This study compared the passive stiffness of wrist flexors and the strength of wrist flexors and extensors in three different wrist positions (30° of flexion, neutral, and 30° of extension) between children with cerebral palsy (CP) and typically developing (TD) comparison children. It also examined associations between these characteristics and manual function in children with CP. Eleven children with spastic hemiplegic CP (six females, five males; mean age 8y 5mo [SD 1y 8mo], range 6–11y) and 11 TD children, matched for age and sex, took part in this study. Passive stiffness of muscles was measured as the torque/angle relation during passive motion. Isometric strength tests were performed and the time needed to complete three tasks based on the Jebsen–Taylor Hand Function Test was recorded. Flexor stiffness was higher in the group with CP. Strength of flexors and extensors in the group with CP was lower with the wrist extended. No difference among test positions was found in the TD group. Moderate correlations were observed between manual function and variables related to strength and stiffness of wrist muscles in the group with CP. Children with CP showed muscle alterations coherent with the use of the wrist in flexion. Intervention on these characteristics could have a positive impact on manual function.

The development of postural adjustments during reaching movements was longitudinally studied in seven infants with cerebral palsy (CP) between 4 and 18 months of age. Five infants developed spastic hemiplegia, one spastic tetraplegia, and one spastic tetraplegia with athetosis. Each assessment consisted of a simultaneous recording of video data and surface EMGs of arm, neck, trunk, and leg muscles during reaching in various lying and sitting positions. The basic organization of postural adjustments of the children developing spastic CP was intact. Their main problem was a deficient capacity to modulate the postural adjustments to task-specific constraints – a deficit which was attributed to a combination of an impaired motor coordination and deficits in sensory integration. The child with spastic-dyskinetic CP showed distinct abnormalities in the basic organization of postural adjustments.

The aim of this study was to examine children with myelomeningocele (spina bifida) who were investigated according to the level of neurosegmental lesion and to determine the number who walked and their average age at commencement/cessation of walking. The data have been collected since 1978 at the Royal Children's Hospital, Melbourne during annual evaluations of patients with spina bifida and high-level paralysis. After screening for established criteria and assigning to a neurosegmental lesion level, 173 children were examined for their ability to walk, with or without aids, in a community setting. Thirty-five children had lesions at the thoracic level, and seven walked at a mean age of 4 years 6 months. Three children with a lesion at the thoracic level ceased walking at a mean age of 7 years 6 months. Ten children had lesions at the high-lumbar level (L1/2), and five walked at a mean age of 5 years 2 months. Three children who had lesions at the high-lumbar level ceased walking at a mean age of 6 years 11 months. Fifteen children had lesions at the mid-lumbar level (L3) and nine walked at a mean age of 5 years. Three ceased walking at a mean age of 7 years. Forty-five children had lesions at the low-lumbar level (L4/5) and 38 walked at a mean age of 3 years 10 months. Five ceased walking at a mean age of 9 years 1 month. Sixty-eight children had lesions at the sacral level; all of whom walked at an average age of 2 years 2 months. None had ceased walking by the end of the study period. Delay in achieving ambulation can be expected in all children with spina bifida, including those with low neurosegmental level lesions. Furthermore, some children with high lesion level, given the opportunity, might be expected to ambulate effectively in the community but may cease walking after 3 to 4 years of this activity, which is earlier than previously recorded.

In this study we describe psychosocial functions and seizure-related factors in a population-based sample of children with epilepsy. Psychosocial problems (Achenbach scales), cognitive function, and socioeconomic status were studied in 117 children with epilepsy aged between 6 and 13 years (mean age 11y [SD 2y 1mo] and 10y 8mo [SD 2y]; 71 males, 46 females) and in randomly selected controls matched with 117 children for sex and age (mean age 11y 2mo [SD 2y 1mo] and 10y 5mo [SD 2y 4mo]; 69 males, 48 females). The children had partial (n=67), generalized (n=43), or undetermined (n=7) epilepsy syndromes, and partial (n=68), generalized (n=47), or other (n=2) main seizure types. Psychosocial problems were more common among children with epilepsy than controls (odds ratio 5–9) and significantly related to epilepsy syndrome, main seizure type, age at onset, and seizure frequency. Mothers and teachers reported males with epilepsy as having more problems than females. Females self-reported psychosocial problems, males did not. Psychosocial problems were common in childhood epilepsy. Females appreciated the problems more realistically than males. Psychosocial problems should be considered an integral part of epilepsy management.

Atraumatic fractures are often seen in children and adolescents with cerebral palsy (CP) and epilepsy in full-time care. Increased bone fragility was postulated to be due to osteopenia resulting from a combination of factors including immobilization and antiepileptic treatment. The aim of this study was to determine the effect of vitamin D and calcium substitution on bone mineral density (BMD) in a group of children with CP in full-time care. Twenty children with the most severe form of CP (spastic quadriplegia) who had been treated with antiepileptic drugs for a relatively long period of time were included in the study. Physical examination and laboratory analyses excluded other possible causes of osteopenia. BMD was measured by dual X-ray absorptiometry. Thirteen patients were treated for 9 months with 1,25-dihydroxy-cholecalciferol vitamin D (0.25 mcg daily) and with calcium (500 mg daily). Seven control children were used for observation only. BMD greatly increased in the treated group, while children with CP in full-time care who did not receive vitamin D and calcium substitution continued to lose their bone mass. It can be concluded that the addition of vitamin D and calcium increases BMD in children with the most severe form of CP, who are receiving antiepileptic drugs.

Diagnosing ischaemic stroke and determining its cause is difficult in children. Both are important for selection of treatment and prediction of outcome. This study explored the diagnostic changes that lead to a delay in the correct diagnosis of paediatric stroke. Case histories of 45 children with ischaemic stroke (31 males, 14 females; median age 6y; age range 2mo–16y) were retrospectively reviewed. The initial clinical diagnosis, based on the interpretation of presenting symptoms, was compared with the final aetiological stroke diagnosis after completion and review of diagnostic work-up. The type of diagnostic change, consequent time delay until correct diagnosis, reasons for change of diagnosis, and alterations to management were evaluated. Twenty-four diagnostic changes were identified; 19 in ‘primary stroke diagnosis’ (symptoms initially not attributed to stroke), and five in ‘aetiological diagnosis’ (incorrect initial determination of type or cause of stroke). The median interval between initial and final diagnosis was 7 days (3h–2y). The change in diagnosis led to therapeutic alterations in 17 patients. Risk factors for childhood stroke differ from those in adults. Stroke is frequently not recognized as the cause of the child's symptoms, and the correct determination of stroke aetiology takes time. We recommend that children with stroke be evaluated in a centre with expertize, using standardized diagnostic protocols and careful follow-up.

Diagnosing cause of pain in children with severe cognitive impairments is difficult due to their problems with communication. Identification of risk factors for specific pain etiologies might help professionals in this task. The aim of this study was to determine whether child-related characteristics increase risk for specific types of pain. Participants were the caregivers of 41 females and 53 males with moderate to profound mental retardation, who were aged 3 to 18 years 8 months (mean 10:1, SD 4:4) but who communicated at the level of a typical child of 13.8 months (SD 10 months): 44 of the children had cerebral palsy (CP) and 59 a seizure disorder. Caregivers reported the cause of children's episodes of pain for four 1-week periods over 1 year. Logistic regression analyses were used to predict occurrence of specific types of pain using children's demographic, medical, and physical characteristics. Children had 406 episodes of pain due to accident, gastrointestinal conditions, musculoskeletal problems, infection, recurrent conditions, and common childhood causes. Results indicated that a unique set of risk factors predicted each pain type in this sample. Significant risk factors for pain included: lack of visual impairment and leg impairment (accidental pain); seizures, leg impairment, and greater number of medications (non-accidental pain); being male and tube fed (musculoskeletal pain); age <7 years, absence of CP, visual impairment, and less frequent medical monitoring (infection pain); being female and with arm impairment (gastrointestinal pain); and being tube fed and taking fewer medications (common childhood pains). In most cases, models were more specific than sensitive, indicating that the significant predictors are more useful for eliminating potential pain causes. These results suggest that population risk factors may be helpful in structuring diagnostic investigations for individual children with severe cognitive impairments.

Gastrostomy tube (GT) feeding in children with cerebral palsy (CP) is associated with significant increases in weight gain and, potentially, with overfeeding. This study aimed to measure energy balance and body composition in children with CP who were fed either orally or by GT. Forty children (27 males, 13 females; median age 8y 6mo; range 1y 4mo–18y 11mo) with spastic quadriplegic CP, of whom 22 were gastrostomy-fed and 18 orally-fed, underwent anthropometry, indirect calorimetry, and total energy expenditure determination (doubly-labelled water method). Total body water content (estimated by the 18O dilution method) was used to determine body composition. The Gross Motor Function Classification System (GMFCS) was used to determine the degree of motor impairment. GMFCS levels ranged from I to V; in the gastrostomy group 19 out of 22 were Level V and two out of 22 were Level IV. Within the orally-fed group, 11 out of 18 were Level V and four out of 18 were Level IV. Resting metabolic rate and total energy expenditure of the gastrostomy-fed children were lower but they had a significantly larger triceps skinfold thickness (p=0.01) and fat mass index (p=0.02) than the orally-fed children. Both groups had consistently higher body-fat content and lower fat-free (i.e. muscle and bone) content than the reference population of age- and sex-matched children without disabilities. This study has demonstrated the relatively low energy expenditure and high body-fat content of children with severe CP and highlighted the potential risk of overfeeding with available enteral feeds administered via GT.

This study was designed to test the hypothesis that systemic hypotension during the first postnatal week increases the risk of ultrasonographic echolucency in the white matter of preterm infants ([les ]28 weeks' gestation) while adjusting for confounders. From a study base of 1607 very-low-birthweight neonates (500 to 1500g), a subsample of 243 preterm infants (122 females; [les ]28 weeks' gestation) was selected for echolucency and data collection prospectively for the entire first postnatal week. Data analyses were performed separately for the first 24 hours of life, for the interval from the end of the first 24 hours to the end of the fourth postnatal day, and for days 5, 6, and 7. Systemic hypotension was defined as the mean arterial blood pressure in the lowest quartile for the infant's week of gestational age. Protocol cranial ultrasounds were those obtained closest to days 1, 7, and 21. A committee of sonologists classified the infants as having either echolucency (echolucency group) or not (control group). Systemic hypotension during the first week of life appeared to be associated with echolucency in univariable analyses but the association did not persist after adjustment for potential confounders. Detailed summaries of 13 previous studies, the majority of which did not show an association between systemic hypotension and white-matter damage, are presented. In sum, these results do not support the hypothesis that systemic hypotension contributes to echolucency among preterm infants.

The purpose of the study was to provide normative data for the Teddy Bear Cancellation Test (TBCT) and to evaluate prospectively the frequency of unilateral spatial neglect (USN) in children with acquired brain injury (ABI). In the control group (n=419; 218 males, 201 females; mean age 5y 1mo [SD 1y 4mo]; range 3 to 8y) omissions were rare and decreased with age. A left displacement of the first three teddy bears cancelled was observed with increasing age. This preferential left-to-right cancelling strategy was interpreted as learned under the influence of reading habits. The same test was used prospectively in 41 children with ABI (24 males, 17 females; mean age 5y 5mo [SD 2y]; range 3 to 8y) admitted to a paediatric rehabilitation department specializing in acquired brain lesions. In patients and controls, children under 6 years of age omitted more items than older children. The localization of omissions was skewed significantly to the left in children with right-sided lesions compared with children with left-sided lesions. USN was observed in seven patients with ABI. Left USN was found in three of the 10 patients with right-sided ABI. Right USN was present in two of the patients with 15 left-sided ABI and two of the 16 patients with non-lateralized ABI. Left USN is frequent in children after right-sided brain injury. The relatively high incidence of right spatial neglect in children is discussed in relation to the development of hemispheric specialization.

Autism and Asperger's disorder (AD) are neurodevelopmental conditions that affect cognitive and social-communicative function. Using a movement-related potential (MRP) paradigm, we investigated the clinical and neurobiological issue of ‘disorder separateness’ versus ‘disorder variance’ in autism and AD. This paradigm has been used to assess basal ganglia/supplementary motor functioning in Parkinson's disease. Three groups (high functioning autism [HFA]: 16 males, 1 female; mean age 12y 5mo [SD 4y 4mo]; AD: 11 males, 2 females; mean age 13y 5mo [SD 3y 8mo]; comparison group: 13 males, 8 females; mean age 13y 10mo [SD 3y 11mo]) completed a cued motor task during electroencephalogram recording of MRPs. The HFA group showed reduced peak amplitude at Cz, indicating less activity over the supplementary motor area during movement preparation. Although an overall significant between-group effect was found for early slope and peak amplitude, sub-analysis revealed that the group with AD did not differ significantly from either group. However, it is suggested that autism and AD may be dissociated on the basis of brain–behaviour correlations of IQ with specific neurobiological measures. The overlap between MRP traces for autism and Parkinson's disease suggests that the neurobiological wiring of motor functioning in autism may bypass the supplementary motor area/primary motor cortex pathway.

This study aimed to investigate whether two distinct motor control processes, i.e. motor planning and parameter setting, were impaired in children with attention-deficit–hyperactivity disorder (ADHD). An experiment was designed in which children copied figures of increasing complexity under increasing accuracy levels on a digitizer. Sixteen children with ADHD (11 males, 5 females; mean age 8y 4mo, SD1y 1mo) and 16 comparison children, without impairment, matched for age and sex participated. ADHD was diagnosed by a psychiatrist following the criteria of the DSM-V. Only children with IQ scores greater than 80 were included. Across all graphic tasks, children with ADHD made slower, inaccurate strokes with relatively high axial pen force compared with the comparison group. No evidence was found for a deficit in motor planning, but parameter setting appeared to be deficient as the ADHD group made less accurate strokes when accuracy demands increased.

Functional electrical stimulation (FES) of the upper limb has been used for patients with a variety of neurological conditions, although few studies have been conducted on its use on the upper limb of children with cerebral palsy (CP). The aim of this study was to investigate the effect of cyclic FES on the wrist extensor muscles of a group of eight children (five boys, three girls) with hemiplegic CP (mean age 10 years). The study design involved a baseline(3 weeks), treatment (6 weeks), and follow-up (6 weeks). FES was applied for 30 minutes daily during the treatment period of the study. Improvements in hand function (p[ges ]0.039) and active wrist extension (p=0.031) were observed at the end of the treatment period. These improvements were largely maintained until the end of the follow-up period. No significant change was observed in the measurements of wrist extension moment during the treatment period (p=0.274). Hand function in this group of children improved after they were exposed to FES of wrist extensor muscles. This suggests that FES could become a useful adjunct therapy to complement existing management strategies available for this patient population.

Intrauterine growth retardation (IUGR) due to placental dysfunction is a risk factor for an impaired neurodevelopmental outcome. This is the case particularly in growth-retarded foetuses whose conditions in utero are so compromised that they have to be delivered before term. In recent years, neonatal treatment and care have improved markedly, and this has led to increased chances of survival of very immature and extremely low-birthweight infants. The optimal time to deliver IUGR infants remains difficult to assess. There is an obvious need for methods that accurately monitor foetal and neonatal condition, and it is important that the implications of these methods are evaluated for the neurodevelopmental outcomes of these infants. It is vitally important to be able to predict the outcome, whether it is normal or abnormal. A method that covers both aspects is the qualitative assessment of spontaneous motility of foetuses and newborn infants.

The aim of this study was to assess psychomotor development, using Griffiths' test, and the incidence of minor anomalies at birth in children who had been exposed to antiepileptic drugs (AEDs) in utero. The study sample comprised 100 children of mothers who were treated with AEDs during pregnancy and 100 matched control children. Women with epilepsy were recruited from a pregnant urban population (450 000 inhabitants). The lowest possible dose of the fewest AEDs to maintain seizure control was used. Drug levels were controlled on a monthly basis. The children were assessed at 9 months of age. The study children had a significant increase in the number of minor anomalies (31 compared with 18 control children; odds ratio 2.4, CI 1.15 to 5.02, P=0.02 McNemars test), and an increased number of facial anomalies after carbamazepine exposure (11 compared with six control children). Drug exposure did not influence the Griffiths' score at 9 months of age. Even a meticulous AED treatment strategy during pregnancy increases the number of minor anomalies. However, treatment with AEDs does not necessarily influence short-term psychomotor development.

Studies on precocious puberty have primarily focused on children with typical patterns of growth and cognitive development. This study reviewed diagnostic data from the records of 15719 patients with neurodevelopmental disabilities for diagnoses associated with premature sexual development/precocious puberty. Thirty-two individuals with premature sexual development were identified, with the earliest changes seen in one girl at 1 year 7 months of age. In this group, the mean age at onset was 7 years 2 months in boys and 5 years 11 months in girls. Central precocious puberty, which was the most common cause of onset of early pubertal changes, was present in 15 of the 32 children. The results of this study suggest that children with a neurodevelopmental disability are at increased risk of premature pubertal changes when compared to children without a neurodevelopmental disability. This study indicates the need for health-care providers to be vigilant in screening for early pubertal changes in children with neurodevelopmental disabilities.

The reliability of visual interpretation of electroencephalograms (EEG) is of great importance in assessing the value of this diagnostic tool. We prospectively obtained 50 standard EEGs and 61 EEGs after partial sleep deprivation from 93 children (56 males, 37 females) with a mean age of 6 years 10 months (SE 5mo; range 4mo–15y 7mo) with one or more newly diagnosed, unprovoked seizures. Two clinical neurophysiologists independently classified the background pattern and the presence of epileptiform discharges or focal non-epileptiform abnormalities of each EEG. The agreement was substantial for the interpretation of the EEG as normal or abnormal (kappa 0.66), almost perfect for the presence of epileptiform discharges (kappa 0.83), substantial for the occurrence of an abnormal background pattern (kappa 0.73), and moderate for the presence of focal non-epileptiform discharges (kappa 0.54). In conclusion, the reliability of the visual interpretation of EEGs in children is almost perfect as regards the presence of epileptiform abnormalities, and moderate to substantial for the presence of other abnormalities.

The study aimed to investigate the development of bladder control in children with cerebral palsy (CP) and to determinate subgroups with deviant development of bladder control and a higher risk of not achieving urinary continence. Children and adolescents between the ages of 4 and 18 years with a diagnosis of CP, from six Dutch rehabilitation centres were included in the study (n=601). Parents of these participants were sent a questionnaire, of whom 76% responded. Prevalence of primary urinary incontinence was 23.5%. Survival curves were made to determine the development of achieving bladder control in CP. For participants who achieved urinary continence, the development was delayed in comparison with normally developing children and adolescents. The most important factors influencing the occurrence of urinary incontinence in CP were tetraplegia and low intellectual capacity. At age six, 54% of participants with spastic tetraplegia and 80% with spastic hemiplegia or diplegia gained urinary continence spontaneously. Of those who had low intellectual capacity, 38% were dry at this age.

Twelve children with vertebrobasilar artery stroke are reported (seven males, five females; aged 6 months to 15 years). Patient 1 showed an arcuate foramen in the posterior arch of the atlas, an anatomical variant occurring in 3 to 15% of the population. It was hypothesized that the presence of the arcuate foramen might cause tethering of the vertebral artery and lead to its dissection by repetitive trauma. Lateral plain films of the cervical spine in cases of posterior circulation stroke were taken. Eight of 11 patients showed aberrant arcuate foramina. Of the remaining three patients, one had normal cervical spine X-rays, one had an absent right posterior arch of the atlas following previous surgery for a cervical meningocele, and one patient had incomplete ossification of the vertebrae. Seven of the nine patients with arcuate foramina had vertebral angiograms. In all cases this showed the vertebral artery passing through the arcuate foramen before entering the brain and an appearance consistent with arterial dissection and occlusion at the same site below the foramen. Most documented cases of posterior circulation stroke in children follow trauma, which may be minimal or repetitive, with thrombotic occlusion of the artery at C1–C2 level. The association with an arcuate foramen and its possible causative role in the genesis of posterior circulation stroke in children has not been previously recognized. There may be a causal association between the presence of an arcuate foramen, tethering of the vertebral artery in the foramen, and dissection from repetitive trauma with movement of the neck.

In this study we describe the development and preliminary psychometric testing of the Spinal Alignment and Range of Motion Measure (SAROMM). Through consultation with pediatric physiotherapists, the items were refined. Subsequently 25 children and adolescents with cerebral palsy (CP; 17 males, 8 females) with a mean age of 9 years 8 months (SD 4y 4mo), stratified by the Gross Motor Function Classification System (GMFCS, n=5 in each group), were recruited. Twenty-two children had spastic CP, and one each was also diagnosed with hypotonic, athetoid, and mixed CP. Three children had hemiplegia, 12 had diplegia, and 10 had quadriplegia. These participants were examined by two physiotherapists on one occasion and by the primary physiotherapist again two weeks later. The intraclass correlation coefficients reflecting interrater and test–retest reliabilities for the spine and range of motion subscales and the total scores were all above 0.80. Validity was supported by a significant contribution of GMFCS level and age to the SAROMM score (r2=0.44). The SAROMM has sufficient reliability and validity for use with children with CP in clinical and research settings by rehabilitation therapists.