Construct validity, reliability, and responsiveness of the Wrist Position Sense Test for use in children with hemiplegic cerebral palsy.

INTRODUCTION: We investigate the construct validity, test re-test reliability, and responsiveness of the Wrist Position Sense Test (WPST) for children with hemiplegic cerebral palsy (CP). METHODS: Twenty-eight children with spastic hemiplegic CP [mean age 10.8 years; SD 2.4 years] and 39 typically developing (TD) children [mean age 11 years; SD 2.9 years] participated in a cross-sectional study to investigate construct validity and association with an upper limb activity measure, the Box and Block Test (BBT). Twenty-two TD children were tested at a second time-point to examine reliability. Test responsiveness was determined by random allocation of 17 children with CP to a treatment (n = 10) or control (n = 7) group with assessments completed at four time-points. RESULTS: Significantly greater differences were observed in mean error of indicated wrist position (p < 0.01) in children with CP at baseline (M = 21.6°, SD = 21.6°) than in TD children (M = 12.8°, SD = 11.0°). Larger WPST errors were associated with poorer performance on the BBT (p < 0.01) indicating a substantial association, and there were no consistent differences between time-points indicating test re-test reliability within a TD population. The WPST demonstrated responsiveness to intervention with a statistically significant reduction in mean error following treatment (p < 0.001), not seen in the control group (p = 0.28). CONCLUSION: The WPST demonstrated construct validity in this preliminary study. Scores were associated with an upper limb activity measure, and scores changed significantly following somatosensory training. These findings support further research and future psychometric investigation of the WPST in children with CP. KEY POINTS FOR OCCUPATIONAL THERAPY: This study provides psychometric knowledge about the WPST tool The WPST shows promise as a discriminative measure with preliminary evidence of responsiveness and intra-rater reliability Until further testing, the WPST can be used cautiously in future research studies to measure wrist position sense.

Congenital anomalies in children with pre- or perinatally acquired cerebral palsy: an international data linkage study.

AIM: To describe the frequency and types of major congenital anomalies present in children with pre- or perinatally acquired cerebral palsy (CP), and compare clinical outcomes for children with and without anomalies. METHOD: This multi-centre total population collaborative study between Surveillance of Cerebral Palsy in Europe, Australian Cerebral Palsy Register, and European Surveillance of Congenital Anomalies (EUROCAT) involved six European and three Australian regions. Data were linked between each region's CP and congenital anomaly register for children born between 1991 and 2009, and then pooled. Children were classified into mutually exclusive categories based on type of anomaly. Proportions of children with congenital anomalies were calculated, and clinical outcomes compared between children with and without anomalies. RESULTS: Of 8201 children with CP, 22.8% (95% confidence interval [CI] 21.9, 23.8) had a major congenital anomaly. Isolated cerebral anomalies were most common (45.2%), with a further 8.6% having both cerebral and non-cerebral anomalies. Cardiac anomalies only were described in 10.5% of children and anomalies associated with syndromes were also reported: genetic (8.0%), chromosomal (5.7%), and teratogenic (3.0%). Clinical outcomes were more severe for children with CP and congenital anomalies, particularly cerebral anomalies. INTERPRETATION: This large, international study reports major congenital anomalies in nearly one-quarter of children with pre- or perinatally acquired CP. Future research must focus on aetiological pathways to CP that include specific patterns of congenital anomalies. WHAT THIS PAPER ADDS: Congenital anomalies were reported in 23% of children with pre- or perinatally acquired cerebral palsy. A higher proportion of children born at or near term had anomalies. The most common type of anomalies were isolated cerebral anomalies. Clinical outcomes were more severe for children with congenital anomalies (particularly cerebral).

Congenital anomalies in children with postneonatally acquired cerebral palsy: an international data linkage study.

AIM: To describe the major congenital anomalies present in children with postneonatally acquired cerebral palsy (CP), and to compare clinical outcomes and cause of postneonatally acquired CP between children with and without anomalies. METHOD: Data were linked between total population CP and congenital anomaly registers in five European and three Australian regions for children born 1991 to 2009 (n=468 children with postneonatally acquired CP; 255 males, 213 females). Data were pooled and children classified into mutually exclusive categories based on type of congenital anomaly. The proportion of children with congenital anomalies was calculated. Clinical outcomes and cause of postneonatally acquired CP were compared between children with and without anomalies. RESULTS: Major congenital anomalies were reported in 25.6% (95% confidence interval [CI] 21.7-29.9) of children with postneonatally acquired CP. Cardiac anomalies, often severe, were common and present in 14.5% of children with postneonatally acquired CP. Clinical outcomes were not more severe in children with congenital anomalies than those without anomalies. Cause of postneonatally acquired CP differed with the presence of congenital anomalies, with cerebrovascular accidents predominating in the anomaly group. Congenital anomalies were likely associated with cause of postneonatally acquired CP in 77% of children with anomalies. INTERPRETATION: In this large, international study of children with postneonatally acquired CP, congenital anomalies (particularly cardiac anomalies) were common. Future research should determine specific causal pathways to postneonatally acquired CP that include congenital anomalies to identify opportunities for prevention. WHAT THIS PAPER ADDS: One-quarter of children with postneonatally acquired cerebral palsy (CP) have a major congenital anomaly. Cardiac anomalies, often severe, are the most common anomalies. Causes of postneonatally acquired CP differ between children with and without congenital anomalies.

A prospective study investigating gross motor function of children with cerebral palsy and GMFCS level II after long-term Botulinum toxin type A use.

BACKGROUND: The aim of this study is to contribute to the knowledge base on the long-term outcomes of evidence-based medical interventions used to improve gross motor function in children and adolescents with Cerebral Palsy. METHOD: Prospective cohort study of children with Cerebral Palsy in the birth years 2000-2009 attending a tertiary level service for children with Cerebral Palsy who's first recorded Gross Motor Function Classification System level was II. RESULTS: A total of 40 children were eligible for the study, of whom 28 (72.7%) enrolled. The Botulinum toxin A treatment for this cohort, (median and interquartile ranges) were: total number of lower limb Botulinum toxin A injections 11 (6.7, 5.5); total dose of Botulinum Toxin A per lower limb treatment 6.95 u/kg (4.5, 11); and dose of Botulinum Toxin u/kg/muscle 2.95 (2.2, 4). For all 28 subjects there was a median of 15 (8.5 to 22) Gross Motor Function Classification System level recordings: six of the 28 children (21.4%) improved from level II to level I, the remaining 22 children remained stable at level II (78.6%). In this highly treated population, the average 66 item Gross Motor Function Measure score for the 22 children in level II was 72.55, which is consistent with the mean of 68.5 reported in the original Ontario cohort. CONCLUSION: This cohort study has confirmed that children with Cerebral Palsy, Gross Motor Function level II treated at a young age with repeated doses of Botulinum Toxin A within an integrated comprehensive service, maintain or improve their functional motor level at a later age.

Normative data of muscle fiber diameter of vastus lateralis during childhood: a field test.

INTRODUCTION: Currently, our knowledge of standard data for muscle morphology in children is largely limited to the 1969 article by Brooke and Engel (BE). In 2016, we reported normal muscle morphology from vastus lateralis biopsies in ambulant children with cerebral palsy (CP). This report compares our normal biopsy results against BE standard value criteria. METHODS: Single-blind prospective cross-sectional study design. RESULTS: Results of biopsies taken in ambulant children with CP were normal according to morphometry and light and electron microscopy; however, only 5 of 10 fulfilled the BE standard value criteria. DISCUSSION: This short report highlights the requirement for contemporary age-specific normative data from a larger number of biopsies, including typically developing children. Review of the literature suggests that biopsy material may be available from typically developing children who were control patients in research trials. This morphometric data could contribute to expanding the normative data set. Muscle Nerve 59:590-590, 2019.

Survival and mortality in cerebral palsy: observations to the sixth decade from a data linkage study of a total population register and National Death Index.

BACKGROUND: Likely duration of survival of children described as having cerebral palsy is of considerable interest to individuals with cerebral palsy, their families, carers, health professionals, health economists and insurers. The aim of this paper is to describe patterns of survival and mortality to the sixth decade in a geographically defined population of people with cerebral palsy stratified according to the clinical description of their impairments in early childhood. METHODS: Identifiers of persons born in Western Australia 1956-2011, registered with cerebral palsy on the Western Australian Register of Developmental Anomalies and surviving at least 12 months, were linked to the Australian National Death Index in December 2014. Patterns of mortality were investigated using survival analysis methods. RESULTS: Of 3185 eligible persons, 436 (13.7%) had died. Of that sample the 22% with the mildest impairment had survival patterns similar to the general population. Mortality increased with increasing severity of impairment. Of 349 (75%) with available cause of death data, 58.6% were attributed to respiratory causes, including 171 (49%) to pneumonia at a mean age of 14.6 (sd 13.4) years of which 77 (45%) were attributed to aspiration. For the most severely impaired, early childhood mortality increased in succeeding decades of birth cohorts from 1950s to 1990 with 20% dying by 4 years of age in the 1981-1990 birth cohort; it then decreased for subsequent birth cohorts, 20% mortality not being attained until 15 years of age. However by 20 years of age mortality of the most severely impaired born in the 1991-2000 birth cohort exceeded that of all other birth cohorts. Remaining life expectancies by age to 50 years have been estimated for two strata with more severe impairments. CONCLUSION: For 22% of individuals with cerebral palsy with mild impairment survival to 58 years is similar to that of the general population. Since 1990 mortality for those with severe cerebral palsy in Western Australia has tended to shift from childhood to early adulthood.

Impact of social disadvantage on cerebral palsy severity.

AIM: To investigate the impact of socio-economic disadvantage on indicators of cerebral palsy (CP) severity - motor impairment, intellectual disability, and the presence of severe comorbidities - in children with CP in Australia. METHOD: Data from the Australian Cerebral Palsy Register were analysed. Socio-economic disadvantage was assessed using maternal age, maternal country of birth, and a measure of neighbourhood socio-economic status (SES) at the time of the child's birth. Descriptive bivariate analysis, trend analysis, risk ratios, and mediation analysis were undertaken to examine the impact of disadvantage on the indicators of CP severity. RESULTS: A socio-economic gradient was demonstrated with an increasing proportion of children with non-ambulant status, at least moderate intellectual disability, and the presence of severe comorbidities (having epilepsy, functional blindness, bilateral deafness, and/or no verbal communication) with decreasing neighbourhood SES, adolescent motherhood, and maternal minority ethnicity. INTERPRETATION: In Australia, socio-economic disadvantage at birth impacts adversely on CP severity at age 5 years. By identifying that socio-economically disadvantaged children with CP are at greater risk of more severe functional outcomes, we can inform targeted interventions at the family and neighbourhood level to reduce these inequities for children with CP. WHAT THIS PAPER ADDS: Socio-economic disadvantage is associated with increased severity of cerebral palsy functional outcomes. This encompasses low neighbourhood socio-economic status, adolescent motherhood, and maternal minority ethnicity.

IMPACTO DE LA DESVENTAJA SOCIAL SOBRE LA SEVERIDAD DE LA PARÁLISIS CEREBRAL: OBJETIVO: Investigar el impacto de la desventaja socioeconómica en los indicadores de severidad de la parálisis cerebral (PC), definida como - deterioro motor, discapacidad intelectual y la presencia de severidad de las comorbilidades: en niños con PC en Australia. MÉTODO: Se analizaron los datos del Australian Cerebral Palsy Register. La desventaja socioeconómica se evaluó utilizando la edad materna, el país de nacimiento de la madre, y una medida de estado socioeconómico del vecindario (SES) en el momento del nacimiento del niño. Se realizaron análisis bivariados, análisis de tendencias, índices de riesgo y análisis de mediación para examinar el impacto de la desventaja en los indicadores de severidad PC. RESULTADOS: Se demostró un gradiente socioeconómico con una proporción creciente de niños con estado no ambulante, al menos discapacidad intelectual moderada, y la presencia de comorbilidades graves (con epilepsia, ceguera funcional, sordera bilateral y / o sin comunicación verbal) con SES vecinales, maternidad adolescente, y etnia de la minoría materna. INTERPRETACIÓN: En Australia, la desventaja socioeconómica al nacer tiene un impacto adverso en la severidad de PC a la edad de 5 años. Al identificar a los niños con desventajas socioeconómicas con PC tienen un mayor riesgo de resultados funcionales más severos. Con esta información podemos guiar intervenciones a nivel familiar y de vecindario para reducir estas inequidades en los niños con PC.

IMPACTO DA DESVANTAGEM SOCIAL NA SEVERIDADE DA PARALISIA CEREBRAL: OBJETIVO: Investigar o impacto da desvantagem sócio-econômica nos indicadores de severidade da paralisia (PC) - comprometimento motor, deficiência intelectual, e a presença de comorbidades graves - em crianças com PC na Austrália. MÉTODO: Dados do Registro Austrliano de Paralisia Cerebral foram analisados. Desvantagens sócio-econômicas foram avaliadas usando-se a idade materna, o país de Nascimento da mãe, e uma medida do estado sócio-econômico (ESE) da vizinhança no momento do nascimento da criança. Análise bivariada descritiva, análise de tendências, taxas de risco, e análise de mediação foram realizados para examinar o impacto da desvantagem nos indicadores da severidade da PC. RESULTADOS: Um gradiente sócio-econômico foi demonstrado com um aumento da proporção de crianças com situação não ambilante, deficiência intelectual pelo menos moderada, e presença de comorbidades severas (epilepsia, cegueira funcional, surdez bilateral, e/ou nenhuma comunicação verbal) quanto menor o ESE da vizinhança, a minoria étnica materna e maternidade na adolescência. INTERPRETAÇÃO: Na Austrália, a desvantagem sócio-econômica ao nascimento impacta adversamente a severidade da PC na idade de 5 anos. Ao identificar que crianças com PC em desvantagem sócio-econômica têm maior risco de resultados funcionais mais severos, podemos informar intervenções específicas voltadas para a família e para a vizinhança para reduzir o nível destas inequidades para crianças com PC.

Cerebral palsy trends in Australia (1995-2009): a population-based observational study.

AIM: To investigate trends in birth prevalence of cerebral palsy (CP) overall and by gestational age, and examine the distribution of motor type, spastic topography, and severity using Australian CP Register data from 1995 to 2009. METHOD: Prenatal and perinatal CP data were collated from state/territory CP registers. Birth prevalence estimates per 1000 live births and per 1000 neonatal survivors (NNS) were calculated in five epochs. Data from three state registers with population-level ascertainment were used to investigate birth prevalence trends by gestational age using Poisson regression. Distribution of motor type, spastic topography, and moderate to severe disability (IQ≤50 and/or Gross Motor Function Classification System levels III-V) were evaluated within birthweight categories. RESULTS: Birth prevalence of CP varied across population-level states but within each state declined significantly over time (p<0.05). Birth prevalence per 1000 neonatal survivors declined amongst children born before 28 weeks (South Australia, Victoria p<0.001) and those born at or after 37 weeks (Victoria p<0.001, Western Australia p<0.002). Across Australia the percentage of children with bilateral spastic CP declined amongst those born less than 1000g. The percentage of children with moderate to severe disability decreased (48%-34%, p<0.001). INTERPRETATION: Birth prevalence of CP declined. Encouragingly, the percentage of children with CP whose disability was moderate to severe also decreased. WHAT THIS PAPER ADDS: Birth prevalence of cerebral palsy (CP) differed but declined across Australian states (1995-2009). Australian CP birth prevalence declined significantly amongst children born before 28 weeks and those born at or after 37 weeks. The percentage of children with moderate to severe disability decreased.

Prenatal and perinatal risks for late language emergence in a population-level sample of twins at age 2.

BACKGROUND: Late Language Emergence (LLE) in the first two years of life is one of the most common parental concerns about child development and reasons for seeking advice from health professionals. LLE is much more prevalent in twins (38%) than singletons (20%). In studies of language development in twins without overt disability, adverse prenatal and perinatal environments have been reported to play a lesser role in the etiology of LLE than adverse postnatal environments. However, there is a lack of population-level evidence about prenatal and perinatal risk factors for LLE in twins. This study investigated the extent to which prenatal and perinatal risk factors were associated with LLE in a population-level sample of twins at age 2 without overt disability. METHODS: The sample comprised 473 twin pairs drawn from a population sample frame comprising statutory notifications of all births in Western Australia (WA), 2000-2003. Twin pairs in which either twin had a known developmental disorder or exposure to language(s) other than English were excluded. Of the 946 twins, 47.9% were male. There were 313 dizygotic and 160 monozygotic twin pairs. LLE was defined as a score at or below the gender-specific 10th percentile on the MacArthur Communicative Development Inventories: Words and Sentences (CDI-WS) (Words Produced). Bivariate and multivariable logistic regression was used to investigate risk factors associated with LLE. RESULTS: In the multivariable model, risk factors for LLE in order of decreasing magnitude were: Gestational diabetes had an adjusted odds ratio (aOR) of 19.5 (95% confidence interval (CI) 1.2, 313.1); prolonged TSR (aOR: 13.6 [2.0, 91.1]); multiparity (aOR: 7.6 [1.6, 37.5]), monozygosity (aOR: 6.9 [1.7, 27.9]) and fetal growth restriction (aOR: 4.6 [1.7, 12.7]). Sociodemographic risk factors (e.g., low maternal education, socioeconomic area disadvantage) were not associated with increased odds of LLE. CONCLUSIONS: The results suggest that adverse prenatal and perinatal environments are important in the etiology of LLE in twins at age 2. It is important that health professionals discuss twin pregnancy and birth risks for delayed speech and language milestones with parents and provide ongoing developmental monitoring for all twins, not just twins with overt disability.

Discovering the sense of touch: protocol for a randomised controlled trial examining the efficacy of a somatosensory discrimination intervention for children with hemiplegic cerebral palsy.

BACKGROUND: Of children with hemiplegic cerebral palsy, 75% have impaired somatosensory function, which contributes to learned non-use of the affected upper limb. Currently, motor learning approaches are used to improve upper-limb motor skills in these children, but few studies have examined the effect of any intervention to ameliorate somatosensory impairments. Recently, Sense© training was piloted with a paediatric sample, seven children with hemiplegic cerebral palsy, demonstrating statistically and clinically significant change in limb position sense, goal performance and bimanual hand-use. This paper describes a protocol for a Randomised Controlled Trial of Sense© for Kids training, hypothesising that its receipt will improve somatosensory discrimination ability more than placebo (dose-matched Goal Directed Therapy via Home Program). Secondary hypotheses include that it will alter brain activation in somatosensory processing regions, white-matter characteristics of the thalamocortical tracts and improve bimanual function, activity and participation more than Goal Directed Training via Home Program. METHODS AND DESIGN: This is a single blind, randomised matched-pair, placebo-controlled trial. Participants will be aged 6-15 years with a confirmed description of hemiplegic cerebral palsy and somatosensory discrimination impairment, as measured by the sense©_assess Kids. Participants will be randomly allocated to receive 3h a week for 6 weeks of either Sense© for Kids or Goal Directed Therapy via Home Program. Children will be matched on age and severity of somatosensory discrimination impairment. The primary outcome will be somatosensory discrimination ability, measured by sense©_assess Kids score. Secondary outcomes will include degree of brain activation in response to a somatosensory task measured by functional MRI, changes in the white matter of the thalamocortical tract measured by diffusion MRI, bimanual motor function, activity and participation. DISCUSSION: This study will assess the efficacy of an intervention to increase somatosensory discrimination ability in children with cerebral palsy. It will explore clinically important questions about the efficacy of intervening in somatosensation impairment to improve bimanual motor function, compared with focusing on motor impairment directly, and whether focusing on motor impairment alone can affect somatosensory ability. TRIAL REGISTRATION: This trial is registered with the Australian New Zealand Clinical Trials Registry, registration number: ACTRN12618000348257. World Health Organisation universal trial number: U1111-1210-1726.

Construct validity and responsiveness of the functional Tactile Object Recognition Test for children with cerebral palsy.

BACKGROUND/AIM: The functional Tactile Object Recognition Test (fTORT) is a measure of haptic object recognition capacity recently adapted for use with children with neurological impairment. The current study aimed to investigate preliminary evidence of construct validity and responsiveness of the fTORT and its association with a measure of upper limb activity. METHODS: A cross-sectional study of 28 children with spastic hemiplegic cerebral palsy (CP) (mean age 10 years 8 months; SD two years four months; 16 male) and 39 typically developing (TD) children (mean age 11 years; SD two years nine months; 19 male) was utilised to investigate construct validity and association between measures. Sixteen children with CP (mean age 10 years 10 months; SD two years 8 months; 9 male) who were randomly allocated to either a treatment (n = 6) or control group (n = 10) were assessed at four time points to assess test responsiveness. RESULTS: There was a very significant difference (P value <0.0001) indicating greater haptic object recognition ability for the TD group (n = 39; median: 40; range: 33-42) than the group with CP (n = 28; median: 32.5; range: 3-41). fTORT scores demonstrated a significant association with scores on the activity measure (Pearson's r: 0.68; P = 0.0001). There were no significant changes over time in fTORT scores (P = 0.22) and no significant difference between the treatment and control groups (P = 0.47). CONCLUSION: The fTORT demonstrated preliminary construct validity, and was positively associated with an upper limb activity measure but scores did not change significantly following somatosensory training. This preliminary paper supports further research and future psychometric knowledge about the tool.

Clinical acceptability of the sense_assess© kids: Children and youth perspectives.

BACKGROUND: The sense_assess© kids is a standardised, norm-referenced assessment designed to measure the functional somatosensation capacity of the upper limb of children with cerebral palsy. The objective of the current study was to determine if the sense_assess© kids was clinically acceptable to children and youth. METHODS: A questionnaire was completed by participants following administration of the sense_assess© kids by a trained occupational therapist. Twenty-six children with spastic hemiplegic cerebral palsy (aged 6-15 years six months; mean 10 years eight months; 16 boys) were recruited. Participants responded to questions regarding the administration and level of difficulty of the sense_assess© kids using a Q-Sort of 'like' and 'dislike', Likert scales and short answers. Content analysis was applied. RESULTS: Twenty-one of twenty-six children, indicated that they were 'very happy' or 'happy' with the administration process of the sense_assess© kids. Most participants indicated that they liked the sensation they felt in the hand when tested. CONCLUSION: This study has demonstrated the acceptability of sense_assess© kids for the population for whom it is intended.

The psychosocial burden of childhood overweight and obesity: evidence for persisting difficulties in boys and girls.

There is evidence that overweight and obese children tend to remain overweight or obese into adolescence and adulthood. However, little is known about the long-term psychosocial outcomes of childhood overweight and obesity. This study aimed to investigate the course of psychosocial difficulties over a 2-year period for children who were overweight or obese at baseline, and a sample of children who were a healthy weight at baseline. Participants were 212 children aged 8 to 13 years at baseline, who were participating in the Childhood Growth and Development (GAD) Study. Questionnaire and interview measures were used to assess children's self-esteem, depressive symptoms, body image, eating disorder symptoms, experiences with bullying, family satisfaction and quality of life. Linear mixed models were used to consider longitudinal changes in psychosocial variables. Overweight and obese children reported greater psychosocial distress than healthy weight children, and these differences were more pronounced for girls than boys. Weight and psychosocial impairment showed stability from baseline to 2-year follow-up. CONCLUSION: The results of this study suggest that psychosocial difficulties show considerable stability in childhood, for overweight/obese and healthy weight children. What is Known: • Childhood obesity tracks into adolescence and adulthood. • Physical health problems associated with childhood obesity also persist to adulthood. What is New: • Overweight and obese children are at risk of ongoing psychosocial distress from childhood into early adolescence.

Somatosensory Discrimination Intervention Improves Body Position Sense and Motor Performance in Children With Hemiplegic Cerebral Palsy.

OBJECTIVE: This study examined the use of the adult neuroscience-based Sense© intervention with children with hemiplegic cerebral palsy (HCP) to improve upper-limb somatosensory discrimination, motor function, and goal performance. METHOD: Seventeen children with HCP (9 boys, 8 girls; mean age = 10.2 yr) participated in this pilot matched-pairs trial with random allocation and 6-mo follow-up (intervention, n = 7; control, n = 10). The intervention group received Sense training 3×/wk for 6 wk (18 hr). Outcome measures included Goal Attainment Scaling, Sense_assess© Kids, and the Assisting Hand Assessment. RESULTS: The intervention group improved in goal performance, proprioception, and bimanual hand use and maintained improvement at 6-mo follow-up. The control group improved in occupational performance by 6-mo follow-up. CONCLUSION: This study established the feasibility of using the Sense intervention in a pediatric setting and adds preliminary evidence to suggest that improving somatosensory function can improve motor function and goal performance among children with HCP.

Factors Associated with Respiratory Illness in Children and Young Adults with Cerebral Palsy.

OBJECTIVE: To describe associations between respiratory illness and its potential predictors in children and young adults with cerebral palsy (CP). STUDY DESIGN: Cross-sectional survey of self- and caregiver-reported respiratory symptoms for individuals aged up to 26 years with CP. Respiratory illness was indicated by 2 outcomes: (1) ≥1 respiratory hospitalizations in the past year; and (2) ≥2 courses of antibiotics for respiratory symptoms in the past year. ORs were calculated using univariate and multivariate logistic regression. RESULTS: There were 551 participants, aged 1-26 years, distributed across all gross motor function classification scale (GMFCS) levels. In univariate analyses, factors significantly associated with respiratory hospitalizations were weekly respiratory symptoms (OR 2.31, 95% CI 1.78-3.00), respiratory symptoms during meals (OR 3.23, 95% CI 1.50-5.80), gastroesophageal reflux (OR 3.01, 95% CI 1.71-5.31), coughing or choking on saliva (OR 4.36, 95% CI 2.38-8.01), current asthma (OR 3.56, 95% CI 1.97-6.42), age (0-3 years) (OR 3.24, 95% CI 1.19-8.80, compared with 13-17 years), seizures (OR 3.45, 95% CI 1.96-6.08), and scoliosis (OR 2.14, 95% CI 1.16-3.97). Nonambulatory individuals (GMFCS IV-V) were at significantly increased risk of hospitalizations only if they had food modifications and/or nasogastric or gastrostomy tube feeds (OR 5.36, 95% CI 2.89-9.96, compared with GMFCS I-III with no food modifications and no tube). All factors, except seizures and scoliosis, were significantly associated with multiple courses of antibiotics in univariate analyses. CONCLUSIONS: Oromotor dysfunction is strongly associated with respiratory illness in patients with CP.

Cerebral palsy and perinatal mortality after pregnancy-induced hypertension across the gestational age spectrum: observations of a reconstructed total population cohort.

AIM: Pregnancy-induced hypertension/pre-eclampsia (PIH/PE) is associated with cerebral palsy (CP) in term births but if sufficiently severe to necessitate preterm delivery predicts a lower risk of CP than observed in gestational peers. We investigated whether this apparent 'protection' was attributable to inappropriately chosen comparison groups and/or an increased risk of perinatal death. METHOD: Perinatal information was collected from medical records of children with CP, individually matched neonatal survivors without CP, and representative samples of perinatal deaths of Western Australian birth cohorts from 1980 to 1995. Compared with these data, the sensitivity of statutorily collected PIH/PE data was assessed for each outcome group. Using these sensitivities, the estimated risks of death and CP in births to all women with and without PIH/PE were compared. RESULTS: Sensitivity of statutory PIH/PE data decreased with increasingly poor outcome. Reconstructed cohorts showed that PIH/PE increased the risks both of CP and of perinatal death in births at lower gestations except in births <27 weeks, where the risk of perinatal death only increased greatly. INTERPRETATION: PIH/PE does not protect against poor outcome at any gestational age. Previously reported protective effects originate from inappropriate control for gestational age and not from higher gestation-specific perinatal mortality.

Strabismus, a preventable barrier to social participation: a short report.

Isolated strabismus does not significantly impair visual functionality and has traditionally been considered a primarily cosmetic defect of little importance. However, even in the absence of strabismus amblyopia, manifest strabismus and its non-surgical treatments can render the person less socially acceptable, creating a barrier to participation and resulting in psychosocial disadvantage that has been documented in the typically developing population. The Australian Cerebral Palsy Register traditionally recorded strabismus only if it were not accompanied by visual impairment; however, even these data indicate that the proportion of cerebral palsy registrants with strabismus is many times higher than in comparable population samples, compounding their challenges to achieve participation. It is therefore inappropriate to continue to consider strabismus as merely a cosmetic defect, but one that deserves surgical correction early in life.

Interobserver reliability of the Australian Spasticity Assessment Scale (ASAS).

AIM: The aim of this paper is to present the Australian Spasticity Assessment Scale (ASAS) and to report studies of its interrater reliability. The ASAS identifies the presence of spasticity by confirming a velocity-dependent increased response to rapid passive movement and quantifies it using an ordinal scale. METHOD: The rationale and procedure for the ASAS is described. Twenty-two participants with spastic CP (16 males; age range 1y 11mo-15y 3mo) who had not had botulinum neurotoxin-A within 4 months, or bony or soft tissue surgery within 12 months, were recruited from the spasticity management clinic of a tertiary paediatric teaching hospital. Fourteen muscles in each child were assessed by each of three experienced independent raters. ASAS was recorded for all muscles. Interrater reliability was calculated using the weighted kappa statistic (quadratic weighting; κqw) for individual muscles, for upper limbs, for lower limbs, and between raters. RESULTS: The weighted kappa ranged between 0.75 and 0.92 for individual muscle groups and was 0.87 between raters. INTERPRETATION: The ASAS complies with the definition of spasticity and is clinically feasible in paediatric settings. Our estimates of interrater reliability for the ASAS exceed that of the most commonly used spasticity scoring systems.

Comparing risks of cerebral palsy in births between Australian Indigenous and non-Indigenous mothers.

AIM: To compare proportions of live births subsequently described as having cerebral palsy (CP), the distributions of associated impairments, and the causes of postneonatal CP between Aboriginal and Torres Strait Islander (Indigenous) and non-Indigenous populations in Australia. METHOD: Data from statutory birth records and CP registers for the 1996 to 2005 birth cohort in Queensland, Western Australia, and the Northern Territory were stratified by Indigenous status and whether the CP was acquired pre/perinatally or postneonatally. Relative risks associated with Indigenous status were estimated and the distributions of causes of postneonatal CP compared. RESULTS: Indigenous births had a relative risk of 4.9 (95% confidence interval [CI] 3.0-7.9) for postneonatal CP but only of 1.42 (95% CI 1.2-1.7) for pre/perinatal CP. Almost half of postneonatal CP in Indigenous infants resulted from infection, whereas for non-Indigenous infants the most frequent cause was cerebrovascular accident. The impairments of Indigenous CP and of postneonatally acquired CP tended to be more numerous and more severe. INTERPRETATION: Indigenous children are at significantly greater risk of CP, particularly postneonatal CP. The predominant cause of postneonatal CP in non-Indigenous children has shifted to cerebrovascular accident over time; however, infections followed by head injury are still the most frequent causes in Indigenous infants.

Congenital anomalies in cerebral palsy: where to from here?

Proportions of cases of cerebral palsy (CP) with congenital anomalies recorded in Australian CP registers range from 15% to 40%. The anomalies seen in CP are extremely variable. We have identified that CP registers often do not have quality data on congenital anomalies, necessitating linkage with congenital anomaly registers. However, a lack of unified processes and definitions in congenital anomaly registers and data collections means that linkages are complex, need to be carefully planned, and limitations acknowledged. Historically in CP research, congenital anomalies have been classified by International Classification of Disease codes, then combined into brain and other major and minor anomalies. Systems have been developed to classify congenital anomalies into aetiologically related groups, but such a classification has yet to be trialled in CP. It is anticipated that primary prevention of a small proportion of cases of CP is possible through the primary prevention of congenital anomalies, especially those due to teratogens. Owing to the anticipated low prevalence of each subgroup, global collaboration will be required to further these lines of enquiry.