The Paediatric Pain Profile (PPP) is a 20-item behaviour rating scale designed to assess pain in children with severe neurological disability. We assessed the validity and reliability of the scale in 140 children (76 females, mean age 9 years 11 months, SD 4 years 7 months; range 1 to 18 years), unable to communicate through speech or augmentative communication. Parents used the PPP to rate retrospectively their child's behaviour when ‘at their best’ and when in pain. To assess interrater reliability, two raters concurrently observed and individually rated each child's behaviour. To assess construct validity and responsiveness of the scale, behaviour of 41 children was rated before and for four hours after administration of an ‘as required’ analgesic. Behaviour of 30 children was rated before surgery and for five days after. Children had significantly higher scores when reported to have pain than ‘at their best’ and scores increased in line with global evaluations of pain. Internal consistency ranged from 0.75 to 0.89 (Cronbach's alpha) and interrater reliability from 0.74 to 0.89 (intraclass correlation). Sensitivity (1.00) and specificity (0.91) were optimized at a cut-off of 14/60. PPP score was significantly greater before administration of the analgesic than after (paired-sample t-tests, p<0.001). Though there was no significant difference in mean pre- and postoperative scores, highest PPP score occurred in the first 24 hours after surgery in 14 (47%) children. Results suggest that the PPP is reliable and valid and has potential for use both clinically and in intervention research.

Clean intermittent catheterization (CIC) is the mainstay of management in neuropathic vesicourethral dysfunction, both to improve continence and, more importantly, to preserve renal function. We looked at the effects of this procedure on children, adolescents, and their families. In particular, we wished to see if there were any differences between those who successfully catheterized and those who did not. Forty families were enrolled into the study. Ages of children and adolescents (23 females, 17 males) ranged from 1 to 20 years. Most participants (n=31) had spina bifida. Other causes of bladder dysfunction included transverse myelitis, spinal cord injury, and spinal neuroblastoma. Parents were assessed using the Effects of Handicap on Parents semi-structured interview, the Socioemotional Functioning Interview, and a semi-structured interview, specifically designed for the study, which looked at family characteristics and experience related to diagnosis and catheterization. In addition, the Rutter Parental ‘A’ Scale Questionnaire was used to screen for emotional and behavioural disorders in the child. Results showed that CIC by carer or self- catheterization itself did not cause major emotional and behavioural problems but the bladder problem may act as a focus that puts considerable strain family relationships. Although most parents disliked CIC they complied with the suggested management. It is important that all those involved understand the aims of management and success can be achieved by combined input from medical, psychological, and specialist nursing staff. The problem is lifelong and continued support from a multidisciplinary team is essential.

Pain frequency and its association with participation, function, and health-related quality of life were evaluated in a sample of 198 children (58% male; mean age 10 years 7 months, SD 3 years 11 months; range 5 to 18 years) with moderate to severe cerebral palsy (CP). Gross Motor Functional Classification System (GMFCS) levels were III to V (23% level III, 23% level IV, 31% level V without a gastrostomy tube, and 23% were level V with a gastrostomy tube). Assessment was carried out using the parent-report Child Health Questionnaire (CHQ) and questions assessing health, medical utilization, and medication use. Pain frequency ranged from ‘none’ to ‘every day’. Eleven percent of parents reported pain almost every day (z score=–0.38, p<0.001). Pain was related to severity of motor impairment and the presence of a gastrostomy; parents of the most severely affected children reported the highest pain frequency (p=0.05). Pain was correlated with school days missed (p=0.03) and days in bed (p=0.01). Children taking gastrointestinal medications were reported to have greater pain frequency (p<0.001). The Parental Impact – Emotional subscale of the CHQ was correlated with reported pain (r=0.38, p<0.001). Pain was frequent in children with moderate or severe CP. Pain was more prevalent with more severe impairment and was associated with educational and social consequences.

Autism is a neurodevelopmental disorder with strong genetic influences. Clinical experience and limited empirical evidence support the view that autism may be associated with aberrant interhemispheric information transfer. This empirical controlled study examined whether, at neuropsychological testing, children with autism showed problems with interhemispheric information transfer. The study included auditory, visual, and motor measures covering information transfer within, as well as across, modalities. Thirty children (24 males, 6 females; mean age 12 years 8 months, SD 2 years 8 months; range 9 years 5 months to 17 years 5 months) without learning disability but with autism spectrum disorders were compared with 30 children from a mainstream school matched for age, sex, and IQ>75. Children with autism spectrum disorder performed significantly worse than the comparison group on most of the tests (p=0.02 for auditory perception and attention, p=0.005 for visual perception, p=0.0001 for motor control, p=0.04 for tactile perception). Results support the notion that aberrant interhemispheric transfer may be involved in the pathogenesis or clinical course of autism. The findings were not accounted for by lower IQ in the group with autism.

This study explored the relation between a motor-free visual perceptual deficit, different visual–motor integration deficits, and different motor skills in children with developmental coordination disorder (DCD). Thirty-six children (22 males), aged 9 or 10 years, with DCD and a control group (n=36), matched for age and sex, were assessed with the Movement Assessment Battery for Children (MABC), a ball-catching test, a jumping test, a timed response task to a visual moving stimulus, and the Beery-Buktenica Developmental Test of Visual–Motor Integration, incorporating copying, visual discrimination, and tracing tasks. Children with DCD performed significantly worse than the control group on all measures. The visual discrimination task did not correlate significantly with any of the motor tasks. The visual timing task correlated significantly with the ball-catching test in the DCD group. The copying test was significantly correlated with the MABC in the DCD group. The association between visual–perceptual deficits and motor tasks was shown to be task specific.

The aim of this study was to assess the association between cocaine or cigarette smoke exposure in utero and visual outcome. A total of 153 healthy infants (89 males, 64 females; gestational age range 34 to 42 weeks) were prospectively enrolled in a masked, race-matched study. Quantitative analyses of urine and meconium were used to document exposure to cigarette smoke and cocaine. Infants with exposure to other illicit drugs, excepting marijuana, were excluded. At 6 weeks of age, grating acuity and visual system abnormalities (VSA; eyelid oedema, gaze abnormalities, and visual inattention) of 96 infants from the original study sample were assessed with the Teller acuity card procedure and a detailed neurological examination. Neither cocaine nor cigarette smoke exposure was associated with acuity or VSA. However, VSAs were associated with abnormal neurological examination, independent of drug exposure and other risk factors (odds ratio 7.9; 95% confidence interval 2.0 to 31.5; p=0.004). This unexpected finding could prove a helpful clinical marker for the infant at risk for neurological abnormalities.

The aim of this study was to investigate the relationship between motor performance, attention deficit, impulsiveness, and hyperactivity in children with attention-deficit–hyperactivity disorder (ADHD). Participants were 42 school-aged children with ADHD (36 males, 6 females; mean age 8 years 2 months, SD 1 year 2 months; range 6 years to 11 years), and 42 age- and sex-matched children without ADHD (mean age 8 years 3 months, SD 1 year 1 month; range 6 years to 11 years). Motor abilities were assessed with the Bruininks-Oseretsky Test of Motor Proficiency. Attention and impulse control were measured with the Gordon Diagnostic System, and assessment of activity level was based on two Activity Level Rating Scales that were completed by parents and teachers. Analysis by t-test revealed a significant difference between children with and without ADHD in fine and gross motor skills, impulse control, and attention. Stepwise regression indicated that attention, impulse control, and parent ratings of activity level were the three best predictors of gross motor skills for children with ADHD, accounting for 55.9% of the variance. Attention and impulse control were the two best predictors of fine motor skills, accounting for 45.7% of the variance. Attention and impulse control were consistently found to be important predictors of both fine and gross motor skills in children with ADHD. However, the fact that activity level was a predictor for gross motor proficiency but not for fine motor tasks suggests that different behavioral processes are involved in fine and gross motor performance to different extents.

Botulinum toxin type A (BTX-A), serial casting, and combined treatment for spastic equinus were compared by chart review. Fifty-five patients with diplegia (n=21), hemiplegia (n=17), quadriplegia (n=6), and other diagnoses (n=11) were reviewed. Thirty-one patients had Gross Motor Function Classification System (GMFCS) scores of I or II, 10 had a GMFCS score of III, and eight had GMFCS scores of IV or V (for six there were no available data). Mean age was 7 years 1 month (SD 4 y 8mo). The combined group showed a significantly greater increase in passive range of motion (ROM) of the ankle joint in comparison with BTX-A alone (p=0.0002), with a mean of 17° (SD 11.2) versus 5° (SD 6.7), and casting alone showed a significantly greater increase in comparison with BTX-A alone (p=0.0001), with a mean of 15° (SD 4.5) versus 5° (SD 6.7). With cast number controlled, change in ROM after casting with and without BTX-A was not significantly different (p=0.37). There was no significant difference between casting with or without BTX-A, and both improved ROM to a greater degree than BTX-A alone. Casting demonstrated a significantly more robust impact on range of motion than BTX-A alone.

We investigated in children with attention-deficit–hyperactivity disorder (ADHD) the ability to programme and execute goal-directed arm movements. The sample consisted of 25 males with ADHD (mean age 11 years 6 months, SD 1 year 11 months, range 8 to 15 years) and 25 age-matched typically developing males. The children moved a cursor on a screen by moving a hand-held indicator on a horizontal digitizing tablet. Start and target positions on the screen were always visible during the movement. The screen cursor, however, could either be visible throughout the movement (visual feedback) or blanked at movement initiation (without visual feedback). Analysis showed that movement control was impaired in children with ADHD and that their problems were especially pronounced during the without-visual-feedback condition. In this condition, the children with ADHD exhibited large end-point errors and prolonged movement durations. As there can be no visual corrections of the movement during this condition, results indicate poorer motor programming in children with ADHD. Moreover, children with ADHD performed jerky movements and showed a reduced capacity to select a movement speed that met with the accuracy demands of the movement.

Deprivation of sensory input affects neurological development. Our objective was to explore clinically the role of hearing in development of sensorimotor integration and non-verbal cognition. The study involved 54 children (15 males, 39 females; 5 to 9 years old) with severe or profound bilateral prelocutive deafness but without neurological or cognitive impairment. Of these, 25 had received an early cochlear implant (CIm). Patients were compared with 40 children with normal hearing. All were given a battery of non-verbal neuropsychological tests and a balance test, and were timed for simple and complex movement of limbs. Deafness, whether treated by CIm or not, resulted in a delay in development of complex motor sequences and balance. Lack of auditory input was also associated with lower, but non-pathological, scores in visual gnoso-praxic tasks and sustained attention. Such differences were not observed in children with CIm. Hearing contributes to clinical development of spatial integration, motor control, and attention. An early CIm enables good verbal development and might also improve non-verbal capacities.

The aim of this study was to determine the reliability of family reports for the Gross Motor Function Classification System (GMFCS), a condition-specific discriminative measure of severity of movement disability for children with cerebral palsy (CP). We conducted a cross-sectional survey using a short questionnaire with families of children with CP for whom we already had ratings of GMFCS level made by a health professional. We assessed the potentially confounding effect of whether the family had discussed the GMFCS with a professional. Two hundred and one questionnaires were posted to families of which 97 (48%) were completed and returned. Mean age of the children (53 males, 40 females) was 9 years 5 months (SD 1 year 1 month), range 6 to 11 years. Children of the families who responded encompassed the spectrum of types and distribution of impairment and severity of movement disability. The intraclass correlation coefficient (ICC) of agreement between professionals and families who had discussed their child's GMFCS level with a health professional (n=35) was 0.97 (95% confidence interval [CI] 0.96 to 0.98); for those who had not (n=52) the ICC was 0.92 (95% CI 0.91 to 0.93); and for the whole sample (n=93) the ICC was 0.94 (95% CI 0.90 to 0.96). Stability between ratings made by health professionals for children when they were in the 4 to 6 year age band of the GMFCS and ratings made by families for the same children when they were in the 6 to 12 year age band (n=35) was ICC=0.96 (95% CI 0.95 to 0.97). The excellent agreement demonstrated in this study suggests that family reports of the GMFCS made by using our questionnaire provide a reliable method for measuring gross motor function in children between 6 and 12 years old. This might be more efficient for observational studies of large populations, experimental research, or community health administration than direct observation, particularly when professional assessment is not feasible.

Parental report may provide an inexpensive alternative to standardized assessments of children's development. We have adapted and validated a parental questionnaire on cognitive development for use with very preterm infants. Sixty-four 2-year-olds (28 males, 36 females) born less than 30 weeks' gestation (median 28.5 weeks, range 23 to 31 weeks), median birthweight 980g (range 500 to 1905g) were assessed using the Mental Development Index (MDI) of the Bayley Scales of Infant Development-II and the parental questionnaire. Significant correlations between parent report and MDI scores (r=0.54–0.68, p<0.001) indicated good concurrent validity. Diagnostic use of the parent report for predicting an MDI score of less than 70 was assessed by using receiver operating characteristic (ROC) curves. The optimal cut off produced equal sensitivity and specificity (81%), indicating good discriminatory power in diagnosing developmental delay. Test–retest reliability was demonstrated and accuracy of parent reporting was not affected by sociodemographic factors. Our questionnaire provides a valid outcome measure for use in randomized trials or large population surveys.

Cortical visual impairment (CVI) refers to bilateral impairment of vision that is usually due to damage occurring perinatally in the visual cortex and/or optic radiations. The most common cause of this damage is hypoxia, and other causes include encephalitis, meningitis, and trauma. Relatively little research has been done to quantify visual abilities in children with CVI. In the present study, we used an electrophysiological technique (visual evoked potentials) to measure two aspects of spatial vision in 35 infants and children with CVI (15 females, 20 males; mean age 3 years 6 months, SD 3 years 5 months; age range 4 months to 16 years). We measured each child's grating acuity (resolution for detecting high-contrast stripe patterns) and vernier acuity (resolution for localizing pattern elements). Performance on grating acuity and vernier acuity in individuals with CVI was compared with that of age-matched individuals with normal vision, and it was found that vernier acuity was relatively lower than grating acuity in children with CVI. Results support the theory that vernier acuity is cortically mediated, and suggest that vernier acuity is a more sensitive measure than grating acuity for quantifying vision deficits in patients with CVI.

The relationships between different levels of severity of ambulatory cerebral palsy, defined by the Gross Motor Function Classification System (GMFCS), and several pediatric outcome instruments were examined. Data from the Gross Motor Function Measure (GMFM), Pediatric Orthopaedic Data Collection Instrument (PODCI), temporal–spatial gait parameters, and oxygen cost were collected from six sites. The sample size for each assessment tool ranged from 226 to 1047 participants. There were significant differences among GMFCS levels I, II, and III for many of the outcome tools assessed in this study. Strong correlations were seen between GMFCS level and each of the GMFM sections D and E scores, the PODCI measures of Transfer and Mobility, and Sports and Physical Function, Gait Velocity, and Oxygen Cost. Correlations among tools demonstrated that the GMFM sections D and E scores correlated with the largest number of other tools. Logistic regression showed GMFM section E score to be a significant predictor of GMFCS level. GMFM section E score can be used to predict GMFCS level relatively accurately (76.6%). Study data indicate that the assessed outcome tools can distinguish between children with different GMFCS levels. This study establishes justification for using the GMFCS as a classification system in clinical studies.

This paper describes part of larger study involving 40 families of children and adolescents with neuropathic bladder looking at the psychosocial impact of catheterization on them and their families. This study focuses on their experiences of clean intermittent catheterization (CIC) and self-catheterization (SC). Twenty-eight children and adolescents (11 males, 17 females; mean age 9 years, SD 2 years 7 months, range 5 to 20 years) with neuropathic bladder (mostly with spina bifida) participated in a semi-structured interview to explore their understanding of micturition and catheterization, and their views about the introduction and practical use of this technique. Participants were invited to draw self-portraits and figure diagrams to depict their understanding of CIC and SC. Age-appropriate self-esteem measures (Harter Pictorial Scale of Perceived Competence, the Self-Perception Profile, and the Culture-Free Self-Esteem Inventory) were administered to see if catheterization status affected emotional well-being. In an extension of this study, children's experiences of SC were explored by a postal questionnaire to all children successfully using SC who were attending the Paediatric Neuropathic Bladder Clinic. Children aged <5 years, those with learning difficulties, and those using indwelling catheters were excluded. From a total sample of 66 (31 females, 35 males), 52 parents and 42 children and adolescents (28 females and 14 males) responded. No significant difference was observed in self-esteem for those successfully catheterizing. Specific challenges involved learning SC and practical use of the technique. Concerns were leakage and being wet, and peers finding out about their continence management. Implications for the education, problem solving, and support of families and young people to promote cooperation and optimal benefits from catheterization are discussed.

We assessed the architecture of the medial gastrocnemius in nine children (five males, four females; age range 6 to 15 years; mean 10 years 10 months, SD 3 years 6 months) with spastic diplegia by ultrasound imaging before and after a gastrocnemius recession. The children were ambulant (seven independent, one with a posterior walker, one using crutches) before and after surgical intervention. We compared values for fascicle lengths and deep fascicular-aponeurosis angles with those from a group of normally developing children (five males, five females; age range 6 to 11 years; mean 8 years 4 months, SD 1 year 4 months). Despite a variable interval between assessments (from 56 to 610 days), fascicles were shorter (p=0.00226) and the deep fascicular-aponeurosis angle increased (p=0.0152) after intervention. Fascicle lengths of patients were similar to those in the group of normally developing children before surgery. After surgery, fascicles in the group of children with spastic diplegia were shorter than in their normally developing peers (p=0.00109). The gastrocnemius recession procedure alters muscle architecture, though the degree of fascicular shortening varied, with four of the participants in our study losing less than 10% of their original fascicular length at maximum dorsiflexion. Increases in ankle-joint power in walking, observed after surgical intervention in children with spastic diplegia, may be due to a more normal ankle position rather than to improvements in the active mechanical performance of the gastrocnemius.

To investigate whether the decrease in mean birthweight of the smallest infants surviving with a very low birthweight (VLBW) in the past two decades is associated with an increase in intellectual deficit rates, we compared the results obtained in two different follow-up studies: one included a cohort of children of VLBW (<1500g; n=102, 52% male) born in the early 1980s; the other included a cohort of children of extremely low birthweight (ELBW; <1000g; n=183, 46% male) born in the mid-1990s. Intellectual development was evaluated in both cohorts. McCarthy Scales of Children's Abilities were used in the cohort from the early 1980s; Wechsler's Preschool and Primary Scale of Intelligence – Revised was used in the cohort from the mid-1990s. The children were assessed at age 4–5 years, and a control group was included for each of the two studies. Mean scores in the measures of intelligence found at the assessment were recalculated to standard deviation scores from the two control groups. We found no difference in the standard deviation scores between the two cohorts despite the mean birthweight being 353g (p<0.001) lower in the cohort from the mid-1990s, and the overall survival rate of infants of VLBW having increased. Our results suggest that the improved survival of the smallest babies in the 1980s and 1990s has been achieved without an increase in the intellectual deficit in the surviving children.

The recently endorsed World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) is proposed as a new tool to describe human functioning and health in a multiprofessional approach for individuals of all ages. Its application to paediatric neuro-rehabilitation may be of great help, especially in children with complex disabilities. However, experience with its application in this field is still limited. We tested the application of ICF in 40 children (26 males, 14 females; age range 3 to 18 years; mean age 11 years 1 month, SD 5 years 3 months) with various types and degrees of disability. We tested the applicability and reliability of the classification, and studied its correlation with well-established measures of functioning (Verbal IQ; gross motor function measure; functional independence measure). The ICF proved to be applicable and reliable, and strongly correlated with established scales. However, several of the Activity and Participation components do not fully capture the developmental nature of many abilities of children. Our study, although acknowledging the universal application of the ICF, and the ICF's value as a clinical tool, calls for its specific adaptation to accommodate better the peculiarities of child functioning and disability.

Most studies of impaired hand function in children with hemiplegic cerebral palsy (CP) have focused on either the involved or the non-involved extremity in isolation. Coordination of the involved and non-involved hand during bimanual tasks in these children is not well understood. The present study examined bimanual coordination using a drawer-opening task under speed and hand constraints in 10 children with hemiplegic CP (5 males and 5 females, mean age 13y 5mo, range 8y to 16y) and 10 age-matched right-handed developing typically children (6 males and 4 females, mean age 13y 1mo). Children were asked to reach forward and open a drawer with one hand and then activate a light switch inside the drawer with the contralateral hand. The role of the two hands (open drawer and activate switch) and speed (self-paced vs fast-as-possible) were varied. The children with hemiplegic CP were slower (p<0.001) and less coordinated in this task, with reduced movement overlap of the two hands (p<0.001) and sequential completion of the two movement objectives (p<0.001). Moreover, the hand used for each task subcomponent affected task performance for the children with hemiplegic CP (p<0.05). Interestingly, faster speed facilitated better bimanual coordination for the children with hemiplegic CP (p<0.001). Results highlight the importance of movement constraints on task performance and suggest that movement speed might facilitate better bimanual coordination.

The Carnegie Council on Adolescent Development, USA has identified activities, beliefs, and perceptions critical for healthy development. The aim of this study was to measure the activities, beliefs and expectations, and perceived outcomes of adolescents with myelomeningocele. In this descriptive study, 66 adolescents with myelomeningocele, aged 12 to 21 years and functioning at grade level, completed a structured interview. Thirty-eight (58%) of the participants were female. The level of lesion was distributed as follows: 30% had thoracic level lesions, 32% had lumbar level lesions, 15% had lumbosacral level lesions, and 23% of the sample had sacral level lesions. Instruments used had both established reliability and validity (WeeFIM, Harter's Self-Perception Profile, Austin's Child Attitude Toward Illness Scale, and Snyder's Hope Scale, Adolescent Decision-Making Inventory, Adolescent Coping Scale) or were scales developed for this study (Adolescent Activities Inventory, Future Expectations Scale, Communication Efficacy, and Adolescent Self-Management and Independence Scale). Scale reliabilities ranged from 0.70 to 0.88. These participants, though hopeful and positive in their attitudes toward myelomeningocele and generally able to perform activities of daily living independently, are not engaging in the full range of adolescent activities (decision making, friendship activities, and household responsibilities) and achieving positive outcomes (self-management and job) necessary to make a successful transition to adulthood. This might explain why so many individuals with myelomeningocele are underemployed and are not living independently as young adults.