A consecutive series of 106 children and adolescents (mean age 10 years, 6 months; SD 4 years, 8 months) with recent traumatic brain injury admitted to a regional hospital-based rehabilitation program was assessed to determine the rate of walking recovery, and characteristics that distinguish between independent walkers, non-walkers, and device-assisted walkers at hospital discharge. Data were collected through a retrospective medical record review of patients admitted between 1994 and 2001. Mean hospital stays were 66.7 days (SD 88.5, range 7 to 140 days). All children (72 male, 34 female) had recent injuries (from 1 to 8 weeks after onset of traumatic brain injury) and were independent walkers before injury. Sixty-four children (60.4%) were discharged as independent walkers, 13 (12.3%) walked with the assistance of a device, and 29 (27.3%) were non-walkers. Non-walkers had a higher proportion of prolonged loss of consciousness, lower-extremity injury, impaired responsiveness, and lower-extremity spasticity than independent walkers. In addition, non-walkers had poorer discharge mobility and social function scores, longer average hospital stays, and a greater proportion of non-community discharges. Device-only walkers were older, more likely to be male, and had a higher proportion of lower-extremity injuries than independent walkers. Results highlight several demographic, clinical, and outcome variables that distinguish independent walkers from device-assisted walkers and non-walkers. These variables might help to determine the prognosis for ambulation, resource needs, and discharge plans for children and adolescents with traumatic brain injury after episodes of inpatient rehabilitation.

This study compared clinical and functional outcomes after treatment with botulinum toxin A (BTX-A) and BTX-A with casting in children with dynamic equinus foot. Ten children (seven males, three females; mean age 6 years 4 months, SD 2 years 7 months; range 4 to 11 years) with mild spastic diplegia and independent walking were divided into two groups: group 1, BTX-A and group 2, BTX-A plus casting. BTX-A was injected in the triceps surae bilaterally at multiple sites while the children were sedated with mask anaesthesia. Dysport® toxin was used, 15 to 20IU/kg in each muscle. Immediately after injection an ankle–foot orthosis was applied to children in group 1 and a cast to children in group 2. Clinical assessment using the Ashworth scale, Gross Motor Function Measure (GMFM), range of movement measurement, and gait analysis was performed before treatment and 1, 4, and 12 months after treatment. Spasticity decreased significantly at 1-month examination in both groups (p=0.002), at 4-month examinations (Wilcoxon test p=0.003), and 12 month (p=0.052) examinations in group 2. GMFM highlighted a significant improvement in group 2 at the 4-month examination(p=0.052 for standing, p=0.007 for walking). Gait analysis showed a significant increase in the walking speed in group 2 (p=0.04). No change was detected in ankle kinematics and kinetics or in muscular activity during the gait cycle. We confirmed that BTX-A reduces spasticity and improves functional performance in standing and walking; association with casting provides more marked and enduring results.

The incidence, survival patterns, and presenting symptoms of children with medulloblastoma were studied. Data were ascertained from the Manchester Tumour Registry which is population-based and has collected data on all childhood malignancies in northwest England since 1954. Incidence rates standardized to the European standard population were calculated and Poisson regression models were used to examine temporal changes in the incidence rates during the period 1954 to 1997. Kaplan–Meier survival curves were derived and used to study changes in survival patterns. World-standardized incidence rates were 5.5 per million child years in males and 3.4 per million child years in females. Incidence rates increased from the 1950s to the 1980s but have declined recently. The 5-year survival rate has improved from 29 to 58% with similar rates for males and females. The 1-year survival rate has also improved, but females had worse survival at this point (58%) than males (75%). The type of symptom or sign at presentation is strongly affected by age, with 10 of the 22 recorded symptoms or signs showing significant age differences. The older the child is, the more likely is the presentation to show pressure features of headache, vomiting, and ophthalmic signs. Younger children present with non-specific features such as lethargy, behavioural disturbance, or increasing head size. Ataxia is seen in about 75% of children across the age range.

The effect of asphyxia on iron metabolism and lipid peroxidation in newborn infants with hypoxic–ischemic encephalopathy (HIE) was investigated. Non-protein-bound iron (NPBI) and lipid peroxidation (thiobarbituric-acid-reactive species; TBARS) in plasma and hematological iron indices were measured in 15 healthy newborn infants (mean gestational age 39 04weeks, SD 1); 15 asphyxiated infants without neurological abnormalities (AS–HIE; mean gestational age 38.8 weeks, SD 0.9); and 15 asphyxiated infants with neurological abnormalities (AS+HIE; mean gestational age 39.75 weeks, SD 1.4). Follow-up was performed at the age of 5 months. It was found that the detectable rates of NPBI in 10 of 15 of the AS–HIE group and 13 of 15 of the AS+HIE group were significantly higher than that of the control group (5 of 15; both p<0.01). Plasma levels of TBARS in the control (9.20µmol/L, SD 1.9) and AS–HIE infants (10.13µmol/L, SD 2.7) were significantly lower than those of the AS+HIE group (13.42µmol/L, SD 2.8). Serum iron, total iron binding capacity, and transferrin saturation in the AS+HIE group was higher than the corresponding values of the control and AS–HIE groups, although no statistical difference was found among them. At 5 months of age, all control and AS–HIE infants were neurologically normal, whether or not their NPBI was detectable. Of the 12 AS+HIE infants, four (all of whom had detectable NPBI) were neurologically impaired. The average Gross Development Quotient of AS+HIE infants was significantly lower than that of the control or AS–HIE groups (p<0.01). Results showed that asphyxia could affect iron metabolism and lead to a significant increase in NPBI and lipid peroxidation in newborn infants with HIE, indicating that iron delocalization induced by asphyxia plays a role in the brain injury of asphyxiated infants.

In this study, 10 sagittal lower limb range-of-motion measures were conducted in a blinded fashion in 25 children with spastic diplegic cerebral palsy and in 25 age- and sex-matched controls. The participants comprised 22 males, mean age 10 years 8 months and 28 females, with mean age 9 years 8 months; age range 6 to 17 years. One paediatric physical therapist performed duplicate goniometric measures at zero time and 7 days later using the same sequence of measures, location, and time of day. Mean absolute differences for measures within one session ranged from 0.7 to 2.9° in controls and from 1 to 4.2° in children with spastic diplegia. Most intraclass correlation coefficients (ICCs) for intra-sessional measures were more than 0.90 in both groups. Measures between sessions were less reliable. Mean absolute differences between sessions were up to 7.1° for children with spastic diplegia and 8.6° for controls, with most ICCs being less than 0.80. Inter-sessional variation in measures was similar in both groups, suggesting that measurement variability is not influenced by the presence of spasticity. Averaging of two measures did not improve inter-sessional reliability compared with the use of a single measure. Dynamic measures (R1) were as reliable as passive measures (R2), but there were inter-sessional differences in calculations using R1 and R2 measures of up to 30°.

Four dimensions of motor competence were evaluated in 4-year-old term and preterm children and were related to academic achievement and use of school services at age 8 years. The objective of the study was to evaluate a graduated ‘stair-step’ effect between perinatal morbidity, mild motor delay, and later school outcomes in 168 children (88 females, 80 males) stratified into four study groups: 134 healthy term infants [ges ] 37 weeks' gestation, birthweight [ges ] 2500g); 134 preterm infants [les ]37 weeks' gestation divided into healthy preterm (n=41), clinically ill preterms (n=59), and preterm infants with neurological illness (n=34). Significant differences were found in total, fine and gross motor performance, and visual–motor integration at age 4 years. Scores for the preterm groups decreased with increasing morbidity. At age 4 years, mild motor delay was found in all preterm groups. Children with mild motor delay had lower academic achievement scores (Wide Range Achievement Test-3) and higher rates of school service use at age 8 years. Perinatal morbidity and compromised motor performance are important precursors of educational underachievement.

The aim of the study was to compare the development of children with and without a programme of psychosocial stimulation in ‘control’ and ‘intervention’ sites in a poorly resourced area of northeast Brazil. The sample (n=156, born 1998) was from a larger cohort. The cohort was tested at 12 months (baseline) with the Bayley Scales of Infant Development. All children in the intervention site with a mental development index (MDI) and/or psychomotor development index (PDI) [les ]100 were enrolled. Each time such a child was enrolled, the next child tested in that site of the same sex and with an index of 101 to 115 was also enrolled, and the next two children matched for sex and scores of [les ]100 and 101 to 115 in the control sites were recruited in parallel. The intervention comprised 14 contacts between 13 and 17 months of age. All children were tested again at age 18 months. The intervention and control groups were similar at baseline for a range of socioeconomic, demographic, environmental, and biological variables, and their MDI and PDI were also similar. At 18 months, the mean differences between the intervention and control groups were +9.4 points for MDI and +8.2 points for PDI (p<0.001 in each case). For children with an initial score of [les ]100, the mean difference between the intervened and control groups was +11.2 points for MDI (p<0.001), and +10.8 points for PDI (p=0.001). The intervention was thus associated with significant improvements in cognitive and motor development.

Everyday functioning is described in 95 children with cerebral palsy (CP; 55 males and 40 females; mean age 58 months, SD 18 months, range 25 to 87 months) using the three scales of the Pediatric Evaluation of Disability Inventory (PEDI): Functional Skills, Caregiver Assistance, and Modifications of the Environment. Types of CP in the children were hemiplegia, (n=19), spastic/ataxic diplegia, (n=44), spastic quadriplegia, (n=16), dyskinetic, (n=9), and mixed (n=7). Symptoms were grouped by severity according to the Gross Motor Function Classification System (GMFCS): 23% were classified at level I, 21% at level II, 10% at level III, 23% at level IV, and 23% level V. A large variability in functioning in mobility, self-care, and social function was seen because of the heterogeneity of children with CP. Limitations in achievement of activities, need for assistance, and use of assistive devices increased progressively with GMFCS level. Furthermore, these children differed to a great extent from the normative sample of the PEDI. Stepwise regression analysis showed that the GMFCS was a good predictor of everyday functioning with age and learning problems as significantly contributing factors, particularly in self-care and social function. In conclusion, the three scales of the PEDI represent different but strongly related aspects of everyday functioning in young children with CP.

The aim of this study was to characterize impairments of hand function in individuals with myelomeningocele (MMC) using a quantitative method. A grip–lift task was studied in 29 individuals with MMC (17 females, 12 males; mean age 12 years 4 months, SD 5 years 6 months; range 4 to 28 years) and 29 age- and sex-matched control participants. A small object (weight 200g) was grasped and lifted with a precision grip of the dominant hand and grip forces (GF) and time intervals were measured. Sensibility was examined with a two-point discrimination test. In those with MMC, the latency between GF onset of the thumb and index finger and ensuing preload duration were both significantly prolonged (p<0.01). Fingertip forces were excessively high and variable. Several parameters differed significantly between those with MMC and control individuals: GF peak, GF at start of loading, mean GF, and the SDs of the GF during static holding. Although the two-point discrimination task indicated deficits of sensibility, these did not correlate with grip force parameters. Results demonstrate that fine motor skills in patients with MMC are impeded by slowness and inadequate adjustments of manipulative forces.

The cognitive status of 168 Dutch young adults (103 females, 65 males; mean age 20 years 9 months, age range 16 to 25 years) with spina bifida (SB) was examined. The main purpose was to establish the effect of the type of SB (occulta or aperta) and the effect of hydrocephalus (HC) within the group with SB aperta (AHC+). Results indicated, on average, a lower cognitive status of persons with AHC+ (n=111) than of persons with SB occulta (n=37) and of persons with SB aperta without HC (AHC– ; n=20). Almost half the young adults with AHC+ had cognitive impairments of some sort. These included more domain specific impairments (70%) as well as a more general cognitive deficit (30%). Cognitive status of persons with SB occulta and of those with AHC– was similar to that in the healthy population. The presence of associated pathology, rather than SB per se, has a negative effect on cognitive status.

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.

This study examined the effect of low birthweight on school achievement and the mediating roles of cognitive and behavioural factors. The sample (115 females, 100 males) was selected from a longitudinal study of first-born singleton children, born between 1986 and 1988 of German-speaking parents, recruited from eight hospitals of the Rhine–Neckar region in Germany. Twenty-nine very-low-birthweight (VLBW; less than 1500g), 74 low-birthweight (LBW; 1500 to 2500g) and 112 normal-birthweight children (NBW; more than 2500g), all without severe neurological disability, were assessed at 11 years on cognitive, motor, scholastic, and behavioural measures. The scholastic measures included a school performance score and a teacher recommendation for type of secondary school the child should attend. LBW and VLBW children performed less well than the NBW group in all areas. LBW and VLBW groups remained at risk for school difficulties into late childhood, even when not neurologically impaired. Arithmetic, vocabulary, concentration, non-verbal intelligence, and attention problems were significant mediators of the effect of low birthweight on the school performance score. Arithmetic, vocabulary, motor skills, and attention problems were found to be mediating factors of birthweight on teacher's recommendations. The poor outcome of the low birthweight children could not be attributed to further obstetric risk factors. Parents and pediatricians should be made aware of specific long-term deficits of low birthweight children that may impair school performance, although they may be within a normal curriculum.

Prevalence of drooling in children with cerebral palsy (CP) and its severity across dental age (i.e. children with primary, mixed, and permanent dentitions) was investigated. One hundred and sixty children and adolescents (91 males, 69 females; mean age 10 years 10 months, SD 4 years 2 months; range 4 to 18 years) with CP who attended special schools were involved in this study. Sixty eight per cent of participants had spastic CP with the majority (45%) diagnosed as having quadriplegia. Presence or absence of drooling was determined by direct observation of the participants. Other data, such as age, sex, learning disability, and type of CP were collected using questionnaires. Results showed 93 of 160 children (58%) with CP had a drooling condition and of these 53 (33%) had severe drooling. It was found that the degree of drooling decreased as the child's dental age increased. Prevalence of drooling in children with primary dentition was found to be 75% as compared with 43% of those with permanent dentition, although this difference was not statistically significant. It was concluded that any invasive treatment to reduce drooling should, perhaps, be postponed until the child's oromusculature has matured.

The aim of the study was to find the epidemiological distribution of tic disorders and Tourette syndrome (TS) in Swedish school children aged 7 to 15 years. A total population of 4479 children and their parents were asked to fill in a questionnaire covering both motor and vocal tics. A three-stage procedure was used: screening, interview, and clinical investigation. Two hundred and ninety-seven children (190 males, 107 females) were found to have tics. TS, according to DSM-IV criteria, was found in 0.6% of the total population, another 0.8% had chronic motor tics, and 0.5% had chronic vocal tics. Further, 4.8% of the children had transient tics. All together 6.6% of 7- to 15-year-old children currently had or had experienced some kind of tic disorder during the last year. Prevalence of different tic disorders was higher among younger children and in males, and was highly associated with school dysfunction. The prevalence of TS was higher than was previously thought but other tic disorders were more common in this childhood population.

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.

To determine the long-term risks and benefits of a separate CSF reservoir in the management of 52 children (23 males, 29 females) with shunted hydrocephalus, a retrospective study was performed comparing the use and complications after separate reservoir insertion, with a prereservoir control period. Median age at first shunt insertion was 1 month and median age at reservoir insertion was 2 years 6 months. Median follow-up for shunt with the additional reservoir was 19 years 1 month. There was no mortality due to shunt failure nor CNS infection, and there were significantly fewer episodes of ventriculitis (p<0.01) and shunt blockage (p<0.0001) compared with the prereservoir period. There was no hemiplegia, epilepsy, visual, nor cognitive loss from the additional reservoir. The reservoir was used for access in 344 attendances (mean 6.62 attendances per patient) for diagnosis or treatment of raised pressure or CNS infection. It was concluded that a separate CSF reservoir is useful in the long-term management of patients with shunted hydrocephalus and is without mortality or significant increased morbidity.

The aim of this study was to document the rate of survival among 288 severely affected pediatric patients (154 females, 134 males) with spasticity and neuromuscular scoliosis who underwent spinal fusion (mean age at surgery 13 years 11 months, SD 3 years 4 months), and to identify exposure variables that could significantly predict survival times. Kaplan–Meier survival analysis was performed demonstrating a mean predicted survival of 11 years 2 months after spinal surgery for this group of globally involved children with cerebral palsy (CP). Cox's proportional hazards model was used to evaluate predictive efficacy of exposure variables, such as sex, age at surgery, level of ambulation, cognitive ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in hospital, and days in the intensive care unit. Number of days in intensive care unit after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. This demonstrated statistically significant predictability for decreased life expectancy after spinal fusion in children with CP.

The recovery of gait, gross motor proficiency, and hand function was examined in 23 children (13 males, 10 females; age 4 years 7 months to 15 years 10 months) with traumatic brain injury (TBI) over five months of in patient rehabilitation. We used gait analysis, the Gross Motor Function Measure, the Developmental Hand Function Test, and the Purdue Pegboard test. Brain injury had been severe (initial Glasgow Coma Scale GCS<8) in 17 children and moderate (GCS 8–10) in six children. Compared with healthy control children of the same age and sex, repeated gait analyses in ambulatory children with brain injury showed significant reductions of velocity, stride length and cadence, and impaired balance. Spatiotemporal gait variables were correlated with Gross Motor Function Measure scores. Hand function tests revealed deficits in fine motor skills, speed, and coordination. Degree of impairment increased with trauma severity. Despite significant improvements, differences in gait velocity, stride length, and hand function of children with brain injuries and controls were still present about 8 months after TBI. Hand motor skills improved less than gait. Young age at injury was not associated with better recovery.

Unexplained crying in infants aged 1 to 3 months is a common concern for Western parents and health services. This study examined the hypothesis that the crying is due to high infant responsivity, and provides evidence about the types of stimulation that trigger crying in infants who present with high responsivity. The sample included 93 eight-day-old infants from a community sample (47 females, 46 males; mean birthweight 3457g; mean gestation 39.4 weeks; mean Apgar scores 8.28 at 1 minute and 9.59 at 5 minutes). Infants were tested for their response to two standard, mildly challenging, procedures: a neurobehavioural test involving undressing, putting down, and handling, and the Guthrie test, involving a painful heel prick to obtain a blood sample. The infants' crying over 24 hours was recorded in parental diaries. Newborn infants who exhibited high responsivity during the neurobehavioural assessment cried, rather than fussed, the most at home. High responsivity during the neurobehavioural assessment also predicted those infants who cried a lot and met a definition of ‘colic’ at home. The findings support the responsivity hypothesis and show that infants with high responsivity are upset by undressing, putting down, and sustained handling. Explanations for this and implications for the management of infant crying and colic are discussed.

The Ely Test (or Duncan-Ely test) has been accepted as a clinical tool to assess rectus femoris spasticity by passively flexing the knee rapidly while the patient lies prone in a relaxed state. In this retrospective review, patients' dynamic knee range of motion (ROM) during gait and an electromyogram (EMG) were compared with the results of the Ely test. Data for 70 patients (44 males, 26 females; 104 limbs) were included. Mean age of patients was 13 years, SD 9 years, range 4 years 5 months to 54 years. All patients were diagnosed with cerebral palsy (spastic diplegia, n=42; spastic quadriplegia, n=15, and hemiplegia, n=13). All patients were ambulatory (50 independent, 20 with assistive devices). A standard matrix was used to calculate sensitivity and specificity of the Ely test as well as its positive and negative predictive value. For the gait variables examined (decreased dynamic knee ROM, timing of peak knee flexion, and abnormal EMG in swing) the sensitivity of the Ely test ranged from 56 to 59% and the specificity ranged from 64 to 85%. For the same variables the positive predictive value ranged from 91 to 98% and the negative predictive value ranged from 4 to 19%. The Ely test was shown to have a good positive predictive value (i.e. the certainty about the presence of rectus spasticity in patients with a positive Ely test result) for rectus femoris dysfunction during gait.