The timed ‘Up & Go’ test (TUG) is a test of basic or functional mobility in adults which has rarely been used in children. Functional mobility was defined for this study as an individual's ability to manoeuvre his or her body capably and independently to accomplish everyday tasks. Reliability and validity of TUG scores were examined in 176 children without physical disabilities (94 males, 82 females; mean age 5y 9mo [SD 1y 8mo]; range 3 to 9y) and in 41 young people with physical disabilities due to cerebral palsy or spina bifida (20 males, 21 females; mean age 8y 11mo [SD 4y 3mo], range 3 to 19y). Mean TUG score for children without physical disability was 5.9s (SD 1.3). Reliability of the TUG test was high, with intraclass correlation coefficients (ICC) of 0.89 within session, and 0.83 for test–retest reliability. Mean score of the group aged 3 to 5 years was significantly higher (6.7s SD 1.2) than that of the older group (5.1s, SD 0.8; p=0.001). Scores in the younger group reduced significantly over a 5-month follow-up period (p=0.001), indicating that the TUG was responsive to change. Within-session reliability of the TUG in young people with disabilities was very high (ICC=0.99). There were significant differences in TUG scores between children classified at levels I, II, and III of the Gross Motor Function Classification System (p=0.001). TUG scores showed a moderate negative correlation with scores on the Standing and Walking dimensions of the Gross Motor Function Measure (n=22, rho=–0.52, p=0.012). There was no significant difference in TUG scores between typically developing male and female children. The TUG can be used reliably in children as young as 3 years using the protocol described in this paper. It is a meaningful, quick, and practical objective measure of functional mobility. With further investigation, the TUG is potentially useful as a screening test, an outcome measure in intervention studies for young people with disabilities, a measure of disability, and as a measure of change in functional mobility over time.

This study examined the evolution of individuals with cerebral palsy (CP) from childhood to adulthood. Seventy-two adults with a diagnosis of CP born between 1934 and 1980 were studied. Individuals were recruited and data comprehensively collected using case notes and through direct assessments of the majority of participants from three rehabilitation units in Bologna, Padua, and Rovigo in Italy. The main findings can be summarized as follows: contact with health and rehabilitation services was radically reduced once individuals reached adulthood; more individuals who were integrated into mainstream schools achieved and maintained literacy than those who had attended special schools; in a high number of participants, motor performance deteriorated once into adulthood. Independent walking or other forms of supported locomotion were lost in many on reaching adulthood. Of those who continued to walk, walking deteriorated in terms of distance. It was concluded that even though CP has been considered as predominantly a childhood pathological condition, the evolution of the effects of CP do not stop at 16 or 18 years of age. For this reason, the traditional child- (or infant-) oriented approach concentrating mainly or exclusively on the achievement of independent walking, may not be an ideal approach to children with CP. Instead a more independence-oriented therapeutic approach would be appropriate.

Constraint-induced (CI) movement therapy is a promising therapy for improving upper limb function in adults after stroke. It involves restraint of the non-involved limb and extensive movement practice with the involved limb. In this study, a single-blinded, randomized, control study was performed to examine the efficacy of CI therapy, modified to be child friendly, in children with hemiplegic cerebral palsy (CP). Twenty-two children (8 females, 14 males; mean age 6y 8mo [SD 1y 4mo]; range 4–8y) were randomized to either an intervention group (n=11) or a delayed treatment control group (n=11). Children wore a sling on their non-involved upper limb for 6 hours per day for 10 out of 12 consecutive days and were engaged in play and functional activities. Children in the treatment group demonstrated improved movement efficiency and dexterity of the involved upper extremity, which were sustained through the 6-month evaluation period, as measured by the Jebsen–Taylor Test of Hand Function and fine motor-subtests of the Bruininks–Oseretsky Test of Motor Proficiency (p<0.05 in both cases). Initial severity of hand impairment and testing compliance were strong predictors of improvement. Caregivers reported significant increases in involved limb frequency of use and quality of movement. However, there was no change in strength, sensibility, or muscle tone (p>0.05 in all cases). Results suggest that for a carefully selected subgroup of children with hemiplegic CP, CI therapy modified to be child-friendly, appears to be efficacious in improving movement efficiency of the involved upper extremity.

The aim of this study was to evaluate the effects of intrathecal bacolfen (ITB) on patients with severe generalized dystonia. Eighty-six participants ranging in age from 3 to 42 years (median age 13 years) with generalized dystonia refractory to oral medications were offered treatment with ITB. Dystonia was associated with cerebral palsy in 71% of participants. Response to ITB was tested by continuous infusions in 72%, and by bolus injections in 17% of participants who had both dystonia and spasticity. Ninety-one percent of participants responded to the screening infusion and 93% to the bolus injections. Pumps were implanted in 77 participants. Dystonia scores at 3, 6, 12, and 24 months were significantly decreased (p<0.005) compared with baseline scores. Dystonia scores were significantly lower in those with intrathecal catheters positioned at T4, or higher than in those with catheters at T6 or lower (p=0.005). Ninety-two percent of participants implanted with a pump retained their responses to ITB during a median follow-up of 29 months. Patient questionnaires indicated that quality of life and ease of care improved in 86% and speech improved in 33%. Side effects of ITB occurred in 26% of participants. Surgical complications occurred in 38% and included CSF leaks, infections, and catheter problems. ITB is probably the treatment of choice for generalized dystonia if oral medications are ineffective.

With an increasing number of paediatric quality of life (QOL) instruments being developed, it is becoming difficult for researchers and clinicians to select the most appropriate instrument. Reviews of QOL instruments tend to report only basic properties of the instruments such as domains and psychometric properties. This paper seeks to appraise critically the conceptual underpinnings of paediatric QOL instruments. A systematic review was conducted to identify QOL instruments for children aged 0 to 12 years, and to examine and compare their conceptual frameworks, definitions employed, and structure. Both generic and condition-specific measures were reviewed. Fourteen generic and 25 condition-specific QOL instruments were identified. Eleven types of definition of QOL and health-related QOL and three theories of QOL were identified. QOL was measured by a variety of domains including emotional, social and physical health, and well-being. Items commonly assessed difficulties, or intensity/frequency of feelings/symptoms, in contrast to positive aspects of life and happiness. The findings highlight the diversity that is apparent in the conceptualization of paediatric QOL and draw attention to the lack of empirical evidence for many of the fundamental assumptions. The impact of the conceptual underpinnings of the instruments on the resulting QOL scores is discussed.

The aim of this study was to describe and analyze gross and fine motor function and accompanying neurological impairments in children with cerebral palsy (CP) born between 1991 and 1998 in western Sweden. A population-based study comprised 411 children with a diagnosis of CP ascertained at 4 to 8 years of age. Gross Motor Function Classification System (GMFCS) levels were documented in 367 children (205 males, 162 females). Bimanual Fine Motor Function (BFMF) classification levels of 345 of the children and information on learning disability, epilepsy, visual and hearing impairments, and hydrocephalus from 353 children were obtained. For spastic CP, a new classification according to the Surveillance of Cerebral Palsy in Europe of uni- and bilateral spastic CP was applied. GMFCS was distributed at Level I in 32%, Level II in 29%, Level III in 8%, Level IV in 15%, and Level V in 16%. The corresponding percentages for BFMF were 30.7%, 31.6%, 12.2%, 11.9%, and 13.6% respectively. Learning disability was present in 40%, epilepsy in 33%, and severe visual impairment in 19% of the children. Motor function differed between CP types. More severe GMFCS levels correlated with larger proportions of accompanying impairments and, in children born at term, to the presence of adverse peri/neonatal events in the form of intracranial haemorrhage/stroke, cerebral infection, and hypoxic–ischaemic encephalopathy. GMFCS Level I correlated positively to increasing gestational age. We conclude that the classification of CP should be based on CP type and motor function, as the two combine to produce an indicator of total impairment load.

A parental questionnaire was used to investigate the outcome for children who had had ischaemic stroke, who were seen at Great Ormond Street Hospital, London between 1990 and 1996. The results of functional assessments carried out by a physiotherapist and an occupational therapist, and of quantitative evaluations carried out by a neuropsychologist were used for validation where possible. The relationship between clinical and radiological factors and outcome were examined. The children were aged between 3 months and 15 years at the time of stroke (median age 5 years) and the period of follow-up ranged from 3 months to 13 years (median duration 3 years). Of the 90 children for whom data were obtained, 13 (14%) had no residual impairments. Outcome was good in 37 children (40%) and poor in 53 (60%) (defined according to whether impairments interfered with daily life). Agreement, as measured by Cohen's kappa, was good or very good between the parents' responses and the qualitative measures provided by the medical professionals and the therapists, but only fair to moderate for the quantitative measures provided by the neuropsychologists. This may reflect different parental perceptions of the physical and cognitive aspects of outcome. Younger age at time of the stroke was the only significant predictor of adverse outcome.

Following agreement on definitions and classification, a central database was set up to include information on over 6000 children with cerebral palsy (CP) from 13 geographically defined populations in Europe. The overall rate for the period 1980 to 1990 was 2.08/1000 live births (95% CI 2.02 to 2.14). One in five children with CP (20.2%) was found to have a severe intellectual deficit and was unable to walk. Among babies born weighing less than 1500g, the rate of CP was more than 70 times higher compared with those weighing 2500g or more at birth. The rate of CP rose during the 1970s, but remained constant during the late 1980s. Future analyses will include data from children born in the 1990s. This collaborative work provides a powerful means of monitoring trends in birthweight-specific rates of CP and an infrastructure for research and service planning.

This study reviews the instruments used for the clinical assessment of spasticity in children with cerebral palsy, and evaluates their compliance with the concept of spasticity, defined as a velocity-dependent increase in muscle tone to passive stretch. Searches were performed in Medline, Embase, and Cinahl, including the keywords ‘spasticity’, ‘child’, and ‘cerebral palsy’, to identify articles in which a clinical method to measure spasticity was reported. Thirteen clinical spasticity assessment instruments were identified and evaluated using predetermined criteria. This review consists of reports on the standardization applied for assessment at different velocities, testing posture, and quantification of spasticity. Results show that most instruments do not comply with the concept of spasticity; standardization of assessment method is often lacking, and scoring systems of most instruments are ambiguous. Only the Tardieu Scale complies with the concept of spasticity, but this instrument has a comprehensive and time-consuming clinical scoring system.

The Children's Communication Checklist (CCC) was developed to provide an objective assessment of pragmatic aspects of children's communication difficulties. We aimed to (1) see whether the checklist provided valid and reliable information when completed by parents, and (2) consider its usefulness in a clinical context. Checklists were completed by parents and a professional who knew the child well for all 5 to 17-year-old referrals to a tertiary developmental paediatrics centre over a 31-month period. Children who were not yet speaking in sentences were excluded. From a sample of 151 children (81% male; mean age 8.7 years) with pervasive or specific developmental disorders, valid checklists were completed by 119 parents and 93 professionals. Reliability, as measured by internal consistency, was 0.7 or higher for most scales. Correlations between ratings for parents and professionals were in the range of 0.30 to 0.58 for individual pragmatic scales, with a correlation of 0.46 (n=82) for the pragmatic composite. For both parents and professionals, the pragmatic composite was lowest for children with a diagnosis of autism; intermediate for those with a diagnosis of Asperger syndrome, pervasive developmental disorder not otherwise specified or attention-deficit–hyperactivity disorder (ADHD); and highest for those with a diagnosis of specific learning disability. The strongest relation between the pragmatic composite and diagnosis was seen when ratings from parents and professionals were combined. Differences between diagnostic groups were not explicable in terms of age or verbal IQ.

The medical findings from a population-based study of Prader-Willi syndrome (PWS) are discussed (in which birth incidence of PWS was estimated at 1:22000 and death rate at over 3% per annum). In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated. Of all people with a possible diagnosis of PWS, only those meeting clinical criteria and/or with a confirmed genetic diagnosis were included in the study. Sixty-six individuals, 40 males and 26 females with a mean age of 19 years (range of 0 to 46 years) agreed to participate in the population-based study group. A prevalence rate of 25% for non-insulin dependent diabetes mellitus (NIDDM) was found in adults. Mean age at onset was 20 years. Those with NIDDM had a higher past maximum body weight and a greater likelihood of positive family history. Nearly 50% across the age groups reported a history of recurrent respiratory infections. High rates of fractures (29%), leg ulceration (22% in adults), sleep disorders (20%), and severe scoliosis (15% in childhood) were also reported. It is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections. Other causes of morbidity, in particular the high rates of NIDDM, may be due to a failure to manage over-eating resulting in severe obesity. Early diagnosis and clear guidance to families about these risks and how they might be prevented is recommended. It is hypothesized that the high pain threshold may result in the presence of some illness not being apparent.

This study is a comparative analysis and meta-analysis of three randomized clinical trials. Children with spastic diplegia received either ‘selective’ dorsal rhizotomy (SDR) plus physiotherapy (SDR+PT) or PT without SDR (PT-only). Common outcome measures were used for spasticity (Ashworth scale) and function (Gross Motor Function Measure [GMFM]). Baseline and 9- to 12-month outcome data were pooled (n=90). At baseline, 82 children were under 8 years old and 65 had Gross Motor Function Classification System level II or III disability. Pooled Ashworth data analysis confirmed a reduction of spasticity with SDR+PT (mean change score difference –1.2; Wilcoxon p<0.001). Pooled GMFM data revealed greater functional improvement with SDR+PT (difference in change score +4.0, p=0.008). Multivariate analysis in the SDR+PT group revealed a direct relationship between percentage of dorsal root tissue transected and functional improvement. SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.

This investigation determined the measurement properties of the Pediatric Quality of Life Inventory (PedsQL) 3.0 Cerebral Palsy (CP) Module. PedsQL 4.0 Generic Core Scales and 3.0 CP Module were administered to 245 families. Mean age of the 134 males and 107 females was 8 years 1 month (SD 4y 4mo; range 2–18y). The sample included children with hemiplegia (n=55), diplegia (n=84), and quadriplegia (n=85). Twenty eight children had a Gross Motor Function Classification System classification at Level I, 40 at Level II, 86 at Level III, 43 at Level IV, and 37 at Level V. Reliability was demonstrated for the PedsQL 4.0 (α=0.86 child, 0.89 parent) and CP Module (α=0.79 child, 0.91 parent). The PedsQL 4.0 distinguished between healthy children and children with CP. Construct validity of the CP Module was supported. Sensitivity of the PedsQL was demonstrated among children with different diagnostic categories and gross motor function.

The objective of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) evidence reports is to provide the biomedical research and clinical practice communities with the current state of evidence about various interventions for the management of developmental disabilities. AACPDM evidence reports aggregate all that has been published about outcomes of an intervention for a medical condition, gauge the credibility (i.e. strength of the internal validity) of that evidence, and identify gaps in our scientific knowledge. The original version of this report was published in the ‘AACPDM Database of Evidence Reports’ on the internet (www.aacpdm.org) where evidence reports are regularly updated to include new research.

A minority of cases of autism has been associated with several different organic conditions, including bioenergetic metabolism deficiency. In a population-based study, we screened associated medical conditions in a group of 120 children with autism (current age range 11y 5mo to 14y 4mo, mean age 12y 11mo [SD 9.6mo], male:female ratio 2.9:1). Children were diagnosed using Diagnostic and Statistical Manual of Mental Disorders criteria, the Autism Diagnostic Interview – Revised, and the Childhood Autism Rating Scale; 76% were diagnosed with typical autism and 24% with atypical autism. Cognitive functional level was assessed with the Griffiths scale and the Wechsler Intelligence Scale for Children and was in the normal range in 17%. Epilepsy was present in 19 patients. Plasma lactate levels were measured in 69 patients, and in 14 we found hyperlactacidemia. Five of 11 patients studied were classified with definite mitochondrial respiratory chain disorder, suggesting that this might be one of the most common disorders associated with autism (5 of 69; 7.2%) and warranting further investigation.

The increased survival of sick and preterm neonates may be associated with long-term problems which must be recognised and managed if outcome is to be optimised. In a prospective study of 35 neonates (median gestational age at birth 34 weeks) admitted to a neonatal intensive care unit over a 3-month period, we have documented a high incidence (14 of 35) of immature or abnormal feeding patterns when infants were assessed at 36 to 40 weeks postmenstrual age. Neonates with prolonged respiratory support and delayed enteral and oral feeding were most affected. Compared with neonates who have normal initial feeding assessments, neonates with disorganised or dysfunctional feeding were six times more likely to vomit and three times more likely to cough when offered solid food at 6 months of age. At 12 months of age significant differences were also found in tolerating lumpy food and enjoying mealtimes. We hypothesise that these feeding problems contribute to failure to thrive and psychosocial distress after discharge from the neonatal unit and propose potential neonatal measures to reduce their incidence.

Muscle function often becomes progressively more compromised in children with spastic cerebral palsy, leading to reduced mobility. This study aimed to examine the role that muscle connective tissue plays in this process. Severity of spasticity as determined by a range of clinical measures was assessed in 26 children (14 males 12 females; age range 4 to 17 years) with either diplegic or quadriplegic cerebral palsy (CP). Muscle biopsies from the vastus laeralis muscle were obtained for biomedical and histological analysis during orthopaedic surgery as part of the child's ongoing care. Total collagen was quantified by hydroxyproline determination. Two clinical measures of severity, Modified Ashworth Scale and Balance, were shown to have a highly significant correlation with collagen content, and Ambulatory Status, Clonus, and Selective Muscle Control all showed positive trends. Collagen I accumulated in spastic muscle's endomysium which appeared to be thickened, and fibrotic regions with sparse muscle fibres were evident in more severe cases. This suggests that collagen may be involved in increases in muscle stiffness observed in spasticity. Once developed, these changes are essentially irreversible and we suggest that future treatments should consider including prevention of muscle fibrosis.

Constraint-induced (CI) movement therapy is a physical intervention that has been receiving increasing attention in pediatric rehabilitation. So far, the evidence suggests that practice associated with CI therapy may improve impaired unimanual hand function in some children with hemiplegic cerebral palsy (CP). However, CI therapy has several important limitations. Most importantly, children with hemiplegia have impairments in bimanual coordination beyond their unilateral impairments. Thus, an intervention approach to increase functional independence during activities of daily living by using both hands in cooperation is needed. Here we briefly review the etiology of hemiplegic CP, describe studies of pediatric CI therapy efficacy in relation to the etiology, discuss the conceptual and practical limitations of CI therapy for this population, and describe bimanual coordination impairments in children with hemiplegia. Finally, we introduce a new intervention for children with hemiplegia, hand–arm bimanual intensive training (HABIT), to address the limitations of CI therapy and to improve bimanual coordination. HABIT retains the two major elements of pediatric CI therapy (intensive structured practice and child-friendliness). The proposed methodology demonstrates that extensive targeted practice can be provided in a child-friendly manner without using a physical restraint, although the efficacy of such an approach remains to be determined.

The reliability and responsiveness of the Barry–Albright Dystonia (BAD) Scale, a 5-point ordinal severity scale for secondary dystonia, was assessed. For interrater reliability, 13 raters scored 10 videotaped patients; for intrarater reliability, two raters rated the videotape again. For test–retest reliability, patients were rated on two occasions. Four inexperienced raters scored patients, received training, then scored additional patients. To assess responsiveness, we compared patient and physician global ratings of change (better, same, and worse) with BAD Scale score changes for 18 patients on intrathecal baclofen (ITB) trials. We assessed reliability with the intraclass correlation coefficient (ICC). The mean ICC for total BAD Scale scores were as follows: interrater reliability 0.866, intrarater reliability 0.967 and 0.978, test–retest reliability 0.978 (before training) and 0.967 (after training). We found the BAD Scale responsive to change, with most improved scores in patients rated by the patient, family, and neurosurgeon as ‘better’. The total scores were reliable for experienced raters. We recommend training for clinicians interested in using the scale.

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.