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Aim: To describe the distribution of neuroimaging patterns in a term/late preterm population‐based cohort with cerebral palsy (CP), ascertain associations between neuroimaging patterns and neonatal well‐being, estimate the proportion with antenatal or perinatal timing of neuropathology, and apply this information to the understanding of common mechanisms of brain injury and causal pathways. Method: The cohort for this observational study comprised 1348 persons born between 1999 and 2017 in Victoria, Australia. Using algorithms designed for the study, neonatal well‐being and timing of brain injury were tabulated for the whole cohort and across neuroimaging patterns and birth epochs. Results: Clinical and demographic profiles, neonatal well‐being, and timing of brain injury differed across neuroimaging patterns. An estimated 57% of the cohort had a complicated neonatal period. Timing of brain injury was antenatal in 57% and perinatal in 41%. A decrease in the relative proportions of perinatal timing of brain injury was observed over a period when the rates of CP in live births at term decreased. Interpretation: This study begins to bridge the knowledge gap about causation in CP, moving towards better description of the main mechanisms of brain injury and their contribution within CP cohorts, and facilitating the ability to monitor changes over time and the success of preventive measures. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. This original article is commented on by O'Shea on pages 829–830 of this issue. [ABSTRACT FROM AUTHOR]

Aim: To better understand parents' beliefs about causation in cerebral palsy (CP) and the emotions related to those beliefs. Method: We surveyed 226 parents of children with CP aged 1 to 18 years, recruited from the Victorian Cerebral Palsy Register, to evaluate their beliefs about the causes of CP, including genetic causes, causes specific to their own child, and their attitudes and emotions in relation to these. Results: Although 92% of participants reported that understanding the causes of their child's CP was important, uncertainty about the cause was expressed by 13%. The most frequently endorsed causal factors, in general and in their own child respectively, were intrapartum hypoxia (81%, 36%) or brain damage (69%, 22%), brain damage during pregnancy (73%, 28%), and preterm birth (66%, 28%). Genetic causes were deemed relevant by 13% of participants and hospital or professional error by 16%. Parents shared related feelings of anger (59%), sadness (80%), guilt (61%), and confusion (53%); parental anger was more likely when their child's CP was attributed to intrapartum events. Interpretation: Substantial parental interest in understanding the causes of CP, together with uncertainty about the causes, parents' causal attributions, and significant emotional sequelae, highlight a strong need for provision of information and support for families of children recently diagnosed with CP. What this paper adds: Understanding the causes of their child's cerebral palsy (CP) was important to parents.Parents most often endorsed intrapartum factors as a cause of CP.Parents reported experiencing strong emotions about the causes of their child's CP. What this paper adds: Understanding the causes of their child's cerebral palsy (CP) was important to parents.Parents most often endorsed intrapartum factors as a cause of CP.Parents reported experiencing strong emotions about the causes of their child's CP. This original article is commented on by Vasyutin on page 148 of this issue. [ABSTRACT FROM AUTHOR]

CP subgroups To explore whether specific aetiological and clinical variables, or combinations thereof, were related to extreme biomarker values in CP cases, additional variables were extracted from the Victorian Cerebral Palsy Register. To assess generalizability to the wider CP population, Table S1 (online supporting information) shows characteristics of the 435 matched CP singleton cases and compares them to the 941 records from the eligible CP sample that were unable to be matched to a cFTS record. Nuchal translucency and CP There was little difference in the mean nuchal translucency MoMs between CP cases (geometric mean 0.96 [95% CI 0.94-0.98]) and non-CP controls (geometric mean 0.95 [95% CI 0.95-0.96]; I p i =0.62 for difference; Table 1). When PAPP-A and -hCG levels were considered together, CP pregnancies were 2.6 times more likely than non-CP pregnancies to have low levels of both PAPP-A and -hCG. [Extracted from the article]

Aim: To determine: the effectiveness of three anticholinergic medications in reducing drooling in children with developmental disabilities (such as cerebral palsy, intellectual disability, and autism spectrum disorder), the frequency and nature of side effects, and their impact on treatment discontinuation.Method: After prescription of benzhexol hydrochloride, glycopyrrolate, or scopolamine patches at a tertiary saliva control clinic, all carers of 110 consecutive, eligible patients were recruited over a 5-year period. They provided data for 52 weeks, or until drug discontinuation, on compliance, drooling, adverse effects, and reasons for cessation. We evaluated and compared best drooling response, side effects, and drug cessation rates using survival analysis, and the effect of baseline variables on the discontinuation rate using proportional hazards regression.Results: Among 110 participants (71 males, 39 females; mean age 8y 5mo [SD 4y 3mo], range 1y 11mo-18y 11mo), benzhexol, glycopyrrolate, and scopolamine were prescribed 81, 62, and 17 times respectively, with respective response rates of 85%, 75%, and 65%. Poor head control and poor oromotor function were predictive of poor response. Side effects frequently prompted drug cessation in males more than females (hazard ratio 1.8 [95% confidence interval 1.0-3.2], p=0.048). Glycopyrrolate had the fewest side effects.Interpretation: Benzhexol, glycopyrrolate, and scopolamine reduce drooling, but improvement is offset by adverse side effects. Overall, glycopyrrolate performs best.What This Paper Adds: In drooling, glycopyrrolate produced the greatest improvement with fewer side effects compared with benzhexol and scopolamine. Poor head control and poor oromotor function were associated with poor response. Medication side effects were common and often led to treatment discontinuation. Behavioural issues instigated cessation of benzhexol more often in males than females. [ABSTRACT FROM AUTHOR]

Aim: A population-based observational study design was used to describe the epidemiology of intellectual disability in cerebral palsy (CP) in terms of clinical and neuroimaging associations, and to report the impact of intellectual disability on utilization of health services and length of survival.Method: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns (n=1141). Data linkage was undertaken to assess usage of hospital inpatient and emergency department services. Survival analysis was performed in a 30-year birth cohort (n=3248).Results: Intellectual disability, present in 45% of the cohort, was associated with non-ambulation (47% vs 8%), later walking (mean 2y 7mo vs 1y 9mo), hypotonic (8% vs 1%) or dyskinetic (9% vs 5%) CP, a quadriplegic pattern of motor impairment (42% vs 5%), epilepsy (52% vs 12%), more emergency and multi-day hospital admissions, and reduced 35-year survival (96% vs 71%). Grey matter injuries (13% vs 6%), malformations (18% vs 6%), and miscellaneous neuroimaging patterns (12% vs 4%) were more common in people with intellectual disability.Interpretation: Intellectual disability adds substantially to the overall medical complexity in CP and may increase health and mortality disparities.What This Study Adds: Cerebral maldevelopments and grey matter injuries are associated with higher intellectual disability rates. Health care is more 'crisis-driven' and 'reactive' in children with co-occurring intellectual disability. Length of survival is reduced in individuals with CP and co-occurring intellectual disability. [ABSTRACT FROM AUTHOR]

Aim: The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) register to an administrative data set for health services research purposes. We sought to compare CP hospital admissions to general childhood population admissions, and identify factors associated with type and frequency of admissions in a CP cohort.Method: The CP register for Victoria, Australia was linked to the state's hospital admissions database. Data pertaining to the admissions of a CP cohort (n=1748) that took place between 2007 and 2014 were extracted. Population data were also obtained.Results: Overall, 80% of the CP cohort (n=1401) had at least admission between 2007 and 2014, accounting for 11 012 admissions or 1.5% of all admissions in their age group. Compared to general population admissions, CP admissions were more costly and more likely to be elective (66% vs 57%; p<0.001), medical (71% vs 57%; p<0.001), and to take place in metropolitan hospitals (92% vs 78%; p<0.001). Increased CP severity and complexity were associated with having more admissions and a higher proportion of admissions attributable to respiratory illness.Interpretation: By linking with administrative data sets, CP registers may be useful for health services research and inform health service delivery. [ABSTRACT FROM AUTHOR]

Aim: Limited information exists on the ability of children with cerebral palsy (CP) to ride a two-wheel bicycle, an activity that may improve health and participation. We aimed to describe bicycle-riding ability and variables associated with ability to ride in children with CP (Gross Motor Functional Classification System [GMFCS] levels I-II) compared with children with typical development.Method: This case-control study surveyed parents of 114 children with CP and 87 children with typical development aged 6 to 15 years (115 males, mean age 9y 11mo, standard deviation [SD] 2y 10mo). Kaplan-Meier methods were used to compare proportions able to ride at any given age between the two groups. Logistic regression was used to assess variables associated with ability to ride for children with CP and typical development separately.Results: The proportion of children with CP able to ride at each level of bicycle-riding ability was substantially lower at each age than peers with typical development (p<0.001). While most children with typical development were able to ride independently by 10 years of age, 51% of children with CP classified as GMFCS level I and 3% of those classified as GMFCS level II had obtained independent riding in the community by 15 years of age. Variables associated with ability to ride for children classified as GMFCS level I were age and parent-rated importance of their child being able to ride.Interpretation: Some independently ambulant children with CP can learn to ride a bicycle, in particular if they are classified as GMFCS level I. Variables associated with ability to ride deserve consideration in shaping future efforts for the majority of this population who are not yet able to ride. [ABSTRACT FROM AUTHOR]

Aim: Males typically outnumber females in cerebral palsy (CP) cohorts. To better understand this 'male disadvantage' and provide insight into causal pathways to CP, this study used 1983 to 2009 Australian CP and population birth cohorts to identify associations and trends with respect to biological sex and CP.Method: Within birth gestation groups, sex ratios were calculated to evaluate any male excess in the CP cohort compared with livebirths, neonatal deaths, neonatal mortality and survival rates, neonatal survivors, and CP rates in survivors. Sex- and gestation-specific trends in neonatal mortality, CP rates, and CP sex ratios were assessed by plotting their annual frequencies and fitting quadratic curves.Results: Over-representation of males in preterm live births partly explained the male excess in the CP cohort after preterm birth, especially at 28 to 31 weeks. Higher CP rates in male neonatal survivors also contributed to the male excess in CP, particularly at <28 and 37+ weeks. Higher neonatal mortality rates in males at all gestations had little impact on the CP sex ratio. There was no clearly discernible change over time in the CP sex ratio.Interpretation: Gestation-specific associations between sex and CP provide insight into causal pathways to CP and suggest sex-specific differences in response to neuroprotective strategies. [ABSTRACT FROM AUTHOR]

Aim: To describe the distribution of impairments among persons with cerebral palsy (CP) in a large Australian cohort.Method: Records of persons on the Australian Cerebral Palsy Register (ACPR) (n=3466) born from 1996 to 2005 were reviewed to extract year of birth, sex, CP subtype, Gross Motor Function Classification System (GMFCS) level, and impairments in vision, hearing, speech, intellect, and epilepsy. The distributions of GMFCS levels and CP subtype were plotted, and the proportions of each level of impairment were tabulated and presented as stacked graphs within the GMFCS and CP subtype distributions.Results: The proportions of persons with CP with each associated impairment increased with increasing GMFCS level. Compared with other spastic CP subtypes, individuals with spastic quadriplegia had higher frequencies of all associated impairments. Other than epilepsy, which was most prevalent in persons with spastic quadriplegia (53%), all impairments were most frequent in non-spastic CP subtypes. Hearing impairment was recorded for 21% of persons with dyskinesia whereas the hypotonic subtype had the highest prevalence of visual impairment (57%), intellectual impairment (90%), and speech impairment (95%).Interpretation: Distributions of associated impairments across all GMFCS levels and CP subtypes in a large cohort are presented in formats suitable for clinical use and discussion with families. [ABSTRACT FROM AUTHOR]

Aim: Our aim was to build on previous research indicating that rates of cerebral palsy (CP) in the Australian state of Victoria are declining, and examine whether severity of impairments is also decreasing.Method: Data on individuals with CP were extracted from the Victorian Cerebral Palsy Register for birth years 1983 to 2009. The yearly rates of dichotomized categories for gross motor function, motor laterality, intellectual impairment, and epilepsy per 1000 neonatal survivors and proportions in the CP cohort were tabulated and plotted by birth gestation. Linear regression modelling was used to fit prediction curves; likelihood ratio tests were used to test for differences in trends between impairment severity groups.Results: Since the mid-1990s, CP rates declined in neonatal survivors of birth at all gestations. Our data suggest that the decreasing CP rates were associated with relatively greater decreases in the rates of Gross Motor Function Classification System levels III to V, bilateral CP, epilepsy, and intellectual impairment (all p<0.005). Some variation was seen between birth gestation groups.Interpretation: Declines in rates of CP of all levels of severity and complexity from the mid-1990s provides 'real-world' support for the effectiveness of concurrent neuroprotective strategies and continual innovation in perinatal practices. [ABSTRACT FROM AUTHOR]

Aims: In a population cohort of children with grey matter injury (GMI) and cerebral palsy (CP), we aimed to describe and classify magnetic resonance imaging characteristics specific to GMI, and to identify key structure-function associations that serve as a basis for rating GMI in clinically relevant ways. Method: Symmetry, extent of cerebral injury, and pathological pattern for 54 children (37 males, 17 females) with CP and a predominant GMI pattern on chronic-phase magnetic resonance imaging were related to gross motor function, motor type and topography, epilepsy, intellectual disability, blindness, and deafness. Results: Relative to mild GMI where there was no pallidal abnormality, severe GMI, comprising pallidal abnormality alone or in conjunction with other deep nuclear and generalized cortical-subcortical involvement, was strongly associated with Gross Motor Function Classification System levels IV to V (OR 35.7 [95% CI 3.5, 368.8]). Involvement of the basal ganglia was associated with non-spastic/mixed motor types, but predominantly where cortical-subcortical grey and white matter involvement was not extensive. The prevalence of epilepsy was highest where there was diffuse cortical-subcortical involvement and white matter loss. Interpretation: Better understanding of structure-function relationships in CP and GMI, and how to rate the severity of GMI, will be helpful in the clinical context and also as a basis for investigation of causal pathways in CP. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to review the distribution of neuroimaging findings from a contemporary population cohort of individuals with cerebral palsy ( CP) and to facilitate standardization of imaging classification. Method Publications from 1995 to 2012 reporting imaging findings in population cohorts were selected through a literature search, and review of the titles, abstracts, and content of studies. Relevant data were extracted, including unpublished data from Victoria, Australia. The proportions for each imaging pattern were tabulated, and heterogeneity was assessed for all individuals with CP, and for subgroups based on gestational age, CP subtype, and Gross Motor Function Classification System level. Results Studies from three geographic regions met the inclusion criteria for individuals with CP, and two additional studies reported on specific CP subtypes. Brain abnormalities were observed in 86% of scans, but were observed least often in children with ataxia (24-57%). White matter injury was the most common imaging pattern (19-45%), although the proportions showed high heterogeneity. Additional patterns were grey matter injury (21%), focal vascular insults (10%), malformations (11%), and miscellaneous findings (4-22%). Interpretation This review suggests areas where further dialogue will facilitate progress towards standardization of neuroimaging classification. Standardization will enable future collaborations aimed at exploring the relationships among magnetic resonance imaging patterns, risk factors, and clinical outcomes, and, ultimately, lead to better understanding of causal pathways and opportunities for prevention. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the distribution of magnetic resonance imaging ( MRI) patterns in a large population sample of children with cerebral palsy ( CP) and to examine associations between MRI patterns, and antenatal and perinatal variables. Method Data were retrieved from the Victorian CP Register for 884 children (527 males, 357 females) born between 1999 and 2006. Postneonatal MRI was classified for 594 children. For 563 children (329 males, 234 females) for whom classification was to a single MRI pattern, the frequency of each variable was compared between patterns and with the population frequency. Results White matter injury was the most common MRI pattern (45%), followed by grey matter injury (14%), normal imaging (13%), malformations (10%), focal vascular insults (9%), and miscellaneous patterns (7%). Parity, birth gestation, level of neonatal care, Apgar score, and time to established respiration varied between MRI patterns ( p<0.01). Nulliparity was most strongly associated with focal vascular insults, whereas multiparity was associated only with malformations. Grey matter injury was not associated with birth in a tertiary unit, but was strongly associated with severe perinatal compromise. The frequency of neonatal seizures and of nursery admissions was lowest among children with malformations. Interpretation As known risk factors for CP are differentially associated with specific MRI patterns, future exploration of causal pathways might be facilitated when performed in pathogenically defined groups. [ABSTRACT FROM AUTHOR]

Aim To establish a prevalence estimate for drooling and explore factors associated with drooling in a population sample of children with cerebral palsy (CP) aged 7 to 14 years living in Victoria, Australia. Method A self-report questionnaire was used to collect data on drooling from parents of children born between 1996 and 2001, and registered with the Victorian Cerebral Palsy Register. Results A total of 385 children (231 males, 154 females; mean age 10y 9mo [SD 1y 7mo], range 8-14y) were studied. The clinical type and distribution of CP were spastic (341), ataxic (16), dyskinetic (17), hypotonic (10), and unknown (1). Distribution in Gross Motor Function Classification System (GMFCS) levels was I (103), II (98), III (52), IV (63), V (61), and unknown (8). After adjustment for topographical pattern of motor impairment and GMFCS level, 40% were reported to have experienced drooling between 4 years of age and the time of completing the questionnaire. A significantly higher prevalence of drooling was found in children with poor gross motor function and in those with more severe presentations of CP, including poor head control, difficulty with eating, and inability to sustain lip closure ( p<0.001 for each). Drooling was shown to be significantly associated with both intellectual disability and epilepsy in this group of children ( p<0.001 for both). Interpretation With a prevalence of 40%, drooling is an important comorbidity in CP. It was considered severe in 15% of children. Poor oromotor function was associated with drooling and could be the target of interventions for this under-researched problem. [ABSTRACT FROM AUTHOR]

Aim This study used data collected prospectively since 1986 from a population-based cerebral palsy registry to explore the rates, predictors, trends, and causes of mortality for individuals born in Victoria, Australia, between 1970 and 2004. Method Data were extracted for 3507 individuals (1972 males; 1535 females). The probability of survival before 31 May 2010 was determined using the Kaplan-Meier method; age-specific mortality rates were calculated per 1000 person-years and related to population rates. Using Cox proportional hazards regression, relative risks of mortality were estimated for different categories of chosen demographic and clinical variables. Causes were tabulated according to the direct cause of death. Results There were 418 deaths. Crude mortality was 20% at the age of 40 years. Relative to the population, mortality was highest in children aged under 15 years and decreased to twice the population rate at the age of 35 years. The strongest independent predictor of mortality was no independent ambulation (adjusted hazard ratio 6.2 [95% confidence interval 3.3-11.8]); additional predictors were severe intellectual impairment (3.0 [1.7-5.2]), epilepsy (1.4 [1.1-1.9]), deafness (2.6 [1.4-4.7]), and term birth (1.8 [1.3-2.4]). No improvement in survival was seen over time (unadjusted hazard ratio 1.00 [95% CI 0.99-1.01]). Respiratory causes were the most common direct causes of death. Interpretation Rates, predictors, and causes of death for individuals with cerebral palsy in Victoria were similar to those found in other population cohorts. Lack of improvement in survival since 1970 was an unexpected finding that warrants further investigation. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the distribution of motor severity levels and temporal trends in an Australian population cohort and to review the distribution of Gross Motor Function Classification System (GMFCS) levels across cerebral palsy (CP) registries worldwide. Methods Data were extracted from the Victorian Cerebral Palsy Register for 3312 individuals (1852 males, 1460 females; mean age 21y 2mo [SD 9y 6mo]) with non-postneonatally acquired CP, born between 1970 and 2003. The proportions of each motor severity level were calculated and logistic regression analyses were used to assess trends over time. A systematic review of the literature was undertaken and GMFCS data were extracted based on previously devised criteria. The proportions were plotted and the degree of heterogeneity was assessed for each level. Results Population data from Victoria suggested a proportional increase in mild motor impairment (GMFCS levels I/II) from 54% of all cases of CP in the 1970s to 61% in the 2000s. For nine CP registries worldwide, the mean proportions of each GMFCS level, from level I to V, were 34.2%, 25.6%, 11.5%, 13.7%, and 15.6% respectively. There was substantial heterogeneity between registries for all levels except level III. Interpretation Despite the usefulness and reported reliability of the GMFCS, substantial variability was found in the distribution of GMFCS levels between population registries, particularly between levels I and II, suggesting greater classification uncertainty between these levels. Further research would be useful to determine whether routine collection of extra clinical information may facilitate more reliable classification. [ABSTRACT FROM AUTHOR]

Aim The aims of this study were to estimate the frequency of hearing loss in children with cerebral palsy (CP), to examine factors associated with hearing loss, and to describe aspects of hearing in a population sample of children with CP and hearing loss. Method A systematic review of the international literature was undertaken, and data on the frequency of hearing loss or severe hearing loss were extracted from 14 data sets based on previously devised criteria. Six hundred and eight-five children with CP (406 males, 279 females) born in Victoria, Australia, between 1999 and 2004 were identified from the Victorian Cerebral Palsy Register. Children were included if they had an established post neonatal cause for their CP before the age of 2 years. Additional information was collected on 48 children with documented hearing loss based on a four-tone pure tone average in the better ear. Results There was considerable variation in the definitions and proportions of hearing loss (range 4-13%) and severe hearing loss (range 2-12%) reported by CP registries in developed countries. In Victoria, 7% of individuals with CP had bilateral hearing loss of a moderate to profound degree, whereas the subgroup with a severe-profound degree of loss constituted 3% to 4% of the CP population. Interpretation These population-based data are likely to more accurately reflect the true frequency of defined hearing loss in children with CP than previous reviews. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to assess overall and gestational age-specific trends in the rate of cerebral palsy (CP) in Victoria, Australia, and to compare these findings with other population data. Method Individuals born in Victoria from 1970 to 2004 with non-postneonatally acquired CP were identified from a population register; 3491 were included in the study (1963 males, 1528 females). After a literature review, comparison data were extracted from publications using previously devised inclusion criteria. Rates were calculated per 1000 live births for all CP and by gestational age group: these were tabulated and plotted by year of birth. Results Data from nine registries, including the Victorian register, showed an increase in the rates of CP over the 1970s and 1980s, consistently seen in extremely preterm (<28wks) survivors but also in those born at term (≥37wks). Since the early 1990s, CP rates either stabilized or decreased, particularly for children born extremely preterm. Interpretation Increases in the rates of CP during the 1970s and 1980s are in part because of the increasing survival of extremely preterm infants that occurred without a concomitant improvement in neurological outcomes. Evidence from population samples now suggests that this trend has been reversed since the mid- to late 1990s. [ABSTRACT FROM AUTHOR]

The aim of this study was to describe the distribution of types of motor disorder (motor type) in a population-based registry of children with cerebral palsy (CP), to examine any trends in motor type distribution over time, and to make comparisons with other populations. Individuals born in Victoria, Australia, between 1970 and 2003 with congenital CP were identified from the Victorian Cerebral Palsy Register, 3297 of whom were included in the study (1840 males, 1457 females). Those who had a hypotonic motor type, those whose brain abnormality was believed to have developed after 28 days of life, and those with missing data were excluded from the study. The proportions of each motor type were calculated and plotted by year of birth. Logistic regression analyses were used to assess the mean change in odds per year of birth. A systematic review of the international literature was undertaken and comparison data were extracted based on previously devised criteria. The degree of heterogeneity was assessed and comparisons were made according to geographical region and reporting period. In Victoria, the predominant motor types for individuals with CP born between 1970 and 2003 were spasticity (91%), ataxia (5%), and dyskinesia (4%). There was no clear trend for any motor type over time. The mean proportions from two other large data sets were similar but there was considerable heterogeneity between 29 individual CP registries, mainly because of differences in the classification of mixed motor types. A more objective method for determining the predominant motor type is required to make the classification of motor type in CP more consistent and reliable. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS). Method The study group comprised 63 participants (41 males, 22 females) from a population-based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome. Results Mean age of participants at the time of study was 10 years 6 months (SD 4y 7mo, range 4-20y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty-four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset. Interpretation Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy. [ABSTRACT FROM AUTHOR]

Aim To determine whether assisted reproductive technologies (ART) were more likely to be the method of conception in singletons with cerebral palsy (CP) than in those without CP. Method Singletons with CP born between 1991 and 2004 were selected from the Victorian Cerebral Palsy Register and matched for birth year to two singletons randomly selected from the Victorian Perinatal Data Collection Unit. Data from both sources were linked to records from three ART centres. Conditional logistic regression was used to assess the association between CP and aspects of conception using ART. Multivariate models were adjusted for parity, previous miscarriages, sex, gestational age, birthweight, and weight for gestational age. Results We identified 1241 singletons with CP (males n=721 [58%], females n=420 [42/100]; motor type: spastic [87%; unilateral 37%; bilateral 63%], ataxic n=60 [5/100], dyskinetic n=46 [4/100], hypotonic n=29 [2/100], unknown n=25 [2/100]. Gross Motor Function Classification System levels were I n=363 [29/100], II n=297 [24/100], III n=137 [11/100], IV n=160 [13/100], V n=192 [15/100], and unknown n=92 [7/100]). Sixteen (1.3/100) of the children with CP and 25 (1.0/100) of 2482 children without CP were conceived using ART. There was no significant increase in the odds of children with CP being conceived using ART (adjusted odds ratio 1.19, 95% confidence interval (CI) 0.63, 2.24) nor in the odds of them being conceived by a subfertile couple without ART (adjusted odds ratio 2.7, 95% CI 0.87, 8.36). Interpretation Singleton conception using ART is not strongly associated with an increased risk of CP. [ABSTRACT FROM AUTHOR]

Aim To describe the development and clinimetric properties of a new scale to evaluate changes in the impact of drooling in children with developmental disabilities. Method After examining the properties of potential items, 10 items were retained for inclusion in the final Drooling Impact Scale. The clinimetric properties of the scale were evaluated using data from two convenience samples of children attending a saliva-control clinic: a stable group ( n=31, 22 males, nine females; mean age 10y 7mo, SD 4y 5mo, range 3y 6mo–18y 3mo; cerebral palsy [CP] n=17, intellectual disability n=10; non-ambulatory n=13, nonverbal n=12) and an intervention group ( n=49, 29 males, 20 females; mean age 11y, SD 3y 6mo, range 3y 4mo–16y 10mo; CP n=31, intellectual disability n=15; non-ambulatory n=27, nonverbal n=28). To assess validity, changes in scores on the Drooling Impact Scale over time were compared with a carer’s global rating of change using Pearson’s correlations and t-tests. A concordance correlation coefficient was used to compute the level of agreement between assessments 1 month apart in stable children. Effect size, standardized response mean, Guyatt responsiveness statistic, and an unpaired t-test were used to estimate responsiveness. Results The correlation between the global rating and change in Drooling Impact Scale scores was 0.69 ( p<0.001). The concordance correlation coefficient was 0.85. An effect size of 1.8, standardized response mean of 1.5, Guyatt responsiveness statistic of 1.4, and mean group difference of 23.5 (95% confidence interval 17.4–29.6) were obtained. Interpretation The Drooling Impact Scale is a valid and reliable subjective measure that is responsive to change. [ABSTRACT FROM AUTHOR]

This commentary is on the original articles by Brooks et al. on pages 1059–1064 and 1065–1071 of this issue. [ABSTRACT FROM AUTHOR]

This commentary is on the original article by Himmelmann and Sundh on pages 762–767 of this issue. [ABSTRACT FROM AUTHOR]

Aim: The aims of this study were to estimate the proportion of emergency department presentations attributable to children with cerebral palsy (CP), investigate the frequency of emergency department presentations in a CP cohort, and compare emergency department presentations among children with CP with those of other children.Method: This was a retrospective cohort study. The Victorian Cerebral Palsy Register was linked to the Victorian Emergency Minimum Dataset. Data on emergency department presentations for the CP cohort occurring between 2007 and 2014 and population control data were obtained.Results: The CP cohort (n=1748) had 7015 emergency department presentations during the 7-year period, accounting for 0.4% of the 1.69 million age-specific presentations during that time. The number of annual presentations per 1000 children rose with increasing CP severity. Compared with presentations among the general population, higher proportions of presentations among the CP cohort were preceded by ambulance arrivals (27% vs 8%), triaged as urgent (66% vs 32%), and required hospital admission (38% vs 12%).Interpretation: The marked differences in presentations between the CP cohort and the general population in the proportions that were urgent and required ambulance arrivals and hospital admissions was an important finding. Strategies to ensure appropriate use of services, including encouragement to seek earlier assistance from primary care providers, may prevent problems escalating to the need for urgent care.What This Paper Adds: Children with cerebral palsy (CP) account for 0.4% of childhood emergency department presentations. More emergency department presentations among children with CP require ambulance arrival. More CP emergency department presentations are urgent and require hospital admission. Traditional emergency department triage scales seem less accurate for this group. [ABSTRACT FROM AUTHOR]

Aim: To provide an updated description of the rates, trends, and predictors of mortality of individuals with cerebral palsy (CP), born in the Australian state of Victoria between 1970 and 2012. Method: Data were extracted for 4807 individuals (2091 females; 2716 males). The probability of survival to 30th June 2017 was calculated using the Kaplan–Meier method. Mortality rates were calculated per 1000 person‐years using age strata and compared with population mortality rates to produce mortality ratios. Cox proportional hazards regression was used to calculate hazard ratios for selected demographic and clinical characteristics and to estimate the effect of birth epoch on 15‐year survival. Results: There were 666 recorded deaths. Compared to the general population, mortality was higher for all persons with CP and highest for children aged 1 to 15 years (45–62 times). We observed 35% improvement in the probability of survival to 15 years for births in the 2000s relative to the 1970s (hazard ratio 0.65, 95% confidence interval [CI] 0.49, 0.86), but only 4% improvement for the subgroup with complex CP (hazard ratio 0.96, 95% CI 0.69, 1.33). Interpretation: The observed improvements in survival for those born in the 2000s is likely related predominantly to a proportional reduction in complex CP within the cohort. What this paper adds: Length of survival improved for Australians with cerebral palsy (CP) born this millennium.Improved survival was mainly because of a proportional reduction in complex CP.A small improvement in length of survival was seen for children with complex CP. [ABSTRACT FROM AUTHOR]

A response by Susan M. Reid and colleagues to a letter to the editor about their article "Survival of individuals with cerebral palsy born in Victoria, Australia, between 1970 and 2004" in the 2012 issue is presented.

Aim: To describe post‐neonatally acquired (PNN) cerebral palsy (CP) in terms of temporal trends in prevalence, clinical and sociodemographic profiles, known causes and associations between causes, and sociodemographic variables. Method: Numerator data, a count of children with PNN‐CP confirmed at 5 years of age (n = 523), was drawn from two Australian state CP registers (birth years 1973–2012). Poisson regression was used to investigate temporal trends in the prevalence of PNN‐CP by 5‐year intervals, calculated per 10 000 live births. Using data from all state and territory Australian CP registers (n = 469), distributions of clinical characteristics, PNN‐CP causes, and sociodemographic factors were tabulated (birth years 1995–2012). χ2 and logistic regression analyses were used to assess associations between sociodemographic profile, Australian reference data, and known causes. Results: A significant temporal decline in PNN‐CP in Victoria (p = 0.047) and Western Australia (p = 0.033) was observed. The most common proximal causes of PNN‐CP were cerebrovascular accidents (34%, n = 158), infection (25%, n = 117), and non‐accidental injuries (12%, n = 58). Children born to teenage mothers, Aboriginal and/or Torres Strait Islander mothers, or children born in remote areas were over‐represented in this cohort compared with reference data (all p ≤ 0.001). Infectious causes were strongly associated with teenage motherhood (odds ratio 3.0 [95% confidence interval 1.1–8.2], p = 0.028) and remote living (odds ratio 4.5 [95% confidence interval 2.0–10.2], p < 0.001). Interpretation: Although prevalence of PNN‐CP has declined, the over‐representation of priority populations, and the relative severity of a condition that is largely preventable, suggest the need for more specific primary preventive measures and support. What this paper adds: Prevalence of post‐neonatally acquired (PNN) cerebral palsy (CP) in Australia significantly declined between 1973 and 2012.Cerebrovascular accidents are the most common proximal cause of PNN‐CP.Children born in remote areas are at greater risk of PNN‐CP. [ABSTRACT FROM AUTHOR]

For the 9% to 16% of children with cerebral palsy (CP) who have normal brain imaging, further testing for metabolic and/or genetic conditions has been recommended. This study aimed to identify a cohort of children with CP with normal magnetic resonance imaging (MRI), clinically review and describe the cases, and assess the value of testing for inherited metabolic disorders in these children. Children with congenital CP born from 1999 to 2005 were selected from a population register. Normal MRI reports were identified and the scans reassessed. Children whose scans were performed before 18 months were excluded, as were children with spastic CP (Gross Motor Function Classification System [GMFCS] level I). The remainder were reviewed clinically and offered investigations. Of 730 children identified, 515 had available imaging and 54 were confirmed as normal. Cases with non-spastic CP and those with milder clinical severity were more likely to have normal imaging. Twenty-three children (17 males, six females; mean age 6y 11mo, SD 1y 10mo, range 3y 0mo to 10y 0mo) were reviewed clinically and offered investigations. Twelve children had spasticity (11 with diplegia, one quadriplegia), three had dyskinesia, five ataxia, and three hypotonia. Two children functioned in GMFCS level I, 11 in level II, seven in level III and three in level IV. Four children with spasticity had unusual features. No alternative diagnoses were made. Although important to consider in individual cases, comprehensive metabolic testing failed to clarify the aetiology of CP further in this large cohort of children with normal MRIs, even those with atypical features. [ABSTRACT FROM AUTHOR]

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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A response by Leonard et al to a letter to the editor about their article " Should children with cerebral palsy and normal imaging undergo testing for inherited disorders?" which appeared in the journal "Developmental Medicine & Child Neurology" is presented.

Aim  To test the effectiveness of repeat botulinum toxin A (BoNT-A) injections in the affected arm of 22 children with hemiplegic cerebral palsy (19 males, three females), aged 1 year 10 months to 4 years 10 months (mean 3y 8mo, SD 9mo) in a randomized controlled trial. Method  Children received either three series of BoNT-A injections plus twice-weekly occupational therapy (OT) or OT alone in 16-week cycles. Muscles targeted at each injection cycle in the 11 children receiving BoNT-A+OT were the adductor pollicis ( n=9), flexor pollicis longus ( n=5), flexor digitorum superficialis ( n=8), flexor digitorum profundus ( n=8), flexor carpi radialis ( n=2), flexor carpi ulnaris ( n=6), pronator teres ( n=10), and biceps brachii ( n=11). Parental perception of treatment efficacy was assessed using the Canadian Occupational Performance Measure (COPM) and the Goal Attainment Scale (GAS), quality of movement using the Quality of Upper Extremity Skills Test (QUEST), fine motor skills using the Peabody Developmental Motor Scale – Fine Motor (PDMS-FM), and spasticity using the Modified Tardieu Scale (MTS). Between-group differences at 12 months were analysed using independent-sample t-tests. Results  All children were at Gross Motor Function Classification System levels I (BoNT-A+OT n=6; OT n=8) or II ( n=5 and n=3 respectively) and were too young to be classified using the Manual Ability Classification System. The BoNT-A+OT group had higher COPM performance scores (mean difference −0.8, 95% confidence interval [CI] −1.5–0.0) and higher GAS T scores (mean difference −6.9, 95% CI −13.8 to −0.1]). No significant difference was found for the COPM satisfaction, PDMS-FM, or QUEST scores. The BoNT-A+OT group showed progressive reduction in spasticity compared with the OT group. At study completion MTS mean difference was 50.0° (95% CI 22.4–77.6) for pronators and 20.9° (95% CI 2.4–39.4) for wrist flexors. Interpretation  Repeat BoNT-A injections in the upper limb combined with OT resulted in progressively reduced spasticity and improved parental perception of performance. [ABSTRACT FROM AUTHOR]

The purpose of this study was to investigate the frequency and spectrum of magnetic resonance imaging (MRI) abnormalities in a population of children with cerebral palsy (CP) who were born in the years 2000 and 2001 in Victoria, Australia. In 2000 and 2001, 221 children (126 males, 95 females; mean age 6y [SD 7mo], range 5–7y) with CP, excluding those with CP due to postneonatal causes (6% of all cases), were identified through the Victorian Cerebral Palsy Register. All medical records were systematically reviewed and all available brain imaging was comprehensively evaluated by a single senior MRI radiologist. MRI was available for 154 (70%) individuals and abnormalities were identified in 129 (84%). The study group comprised 88% with a spastic motor type CP; the distribution was hemiplegia in 33.5%, diplegia in 28.5%, and quadriplegia in 37.6% of children. Overall, pathological findings were most likely to be identified in children with spastic hemiplegia (92%) and spastic quadriplegia (84%). Abnormalities were less likely to be identified in non-spastic motor types (72%) and spastic diplegia (52%). The most common abnormalities identified on MRI were periventricular white matter injury (31%), focal ischaemic/haemorrhagic lesions (16%), diffuse encephalopathy (14%), and brain malformations (12%). Dual findings were seen in 3% of patients. This is the first study to document comprehensively the neuroimaging findings of all children identified with CP born over a consecutive 24-month period in a large geographical area. [ABSTRACT FROM AUTHOR]

The aim of this study was to determine the effectiveness of an intraoral appliance, the Innsbruck Sensorimotor Activator and Regulator (ISMAR), in improving drooling and eating skills in a group of children with cerebral palsy, and to determine which factors might indicate good candidates for this type of treatment. Eighteen children (13 males, five females; mean age 7 years 10 months, range 4 to 13 years) were selected. Measures of drooling and feeding skills were taken at baseline, at the completion of a 6-month control phase, and at two more 6-monthly time points after the ISMAR was fitted. Children varied greatly in both the length of time taken to tolerate wearing the ISMAR and duration for which the appliance was worn. Only six children (four females, two males) completed the full study. Their motor disabilities were athetosis (n=3), spastic quadriplegia (n=2), and hypotonia (n=1); four of the six children used a wheelchair for locomotion. None had epilepsy and none had greater than mild cognitive impairment. For these children drooling severity scores and eating and drinking skills improved significantly over the treatment period in comparison with the control phase. We conclude that the ISMAR remains a valid option in improving drooling in children and merits further study. [ABSTRACT FROM AUTHOR]