The aim of this study was to investigate long-term survival and examine causes of death in adult patients with cerebral palsy (CP). A 1940–1950 birth cohort based on paediatric case referral allows for long-term survival follow-up. Survival is analyzed by birth characteristics and severity of disability from age 20 years (and age 2y for a subset of the data). Survival outcome compared with that expected in the general population based on English life tables. The main cohort consisted of 341 individuals, with 193 males and 148 females. Conditional on surviving to age 20 years, almost 85% of the cohort survived to age 50 years (a comparable estimate for the general population is 96%). Very few deaths were attributed to CP for those people dying over 20 years of age. Females survived better than males. However, females faced a greater increase in risk relative to the general population than did males. We conclude that survival outlook is good though lower than in the general population. The relative risk of death compared with the UK population decreases with age, although it shows some indication of rising again after age 50 years. Many more deaths were caused by diseases of the respiratory system among those dying in their 20s and 30s than would be expected in the general population. Many fewer deaths than expected in this age group are caused by injuries and accidents. For those people who die in their 40s and 50s, an increase in deaths due to diseases of the circulatory system and neoplasms is observed. More deaths than expected in this age group are due to diseases of the nervous system.

The aims of this study were to investigate fine motor skills of children with both attention-deficit–hyperactivity disorder (ADHD) and developmental coordination disorder (DCD) and those of a control group, and to examine the effects of methylphenidate on these skills. A group of 12 children with ADHD–DCD (11 males, one female; mean age 9y 8mo [SD 1y 7mo]) and 12 age- and sex-matched controls (mean age 9y 7mo [SD 1y 2 mo]) participated. The manual dexterity subtests of the Movement Assessment Battery for Children, the concise assessment method for children's handwriting, and a computerized graphomotor task were used. Results demonstrated that children with ADHD–DCD performed more poorly on the manual dexterity subtests, had poorer quality of handwriting, and drew more rapidly, more fluently, but less accurately than controls on the graphomotor task. On methylphenidate, manual dexterity and quality of handwriting improved, and strokes on the graphomotor task became less fluent but more accurate. ADHD is characterized by persistent symptoms of inattention, impulsivity, and hyperactivity, affecting 3 to 5% of school-age children. Up to 50% of children with ADHD also have motor coordination problems that are severe enough to meet criteria for DCD. In DCD, children demonstrate functional motor performance deficits not explained by the child's (chronological) age or intellect, or by other neurological or psychiatric disorders.

The aim of this study was to determine the reliability of family and professional assessment of manual ability using the Manual Ability Classification System (MACS) for children with cerebral palsy (CP) in the UK. Families who were taking part in a study measuring the activities and participation of children with CP were invited to classify their child's manual ability using the MACS. Postal surveys were conducted with the families and health professionals nominated by the families. Perfect agreement was assessed as a percentage; chance-corrected agreement was measured using Cohen's kappa (κ), and reliability was determined using the intraclass correlation coefficient (ICC). Families of 91/128 (71%) children responded to the survey (53 males, 38 females; mean age 9y 11mo [SD 1y 11mo], range 6–12y) out of whom 88 indicated a single MACS level. Seventy-two children (82%) were classified with spastic CP, 12 (14%) with dyskinesia, two (2%) with ataxia, and two (2%) were not classified. There were 21, 27, 11, 10, and 19 children who were classified by their families in Gross Motor Function Classification System Levels I to V respectively; 14, 30, 18, 13, and 13 children classified by their families in MACS levels I to V. The survey of health professionals generated 60/71 (85%) responses from physiotherapists, 55/58 (93%) responses from paediatricians, and 21/24 (88%) responses from occupational therapists. There was perfect agreement between families and professionals for more than 50% of children; the indices of chance-corrected agreement ranged from κ=0.3 to 0.5, and the reliability coefficients ranged from ICC 0.7 to 0.9. Indices of agreement and reliability between families and professionals were equivalent to those between different professionals. The MACS, therefore, offers a valid and reliable method for communicating about the manual ability of children with CP. Families and professionals may not always agree precisely on a MACS level, particularly if children's performance of manual tasks varies in different environments.

The aim of this study was to describe the social experience of children with cerebral palsy (CP) in mainstream classes in Canada and compare it with that of their classmates without disability. The CP group included 25 females and 35 males (mean age 10y 5mo [SD 0.95], range 10y 4mo–10y 10mo) diagnosed as having hemiplegia (n=44) or diplegia (n=16) and classified as Level I on the Gross Motor Function Classification System (GMFCS). Fifty-seven comparison children, born at term and without any motor and/or sensory impairment, were recruited from the classes of the children with CP during a school visit (mean age 10y 3mo, [SD 1.0], range 10y–10y 6mo). They were matched to children with CP for sex, age, parents' education level, and family income. Social adjustment measures (social status, reciprocated friendships, social isolation, aggression, sociability/leadership, and verbal and/or physical victimization) were obtained by conducting a class-wide sociometric interview (n=943) in the classes of the children with CP. Findings showed that children with CP (specifically females with CP and irrespective of their type of disability) had fewer reciprocated friendships, exhibited fewer sociable/leadership behaviours, and were more isolated and victimized by their peers than their classmates without a disability. This seems to suggest that females and males with CP are perceived differently from their peers in a mainstreaming context. The discussion addresses the issue of age- and sex-related differences and provides avenues of intervention relating to personal and environmental factors that could facilitate or interfere with the social experience of children with CP in a mainstream environment.

The aim of this cross-sectional study was to determine the prevalence of cerebral palsy (CP) among children in Turkey between the ages of 2 and 16 years. Samples were selected from cities, towns, districts, and villages using the cluster sampling method; 41861 children were selected. Data was collected by parental interview and physical examination. One hundred and eighty-six children were identified with CP. The prevalence of CP was determined as 4.4 per 1000 live births and included postnatally acquired CP. Origin of CP was classified as prenatal in 49 (26.6%), perinatal/neonatal in 34 (18.5%), postnatal in 11 (5.9%), and unclassifiable in 90 participants (48.9%; data was unobtainable for two individuals). Type of CP was diplegia in 39.8% of children, hemiplegia in 28%, tetraplegia in 19.9%, ataxia in 5.9%, and dyskinetic in 6.4%. Prenatal factors were seen more frequently in the groups with a high socioeconomic status while perinatal factors were encountered more often in those with a low socioeconomic status (p<0.05). Place of residence and sex had no significant effect on the prevalence of CP (p>0.05). This cross-sectional study shows that the prevalence of CP in Turkey is higher than that in developed countries but the aetiology is probably similar. Although the high prevalence of CP in Turkey could originate from an increased level of obstetric and neonatal problems, the lack of a possible aetiological factor in approximately half the children suggests that the high level might also be due to other factors, such as genetic disorders.

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 assess the therapeutic efficacy of osteopathic treatment in infants with postural asymmetry. A randomized clinical trial of efficacy with blinded videoscoring was performed. Sixty-one infants with postural asymmetry aged 6 to 12 weeks (mean 9wks) were recruited. Thirty-two infants (18 males, 14 females) with a gestational age of at least 36 weeks were found to be eligible and randomly assigned to the intervention groups, 16 receiving osteopathic treatment and 16 sham therapy. After a treatment period of 4 weeks the outcome was measured using a standardized scale (4–24 points). With sham therapy, five infants improved (at least 3 points), eight infants were unchanged (within 3 points), and three infants deteriorated (not more than –3 points); the mean improvement was 1.2 points (SD 3.5). In the osteopathic group, 13 infants improved and three remained unchanged; the mean improvement was 5.9 points (SD 3.8). The difference was significant (p=0.001). We conclude that osteopathic treatment in the first months of life improves the degree of asymmetry in infants with postural asymmetry.

This study describes ages of acquisition (AOA) of typical adolescent autonomy skills in a regional cohort of individuals with myelomeningocele (MM), aged 12 to 18 years, with a mean age of 14 years 11 months (SD 2y 5mo). Prospectively collected data over 10 years were analyzed. Regression analysis, using Generalized Estimation Equation, provided 50th centile and 75th centile AOA for each skill. One hundred and fifty-eight participants (90 males, 68 females) attended 378 annual patient visits. Patient contacts were equally distributed across age and physical severity groupings. Twenty-four percent of participants had functional lesion levels at or above L2, and 38% between L3–L5, 38% at S1 or below. Eighty-four percent had shunted hydrocephalus, 24% were independent in ambulation, and 69% achieved toileting independence before adolescence. AOA for autonomy skills were delayed by 25 to 30% when compared with typically developing adolescents. Differences in cognitive ability explained the variance in median ages for skill acquisition (p=0.01) more than physical lesion level. Participants acquired community skills at a median age of 16 years 6 months. Twelve percent of 18-year-olds drove cars (9% with supervision, 3% independently), regardless of physical lesion severity. We conclude that adolescents with MM acquire the majority of autonomy skills 2 to 5 years later than their typically developing peers. This study establishes AOA timelines for autonomy skills in adolescents with MM.

A prospective study was designed to determine whether the combined use of neuromuscular electrical stimulation (NMES) and dynamic bracing was more effective than use of either alone in reducing upper-extremity spasticity in children with spastic hemiplegic cerebral palsy. Twenty-four patients (12 males, 12 females; mean age 8y 7mo [SD 4y 2mo]; age range 3–18y) diagnosed with spastic hemiplegic CP were randomly allocated to three groups: group 1 had two 30-minute sessions of NMES a day applied on the antagonist extensors without bracing; group 2 had two 30-minute sessions of dynamic bracing per day; and group 3 had two 30-minute sessions of NMES and dynamic bracing every day. Treatment was continued for 6 months in all groups and applied only to the affected extremity. Patients were evaluated before therapy, at monthly intervals during the therapy, and 3 months after completion of the therapy. Three measures of outcome were taken: the Melbourne Assessment, grip strength, and posture evaluation with Zancolli's classification. The therapist performing the outcome assessments was blinded as to groups. Statistically significant differences were found in all three measures for only those treated with combined NMES and dynamic bracing. However, this significant effect lasted for only 2 months after discontinuation of the treatment. We conclude that the combined use of NMES and bracing is more effective than either alone but requires continuous application.

The objective was to determine the effects of low-dose, high-concentration, dual localized botulinum toxin A (BTX-A) injections on upper limb movement quality and function. Study design was an evaluator-blinded, randomized, controlled trial. Forty-two children (31 males, 11 females; range 2–8y, mean 4y [SD 1.6]) with hemiplegic cerebral palsy (Gross Motor Function Classification System level I) participated. All received occupational therapy. The treatment group (n=21) received one injection series (mean muscles injected 6 [SD 1.05]; total dose 82–220 units, mean 139 [SD 37.48]; dilution 100 units/0.5ml). Primary outcome of Quality of Upper Extremity Skills Test (QUEST) at 6 months was not significant (p=0.318). Secondary outcomes were average treatment effects at 1, 3, and 6 months, which favoured the treatment group: QUEST (p<0.001); Canadian Occupational Performance Measure (performance, p=0.002; satisfaction p=0.007); parent Goal Attainment Scaling (GAS; p=0.001), therapist GAS (p<0.001); Pediatric Evaluation of Disability Inventory (PEDI) functional skills (p=0.030); Ashworth (p<0.001). PEDI caregiver assistance was not significant (p=0.140). Therapy alone is effective, but at 1 and 3 months movement quality is better where BTX-A is also used. Moreover, function is better at 1, 3, and 6 months, suggesting BTX-A enhances therapy outcomes beyond the pharmacological effect. One- and 3-month Ashworth and QUEST scores suggest precise needle placement accuracy.

We obtained information about the behavioral, psychiatric, and functional status of 26 children (13 males, 13 females) with juvenile neuronal ceroid lipofuscinosis (JNCL; mean age 12y 3mo [SD 3y 4mo]; range 6y 9mo to 18y 8mo). Twenty-five children had visual impairment and 18 were known to have a positive seizure history before enrollment. Parents completed the Child Behavior Checklist, Scales of Independent Behavior – Revised, and a structured interview to assess obsessive–compulsive symptoms. Participants exhibited a broad range of behavioral and psychiatric problems, rated as occurring frequently and/or as severe in more than half of the sample. Males and females did not differ with regard to the number of behavioral and psychiatric problems. Children were also limited in their ability to perform activities of daily living, including self-care, hygiene, socialization, and other age-appropriate tasks. Results provide a quantitative baseline for behavioral and psychiatric problems and functional level in JNCL, against which further decline can be measured. Longitudinal assessment of behavioral and psychiatric symptoms and functional abilities is continuing and will provide much-needed data on the natural history of JNCL.

Using a semi-structured questionnaire, this descriptive study examined perceptions of feeding and adherence to feeding recommendations for caregivers (26 females; mean age 32y 7mo [SD 9.4y], range 20–59y) of children with cerebral palsy (CP) and a gastrostomy tube (GT). Children in the study (15 females, 11 males; mean age 4y 8mo [SD 3y 11mo], range 8mo–16y) had had a GT in place for at least 1 month and been assessed at Level II (n=2), Level III (n=2), Level IV (n=5), and Level V (n=17) of the Gross Motor Function Classification System. A negative response was reported by 18 caregivers when the GT was recommended; however, 21 caregivers reported improvement in the children following placement. All children received formula through the GT that was adequate for complete nutrition, yet 14 caregivers gave other foods through the GT (e.g. juice, cereal, soup, or table food). Of the 17 children receiving oral feedings, meals were an unpleasant experience for over half. Of the remaining nine children, in spite of a strict nil by mouth recommendation by physicians, five continued to receive some oral feedings. Generally, caregivers perceived GT feeding as ‘unnatural’. Understanding these perceptions will help clinicians to develop effective, family-centered, patient-appropriate intervention and adherence strategies for GT-fed children with CP.

We report on seven children (five males, two females) who presented with marked, often asymmetrical, toe-walking from onset of independent walking, associated with abnormal foot postures and increased tone at the ankles with characteristics of dystonia. Most of the children had presented with unusual pre-walking locomotion and a mild delay in independent walking. They did not fit into the usual categories of ‘habitual’ toe-walking or congenital short tendo calcaneus but nor did they have the clinical signs of spastic diplegia or of a peripheral neuromuscular disease. Normalization occurred progressively in the second to fourth years of life. The children were re-examined several years later (1 to 11y) and were normal. We believe that their persistent toe-walking corresponded to a variant of ‘transient focal dystonia of infancy’. Knowledge of its existence may justify a period of observation without special investigations, surgery, or casting.

Social integration and independence is the ultimate goal of habilitation and social support for patients with cerebral palsy (CP). Having a partner and having children provide support for social integration of adults with or without a disability. We studied 416 participants with CP born between 1965 and 1970 (243 males, 173 females; mean age 32y 2mo [SD 2y]; age range 29–35y) and compared them with 2247 age-matched comparison individuals. Diagnostic subtypes of the 416 participants were: 31% hemiplegia, 49% diplegia, 11% tetraplegia, and 9% other types. The level of motor impairment, estimated in childhood, with regard to walking ability was 65% able to walk without assistance, 22% with assistance, and 12% not able to walk (for 1% of the participants their walking ability was not known). We found no sign of increased social integration over the past two or three decades in Denmark. Sixty-eight per cent lived independently, 13% lived with their parents, and 16% lived at an accommodation facility arranged by the county (institution). Twenty-eight per cent of the participants were cohabiting and 19% had children. The presence of epilepsy and the severity of physical or cognitive impairment as assessed in childhood predicted independent living and physical and cognitive impairment predicted cohabitation, but parents' socioeconomic position and region of living did not. Fifty-five percent of the participants, compared with 4% of the comparison group, had no competitive employment, cohabiting partner, or biological children. The remaining participants had at least one of these types of social contact, but this more optimally socially integrated half of the participants only combined all three types of social contact half as often as the comparison group. This could be due to cognitive difficulties or premature ageing.

Children with physical disabilities are at increased risk of limitations to participation in everyday activities. This study describes research examining the participation of children in day-to-day formal and informal activities (excluding mandated academic schooling). Using the Children's Assessment of Participation and Enjoyment (CAPE) measure, data on participation patterns were collected from 427 children (229 males, 198 females; mean age 10y [SD 2y 4mo]; range 6-14y) with physical limitations and from their families. The primary types of physical disability in the sample included cerebral palsy, spina bifida, acquired brain injury, and musculoskeletal limitations. Findings indicate a broad range of diversity and intensity of participation, with proportionately greater involvement in informal rather than formal activities. Significant differences in participation and enjoyment were found between males and females, and for children more than 12 years of age. Children's participation was less diverse in families reporting lower income, single-parent status, and lower respondent parent education. These findings provide a foundation for an improved understanding of the participation of children with physical disabilities, which can assist families and service providers in planning activities that fit with their child's preferences and ensure active participation.

Adductor spasticity in children with cerebral palsy (CP) impairs motor function and development. In a placebo-controlled, double-blind, randomized multicentre study, we evaluated the effects of botulinum toxin A(BTX-A) in 61 children (37 males, 24 females; mean age 6 years 1 month [SD 3y 1mo]) with CP (leg-dominated tetraparesis, n=39; tetraparesis, n=22; GMFCS level I, n=3; II, n=6; III, n=17; IV, n=29; V, n=6). Four weeks after treatment, a significant superiority of BTX-A was observed in the primary outcome measure (knee–knee distance ‘fast catch’, p=0.002), the Ashworth scale (p=0.001), and the Goal Attainment Scale (p=0.037).

The goal was to compare children with hemiplegia with those with diplegia within Gross Motor Functional Classification System (GMFCS) levels using multiple validated outcome tools. Specifically, we proposed that children with hemiplegia would have better gait and gross motor function within levels while upper extremity function would be poorer. Data were collected on 422 ambulatory children with cerebral palsy: 261 with diplegia and 161 with hemiplegia, across seven centers. Those with hemiplegia in each level performed significantly and consistently better on gait or lower extremity function and poorer on upper extremity and school function than those with diplegia. In GMFCS Level II, the group with hemiplegia walked faster (p=0.017), scored 6.6 points higher on Dimension E of the Gross Motor Function Measure (p=0.017), 6.7 points lower on Upper Extremity subscale of the Pediatric Outcomes Data Collection Instrument, and 9.1 points lower on WeeFIM self-care (p=0.002). Basing motor prognosis on GMFCS level alone may underestimate lower extremity skills of children with hemiplegia, and overestimate those of children with diplegia.

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.

The aim of this study was to use family-assessed instruments and details of children's impairments to explore factors affecting the activities and participation of children with cerebral palsy (CP). A postal survey was conducted with families of a geographically defined population of children with CP aged 6 to 12 years. Family-assessed indices of children's activities and participation were the Activities Scale for Kids (ASK) and Lifestyle Assessment Questionnaire (LAQ-CP). Families also assessed children's abilities using the Gross Motor Function and Manual Ability Classification Systems (GMFCS; MACS). Details of children's impairments were available from the 4Child epidemiological database and used with the GMFCS and MACS as explanatory variables in multiple regression analyses to identify their effect on children's activities and participation. Families of 175/314 (56%) children returned an assessment using the GMFCS and 129 (41%) children participated fully by returning all the questionnaires. Full participants (72 males, 57 females) did not differ from those who did not take part by their age, sex, CP characteristics, or associated impairments: GMFCS Level I–25, Level II–43, Level III–15, Level IV–14, Level V–23; MACS Level I–14, Level II–30, Level III–18, Level IV–13, Level V–13. Scores for the ASK and LAQ-CP Physical Independence and Mobility domains were predicted well by children's movement, manual, and intellectual disability, and also, to some extent, by the presence of seizures or speech problems. LAQ-CP domains for Economic and Clinical Burden and Social Integration were not well explained by children's abilities and impairments. Family assessment, therefore, offers a useful method for measuring children's activities and participation; however, currently available instruments do not fully represent all the domains in the International Classification of Functioning, Disability and Health. Children's abilities only partially explain their activities and participation.

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.