ipsilateral to the lesion first and the contralateral side last. These results suggest that spatial neglect may be found in young children even after very early unilateral brain damage. Further, in contrast to the adult pattern, contralateral neglect is present for up to 6 years after either LH or RH damage. This pattern suggests that there may be a different distribution of attention to space in the developing brain as compared with the mature brain. The persistence of spatial neglect suggests that there are some limitations on plasticity in the developing human brain.

Lesch–Nyhan disease (LND) is a rare X-linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self-mutilation. This study examines data gathered from a survey of 64 families in the USA and abroad regarding the self-injury of their family members who have LND. The individuals with LND ranged in age from 1 to 40 years (mean 16 years 7 months, SD 11 years 2 months) and, with the exception of one, were males. The most common initial mode of self-mutilation, and the most frequently cited past or current behavior, was biting of lips and/or fingers. Other behaviors, in order of frequency, included head banging, extension of arms when being wheeled through doorways, tipping of wheelchairs, eye-poking, fingers in wheelchair spokes, and rubbing behaviors. Hierarchical cluster analysis identified patterns of association among the types of self-mutilation. Modes of self-mutilation in which external surfaces (such as a wheelchair component) served as instruments of self-injury tended to co-occur, as did biting of lips and fingers.

In contrast to physical characteristics, the developmental and behavioural characteristics of children with Sotos syndrome are not well documented. Data from a survey of 27 children (17 males and 10 females; mean age 10 years 7 months; range 6 to 15 years) with mild and moderate learning disabilities were obtained concerning school placement, social networks, adaptive competence, and behavioural problems and compared with a control group matched for cognitive level and age. Ten children attended a mainstream school (with remedial assistance), eight were placed in a school for children with learning disabilities, nine children attended other special schools. Social contact problems and anxious behaviour were reported as the most prominent characteristics of children with Sotos syndrome. Some implications for psychological counselling and educational support are discussed.

A sample of 12 children (seven males, five females; mean age 6 years 7 months, SD 2 years 6 months, range 2 years 4 months to 12 years 1 month) with moderate-to-severe foetal alcohol syndrome (FAS) and another sample of 26 children (12 males, 14 females; mean age of 6 years 2 months SD 2 years 10 months, range 2 years 6 months to 12 years 8 months)with mild FAS or foetal alcohol effects (FAE) as well as a sample of 15 age- and sex-matched control children with unspecific intellectual disability were compared using the Developmental Behaviour Checklist (DBC). There were significant differences (p=0.01) between the groups on five of six subscales of the DBC with controls scoring lower on the disruptive, self-absorbed, anxiety, antisocial behaviour, and communication disturbance scales. The DBC profiles of the two foetal alcohol exposed groups did not differ from each other. It is concluded that quantitative behaviour measurement provides insights into specific behavioural phenotypes of FAS/FAE.

This was a qualitative research study of parental perceptions of gastrostomy feeding before surgery using an in-depth interview in the parental home to examine the factors parents consider when gastrostomy feeding is recommended and to identify the need for support. Participants were a subgroup of families taking part in a larger research study evaluating gastrostomy placement in children with severe neurodevelopmental disabilities at a major paediatric centre in the UK between 1998 and 2000. Parents of 22 children (13 males, nine females; mean age 4 years 8 months [SD 3 years 6 months]; age range 1 year 1 month to 13 years 3 months) were interviewed. Categories of disability were: cerebral palsy (n=10), a syndrome of chromosomal or genetic origin (n=10), and unconfirmed diagnoses (n=2). Thirteen children were recommended for a gastrostomy and seven were recommended for a gastrostomy with an antireflux procedure. Four families were undecided about surgery at the time of the interview, two of whom went ahead with the procedure some time later. Parental accounts of oral and tube feeding were both contradictory and ambivalent. Concerns about the loss of oral feeding, which was regarded as having a range of psychosocial effects for the child and family, were raised. Both oral and tube feeding have multiple meanings for parents and signify more than obtaining an adequate nutritional intake. The need for additional information about the risks and benefits of gastrostomy and how tube feeding fits into the context of everyday life were dominant themes. The biomedical emphasis on health and weight-gains may fail to reflect parental concerns about tube and oral feeding. There is a need for greater practical and emotional support for families feeding children with severe disabilities.

The ‘Uptimer’ is a custom-made lightweight battery-operated remote activity monitor that records the amount of time an individual spends in the upright position, which is also known as ‘uptime’. The aims of this study were to determine levels of uptime over 24 hours and the relation between uptime and a child's age, sex, height, weight, and body mass index (BMI). Uptime was recorded in 529 normally developing children (318 females, 211 males), aged between 8 and 15 years. All children wore an Uptimer continuously for a 24-hour period that included a typical school day. Mean uptime for children in this study was 5.4 hours (SD 1.3; range 1.5 to 10.3 hours), over a 24-hour period. Uptime followed approximately a normal distribution in this population and did not have a linear relation to age, height, weight, nor BMI. Results of this study may be used as a normative database for the evaluation of uptime in children with physical disabilities.

The aim of this study was to describe child health and well-being across the spectrum of cerebral palsy (CP) and to report on psychometric properties of the Child Health Questionnaire (CHQ) in this population. Parents of 80 individuals (45 males, 35 females; mean age 11 years 4 months, SD 3 years 6 months; range 5 to 18 years) attending a CP clinic in Victoria, Australia were surveyed. Measures included the CHQ PF-50, a 50-item parent-reported heath status measure; severity of CP, based on the Gross Motor Function Classification System; and cognitive status and presence of epilepsy. CHQ data were compared with normative data collected in Victoria two years earlier. The CHQ demonstrated good psychometric properties for children with CP. Children with CP had markedly poorer health on every CHQ scale than those in the normative sample. Health status did not vary by cognitive status or epilepsy. Children with severe CP had the poorest physical health, but psychosocial health and emotional impact on parents were similar for mild and severe CP showing that these should not be assumed to be less prevalent when CP is mild.

The purpose of this study was to evaluate the effects of botulinum toxin A (BTX-A, Botox) dilution volume and post-injection exercise with electrical stimulation on muscle paralysis. We injected 10 units of BTX-A diluted with 0.1 ml (B1, n=8) or 0.5 ml (B5, n=8) normal saline into both gastrocnemius muscles of 16 New Zealand white rabbits; two controls received no BTX-A. After BTX-A injection, all rabbits received calf muscle stretching exercise and electrical stimulation for 2 hours on the left leg. The compound muscle action potential (CMAP) decrease was most pronounced at 1 week and progressive recovery was observed (i.e. recovery from paralysis, increase of CMAP). There was a significant decrease of CMAP amplitudes in the B5 group compared with the B1 group at week 1 and week 4 (p<0.001). Left limbs with stretching exercise and electrical stimulation showed lower CMAP amplitudes compared with control right limbs of all rabbits. To maximize the muscle paralysis effect of BTX-A, increasing dilution volume and performing post-injection stretching exercise with electrical stimulation may be a promising strategy for increasing the beneficial effect of BTX-A treatment. Future studies are needed to investigate the clinical application of this finding.

We describe the coexistence of Asperger and Tourette syndromes (AS and TS) caused by discrete hypoxic–ischaemic necrosis of the midbrain, infrathalamic and thalamic nuclei, and striatum in an adolescent male with positive family history for tics and obsessive–compulsive disorder. Behavioural ratings, cognitive tests, and volumetric measurements of the basal ganglia were performed in the patient and five other individuals with AS–TS unassociated with MRI lesions. Cognitive deficits in attentional, executive, and visual–spatial domains were found both in the patient and control AS–TS group, though deficits were more severe in the former. MRI showed reduction of the left basal ganglia volume compared with the right in the patient, whereas the control group showed reduction of right basal ganglia volume compared with the left. It is suggested that individuals with a genetic predisposition to TS may develop AS and TS after involvement of midbrain and related components of basal ganglia-thalamocortical circuits normally implicated in the integration of emotional, cognitive, and motor functions.

Any good paediatrician will point out to the parents of a child with cerebral palsy (CP) that their child's brain damage is fixed and cannot be altered. This is not being over pessimistic but simply honest. The paediatrician then suggests a variety of treatments/therapies aimed at maximizing the child's potential. However, it is understandable if parents still go on to seek treatments which they hope can alter their child's condition. Such treatments include conductive education, cranial osteopathy, and hyperbaric oxygen (HBO) – to name a few. This article seeks to review available information on the use of HBO in the treatment of children with CP.