Showing papers in "Developmental Medicine & Child Neurology in 2006"
TL;DR: Reliability was tested between pairs of therapists for 168 children between 4 and 18 years and between 25 parents and their children's therapists, demonstrating that MACS has good validity and reliability.
Abstract: The Manual Ability Classification System (MACS) has been developed to classify how children with cerebral palsy (CP) use their hands when handling objects in daily activities. The classification is designed to reflect the child's typical manual performance, not the child's maximal capacity. It classifies the collaborative use of both hands together. Validation was based on the experience within an expert group, a review of the literature, and thorough analysis of children across a spectrum of function. Discussions continued until consensus was reached, first about the constructs, then about the content of the five levels. Parents and therapists were interviewed about the content and the description of levels. Reliability was tested between pairs of therapists for 168 children (70 females, 98 males; with hemiplegia [n=52], diplegia [n=70], tetraplegia [n=19], ataxia [n=6], dyskinesia [n=19], and unspecified CP [n=2]) between 4 and 18 years and between 25 parents and their children's therapists. The results demonstrated that MACS has good validity and reliability. The intraclass correlation coefficient between therapists was 0.97 (95% confidence interval 0.96-0.98), and between parents and therapist was 0.96 (0.89-0.98), indicating excellent agreement.
1,778 citations
TL;DR: 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.
Abstract: 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 10 y [SD 2 y 4 mo]; range 6-14 y) 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.
477 citations
TL;DR: There was a tendency for children younger than 6 years who were reclassified to be done so to a lower level of ability, and the results provide evidence of stability of the GMFCS.
Abstract: The aim of this study was to assess the stability of the Gross Motor Function Classification System (GMFCS) by examining whether children with cerebral palsy (CP) remain in the same level over time. Participants were 610 children with CP (342 males, 268 females; mean age 6y 9mo [SD 2y 10mo]), range 16mo-13y). Children were assessed 2 to 7 times (mean 4.3) at 6-month (children <6y old) or 12-month (children ≥6y old) intervals. Seventy-three per cent of children remained in the same level for all ratings. The weighted kappa coefficient between the first and last ratings was 0.84 for children less than 6 years old and 0.89 for children at least 6 years old, indicating excellent chance-corrected agreement. Children initially classified in Levels I and V were least likely to be reclassified. There was a tendency for children younger than 6 years who were reclassified to be done so to a lower level of ability. The results provide evidence of stability of the GMFCS.
351 citations
TL;DR: 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.
Abstract: 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.
323 citations
TL;DR: 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.
Abstract: 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 6 y 8 mo [SD 1 y 4 mo]; range 4-8 y) 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 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.
305 citations
TL;DR: It is indicated that there are important neurobiological differences between males and females with respect to their response to brain injuries, relevant to understanding the pathogenesis of CP as well as to the design of future clinical trials of potential neuroprotective strategies.
Abstract: Cerebral palsy (CP) and related developmental disorders are more common in males than in females, but the reasons for this disparity are uncertain. Males born very preterm also appear to be more vulnerable to white matter injury and intraventricular hemorrhage than females. Experimental studies in adult animals and data from adult patients with stroke indicate that sex hormones such as estrogens provide protection against hypoxic-ischemic injury, and the neonatal brain is also influenced by these hormones. However, hormonal influences on the fetus and neonates are substantially different from those on adults. Recent data from neonatal rodents subjected to hypoxia-ischemia also demonstrate differences between males and females. Knockout of the gene for poly (ADP-ribose) polymerase (PARP-1), a major step in the cascade of injury, protected male but not female mouse pups from hypoxic-ischemic injury. Other reports demonstrated major differences between male and female neurons grown separately in cell culture, suggesting that sex differences in the fetal or neonatal period result from intrinsic differences in cell death pathways. This new information indicates that there are important neurobiological differences between males and females with respect to their response to brain injuries. This information is relevant to understanding the pathogenesis of CP as well as to the design of future clinical trials of potential neuroprotective strategies.
296 citations
TL;DR: Sensory organization according to each sensory component in relation to age and sex reached adult level at 15 to 16 years of age, revealing differences between young males and females.
Abstract: Maintenance of postural balance requires an active sensorimotor control system. Current data are limited and sometimes conflicting regarding the influence of the proprioceptive, visual, and vestibular afferent systems on posture control in children. This study investigated the development of sensory organization according to each sensory component in relation to age and sex. A total of 140 children (70 males, 70 females; mean age 10y [SD 4y]; age range 3y 5mo-16y 2mo) and 20 adults (10 males, 10 females; mean age 30y 6mo [SD 8y 4mo]; age range 17y 2mo-49y 1mo) were examined using the Sensory Organization Test. Participants were tested in three visual conditions (eyes open, blindfolded, and sway-referenced visual enclosure) while standing on either a fixed or a sway-referenced force platform. Mean equilibrium scores for the six balance conditions showed rapid increases and maturation ceiling levels for age-related development of the sensorimotor control system. Proprioceptive function seemed to mature at 3 to 4 years of age. Visual and vestibular afferent systems reached adult level at 15 to 16 years of age, revealing differences between young males and females. Characterizing balance impairments can contribute to the diagnostic evaluation of neuromotor disorders.
270 citations
TL;DR: 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.
Abstract: 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.
254 citations
TL;DR: A new intervention for children with hemiplegia, hand-arm bimanual intensive training (HABIT), is introduced, to address the limitations of CI therapy and to improve bimanUAL coordination.
Abstract: 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.
231 citations
TL;DR: Sensitivity of the PedsQL was demonstrated among children with different diagnostic categories and gross motor function, and construct validity of the CP Module was supported.
Abstract: 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.
226 citations
TL;DR: 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.
Abstract: 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 13 y 5 mo [SD 3 y 4 mo]) 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.
TL;DR: Three subtypes of children with NF1 having distinct cognitive profiles are defined, each with important implications for assessment and remediation, and Verbal IQ
Abstract: Cognitive deficits are the most common complication in children with neurofibromatosis type 1 (NF1), and academic achievement is broadly affected. There is a lack of consensus in the literature regarding the frequency of general and specific learning disabilities, which seems to be related to the lack of a consensus on diagnostic criteria. The present study examined the frequency of specific learning disabilities (SLDs) in NF1, using an intellect-achievement discrepancy for diagnosis, as well as general learning difficulty associated with a lowering of general intellectual ability. The cohort consisted of 81 children with NF1 (43 males, 38 females; mean age 11y 6mo [SD 2y 4mo]; range 8y-16y 9mo) and 49 comparison children (20 males, 29 females; mean age 12y [SD 2y 6mo]; range 8y 2mo-16y 8mo). Problems with academic achievement were present in 52% of children with NF1; however, only 20% of the children with NF1 were diagnosed with an SLD (32% had more general learning problems). Only males with NF1 were at significant risk for SLD, and Verbal IQ
TL;DR: Spatiotemporal gait data for children with autism were compatible with findings from patients with cerebellar ataxia: specifically, greater difficulty walking along a straight line, and the coexistence of variable stride length and duration.
Abstract: We investigated gait in newly diagnosed children with autism. From our previous study with 6- to 14-year-olds, we hypothesized that motor symptoms indicative of basal ganglia and cerebellar dysfunction would appear across the developmental trajectory of autism. Two groups were recruited: children with autism (eight males, three females; mean age 5 y 10 mo [SD 9 mo]; range 4 y 4 mo-6 y 9 mo) and a comparison group of typically developing children (eight males, three females; mean age 5 y 9 mo [SD 1 y 1 mo]; range 4 y 3 mo-7 y 2 mo). The GAITRite Walkway was used to gather data from average gait and intra-walk measurements. Experienced physiotherapists analyzed gait qualitatively. Groups were matched according to age, height, weight, and IQ; although not statistically significant, IQ was lower in the group with autism. Spatiotemporal gait data for children with autism were compatible with findings from patients with cerebellar ataxia: specifically, greater difficulty walking along a straight line, and the coexistence of variable stride length and duration. Children with autism were also less coordinated and rated as more variable and inconsistent (i.e. reduced smoothness) relative to the comparison group. Postural abnormalities in the head and trunk suggest additional involvement of the fronto-striatal basal ganglia region. Abnormal gait features are stable across key developmental periods and are, therefore, promising for use in clinical screening for autism.
TL;DR: It appeared that sustained stretching of longer duration was preferable to improve range of movements and to reduce spasticity of muscles around the targeted joints.
Abstract: Passive stretching is widely used for individuals with spasticity in a belief that tightness or contracture of soft tissues can be corrected and lengthened. Evidence for the efficacy of passive stretching on individuals with spasticity is limited. The aim of this review was to evaluate the evidence on the effectiveness of passive stretching in children with spastic cerebral palsy. Seven studies were selected according to the selection criteria and scored against the Physiotherapy Evidence Database scale. Effect size and 95% confidence intervals were calculated for comparison. There was limited evidence that manual stretching can increase range of movements, reduce spasticity, or improve walking efficiency in children with spasticity. It appeared that sustained stretching of longer duration was preferable to improve range of movements and to reduce spasticity of muscles around the targeted joints. Methods of passive stretching were varied. Further research is required given the present lack of knowledge about treatment outcomes and the wide use of this treatment modality.
TL;DR: References 1.
Abstract: References 1. Rosenbaum P, Dan B, Leviton A, Paneth N, Jacobsson B, Goldstein M, Bax M. (2005) Proposed Definition and Classification of Cerebral Palsy. Dev Med Child Neurol 47:571–576. 2. Graham HK. (2004) Management of the Motor Disorders of Children with Cerebral Palsy 2nd edn. Clinics in Developmental Medicine No 161 London: Mac Keith Press p105–129. 3. Graham HK, Selber P. (2003) Musculoskeletal aspects of cerebral palsy. J Bone Joint Surg 85: B157–B166.
TL;DR: The evidence suggests that HBRT and hippotherapy are individually efficacious, and are both medically indicated as therapy for gross motor rehabilitation in children with CP.
Abstract: Quantitative (not qualitative) studies were sought investigating whether horseback riding used as therapy improves gross motor function in children with cerebral palsy (CP). Eleven published studies on instructor-directed, recreational horseback riding therapy (HBRT) and licensed-therapist-directed hippotherapy were identified, reviewed, and summarized for research design, methodological quality, therapy regimen, internal/external validity, results, and authors'conclusions. Methodological quality was moderate to good for all studies; some studies were limited by small sample size or lack of non-riding controls. HBRT improved gross motor function in five of six studies (one study was inconclusive); hippotherapy improved gross motor function in all five studies. The studies found that during HBRT and hippotherapy: (1) the three-dimensional, reciprocal movement of the walking horse produced normalized pelvic movement in the rider, closely resembling pelvic movement during ambulation in individuals without disability; (2) the sensation of smooth, rhythmical movements made by the horse improved co-contraction, joint stability, and weight shift, as well as postural and equilibrium responses; and (3) that HBRT and hippotherapy improved dynamic postural stabilization, recovery from perturbations, and anticipatory and feedback postural control. The evidence suggests that HBRT and hippotherapy are individually efficacious, and are both medically indicated as therapy for gross motor rehabilitation in children with CP. Recommendations for future research are discussed.
TL;DR: Data indicate that unlike the stabilization of suck and suck-swallow rhythms, which occur before about 36 weeks' PMA, improvement in coordination of respiration and swallow begins later.
Abstract: To study the coordination of respiration and swallow rhythms we assessed feeding episodes in 20 preterm infants (gestational age range at birth 26-33wks; postmenstrual age [PMA] range when studied 32-40wks) and 16 term infants studied on days 1 to 4 (PMA range 37-41wks) and at 1 month (PMA range 41-45wks). A pharyngeal pressure transducer documented swallows and a thoracoabdominal strain gauge recorded respiratory efforts. Coefficients of variation (COVs) of breath-breath (BR-BR) and swallow-breath (SW-BR) intervals during swallow runs, percentage of apneic swallows (at least three swallows without interposed breaths), and phase of respiration relative to swallowing efforts were analyzed. Percentage of apneic swallows decreased with increasing PMA (16.6% [SE 4.7] in preterm infants 35wks; 1.5% [SE 0.4] in term infants; p 35wks' PMA; 0.693 [SE 0.059] at
TL;DR: The validity of the CP QOL is supported by the pattern of correlations between CP Q OL‐Child scales with the CHQ, KIDSCREEN, and GMFCS, and preliminary statistics suggest that the child self‐report questionnaire has acceptable psychometric properties.
Abstract: This paper describes the development and psychometric properties of a condition-specific quality of life instrument for children with cerebral palsy (CP QOL-Child). A sample of 205 primary caregivers of children with CP aged 4 to 12 years (mean 8y 5mo) and 53 children aged 9 to 12 years completed the CP QOL-Child. The children (112 males, 93 females) were sampled across Gross Motor Function Classification System (GMFCS) levels (Level I=18%, II=28%, III=14%, IV=11%, V=27%). Primary caregivers also completed other measures of child health (Child Health Questionnaire; CHQ), QOL (KIDSCREEN), and functioning (GMFCS). Internal consistency ranged from 0.74 to 0.92 for primary caregivers and from 0.80 to 0.90 for child self-report. For primary caregivers, 2-week test-retest reliability ranged from 0.76 to 0.89. The validity of the CP QOL is supported by the pattern of correlations between CP QOL-Child scales with the CHQ, KIDSCREEN, and GMFCS. Preliminary statistics suggest that the child self-report questionnaire has acceptable psychometric properties. The questionnaire can be freely accessed at http://www.deakin.edu.ac/hmnbs/chase/cerebralpalsy/cp_qol_home.php.
TL;DR: Difficulty in initiating and maintaining sleep, sleep-wake transition, and sleep breathing disorders were the most frequently identified problems.
Abstract: To determine the frequency and predictors of sleep disorders in children with cerebral palsy (CP) we analyzed the responses of 173 parents who had completed the Sleep Disturbance Scale for Children. The study population included 100 males (57.8%) and 73 females (42.2%; mean age 8y 10mo [SD 1y 11mo]; range 6y-11y 11mo). Eighty-three children (48.0%) had spastic diplegia, 59 (34.1%) congenital hemiplegia, 18 (10.4%) spastic quadriplegia, and 13 (7.5%) dystonic/dyskinetic CP. Seventy-three children (42.2%) were in Gross Motor Function Classification System Level I, 33 (19.1%) in Level II, 30 (17.3%) in Level III, 23 (13.3%) in Level IV, and 14 (8.1%) in Level V. Thirty children (17.3%) had epilepsy. A total sleep problem score and six factors indicative of the most common areas of sleep disorder in childhood were obtained. Of the children in our study, 23% had a pathological total sleep score, in comparison with 5% of children in the general population. Difficulty in initiating and maintaining sleep, sleep-wake transition, and sleep breathing disorders were the most frequently identified problems. Active epilepsy was associated with the presence of a sleep disorder (odds ratio [OR]=17.1, 95% confidence interval [CI] 2.5-115.3), as was being the child of a single-parent family (OR=3.9, 95% CI 1.3-11.6). Disorders of initiation and maintenance of sleep were more frequent in children with spastic quadriplegia (OR=12.9, 95% CI 1.9-88.0), those with dyskinetic CP (OR=20.6, 95% CI 3.1-135.0), and those with severe visual impairment (OR=12.5, 95% CI 2.5-63.1). Both medical and environmental factors seem to contribute to the increased frequency of chronic sleep disorders in children with CP.
TL;DR: Efficacy and overall tolerability for children are maintained after prolonged use of the ketogenic diet, however, side effects, such as slowed growth, kidney stones, and fractures, should be monitored closely.
Abstract: Long-term outcomes of the ketogenic diet in the treatment of epilepsy have not previously been reported. A retrospective chart review of children treated with the ketogenic diet for more than 6 years at the Johns Hopkins Hospital was performed. The response was documented at clinic visits and by telephone contacts; laboratory studies were obtained approximately every 6 to 12 months. Satisfaction and tolerability were assessed by means of a brief parental telephone questionnaire. In all, 28 patients (15 males, 13 females), currently aged 7 to 23 years, were identified. The median baseline seizure frequency per week at diet onset was 630 (range 1-1400). Diet duration ranged from 6 to 12 years; 19 remain on the diet currently. After 6 years or more, 24 children experienced a more than 90% decrease in seizures, and 22 parents reported satisfaction with the diet's efficacy. Ten children were at less than the 10th centile for height at diet initiation; this number increased to 23 at the most recent follow-up (p=0.001). Kidney stones occurred in seven children and skeletal fractures in six. After 6 years or more the mean cholesterol level was 201mg/dl, high-density lipoprotein was 54mg/dl, low-density lipoprotein was 129mg/dl, and triglycerides were 97mg/dl. Efficacy and overall tolerability for children are maintained after prolonged use of the ketogenic diet. However, side effects, such as slowed growth, kidney stones, and fractures, should be monitored closely.
TL;DR: The DCD-Q is reliable and valid in the age range for which the questionnaire was developed and in a younger age range (4-8 y), and met the standard for sensitivity in the clinic-referred sample, but not in the population-based sample.
Abstract: Reliability and validity of the Developmental Coordination Disorder Questionnaire (DCD-Q) was assessed using a population-based sample of 608 children (311 males, 297 females; mean age 7y 8mo [SD 2y 4mo]), a sample of 55 children with DCD referred to a rehabilitation clinic, and a control sample of 55 children matched for age and sex (48 males, seven females in each sample; mean age 8y 3mo [SD 2y]). The DCD-Q is reliable and valid in the age range for which the questionnaire was developed (8y-14y 7mo) and in a younger age range (4-8y). Sensitivity and specificity of the DCD-Q was assessed using the Movement Assessment Battery for Children as the criterion standard. The DCD-Q met the standard for sensitivity (80%) in the clinic-referred sample (81.6%), but not in the population-based sample (28.9%). Specificity almost reached the standard of 90%: 89% in the population-based sample and 84% in the clinic-referred sample.
TL;DR: For children with severe BPD, better recognition and subsequent remediation of neuromotor impairments that manifest at school age may help maximize their functional potential to minimize disability in preterm children.
Abstract: Preterm children who develop severe chronic lung disease may be developmentally compromised by exposure to hypoxic episodes. This study aims to determine if children with severe bronchopulmonary dysplasia (BPD) who required home oxygen therapy were at greater risk for neurological and motor deficits at school age than preterm peers without BPD. This study evaluated 27 subjects with BPD and 27 preterm control infants matched for gestational age, birthweight, sex, and year of birth at a mean age of 9.9 years (2.0 SD) using standardized neuromotor outcome measures. Pair-matched comparisons and regression analyses were used to determine if subjects with BPD were at increased risk for neuromotor sequelae. Neurological abnormalities, including subtle neurological signs, cerebral palsy, microcephaly, and behavioral difficulties were highly prevalent in the BPD group (71% compared with 19% in control group, P<0.005). Over half the BPD cohort had difficulties in gross and/or fine motor skills. There were significant differences in postural stability between groups. Duration of hospitalization and home oxygen treatment, and decreased lung function at school age, markers of severity of illness, correlated with motor outcomes. The findings underline the importance of preventing the cardiorespiratory complications associated with chronic lung disease to minimize disability in preterm children. For children with severe BPD, better recognition and subsequent remediation of neuromotor impairments that manifest at school age may help maximize their functional potential.
TL;DR: Investigating fine motor skills of children with both attention‐deficit–hyperactivity disorder (ADHD) and developmental coordination disorder (DCD) and those of a control group found 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.
Abstract: 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.
TL;DR: Investigation of long‐term survival and causes of death in adult patients with cerebral palsy finds that survival outlook is good though lower than in the general population, although it shows some indication of rising again after age 50 years.
Abstract: 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.
TL;DR: The MACS offers a valid and reliable method for communicating about the manual ability of children with cerebral palsy and may not always agree precisely on a MACS level, particularly if children's performance of manual tasks varies in different environments.
Abstract: 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 (kappa), 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 kappa=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.
TL;DR: It is concluded that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.
Abstract: The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. We conclude that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.
TL;DR: The hypothesis of a dissociation within the sketch‐pad slave system in the WM model is supported and the view of intellectual disability as a non‐unitary condition is reinforced.
Abstract: This study aimed at investigating the possible dissociation between visual-object and visual-spatial working memory (WM) in individuals with Williams syndrome (WS) and Down syndrome (DS). Four study groups were included: WS group (10 males, 5 females) with a mean chronological age (CA) of 19 years 8 months (SD 6y 1mo) and a mean mental age (MA)of 6 years 11 months (SD 1y 5mo); WS comparison group (7 males, 8 females) comprised of typically developing children with a mean CA of 6 years 10 months (SD 10mo) and a mean MA of 6 years 11 months (SD 8mo)matched as a group with the participants with WS on the basis of mental age; DS group (11 males, 7 females) with a mean CA of 15 years 10 months (SD 5y 8mo) and a mean MA of 5 years 2 months (SD 8mo); and DS comparison group (10 males, 8 females) with a mean CA of 5 years and 1 month (SD 7mo)and a mean MA of 5 years 2 months (SD 8mo) selected to match the DS group on the basis of mental age. They were all administered tests that explored visual perception (Visual Perception Test - Subtest 4 and Line Orientation tests), visual imagery (imaging the colour of objects and the tail length of well-known animals), spatial imagery (mental rotation of visually presented or verbally evoked objects), and WM for visual-object and visual-spatial information. Individuals with WS exhibited specific difficulties in the visual-spatial, but not the visual-object, WM task. Instead, people with DS showed reduced performance in both tests. However, whereas the observed deficit in individuals with DS persisted when perceptual abilities were taken into account, the deficit in individuals with DS was compensated when their scores were adjusted for performance on perceptual tasks. These results support the hypothesis of a dissociation within the sketch-pad slave system in the WM model and reinforce the view of intellectual disability as a non-unitary condition.
TL;DR: In this article, convergence evidence is presented suggesting that, in addition to movement execution impairments, impairments in movement planning may also limit the performance of activities of daily living, based on studies of fingertip-force planning and anticipatory planning in object manipulation tasks.
Abstract: Review of the definition of cerebral palsy (CP) has focused on disorders of movement and posture development that cause activity limitations. Recent research provides new insights into CP, showing that activity limitation in individuals with hemiplegic CP is not exclusively caused by disorders related to movement execution, but is also related to the planning of movements. In this review article, converging evidence is presented suggesting that, in addition to movement execution impairments, impairments in movement planning may also limit the performance of activities of daily living. Evidence from studies of fingertip-force planning and anticipatory planning in object manipulation tasks is discussed.
TL;DR: Evaluated correlative magnetic resonance imaging (MRI) and histological parameters of development of cortical afferents during pathfinding and target selection in transient fetal cerebral laminas in human fetuses and preterm infants show a developmental potential for plasticity after perinatal cerebral lesions.
Abstract: The aim of this paper is to evaluate correlative magnetic resonance imaging (MRI) and histological parameters of development of cortical afferents during pathfinding and target selection in transient fetal cerebral laminas in human fetuses and preterm infants. The transient fetal subplate zone, situated between the fetal white matter (i.e. intermediate zone) and the cortical plate, is the crucial laminar compartment for development of thalamocortical and corticocortical afferents. The prolonged coexistence of transient (endogenously active) and permanent (sensory-driven) circuitry within the transient fetal zones is a salient feature of the fetal and preterm cortex; this transient circuitry is the substrate of cerebral functions in preterm infants. Another transient aspect of organization of developing fibre pathways is the abundance of extracellular matrix and guidance molecules in periventricular crossroads of projection and corticocortical pathways. Both the subplate zone and periventricular crossroads are visible on MRI in vivo and in vitro. Hypoxic-ischaemic lesions of periventricular crossroads are the substrate for motor, sensory, and cognitive deficits after focal periventricular leukomalacia (PVL). Lesions of distal portions of the white matter and the subplate zone are the substrate for diffuse PVL. The neuronal elements in transient fetal zones form a developmental potential for plasticity after perinatal cerebral lesions.
TL;DR: A high prevalence of significant sleep problems was reported in Chinese children in Hong Kong with PDD and a higher stress level among the parents of those children with PDd with sleep problems suggests the need for systematic early detection and management of sleep problems in children withPDD.
Abstract: This study aimed to investigate the prevalence of sleep problems in Chinese children with pervasive developmental disorders (PDD) in Hong Kong and their relationship to parental stress. A cross-sectional survey was conducted in six child assessment centres. All parents of the children with PDD completed the Children's Sleep Habits Questionnaire, the Parenting Stress Index-Short Form, and questions on sleep practice. A total of 210 out of 250 questionnaires (response rate 84%) were returned. Mean age of the children was 3 years 6 months (SD 1 y 4 mo; range 2 y-7 y 7 mo; 168 males, 25 females). The prevalence of parent-defined sleep problems in various sleep domains ranged from 9.3 to 45.6%, with 67.9% of children having significant problems in at least one sleep domain. The most common problems reported were bedtime resistance and parasomnias. Similar sleep problems occurred in all the PDD subgroups. The factor most significantly associated with sleep problems was the occurrence of sleep problems before the age of 2 years. The parents of children with PDD with sleep problems experienced a higher level of stress than those whose children had no sleep problems. A high prevalence of significant sleep problems was reported in Chinese children in Hong Kong with PDD. A higher stress level among the parents of those children with PDD with sleep problems suggests the need for systematic early detection and management of sleep problems in children with PDD.