Event-related functional magnetic resonance imaging (fMRI) was used to investigate the hypothesis that males who were born very preterm may show differences in relative strength of blood oxygen level dependent (BOLD) signals in selective brain areas during performance of a simple response inhibition task compared with term-born controls. Participants were eight males (mean gestational age at birth 28wks, [SD 2]; mean age at testing 16y, [SD 1] and 14 controls matched for sex, age (mean age 17y, [SD 1]), and IQ. A ‘go-no-go’ task was used to assess response selection and motor response inhibition in response to a visual stimulus. When the ‘no-go’ condition was contrasted with an attentional control condition, preterm participants showed reduced BOLD signal response bilaterally in the cerebellum, right caudate nucleus, and thalamus, and prefrontal areas including left inferior prefrontal and left anterior cingulate gyri. They also showed increased response mainly in temporal regions. These results suggest that despite good task performance, individuals who were born very preterm exhibit different BOLD signal responses in selective brain areas compared with controls which may underline the use of alternative strategies when challenged with motor response inhibition processing.

This study sought to describe change and stability in health-related quality of life (HRQL) over time in a cross-section of children with cerebral palsy (CP) using the Child Health Questionnaire (CHQ) through repeated surveys of the children's parents/caregivers. A total of 177 children with CP (98 males, 79 females); age range 3 to 18 years (mean age 8y 6mo, [SD 4y 2mo]; Gross Motor Function Classification System: Level I 40%, Level II 14%, Level III 14%, Level IV 16%, and Level V 17%) were enrolled as a convenience sample from the outpatient clinics at a tertiary-care children's hospital. The main outcome measure was HRQL as determined by the CHQ – Parent Form 50. Parents reported reduced HRQL for their children with CP relative to norms for the CHQ. Although Role – Physical functioning (reflecting how physical impairment interferes with daily activity) declined with time, all other subscales of the CHQ were stable over the course of 1 year. CHQ scores were not measurably affected by common medical interventions. Children with CP have lower CHQ scores than other children that are, on average, stable over the course of 1 year.

Children with autism have a relatively shorter index finger (2D) compared with their ring finger (4D). It is often presumed that the 2D:4D ratio is associated with fetal testosterone levels and that high fetal testosterone levels could play a role in the aetiology of autism. It is unknown whether this effect is specific to autism. In this study, 2D:4D ratios of 144 males aged 6 to 14 years (mean age 9y 1mo [SD 1y 11mo]) with psychiatric disorders were compared with those of 96 males aged 6 to 13 years from the general population (mean age 9y 1mo [SD 1y 10mo]). Psychiatric disorders were divided into autism/Asperger syndrome (n=24), pervasive developmental disorder–not otherwise specified (PDD–NOS; n=26), attention-deficit–hyperactivity disorder (ADHD)/oppositional defiant disorder (ODD; n=68), and anxiety disorders (n=26). Males with autism/Asperger syndrome (p<0.05) and ADHD/ODD (p<0.05) had significantly lower (though not significantly; p=0.52) ratios than males with an anxiety disorder, and males with autism/Asperger syndrome had lower ratios than those in the comparison group. These results indicated that higher fetal testosterone levels may play a role, not only in the origin of autism, but also in the aetiology of PDD–NOS and of ADHD/ODD. Males with anxiety disorders might have been exposed to lower prenatal testosterone levels.

Rotation characteristics in gait and passive rotation of the lower limbs were evaluated retrospectively in 105 patients with diplegic cerebral palsy (65 males, 40 females; mean age 13y [SD 6y 9mo]; range 4y 4mo–40y 5mo). Of 105 patients, 22 (20.9%) required crutches, sticks, tripods, or a K-walker for their daily ambulation. Twelve (11.5%) patients used a wheelchair or buggy for community distances, e.g. shopping. Significant differences in rotational characteristics were found at the pelvis, hip, knee, and foot between left and right legs. Patients who were more affected on the right (group R, n=33) or the left side (group L, n=39) were re-evaluated. There was also a group of patients who were not asymmetrically affected (group S, n=33). In group L, maximum passive internal rotation was significantly greater on the left side, while no difference between the sides was found in group R. Peak internal rotation in gait was significantly higher on the right side in group R, but did not differ significantly between the sides in group L. Right hindfoot–thigh angle and transmalleolar axis were more external on the right, irrespective of which leg was more affected. These findings may have implications for the early non-operative management of limb posture in infants with diplegia and the surgical management of established lower extremity malrotation.

This paper reports the development and validation of a disease-specific measure of health status and well-being of children with severe cerebral palsy (CP). The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) was constructed from recommendations from caregivers, healthcare providers, and review of other measures. Items spanning six domains are rated on an ordinal scale. Standardized scores (0–100) are reported for each domain and in total. Primary caregivers (n=77) of 45 males and 32 females between 5 and 18 years of age (mean age 13y 5mo [SD 3y 4mo]) with CP, categorized by the Gross Motor Function Classification System (GMFCS) level, completed the CPCHILD. Caregivers of children with severe CP (GMFCS Levels IV and V) also completed a second administration of the CPCHILD 2 weeks after the first. The mean CPCHILD score for children with severe CP was 56.2 (SD 15.7; range 24–93). The mean CPCHILD scores for children in GMFCS Levels I to V were 22.0, 38.2, 23.0, 44.5, and 59.3 respectively (p<0.001). Reliability was tested in 41/52 caregivers who reported no change in health status between the two administrations of the CPCHILD. The intraclass correlation coefficient was 0.94 (95% confidence interval 0.90–0.97). The CPCHILD seems to be a reliable and valid measure of caregivers' perspectives on the health status, functional limitations, and well-being of these children.

The purpose of this study was to investigate the effect of salivary flow reduction on daily life and provision of care in children with cerebral palsy (CP). Parents of children with CP were asked to fill in a questionnaire on the impact of drooling on the daily life of their children and their families and the data were then analyzed. Forty-five children with severe drooling (28 males, 17 females; mean age 9y 5mo [SD 3y 7mo]; range 3 to 16y) were monitored before and after receiving medication (scopolamine and botulinum toxin) to reduce salivary flow. Type of CP included hypotonia (n=1), spastic paresis (n=27), and mixed motor disorders with spastic and dyskinetic paresis (n=17). Eight children were independently ambulant and 37 children were wheelchair users. Thirty-four children had learning disability with a developmental age of below 6 years. Six participants dropped out of the study; data on 39 children were analyzed. Results showed that anticholinergic agents effectively reduced salivary flow. Drooling diminished substantially and this was accompanied by a significant reduction in care needs, making daily care less demanding. The amount of reported damage to communication devices and computers decreased. In addition to the evaluation of primary variables, such as the salivary flow rate, investigation of impact of drooling on daily life provides useful information about the outcome of treatment for reduction in drooling.

To determine the frequency and predictors of sleep disorders in children with cerebral palsy (CP) we analyzed the responses of 173 parents who had completed the Sleep Disturbance Scale for Children. The study population included 100 males (57.8%) and 73 females (42.2%; mean age 8y 10mo [SD 1y 11mo]; range 6y–11y 11mo). Eighty-three children (48.0%) had spastic diplegia, 59 (34.1%) congenital hemiplegia, 18 (10.4%) spastic quadriplegia, and 13 (7.5%) dystonic/dyskinetic CP. Seventy-three children (42.2%) were in Gross Motor Function Classification System Level I, 33 (19.1%) in Level II, 30 (17.3%) in Level III, 23 (13.3%) in Level IV, and 14 (8.1%) in Level V. Thirty children (17.3%) had epilepsy. A total sleep problem score and six factors indicative of the most common areas of sleep disorder in childhood were obtained. Of the children in our study, 23% had a pathological total sleep score, in comparison with 5% of children in the general population. Difficulty in initiating and maintaining sleep, sleep–wake transition, and sleep breathing disorders were the most frequently identified problems. Active epilepsy was associated with the presence of a sleep disorder (odds ratio [OR]=17.1, 95% confidence interval [CI] 2.5–115.3), as was being the child of a single-parent family (OR=3.9, 95% CI 1.3–11.6). Disorders of initiation and maintenance of sleep were more frequent in children with spastic quadriplegia (OR=12.9, 95% CI 1.9–88.0), those with dyskinetic CP (OR=20.6, 95% CI 3.1–135.0), and those with severe visual impairment (OR=12.5, 95% CI 2.5–63.1). Both medical and environmental factors seem to contribute to the increased frequency of chronic sleep disorders in children with CP.

The aim of this study was to assess the stability of the Gross Motor Function Classification System (GMFCS) by examining whether children with cerebral palsy (CP) remain in the same level over time. Participants were 610 children with CP (342 males, 268 females; mean age 6y 9mo [SD 2y 10mo]), range 16mo–13y). Children were assessed 2 to 7 times (mean 4.3) at 6-month (children <6y old) or 12-month(children [ges ]6y old) intervals. Seventy-three per cent of children remained in the same level for all ratings. The weighted kappa coefficient between the first and last ratings was 0.84 for children less than 6 years old and 0.89 for children at least 6 years old, indicating excellent chance-corrected agreement. Children initially classified in Levels I and V were least likely to be reclassified. There was a tendency for children younger than 6 years who were reclassified to be done so to a lower level of ability. The results provide evidence of stability of the GMFCS.

Motor performance and movement quality were quantitatively examined (Zurich Neuromotor Assessment: timed motor performances and associated movements) in 87 prospectively enrolled very-low-birthweight (VLBW; <1250g) children (38 males, 49 females; mean birthweight 1016.2g [SD 141.5]:, range 720–1240g; mean gestational age 28.7wks [SD 2], range 25.7–33.4wks) at 6 years of age. All motor tasks were below the reference population: pure motor (median z-score) –0.46; adaptive fine motor (pegboard) –0.99; adaptive gross motor –0.88; static balance –0.48; and associated movements –1.90. All tasks correlated with the degree of neurological abnormalities (p[les ]0.004). VLBW children with no neurological abnormality also performed below the 10th centile and associated movements occurred more frequently than in the reference population (odds ratio 18, 95% confidence interval 6.7–47.9). Severity of periventricular leukomalacia and intraventricular haemorrhage assessed by ultrasound was associated with adaptive fine and gross motor tasks. We conclude that speed of motor performance and movement quality in particular were substantially impaired in VLBW children and are related to the degree of neurological abnormalities and neonatal cerebral injury.

Fifty-seven children with cerebral palsy (CP) and imaging evidence of vascular thrombosis (study group) and 167 children with CP and other imaging finds (control group)were selected. Sixty-one per cent of the study group were male and 53 (93%) had spastic hemiplegia compared with the control group, of whom 55% were male and 54 (32%) had a diagnosis of spastic hemiplegia. Mean age was 5 years 11 months (SD 5y 1mo) for the study group and 7 years 7 months (SD 4y 7mo) for the control group. Blood spots on Guthrie cards or buccal swabs were used to test both groups and their mothers for the factor V Leiden (fVL) mutation, which predisposes carriers to thrombophilia. Mothers were interviewed to gather antenatal, perinatal, demographic, and socio-economic data. The frequency of the fVL mutation in children with evidence of vascular thrombosis and their mothers was not statistically different from the frequency in children with CP with other imaging findings and their mothers. The frequency of the fVL mutation was significantly higher than the expected population frequency of 4% in the study group (10.5%, p=0.012) and in mothers of the control group (7.2%, p=0.036).

This study aimed to investigate the prevalence of sleep problems in Chinese children with pervasive developmental disorders (PDD) in Hong Kong and their relationship to parental stress. A cross-sectional survey was conducted in six child assessment centres. All parents of the children with PDD completed the Children's Sleep Habits Questionnaire, the Parenting Stress Index – Short Form, and questions on sleep practice. A total of 210 out of 250 questionnaires (response rate 84%) were returned. Mean age of the children was 3 years 6 months (SD 1y 4mo; range 2y–7y 7mo; 168 males, 25 females). The prevalence of parent-defined sleep problems in various sleep domains ranged from 9.3 to 45.6%, with 67.9% of children having significant problems in at least one sleep domain. The most common problems reported were bedtime resistance and parasomnias. Similar sleep problems occurred in all the PDD subgroups. The factor most significantly associated with sleep problems was the occurrence of sleep problems before the age of 2 years. The parents of children with PDD with sleep problems experienced a higher level of stress than those whose children had no sleep problems. A high prevalence of significant sleep problems was reported in Chinese children in Hong Kong with PDD. A higher stress level among the parents of those children with PDD with sleep problems suggests the need for systematic early detection and management of sleep problems in children with PDD.

The aim of this study was to determine whether botulinum toxin A (BTX-A) injected into the parotid and submandibular salivary glands of children aged 6 to 16 years with cerebral palsy (CP) and relative sialorrhoea significantly decreases their drooling and improves their quality of life. Twenty children (10 males, 10 females; mean age 10y 1mo [SD 3y 8mo]; range 6y 1mo–16y 7mo) with CP, identified as having significant daily drooling (scoring at least 6 on drooling frequency/severity scales) were recruited. BTX-A (2U/kg; maximum 70U) was injected under sedation, using ultrasound guidance (1.4U/kg and 0.6U/kg divided between parotid and submandibular glands respectively). Nineteen children completed the study: 15 with spastic quadriplegia (Gross Motor Function Classification System [GMFCS] Level V); one with dystonia (GMFCS Level V); one with hemiplegia (GMFCS Level II); and two with a mixed pattern of CP (GMFCS Levels III and V). Drooling was assessed by five methods at baseline, and 4 and 12 weeks after injection. Qualitative assessment of drooling frequency and severity scores showed statistically significant reductions at 4 weeks (p<0.001) and 12 weeks (p=0.006). Qualitative assessment of quality of life scores (rated by parents and teachers separately) also significantly improved (p<0.001 and p=0.023 respectively). Quantitative assessments showed that the number of bibs/scarves changed per day was significantly reduced at 4 weeks (p<0.001). There was no side effect from the injections themselves; 89% of parents and children wished for further intrasalivary BTX-A injections in the future. We conclude that percutaneous intrasalivary BTX-A injections into the parotid and submandibular salivary glands can reduce drooling in children with CP and relative sialorrhoea, leading to an improvement in their quality of life.

Upper limb impairment can affect the ability to perform and participate in activities of daily living. The level of activity and participation limitation in the home environment for children with hemiplegic cerebral palsy (CP) is poorly understood. A greater understanding of these limitations could be used to provide targeted and appropriate intervention programmes. Level of activity and participation limitation were investigated, with the use of the Assessment of Motor and Process Skills (AMPS) in a representative sample of 54 South Australian children (31 males, 23 females) with hemiplegic CP, aged 3 to 12 years (mean age 7y 4mo [SD 2y 5mo]). Two AMPS tasks that were familiar to the child were performed in the home under the supervision of a trained occupational therapist. Findings suggested that younger children (3–8y) performed significantly better than older children (9–12y) for motor skill ability (0.46 vs 0.09, p=0.041) but not for process skill ability (–0.25 vs –0.28, p=0.885). Functional performance for the sample was below that of age-matched normative data. Motor performance in activities of daily living as detected by the AMPS seemed to worsen with age in children with hemiplegic CP. Further investigation into what can influence this outcome is required.

The level of agreement between clinicians' ratings of gait patterns in children with spastic hemiplegia was investigated using the Winters, Gage, and Hicks (WGH) classification scale. Sixteen clinicians (nine physiotherapists, seven orthopaedic surgeons) from six gait laboratories in five different countries rated gait patterns in 34 children with spastic hemiplegia (22 males, 12 females; mean age 10y [SD 3y], range 6–18y). Archived three-dimensional gait reports (kinematic and video data) and videos (video clips only) were rated for each child. Agreement between clinicians was substantial for reports (weighted kappa [wκ] 0.77, range 0.62–0.89). Agreement was lower for videos (wκ 0.63, range 0.39–0.97) and for clinicians' agreement between reports and corresponding videos (wκ 0.62, range 0.47–0.76). Exact agreement was unacceptable for some gait patterns using reports (mean 65%, range 32–74%) and videos (mean 53%, range 35–94%). Not all gait patterns could be rated using the original WGH categories. It was concluded that: (1) agreement is acceptable using the WGH tool; (2) kinematic data from 3D instrumented gait analysis and video should be used together when using the WGH scale; and (3) further refinement of this classification is required.

The many concerns of parents of children with hydrocephalus have not previously been assessed using a reliable, quantitative measurement. The development and preliminary use of a questionnaire to measure the concerns of these parents is reported. A general health status measure for hydrocephalus was developed concurrently. Items were generated primarily from parent focus groups resulting in a comprehensive list of 22 unique parental concern items. The most important of these items, as selected by another sample of parents of 60 children (27 females, 33 males; mean age 8y 4mo, [SD 4y 6mo]) were retained for the final questionnaire. This 9-item parental concerns questionnaire was tested for reliability and construct validity (by assessing correlations with various independent measures of health status, including Health Utilities Index–2 and Impact-on-Family Scale) using a cohort consisting of the parents of 80 children (39 females, 41 males; mean age 10y, [SD 3y 6mo]). An exploratory analysis was performed to determine which patient variables were associated with greater parental concern. The parental concerns questionnaire demonstrated excellent test–retest reliability (0.86, 95% CI 0.77–0.92) and internal consistency (Cronbach's alpha=0.89). As expected, interrater reliability was somewhat lower (0.67, 95% CI 0.49–0.79). Construct validity was demonstrated by consistently moderate Pearson's correlations with several independent measures of the child's health. The presence of epilepsy and younger patient age appeared to be significantly associated with greater parental concern. This new questionnaire has demonstrated very good psychometric properties. It can be used to broaden the scope of future clinical research in pediatric hydrocephalus to include the impact on parents.

The Unilateral Below Elbow Test (UBET) was developed to evaluate function in bimanual activities for both the prosthesis wearer and non-wearer. Nine tasks were chosen for each of four age-specific categories defined by development stages of hand function (2–4y, 5–7y, 8–10y, and 11–21y). Two scales, Completion of Task and Method of Use, were designed to rate performance. To measure reliability, four occupational therapists scored samples of videotaped UBET performances. For Completion of Task, an interval scale, agreement in scoring was measured with interclass correlation coefficients (ICC; n=9; five females, four males). For Method of Use, a nominal scale, chance-adjusted association was calculated with Cohen's kappa coefficients (interobserver n=198; 111 females, 87 males; intraobserver n=93; 56 females, 37 males). For Completion of Task, the average ICC was 0.87 for the prosthesis-on condition, and 0.85 for the prosthesis-off condition. ICCs exceeded 0.80 for eight out of nine tasks for the two older age groups, but for only five out of nine tasks in the younger age groups. Higher inter- and intraobserver kappa coefficients for Method of Use resulted when scoring children with their prostheses on versus off. The oldest age group had lower kappa values than the other three groups. The UBET is recommended for the functional evaluation of Completion of Task in children with unilateral congenital below elbow deficiency with and without their prostheses. Method of Use scoring can evaluate individuals for directed therapy interventions or prosthetic training.

The lack of evidence regarding functional and quality of life benefits resulting from tone reduction with intrathecal baclofen (ITB) infusion treatment relates to the lack of validated and responsive measures. We integrated our scale/questionnaire, developed from chart review, with the non-validated Caregiver Questionnaire (CQ) to yield a final document, the Care and Comfort Hypertonicity Questionnaire (CCHQ). Convergent validity was achieved by administering the CCHQ to 47 patients with spastic/dystonic cerebral palsy (CP) who were being evaluated for tone management. The population studied included 18 females and 29 males (mean age 10y [SD 4y 10mo]; range 3y 1mo–21y 1mo). Twenty-five patients were subsequently referred for botulinum toxin (BTX-A) injections (mean Gross Motor Function Classification System [GMFCS] 3.2); 11 patients were referred for ITB (mean GMFCS 4.4); four were referred for orthopedic surgery (mean GMFCS 3.3); 3 were referred for selective dorsal rhizotomy (mean GMFCS 2.7); one was recommended for oral baclofen (GMFCS 5); and three were recommended for no treatment (mean GMFCS 3.7). Blinded to the score, those with the highest scores (severe hypertonicity) were recommended for ITB; those with the lowest scores were recommended for BTX-A injections. Responsiveness of the CCHQ was established by administering the questionnaire to patients who already had an implanted ITB pump. The children with the largest dose increase demonstrated a statistically significant improvement in the CCHQ score. This scale can be used to document the efficacy of treating severe hypertonicity both in clinical and research protocols.

This study investigated whether in preterm children who had ventricular dilatation (VD) on neonatal cranial ultrasound outcome at age 8 years was influenced by the additional presence of germinal matrix haemorrhage – intraventricular haemorrhage (GMH–IVH). Six-hundred and ninety-nine preterm infants (<33wks' gestation, mean 29.6wks [SD 2.1]) with either normal cranial ultrasound (n=616; 286 females, 330 males), or with VD with (n=66; 32 females, 34 males) or without (n=17; 4 females, 13 males) GMH–IVH were enrolled in the study. At age 8 years outcome was assessed in 567 (81%) of the 699 children by neurological examination, the Test of Motor Impairment (TOMI), the test of Visuo-Motor Integration (VMI), and the Wechsler Intelligence Scales for Children. Results showed that the proportion of children with disabling impairments was higher in the group with VD and GMH–IVH. Performance on TOMI and VMI (even in those without disabling impairments) was poorer in those with VD and GMH–IVH than in children with normal scans or those with VD only. Children with VD and GMH–IVH had significantly lower performance IQ than children with normal ultrasound, whereas those with VD only were not different from those with normal scans. Results suggest the presence of subtle white matter injury that has not been identified by neonatal cranial ultrasound. Although this study did not investigate biochemical markers of haemorrhage, we hypothesize that non-protein-bound iron is likely to be a contributing factor to white matter damage in preterm infants.

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.

To spare more children from painful muscle biopsy, a new non-invasive diagnostic motor performance test is undergoing development. Fifteen functional items were used to measure muscle strength and muscle endurance in 68 patients (47 males, 21 females; mean age 7y 8mo, SD 2y 2mo; range 4 to 11y), who had been referred to our specialist centre in the past 3 years on suspicion of myopathy. All the patients had undergone muscle biopsy. To correct the patients' outcomes for age, sex, and body size, regression prediction equations were obtained from a stratified random sample of 64 normally developing primary-school children aged 4 to 11 years (32 males, 32 females; mean age 8y 1mo, SD 2y 4mo). Feasibility was evaluated on the basis of five criteria. Validity was assessed using logistic regression analysis, receiver operating characteristic analysis, and sensitivity and specificity at a specifically chosen cut-off point. Reproducibility was evaluated by test–retest reliability in a stratified random sample of 40 patients who returned for re-measurements using the intraclass correlation coefficient. Seven items satisfied all five feasibility criteria, had high diagnostic power, and high test–retest reliability. The motor performance test can improve diagnostic procedure in children suspected of having myopathy.