The purpose of this study was to evaluate the effects of botulinum toxin A (BTX-A, Botox) dilution volume and post-injection exercise with electrical stimulation on muscle paralysis. We injected 10 units of BTX-A diluted with 0.1 ml (B1, n=8) or 0.5 ml (B5, n=8) normal saline into both gastrocnemius muscles of 16 New Zealand white rabbits; two controls received no BTX-A. After BTX-A injection, all rabbits received calf muscle stretching exercise and electrical stimulation for 2 hours on the left leg. The compound muscle action potential (CMAP) decrease was most pronounced at 1 week and progressive recovery was observed (i.e. recovery from paralysis, increase of CMAP). There was a significant decrease of CMAP amplitudes in the B5 group compared with the B1 group at week 1 and week 4 (p<0.001). Left limbs with stretching exercise and electrical stimulation showed lower CMAP amplitudes compared with control right limbs of all rabbits. To maximize the muscle paralysis effect of BTX-A, increasing dilution volume and performing post-injection stretching exercise with electrical stimulation may be a promising strategy for increasing the beneficial effect of BTX-A treatment. Future studies are needed to investigate the clinical application of this finding.

This study compared performance on the Functional Independence Measure for Children (WeeFIMTM), the Battelle Developmental Inventory Screening Test (BDIST), and the Vineland Adaptive Behavior Scales (VABS) in children with developmental disabilities. The three instruments were administered to 205 children with identified disabilities. All 205 children were tested using the WeeFIM instrument. The BDIST was administered to 101 children and the VABS to the remaining 104 children. Administration was counterbalanced and randomized across all three instruments. A proportional sampling plan was used to select the 205 children, who ranged in age from 11 to 87 months. A variety of medical diagnoses and levels of severity of motor, cognitive, and communication impairments were systematically included in the sample. Correlations (r) among subscales for all three instruments ranged from 0.42 to 0.92. Correlations for total scores ranged from 0.72 to 0.94. Analyses of potential moderator variables found no significant relation between age and severity of disability (r=0.05) or between socioeconomic status (SES) and severity of disability (r=0.21). Correlations with age were strongest for those subscale scores involving gross and fine motor skills. Correlations with SES and subscale scores ranged from 0.03 to 0.18. The three instruments provide important information regarding childhood performance in motor, self-care, communicative, cognitive, and social skills. The WeeFIM instrument requires less administration time and provides information directly relevant to evaluating functional outcomes for children with disabilities and their families.

Individuals with Angelman syndrome (AS) have severe learning disability, ataxia, seizures, dysmorphic facies, a happy, sociable disposition, and inability to speak. Most of the literature concerning the clinical features of AS has concentrated on younger children. This study aimed to look at the natural history of AS by documenting the clinical features in a group of 28 adolescents and adults with AS (12 males, 16 females; age range 16 to 40 years). Specific aspects studied included physical characteristics, general health, mobility, seizure disorder, behaviour and communication, and self-help skills. Problems seen in this older group of individuals differed significantly from those typically observed in younger children. The incidence of scoliosis and joint contractures increased with age. Facial features were more striking in adults. Hyperactivity and concentration improved and the sociable disposition persisted. There were subtle clinical differences between the groups of individuals with different underlying genetic abnormalities. Information obtained from this study is relevant to diagnosis and management of older individuals with AS.

This study aimed to determine whether supine sleep-positioned infants have delayed motor skills at age 4 and 6 months, and if delays are associated with decreased exposure to prone position. One 4 and one 6-month-old sample of healthy infants born at term were recruited. Motor assessments included the Peabody Developmental Motor Scale (PDMS) and Alberta Infant Motor Scale (AIMS). Parents completed an infant behavior diary for 3 consecutive days. Seventy-one 4-month-old infants were assessed (38 males; mean age 4.4mo, standard deviation [SD] 0.2). Mean motor scores were close to normative standards (AIMS 47.7, SD19.6; PDMS gross motor quotient [GMQ] 96.3, SD 6.5; PDMS fine motor quotient [FMQ] 99.2, SD4.8). No infant scored below cut-off values used to identify motor delay. Milestones less likely to be achieved included extended arm support in prone, hands to feet in supine, and sitting with arm support. Exposure to ‘tummy time’ while awake was correlated with AIMS scores (r=0.38, p<0.01). Fifty 6-month-old infants were assessed (21 males; mean age 6.4mo, SD0.4). Mean scores were shifted down for all scales, and as much as 1SD for PDMS (AIMS 44.5, SD 21.6; PDMS GMQ 85.7, SD7.6; PDMS FMQ 88.9, SD9.0). Only 22% of 6-month-olds could sit without arm support versus 50% expected in a normative sample. Remarkably, 22% of our sample exhibited gross motor delays (quotient<78). Tummy time (awake) was significantly associated with the AIMS (r=0.64) and PDMS GMQ (r=0.55) and FMQ (r=0.33) quotients, even after adjusting for confounders. Typically developing infants who were sleep-positioned in supine had delayed motor development by age 6 months, and this was significantly associated with limited exposure to awake prone positioning. This has important implications for interpreting motor assessments of infants at risk and for preventing inappropriate referrals.

To assess time costs of caring for children with severe disabilities in the community compared to caring for children without disabilities, a diary- and questionnaire-based study was carried out. Sixteen complete data sets were obtained from families with children who have disabilities (mean age 8.7 years) and 31 complete data sets from families with normally developing children (mean age 4.9 years). Diagnoses in the study group included cerebral palsy, autism, Sanfillipo syndrome, lissencephaly, and osteogenesis imperfecta. Items of personal care per waking hour were significantly greater in children with disabilities than non-disabled children (p<0.001). In the study group, there was no correlation (r=–0.12) between age and frequency of care whereas a significant correlation was observed between degree of disability as measured by the Functional Independence Measure for children (WeeFIM) and frequency of care items (r=0.89). Twelve of the 16 mothers in the study group were not in paid employment. Twelve had little or no extended family support. Benefits awarded did not correlate with the degree of disability as measured by the WeeFIM (r=–0.11). Care needs of children with severe disabilities are significantly greater than those of non-disabled children and do not decrease with advancing age. Mothers of children with disabilities are unable to work outside the home because of these care needs. This brings the family income, even when benefits are included, to a level that is less than peer families with non-disabled children. A Functional Disability Score may help to achieve more appropriate allocation of state resources.

The sensitivity of the pendulum test to variation in spasticity in persons with spastic cerebral palsy (CP) was tested in 30 participants with CP and 10 participants without CP (controls) (mean age 13.8 years). The participants with CP were classified into three groups, with normal (mean age 15.9 years), mild/moderate (13.0 years), or severe (23.0 years) muscle tone, as assessed clinically using a modified Ashworth scale. Joint motion during the pendulum test was measured with an electrogoniometer. Muscle relaxation was confirmed using surface EMG. Outcome measures from the pendulum test were (1) number of oscillations, (2) duration of oscillations, (3) excursion of the first backward swing, and (4) relaxation index (first swing excursion/difference between the starting and resting angles). Data were assessed using one-way analysis of variance. Outcome measures 1 to 3 differed significantly between control participants and participants with CP (p<0.05). The first swing excursion was the best predictor of the degree of spasticity in persons with CP, being significantly different between all groups (p<0.05). The number of oscillations and their duration differentiated between control participants and all participants with CP (p<0.05) but not between participants with CP who had mild/moderate versus severe spasticity (p>0.05). The relaxation index was not a sensitive measure (p>0.05 between most study groups). We conclude that the pendulum test is a valid tool for assessing spasticity in persons with CP and that the first swing excursion is the most sensitive outcome measure.

Young people with cerebral palsy (CP) are often assumed to have low self-concept, in other words, they do not feel good about themselves. We systematically searched the literature to determine whether this assumption was supported by empirical research. Relevant trials were identified by searching electronic databases, and this was supplemented by citation tracking. Of 1355 papers initially identified, six met the criteria for review. Results showed that adolescent females with CP have a lower self-concept than females without disability in the domains of physical appearance (d=–1.16; 95% confidence interval [CI] –2.06 to –0.26); social acceptance (d=–1.24; 95% CI –2.15 to –0.33); athletic competence (d=–0.93; 95% CI –1.79 to –0.07); and scholastic competence (d=–0.86; 95% CI, –1.71 to –0.01). Adolescent females with CP may be an at-risk group owing to their vulnerable self-concept. Clinicians may need to monitor and implement appropriate intervention strategies with this group. There was insufficient evidence to conclude that children with CP, in general, have a lower global self-concept compared with those without disability.

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.

To evaluate the clinical results of the treatment and to assess the factors that influenced the rate of scoliosis progression, a retrospective study of spinal orthosis in 86 patients with spastic quadriplegic cerebral palsy was performed. The mean age of the patients was 13.8 years (range 5 to 33 years). Their scoliotic deformities were treated with custom-moulded, polypropylene thoraco-lumbar-sacral orthoses. Cobb angles were measured on radiographs taken in a sitting position before treatment, in orthosis, and during follow-up. The mean initial Cobb angle was 68.4° (range 25 to 131°). The mean correction in orthosis was 25° (range 3 to 60°). Seventy-two patients had a follow-up period of more than 2 years. At the latest follow-up, average 6.3 years (range 2 to 14 years) after the start of treatment, the mean Cobb angle without orthosis was 93.1° (range 40 to 145°). The mean progression per year was 4.2° (range –3 to 21°). Linear multiple regression revealed that age and initial correction in orthosis were the only variables that significantly influenced the rate of progression. Twenty-two patients had no progression or progression <1.0° per year. Correction in orthosis was the only variable that predicted progression <1.0° per year in both age groups (<15 years and [ges ]15 years). Of the 57 patients who were still alive and had not undergone surgical fusion, 72% used their orthoses at a mean age of 22 years. Parents and caregivers expressed satisfaction with the use of orthosis, mainly because of improved sitting stability which gave better overall function.

Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re-examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge-Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow-up. We recruited a total of 15 patients with unilateral Sturge-Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow-up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation and two had moderate mental retardation. Eight of the ten non-operated patients still experience seizures at follow-up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge-Weber syndrome is a prognostic marker for mental deterioration.

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.

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall-Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach-and-grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon χ2 test, self-help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.

To document the characteristics of early posttraumatic seizures (EPTS) in non-accidental head injury (NAHI), and examine their relation with outcome, a retrospective study was carried out. All children with NAHI admitted to the Royal Hospital for Sick Children, Edinburgh since 1981 were identified. The characteristics of EPTS, EEG, and outcome were noted. Forty-four cases were identified. The average age of children at presentation was 5.9 months. Thirty-two of these children had EPTS. The median length of follow-up was 3.0 years. The mortality rate was six in 44 (14%). The neurodevelopmental outcome correlated significantly with the presence and severity of EPTS (Tau = 0.317, p=0.017). Of survivors, 22% developed late posttraumatic epilepsy, the outcome in those with epilepsy was significantly worse than those without (p<0.0001). It was concluded that the severity of the primary brain injury dictates the severity of the EPTS and neurodevelopmental status at follow-up.

Language disorders have been reported after severe falciparum malaria but the deficits have not been described in detail. We assessed language outcome in three groups of children aged 6 to 9 years (n=487): those previously admitted to Kilifi District Hospital, Kenya, with cerebral malaria (CM; n=152; mean age 7y 4 mo [SD 1y 1mo]; 77 males, 75 females); or those with malaria and complicated seizures (M/S; n=156; mean age 7y 4mo [SD 1y 2mo]; 72 males, 84 females); and those unexposed to either condition (n=179; mean age 7y 6mo [SD 1y 1mo]; 93 males, 86 females). Median age at hospital admission was 28 months (interquartile range [IQR] 19 to 44 mo) among children with a history of CM and 23 months (IQR 12 to 35mo) among children with a history of M/S. A battery of eight assessments covering the major facets of speech and language was used to measure language performance. Cognitive performance, neurological/motor skills, behaviour, hearing, and vision were also measured. Eighteen (11.8%) of the CM group, 14 (9%) of the M/S group, and four (2.2%) of the unexposed group were found to have a language impairment. CM (odds ratio 3.68, 95% confidence interval 1.09 to 12.4, p=0.04) was associated with significantly increased odds of an impairment-level score relative to the unexposed group. The results suggest that falciparum malaria is one of the most common causes of acquired language disorders in the tropics.

The incidence of childhood autism and other autistic spectrum disorders (ASDs) in preschool children was determined for two areas of the West Midlands between 1991 and 1996. Children diagnosed before the age of 5 years and residing within the study areas at diagnosis were detected from the records of four child development centres. The incidence rate per 10000 children per year for the combined areas was 8.3 for all children with ASDs, 3.5 for classical childhood autism (CA), and 4.8 for other ASDs. Rates were similar in both areas, despite differences in social deprivation and proportions of ethnic minorities. While rates for classical CA increased by 18% per year, a much larger increase (55% per year) was seen for ‘other ASDs’, suggesting that clinicians are becoming increasingly able and/or willing to diagnose ASDs in preschool children.

Idiopathic toe-walking (ITW) is a term used to describe the condition in which children walk with a toe–toe gait pattern in the absence of any known cause. It has also been referred to as habitual toe-walking and congenital short tendo calcaneus (tendo Achilles).

The aim of the study was to compare sleep patterns in children with epilepsy with those of their non-epileptic siblings and to determine which epilepsy-specific factors predict greater sleep disturbance. We conducted a case-control study of 55 children with epilepsy (mean age 10y, range 4 to 16y; 27 males, 28 females) and their nearest-aged non-epileptic sibling (mean age 10y, range 4 to 18y; 26 males, 29 females). Epilepsy was idiopathic generalized in eight children (15%), symptomatic generalized in seven (13%), and focal in 40 (73%); the mean duration was 5 years 8 months. Parents or caregivers completed the Sleep Behavior Questionnaire (SBQ) and Child Behavior Checklist (CBCL) for patients and controls, and the Quality of Life in Childhood Epilepsy (QOLCE) for patients. Patients had a higher (more adverse) Total Sleep score (p<0.001) and scored worse than controls on nearly all subscales of the SBQ. In patients, higher Total Sleep scores were correlated with higher scores on the Withdrawn, Somatic complaints, Social problems, and Attention subscales of the CBCL, and significantly lower Total Quality of Life Scores. Refractory epilepsy, mental retardation, and remote symptomatic etiology predicted greater sleep problems in those with epilepsy. We conclude that children with epilepsy in this current study had significantly greater sleep problems than their non-epileptic siblings.

Results of surgery to correct fixed flexion contracture of the knee and improve voluntary knee extension in 39 knees in 20 children (11 females, 9 males; mean age 12 years 8 months, age range 5 to 20 years) with cerebral palsy were analyzed. All patients had neuromotor disease and 18 children had spastic diplegia or quadriplegia. All patients could initiate voluntary knee extension but lacked full passive extension. Five patients (10 knees) were free walkers with a mean motor severity index of 19 and mean fixed knee contracture of 20 degrees. Fifteen patients (29 knees) were not free walkers and 13 were wheelchair ambulators. They had a mean motor severity index of 8 and mean fixed knee contractures of 30 degrees. Surgical procedures included various combinations of hamstring lengthening and/or posterior capsulotomy to allow free passive knee extension, with or without quadriceps mechanism shortening, to enhance voluntary extension. The best results were in patients who had hamstring lengthening, posterior capsulotomy, and quadriceps mechanism shortening.

The aims of the study were: (1) to determine the feasibility of a rehabilitation program combining intensive therapy periods (4 times/week for 4 weeks) with periods without therapy (8 weeks) over a 6-month period in severely impaired children with cerebral palsy (CP); and (2) to measure changes in gross motor function after intensive therapy periods (immediate effects) and rest periods (retention). A convenient sample included five children (two females, three males; mean age 22.6 months [SD 9.9]) with severe forms of CP with impairment of four limbs and trunk (GMFCS levels IV and V). A multiple-baseline design was used. Changes in motor performance were assessed by a blind evaluator using the Gross Motor Function Measure. Visual and statistical analyses followed. Level of compliance during intensive therapy was 93.1%. Children received a mean of 30 treatments over the 24 weeks of the experimental phase compared with the 48 treatments they would have received routinely. Increases in GMFM scores (mean 9.2%; range 3 to 15%) were significant in three children (p<0.05) and all participants maintained their motor performance during the two 8-week rest periods. Results showed that four treatments per week over a 4-week period were well tolerated when separated by rest periods. The intermittent program led to improvements in motor function that were maintained over the rest periods. Results underline the need to reconsider the organization of physical rehabilitation programs. A regime that is intensive enough without being tiring and one that provides practice conditions for consolidating motor skills learned during the intensive therapy period may best optimize motor training.

The relationships between quality of life (QoL), participation, and environment are being studied within a multicentre European study of 8- to 12-year-old children with cerebral palsy (CP; Study of PARticipation of Children with cerebral palsy Living in Europe [SPARCLE], www.ncl.ac.uk/sparcle/). The international group was determined that children with CP who also had intellectual impairment (IIm) should not be excluded from the study, but the group realized that there was little information available on how to assess subjective QoL in children with IIm. This annotation reviews and discusses the literature on conceptual and methodological issues in relation to such assessment.