Management of a painful or contracted hip dislocation in individuals with severe spastic quadriplegia is difficult. Clinical and radiographic results of 12 proximal femoral resection–interposition operations performed in seven non-ambulatory persons (five males, two females; mean age 14 years, 8 months; age range 6 years 11 months to 19 years 8 months) with severe spasticity were reviewed to determine if pain relief and restoration of motion were maintained. At a mean follow-up of 7 years 7 months (median 9 years 6 months) all participants maintained a good sitting position and a functional range of motion with improved hygiene. Hip pain was improved in all participants compared with their preoperative status. Proximal femur migration occurred causing slight pain in one person. Heterotopic ossification was observed but was not clinically significant. Complications included traction pin loosening and infection and a late supracondylar femur fracture 3 months after the operation. Proximal femoral resection effectively decreased pain and restored hip motion in those with severe spastic quadriplegia leading to improved sitting and perineal care.

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.

This randomized clinical trial evaluated the effects of a home-based, six-week strength-training programme on lower limb strength and physical activity of 21 young people (11 females, 10 males; mean age 13 years 1 month, SD 3 years 1 month; range 8 to 18 years) with spastic diplegic cerebral palsy (CP) with independent ambulation, with or without gait aids; (Gross Motor Function Classification System levels I to III). Compared with the 10 controls, the 11 participants in the strength-training programme increased their lower limb strength (combined ankle plantarflexor and knee extensor strength as measured by a hand-held dynamometer) at 6 weeks (F(1,19)=4.58, p=0.046) and at a follow-up 12 weeks later (F(1,18)=6.25, p=0.041). At 6 weeks, trends were also evident for improved scores in Gross Motor Function Measure dimensions D and E for standing, running and jumping, and faster stair climbing. A relatively short clinically feasible home-based training programme can lead to lasting changes in the strength of key lower-limb muscles that may impact on the daily function of young people with CP.

Cervical accelerometry with digital signal processing (DSP) can identify signals that are consistently associated with swallowing during feeding of infants. It is shown that these signals, called initial discrete sounds (IDSs), become more uniform with advancing postmenstrual age (PMA) in healthy preterm infants. The objectives of this study were to determine if cervical accelerometry and DSP at a higher sample rate than previously used (22kHz versus 16kHz), in conjunction with a DSP software package that allows manipulation of the sound files, would improve the accuracy of the previously developed variance index (VI) method of analyzing accelerometric information. The modified VI method was then used to measure developmental differences in the IDS morphology of infants with and without bronchopulmonary dysplasia (BPD). VIs were calculated for 24 feeding studies of infants of between 32 and 39 weeks PMA: 12 studies on healthy preterm infants (n=10: three males, seven females; mean gestational age [GA] 28.6 weeks, SD 0.4; mean birthweight [BW] 1080g, SD 82; PMA mean 35.2 weeks, SD 0.6) and 12 studies on infants with BPD (n=7: five males, two females; GA 27.1 weeks, SD 0.4; BW 911g, SD 71; PMA 36.2 weeks, SD 0.6). There was a significant inverse correlation between VI and PMA for the healthy preterm group (r=0.66, m=–2.31/week, p=0.02). There was no significant correlation between VI and PMA for the BPD cohort. The VI of infants with BPD was significantly different from that of infants without BPD (p<0.007, multiple regression analysis, interaction PMA×Group). Additionally, using our modified VI technique, 100% of swallows were found to have the expected IDS signals.

The 1999 British Child and Adolescent Mental Health Survey, a nationwide epidemiological study of rates of psychiatric disorder in children aged 5 to 15 years, provided the opportunity to investigate the mental health of children with epilepsy. These children and their families experience disability specifically because of additional emotional, behavioural, and relationship problems, and this is the first epidemiological study that directly measures these impairments. Information was obtained by interviewing a main carer and teacher for 10316 children; 67 children with epilepsy were identified (35 males, 32 females; mean age 10 years 2 months, SD 2 years 11 months, range 5 to 15 years), and compared with the 47 children with diabetes (27 females, 20 males; mean age 10 years 4 months, SD 3 years 4 months, range 5 to 15 years) and 10202 controls (50% male; mean age 9 years 11 months, SD 3 years 1 month, range 5 to 15 years). DSM-IV psychiatric diagnoses were derived from the Development and Well-Being Assessment in combination with the interview and a specialist clinician rating. Parental reports of emotional and behavioural problems, their impact, and associated peer problems were also obtained. Rates of psychiatric disorder were 37% (95% confidence interval [CI] 22 to 49) in epilepsy, 11% (95% CI 2 to 19%) in diabetes, and 9% (95% CI 9 to 10%) in control children. Parents of children with epilepsy consistently reported more problems, with greater impact and associated peer problems. Epilepsy, but not diabetes, was independently (adjusted for age, sex, and severe learning difficulties) associated with all behavioural variables in regression analyses. Emotional, behavioural, and relationship difficulties are common in children with epilepsy, and constitute a significant burden to the children and their families, indicating the need for effective mental health services for these children.

Precision grip and concomitant anticipatory postural adjustments were investigated in 11 children (three females, eight males; mean age 9 years 1 month, SD 11 months) with attention-deficit–hyperactivity disorder (ADHD); 12 children (three females, nine males; mean age 9 years, SD 7 months) with developmental coordination disorder (DCD), and 13 children (two females, 11 males; mean age 9 years 9 months, SD 11 months) with a combination of ADHD and DCD (ADHD+). There were two comparison groups: an age-matched group (four females, 11 males; mean age 9 years 1 month, SD 14 months) and a younger age group (five females, six males; mean age 6 years 5 months, SD 8 months). Adaptation to different weights was evaluated by lifting a specialized grip instrument monitoring grip force, load force, and centre of foot pressure displacements. Children with ADHD+ showed: (1) excessive grip forces, (2) decreased amplitude and prolonged onset of postural adjustments, and (3) reduced ability to adapt the motor output. Children with ADHD and DCD did not scale manual and postural forces in amplitude and time domains. Children with DCD also differed in delayed timing of postural adjustments. Results indicate that children with ADHD and DCD show a spectrum of neural dysfunctions underlying poor motor coordination, which are not specific to the clinical disorder.

The aims of this retrospective and population-based study were to describe the frequency and characteristics of epilepsy in 146 children (82 males and 64 females) with cerebral palsy (CP) born from 1987 to 1994 in the Göteborg area of Sweden. The frequency of epilepsy was found to be 38% (55 children). All children with tetraplegic CP and about one-third of the children with other CP types developed epilepsy. Age at onset of epilepsy varied with type of CP: children with tetraplegic CP tended to have an earlier onset of epilepsy than children with other CP types. Partial seizures were the most common seizure type; all children with hemiplegic CP had partial seizures. Children with cognitive impairment had a higher frequency of epilepsy than those without cognitive impairment. CP aetiology may predict the development and outcome of epilepsy, as children with CP caused by CNS malformation, CNS infection, and grey matter damage all showed a higher frequency of epilepsy than children with CP of other aetiology, and also had less chance of becoming seizure-free.

The spectrum of language regression in childhood is incompletely understood. To describe the features of this disorder more fully, we reviewed the records of 196 consecutive children (143 males and 53 females) with language regression or perceived plateau evaluated between 1988 and 1994 by a child neurologist. Mean age at regression was 21.2 months and the mean interval to referral was 34.8 months. A trigger for the regression was identified in 74 of the children (38%) and was associated with a more rapid regression. Mean age at follow-up was 64 months (SD 55). Seventy per cent of the children became nonverbal, and 75% were cognitively impaired. Language regression was associated with a more global autistic regression in 93% of children. There was a history of seizures in 15% of the children. Some recovery occurred in 61% but only one child recovered fully. Improvement was more likely in the 49% who were entirely developmentally normal before the regression. We conclude that language regression in childhood is a serious disorder with significant long-term morbidity.

The neonatal predictors of microcephaly, defined as a head circumference <5th centile in children born preterm, has not been systematically assessed. Children were drawn from the Developmental Epidemiology Network (DEN) cohort of very low-birthweight children (VLBW: 500–1500g) born from 1991 to 1993 at three sites in the USA. Neurological assessments were carried out among 198 singleton children (mean age 6 years 8 months, SD 0.5 years). Ninety-six children (48.5%) were male. Microcephaly was observed in 30 children (15%) and, using multivariate analysis, it was found to be associated with gestational age <26 weeks and bronchopulmonary dysplasia (BPD). Sonography-defined white-matter damage (WMD, i.e. echolucency or echodensities) was not associated with increased odds of microcephaly, while occurrence of intraventricular hemorrhage (IVH) was in univariate but not multivariate analysis. In analyses that excluded children with IVH/WMD, odds of microcephaly increased in dose-related fashion according to number of days on ventilator: >5 days, OR=4.5; 95%CI=1.4 to 15; >10 days, OR=5.7; 95%CI=1.7 to 19; >15 days OR=8.3; 95% CI=2.3 to 29.2. Among children without BPD, microcephaly was not associated with differences in IQ, while IQ scores among children with BPD or any ventilation were disproportionately lower among those with microcephaly. In multivariate analyses predicting IQ at age 7 years, microcephaly was found to modify the association between neonatal lung disease and IQ.

Ischaemic stroke subtypes in children and adults were compared to determine the similarity in aetiologies. Thirty-six children (22 females, 14 males; median age 5 years 7 months, range 6 weeks to 15 years 10 months) and 50 adults (35 males, 15 females; median age 44 years, range 17 years 2 months to 49 years 11 months) who had presented with ischaemic stroke between 1995 and 2000, were categorized using a modified version of the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification. Proportions of patients in the subtypes of the TOAST classification system were significantly different in the two groups (χ2 test, p<0.01). The first three subtypes (large artery atherosclerosis, cardioembolic, and small vessel disease) accounted for the majority of adult strokes (27 of 50). In contrast, only three of 36 children were accounted for within these three subtypes. The majority of children (29 of 36) were classified within the ‘other determined aetiology’ subtype. Aetiology was undetermined in 12 of 50 adults compared with three of 36 children. Causes of ischaemic stroke in children and adults are distinct. A classification system for ischaemic stroke in children would be useful for collaborative studies.

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.

The aim of this study was to establish the reliability and validity of visual gait assessment in children with spastic diplegia, who were community or household ambulators, using a modified version of the Physicians Rating Scale, known as the Observational Gait Scale (OGS). Two clinicians viewed edited split-screen video recordings of 20 children/adolescents (11 males, 9 females; mean age 12 years, range 6 to 21 years) made at the time of three-dimensional gait analysis (3-DGA). Walking ability in each child was scored at initial assessment and reassessed from the same videos three months later using the first seven sections of the OGS. Validity of the OGS score was determined by comparison with 3-DGA. The OGS was found to have acceptable interrater and intrarater reliability for knee and foot position in mid-stance, initial foot contact, and heel rise with weighted kappas (wk) ranging from 0.53 to 0.91 (intrarater) and 0.43 to 0.86 (interrater). Comparison with 3-DGA suggests that these sections might also have high validity(wk range 0.38–0.94). Base of support and hind foot position had lower interrater and intrarater reliabilities (wk 0.29 to 0.71 and wk 0.30 to 0.78 respectively) and were not easily validated by 3-DGA.

A detailed study was made of the pathology of specimens removed by hemispherectomy for the treatment of intractable epilepsy in children with unilateral middle cerebral artery stroke. Neuropathological criteria were used to differentiate strokes that occurred in early intrauterine development (before 28 weeks gestational age) from those occurring in the last trimester, at birth, or after birth: 19 children had early strokes and 21 late. There was no difference in seizure history or occurrence of febrile convulsions in these two groups. Hippocampal tissue was available in 20 patients; pathology in the hippocampus, remote from the infarcted area, showed a marked difference between early-onset and late-onset groups. Hippocampal sclerosis was uncommon in children with early-onset strokes but developed in most of the children whose strokes were of later origin. However, hippocampal sclerosis was more closely related to a clinical history of a late initial precipitating insult irrespective of infarct timing. These findings demonstrate the changing vulnerability of the developing brain and show that hippocampal pathology is more closely related to the timing of an insult than seizure history or the occurrence of febrile convulsions.

The aim of this prospective study was to determine the outcome of affected hand impairment and disability in a group of 31 children (16 males, 15 females) with hemiplegic cerebral palsy who were referred consecutively to our rehabilitation service and followed from a mean age of 2 years and 7 months to a mean age of 12 years and 9 months. The hand function assessment protocol consisted of video-recorded procedures: one to assess grip and the other to assess the extent of spontaneous use of the affected hand. Assessments at outset and at latest follow-up showed that the series as a whole had greater hand impairment in spontaneous manipulation than in gripping tasks. Comparison of hand function before age 4 years with the latest assessment over age 11 years revealed a non-significant improvement in grip, whereas spontaneous hand use remained stable. All but one of the children who had good grip and hand use scores at first assessment maintained them over time unless they were not treated for hand dysfunction. Among the children with low scores at outset, the improvement over time was more marked in grip than spontaneous hand use and occurred mainly in the early years. These results suggest that to evaluate the real disability of the affected hand in children with hemiplegia, grip assessment is insufficient and that an instrument assessing spontaneous hand use in bilateral manipulation is required. Furthermore, intensive treatment focused on hand function should be planned in the early years for children with more severe hand impairment, whereas regular follow-up is sufficient for less affected children.

Little is known about long-term physical sequelae, cognitive functioning, and quality of life of children who have had a haemorrhagic stroke. Fifty-six patients (29 females, 27 males) under 16 years of age at time of the bleeding were studied. Mean age at time of bleeding was 7.7 years (range 1 month to 15.9 years). The primary site and cause of the bleeding at baseline were determined. Occurrences of death, re-bleedings, and seizures during follow-up were recorded. Patients who survived were invited for a follow-up examination including physical check-up, general screening of cognition, and an inventory of subjective health perception. Thirteen children died directly as a result of the haemorrhage; nine experienced a recurrent bleeding, which was fatal in three; six children developed epileptic seizures. At follow-up 36 of 56 patients were still alive. Mean follow-up time was 10.3 years (range 1.3 to 19.9 years) and mean age was 18.6 years (range 1.8 to 34.1 years). There was no patient lost to follow-up. Five patients declined to visit the hospital. In 15 out of 31 patients who could be examined, no physical impairment was observed, 11 had a hemiparesis of varying severity, and three had symptoms of cerebellar ataxia. One child had persisting tetraparesis and one persisting paraparesis. Signs of cognitive deficits were found in 15 patients. Of the children who survive haemorrhagic stroke, the physical and functional prognosis is relatively good, as almost all children were independent at follow-up. However, only a quarter of the surviving children had no physical or cognitive deficit after a mean follow-up period of 10 years. The majority had low self-esteem as well as emotional, behavioural, and health problems.

This study was designed to test the hypothesis that non-ambulatory patients with spastic quadriplegia will have reduced bone mass which worsens with increasing age. Forty-eight patients (age 5 to 48 years, median age 15 years; 19 females and 29 males) were studied. Anticonvulsants were used in 29 patients (60.4%). Lumbar spine bone mineral density (LS-BMD) was markedly reduced compared with age- and sex-matched control individuals with a z score of –2.37±0.21. Twenty-eight (58%) had z scores of less than –2. A history of documented previous fracture was present in 19 patients (39%). Patients with a history of fracture had significantly lower (p=0.05) LS-BMD z scores (–2.81±0.29) compared with those without a history of fracture (–2.11±0.26). Mean serum 25-OH vitamin D was 29.6±1.9ng/mL (normal 9 to 37.6ng/mL) with three patients having serum 25-OH vitamin D levels less than 15ng/mL. These findings indicate that BMD is markedly reduced in non-ambulatory children and adults with neuromuscular disease. Reductions in bone mass put them at greater risk for non-traumatic fractures.

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.

This qualitative study investigated the positive and negative outcomes of a home-based strength-training programme for young people with cerebral palsy (CP). Eleven young people with spastic diplegic CP (seven females, four males; mean age 12 years 9 months, SD 2 years 10 months; range 8 to 18 years) and their parents were interviewed. Gross Motor Function Classification System scores ranged from I (walks without limitations) to III (walks with assistive device), with a mode of III. The strength-training programme, which was conducted in the participants' homes three times per week for 6 weeks (total of 18 prescribed sessions), comprised three exercises targeting the major support muscles of the lower limbs. Exercises were bilateral half squats, heel raises, and step-ups. The training load was increased by adding free weights to a backpack so that 8 to 10 repetitions of each exercise could be performed. Using thematic coding, three categories of outcome emerged: body function and structure, activity, and participation, which were influenced by environmental and personal contextual factors. The programme generated overwhelmingly positive outcomes with only minor negative responses about some equipment and the need for parental involvement. Benefits included perceptions that strength, flexibility, posture, walking, and the ability to negotiate steps had improved. In addition, participants reported psychological benefits such as a feeling of increased well-being and improved participation in school and leisure activities. The contextual factors highlighted the fact that sufficient clinician resources must be allocated to solve individual exercise and equipment problems. As well as providing further evidence that strength training can be beneficial, this study provides useful indicators to guide future quantitative studies of outcomes that are meaningful for people with CP.