Severe Congenital Heart Defects and Cerebral Palsy.

OBJECTIVE: To report the prevalence of cerebral palsy (CP) in children with severe congenital heart defects (sCHD) and the outcome/severity of the CP. METHODS: Population-based, data linkage study between CP and congenital anomaly registers in Europe and Australia. The EUROCAT definition of severe CHD (sCHD) was used. Linked data from 4 regions in Europe and 2 in Australia were included. All children born in the regions from 1991 through 2009 diagnosed with CP and/or sCHD were included. Linkage was completed locally. Deidentified linked data were pooled for analyses. RESULTS: The study sample included 4989 children with CP and 3684 children with sCHD. The total number of livebirths in the population was 1 734 612. The prevalence of CP was 2.9 per 1000 births (95% CI, 2.8-3.0) and the prevalence of sCHD was 2.1 per 1000 births (95% CI, 2.1-2.2). Of children with sCHD, 1.5% (n = 57) had a diagnosis of CP, of which 35 (61%) children had prenatally or perinatally acquired CP (resulting from a brain injury at ≤28 days of life) and 22 (39%) children had a postneonatal cause (a brain injury between 28 days and 2 years). Children with CP and sCHD more often had unilateral spastic CP and more intellectual impairments than children with CP without congenital anomalies. CONCLUSIONS: In high-income countries, the proportion of children with CP is much higher in children with sCHD than in the background population. The severity of disease in children with CP and sCHD is milder compared with children with CP without congenital anomalies.

Temporal trends, clinical characteristics, and sociodemographic profile of post-neonatally acquired cerebral palsy in Australia, 1973-2012: A population-based observational study.

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.

Prevalence and trends for Aboriginal and Torres Strait Islander children living with cerebral palsy: A birds-eye view.

AIM: To provide a birds-eye view of the trends of cerebral palsy (CP) for Australian Aboriginal and Torres Strait Islander children and young adults. METHOD: Data were obtained for this population-based observational study from the Australian Cerebral Palsy Register (ACPR), birth years 1995 to 2014. The Indigenous status of children was classified by maternal Aboriginal and Torres Strait Islander or non-Indigenous status. Descriptive statistics were calculated for socio-demographic and clinical characteristics. Prenatal/perinatal and post-neonatal birth prevalence was calculated per 1000 live births and per 10 000 live births respectively, and Poisson regression used to assess trends. RESULTS: Data from the ACPR were available for 514 Aboriginal and Torres Strait Islander individuals with CP. Most children could walk independently (56%) and lived in urban or regional areas (72%). One in five children lived in socioeconomically disadvantaged remote/very remote areas. The birth prevalence of prenatal/perinatal CP declined after the mid-2000s from a high of 4.8 (95% confidence interval 3.2-7.0) to 1.9 per 1000 live births (95% confidence interval 1.1-3.2) (2013-2014), with marked declines observed for term births and teenage mothers. INTERPRETATION: The birth prevalence of CP in Aboriginal and Torres Strait Islander children in Australia declined between the mid-2000s and 2013 to 2014. This birds-eye view provides key stakeholders with new knowledge to advocate for sustainable funding for accessible, culturally safe, antenatal and CP services. WHAT THIS PAPER ADDS: Birth prevalence of cerebral palsy (CP) is beginning to decline for Aboriginal and Torres Strait Islanders. Recent CP birth prevalence for Aboriginal and Torres Strait Islanders is 1.9 per 1000 live births. Most children with CP live in more populated areas rather than remote or very remote areas. One in five Aboriginal and Torres Strait Islander children with CP live in socioeconomically disadvantaged remote areas.

Cerebral palsy registers around the world: A survey.

AIM: To provide a description of cerebral palsy (CP) registers globally, identify which aim to report on CP epidemiology, and report similarities and differences across topics of importance for the sustainability and collaboration between registers. METHOD: Representatives of all known CP registers globally (n = 57) were invited to participate. The online survey included 68 questions across aims, methodologies, output/impact, and stakeholder involvement. Responses were analysed using descriptive statistics. RESULTS: Forty-five registers participated, including three register networks. Twenty were newly established or under development, including 12 in low- and middle-income countries (LMICs). An epidemiological aim was reported by 91% of registers. Funding is received by 85% of registers, most often from not-for-profit organizations. CP definitions are comparable across registers. While the minimum data set of a register network is used by most registers, only 25% of identified items are collected by all three register networks. Ninety per cent of registers measure research activities/output, and 64% measure research impact. People with lived experience are involved in 62% of registers. INTERPRETATION: There has been a recent surge in CP registers globally, particularly in LMICs, which will improve understanding of CP epidemiology. Ongoing efforts to address identified methodological differences are essential to validate comparison of results and support register collaboration.

Declining trends in birth prevalence and severity of singletons with cerebral palsy of prenatal or perinatal origin in Australia: A population-based observational study.

AIM: To investigate temporal trends in birth prevalence, disability severity, and motor type for singletons with prenatal or perinatally acquired cerebral palsy (CP). METHOD: Numerator data, number of children with CP born a singleton between 1995 and 2014, confirmed at 5 years of age, were drawn from three state registers with population-level ascertainment. Birth prevalence estimates and 95% confidence intervals (CI) were calculated per 1000 singleton live births for the three states combined, overall, by gestational age group, by dichotomized disability severity, and spastic laterality. Poisson regression models were used to analyse trends. Using data from all eight registers, trends in the proportional distribution of CP subtypes overall and stratified by gestational age were examined. RESULTS: Birth prevalence of CP declined from 1.8 (95% CI 1.6-2.0) in 1995 to 1996 to 1.2 (95% CI 1.1-1.4) in 2013 to 2014 (average 5% per 2-year epoch, p < 0.001). Declines in birth prevalence were observed across all gestational age groups with the largest decline in children born at <28 weeks (average 8% per epoch, p < 0.001). Prevalence of moderate-severe disability declined for children born at <28 and ≥37 weeks (average 11% and 7% per epoch respectively, p < 0.001). The proportions of bilateral spastic CP declined (p < 0.001) at <28 weeks (p = 0.014) and ≥37 weeks (p < 0.001). The proportion of children with dyskinesia increased (28-31 weeks: p = 0.021, 32-36 weeks: p = 0.001, and ≥37 weeks: p < 0.001). INTERPRETATION: Birth prevalence of CP and moderate-severe disability (<28 and ≥37 weeks) declined in Australian singletons between 1995 and 2014, reflecting changes in prenatal and perinatal care over time. WHAT THIS PAPER ADDS: Declines in birth prevalence of prenatal or perinatally acquired cerebral palsy were observed for singletons born in Australia between 1995 and 2014. These declines were evident across all gestational age groups. Declines in birth prevalence of moderate-severe disability were observed for children born at <28 weeks and ≥37 weeks.

Common data elements to standardize genomics studies in cerebral palsy.

AIM: To define clinical common data elements (CDEs) and a mandatory minimum data set (MDS) for genomic studies of cerebral palsy (CP). METHOD: Candidate data elements were collated following a review of the literature and existing CDEs. An online, three-round Delphi survey was used to rate each data element as either 'core', 'recommended', 'exploratory', or 'not required'. Members of the International Cerebral Palsy Genomics Consortium (ICPGC) rated the core CDEs as either mandatory or not, to form the MDS. For both the CDEs and the MDS, a data element was considered to have reached consensus if more than 75% of respondents agreed. RESULTS: Forty-six individuals from around the world formed the Delphi panel: consumers (n=2), scientists/researchers (n=17), medical (n=19), and allied health professionals (n=8). The CDEs include 107 data elements across six categories: demographics, diagnostics, family history, antenatal and neonatal details, clinical traits, and CP-specific assessments. Of these, 10 are mandatory, 42 core, 41 recommended, and 14 are exploratory. INTERPRETATION: The ICPGC CDEs provide a foundation for the standardization of phenotype data captured in CP genomic studies and will benefit international collaborations and pooling of data, particularly in rare conditions. WHAT THIS PAPER ADDS: A set of 107 common data elements (CDEs) for genomics studies in cerebral palsy is provided. The CDEs include standard definitions and data values domains. The CDEs will facilitate international data sharing, collaboration, and improved clinical interpretation of findings.

OBJETIVO: Definir elementos de dados clínicos comuns (DCC) e um conjunto mínimo de dados obrigatórios (CMDO) para estudos genômicos de paralisia cerebral (PC). MÉTODO: Os elementos de dados do candidato foram coletados seguindo uma revisão da literatura e através dos DCC existentes. Uma pesquisa on-line de três rodadas Delphi foi usada para classificar cada elemento de dados como 'essencial', 'recomendado', 'exploratório' ou 'não obrigatório'. Os Membros do Consorcio Internacional de Genoma na Paralisia Cerebral (MCIGPC) classificaram os DCC do núcleo como obrigatórios ou não, para formar o CMDO. Tanto para os DCC quanto para o CMDO, um elemento de dados foi considerado como tendo chegado a um consenso se mais de 75% dos respondentes concordassem. RESULTADOS: Quarenta e seis indivíduos de todo o mundo formaram o painel Delphi: consumidores (n=2), cientistas/pesquisadores (n=17), médicos (n=19) e profissionais de saúde aliados (n=8). Os DCC incluem 107 elementos de dados em seis categorias: demografia, diagnóstico, história familiar, detalhes pré-natais e neonatais, características clínicas e avaliações específicas de PC. Destes, 10 são obrigatórios, 42 essenciais, 41 recomendados e 14 são exploratórios INTERPRETAÇÃO: Os DCC do MCIGPC fornecem uma base para a padronização de dados de fenótipo capturados em estudos genômicos de PC e beneficiarão colaborações internacionais e agrupamento de dados, particularmente em condições raras.

Global prevalence of cerebral palsy: A systematic analysis.

AIM: To determine trends and current estimates in regional and global prevalence of cerebral palsy (CP). METHOD: A systematic analysis of data from participating CP registers/surveillance systems and population-based prevalence studies (from birth year 1995) was performed. Quality and risk of bias were assessed for both data sources. Analyses were conducted for pre-/perinatal, postnatal, neonatal, and overall CP. For each region, trends were statistically classified as increasing, decreasing, heterogeneous, or no change, and most recent prevalence estimates with 95% confidence intervals (CI) were calculated. Meta-analyses were conducted to determine current birth prevalence estimates (from birth year 2010). RESULTS: Forty-one regions from 27 countries across five continents were represented. Pre-/perinatal birth prevalence declined significantly across Europe and Australia (11 out of 14 regions), with no change in postneonatal CP. From the limited but increasing data available from regions in low- and middle-income countries (LMICs), birth prevalence for pre-/perinatal CP was as high as 3.4 per 1000 (95% CI 3.0-3.9) live births. Following meta-analyses, birth prevalence for pre-/perinatal CP in regions from high-income countries (HICs) was 1.5 per 1000 (95% CI 1.4-1.6) live births, and 1.6 per 1000 (95% CI 1.5-1.7) live births when postneonatal CP was included. INTERPRETATION: The birth prevalence estimate of CP in HICs declined to 1.6 per 1000 live births. Data available from LMICs indicated markedly higher birth prevalence. WHAT THIS PAPER ADDS: • Birth prevalence of pre-/perinatal cerebral palsy (CP) in high-income countries (HICs) is decreasing. • Current overall CP birth prevalence for HICs is 1.6 per 1000 live births. • Trends in low- and middle-income countries (LMICs) cannot currently be measured. • Current birth prevalence in LMICs is markedly higher than in HICs. • Active surveillance of CP helps to assess the impact of medical advancements and social/economic development. • Population-based data on prevalence and trends of CP are critical to inform policy.

Program for the Education and Enrichment of Relational Skills for adolescents with an acquired brain injury: A randomized controlled trial.

AIM: To test the efficacy of a group social skills intervention on social functioning in adolescents with a brain injury. METHOD: Thirty-six adolescents (mean age 14y, SD 1y 8mo, age range 12y 1mo-16y 3mo; 17 females) with acquired brain injury (ABI; ≥12mo postintervention; n=19) or cerebral palsy (n=17) were randomly allocated to the Program for the Education in Enrichment of Relational Skills (PEERS) or usual care. The primary outcome was the Social Skills Improvement System-Rating Scales (SSIS-RS). Secondary outcomes were scores derived from the Test of Adolescent Social Skills Knowledge-Revised (TASSK-R), Social Responsiveness Scale, Second Edition, and Quality of Socialization Questionnaire. Between-group differences postintervention and at the 26-week retention time point were compared using linear mixed modelling for continuous outcomes and Poisson regression for count data. RESULTS: There were no between-group differences on the primary outcome (SSIS-RS). Regarding the secondary outcomes, the PEERS-exposed group achieved significantly greater improvements on the TASSK-R (mean difference [MD]=6.8, 95% confidence interval [CI]=4.8-8.8, p<0.001), which were maintained at the 26-week retention time point (MD=8.1, 95% CI=6.0-10.2, p<0.001). PEERS was also associated with a significant increase in parent-reported invited get-togethers at 26 weeks (incidence rate ratio=4.0, 95% CI=1.0-16.0, p=0.05). INTERPRETATION: Adolescents with brain injury who completed the PEERS learned and retained social knowledge and increased social participation.

eLearning significantly improves maternity professionals' knowledge of the congenital cytomegalovirus prevention guidelines.

AIMS: Cytomegalovirus (CMV) is a preventable cause of neurodevelopmental disability. Australian guidelines recommend that pregnant women are informed about CMV to reduce their risk of infection; however, less than 10% of maternity health professionals routinely provide prevention advice. The aim was to develop and evaluate the effectiveness of an eLearning course for midwives to improve knowledge and confidence about CMV. MATERIALS AND METHODS: Participants undertaking the course between March and November 2020 were invited to complete an evaluation questionnaire: before the course (T1), immediately after (T2) and three months post completion (T3). A linear mixed model was used to evaluate change in participant scores; P < 0.05 was considered statistically significant. RESULTS: Midwives (316/363, 87%), midwifery students (29/363, 8%) and nurses (18/363, 5%) participated. At T1 80% indicated they had not received education about CMV. Total adjusted mean scores for questionnaires completed between T1 (n = 363) and T2 (n = 238) increased significantly (from 17.2 to 22.8, P < 0.001). Limited available T3 scores (n = 27) (-1.7, P < 0.001), while lower than T2, remained higher than at T1 (+3.6, P < 0.001). Participants' awareness of CMV information resources improved from 10 to 97% from T1 to T2. Confidence in providing CMV advice increased from 6 to 95% between T1 and T2 (P < 0.001) and was maintained at T3. Almost all (99%) participants indicated they would recommend the course to colleagues. CONCLUSION: Participants who completed the eLearning course had significantly improved knowledge and confidence in providing advice about CMV. Programs targeting other maternity health professionals should be considered, to further support the implementation of the congenital CMV prevention guidelines.

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.

Cerebral palsy in twins and higher multiple births: a Europe-Australia population-based study.

AIM: To describe the birth prevalence, temporal trends, and clinical outcomes of twins, triplets, or quadruplets with cerebral palsy (CP). METHOD: This was a cross-sectional study using data for twins, triplets, and quadruplets with prenatally or perinatally acquired CP and pooled from the Surveillance of Cerebral Palsy in Europe network (born 1992-2009) and Australian Cerebral Palsy Register (born 1993-2009). Children were at least 4 years old at time of registration. Children born in regions with population ascertainment and available denominator data were included in prevalence calculations (n=1033 twins, 81 triplets, and 11 quadruplets). Clinical data from children registered in all participating registers were described, including 2163 twins (56% male), 187 triplets (59% male), and 20 quadruplets (45% male). RESULTS: The birth prevalence of CP was higher with increasing plurality (twins 6.5 per 1000 live births [95% confidence interval {CI} 6.1-6.9], triplets 17.1 [95% CI 13.6-21.2], quadruplets 50.7 [95% CI 25.6-88.9]); however, prevalence by gestational age was similar across all pluralities. Between 1992-1994 and 2007-2009, prevalence of CP among twins declined (p=0.001) but prevalence of CP among triplets did not change significantly over time (p=0.55). The distributions of Gross Motor Function Classification System, epilepsy, and impairments of intellect, vision, and hearing were similar regardless of plurality. INTERPRETATION: The data combined from two CP register networks indicated that triplets and quadruplets had increased risk of CP compared to twins. The higher prevalence of CP in triplets and quadruplets is due to their higher risk of preterm birth. Prevalence of CP among twins significantly declined in Europe and Australia. Clinical outcomes were similar for all multiple births.

Epidemiology of cerebral palsy in low- and middle-income countries: preliminary findings from an international multi-centre cerebral palsy register.

AIM: To describe the epidemiology of cerebral palsy (CP) in children from low- and middle-income countries (LMICs) using data from the Global Low- and Middle-Income Country CP register (GLM-CPR). METHOD: The GLM-CPR is a multi-country initiative that combines and compares data from children with CP (<18y) in LMICs. Children with CP are registered after detailed neurodevelopmental assessment by a multidisciplinary medical team using a harmonized protocol. Data are collected on agreed core variables. Descriptive analyses are completed to report findings from participating countries. RESULTS: Between January 2015 and May 2019, 2664 children were recruited from Bangladesh, Nepal, Indonesia, and Ghana (mean age [SD] at assessment: 7y 8mo [4y 8mo], 95% confidence interval 7y 6mo-7y 11mo; male [n=1615] 60.6%, female [n=1049] 39.4%). Overall, 86.6% children acquired CP prenatally and perinatally (e.g. preterm birth, birth asphyxia, neonatal encephalopathy). Median age at CP diagnosis was 3 years. Moreover, 79.2% children had spastic CP and 73.3% were classified in Gross Motor Function Classification System levels III to V. Notably, 47.3% of children never received rehabilitation services (median age at receiving rehabilitation services was 3y; 12.7% received assistive devices) and 75.6% of school-age children had no access to education. INTERPRETATION: Population-based data show that the proportion of severe cases of CP is very high in LMICs. Children with CP in LMICs lack access to rehabilitation and educational services and a large proportion of children have potentially preventable risk factors, for example, birth asphyxia and neonatal infections. Delayed diagnosis, severe motor impairments, and lack of rehabilitation in most children call for urgent action to identify preventive opportunities and promote early diagnosis and intervention for children with CP in LMICs. What this paper adds The proportion of severe cases of cerebral palsy (CP) is very high in rural low- and middle-income countries (LMICs). Children with CP in LMICs lack access to rehabilitation and educational services. A large proportion of children with CP in LMICs have potentially preventable risk factors.

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).

Early Life Parechovirus Infection Neurodevelopmental Outcomes at 3 Years: A Cohort Study.

OBJECTIVE: To investigate the long-term developmental and behavioral outcomes in an established cohort of children hospitalized as infants with human parechovirus (HPeV) infection and sepsis-like illness. STUDY DESIGN: The HPeV cohort was composed of children 3 years of age after HPeV infection and hospitalization in early infancy that occurred during a well-documented HPeV genotype 3 outbreak in Australia. We assessed neurodevelopmental and behavioral outcomes using the Bayley Scales of Infant and Toddler Development-III and the Child Behavior Checklist. We compared their outcomes with a subsample of healthy control infants drawn from the independently sampled Triple B Pregnancy Cohort Study. RESULTS: Fifty children, with a mean age of 41 months, were followed for 3 years after hospital admission with HPeV infection. There were 47 children whose original illness was fever without source or sepsis-like illness and 3 who had encephalitis. All children in the HPeV cohort showed age-specific development within the population normal range on the Bayley Scales of Infant and Toddler Development-III. There was no difference in developmental attainment compared with 107 healthy control infants after adjusting for measured confounders. The HPeV cohort showed higher average scores on the Child Behavior Checklist and a higher frequency of clinical range scores compared with healthy controls. CONCLUSIONS: Although HPeV sepsis-like illness did not result in neurodevelopmental delay at 3 years of age, it was associated with increased behavioral problems compared with healthy controls. The behavioral problems reached a clinical threshold in a minority of children. Results inform clinical management and planning for children after severe HPeV infection in infancy.

European and Australian Cerebral Palsy Surveillance Networks Working Together for Collaborative Research.

AIMS: This study aims to describe and compare goals and methods, characteristics of children with cerebral palsy (CP), and to compare prevalence of CP in the Surveillance of Cerebral Palsy in Europe (SCPE) and the Australian Cerebral Palsy Register (ACPR). METHODS: This study compares the objectives of the two networks and their working practices; key documents from both above-mentioned networks were used. Children included in the comparison of the descriptive profile and prevalence measures were born between 1993 and 2009 for Australian data and between 1980 and 2003 for SCPE. RESULTS: SCPE contributed 10,756 cases and ACPR 6,803. There were similar distributions of motor type, severity, and gestational age groups, except for the proportion of the lowest gestational age category (range, 20-27 weeks) which was twice higher in the ACPR (13 vs. 7%). Associated impairment proportions were also similar except for severe vision impairment which was more than twice as high in SCPE as in the ACPR (11 vs. 4%), but most likely due to a subtle difference in definitions. Prevalence rates were comparable at the same time point in the different groups of birth weight, and declined over time, except for the moderately low birth weight in ACPR. CONCLUSION: Two CP networks representing two continents have compared their major characteristics to facilitate the comparison across their study populations. These characteristics proved to be similar with only marginal differences. This gives additional strength to the observation in both networks that CP prevalence is decreasing which is of great importance for families and health care systems.

Congenital anomalies in children with cerebral palsy in rural Bangladesh.

AIM: To determine the proportion of children with cerebral palsy (CP) who had major congenital anomalies, describe the types of disorders, and report on the children's functional outcomes. METHOD: Data were extracted from the Bangladesh Cerebral Palsy Register (BCPR). Descriptive analyses were conducted on children with CP and major congenital anomalies. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated to measure the association between major congenital anomalies, clinical severity, and presence of comorbidities. RESULTS: Between January 2015 and December 2016, 726 children with CP were newly registered with the BCPR (277 females, 449 males; mean age [SD] at registration 90mo [54mo], 4mo-18y). Seventy-eight children (11%) had a major congenital anomaly. Neurological (86%) and musculoskeletal congenital anomalies (10%) were the most common. Microcephaly was the most common congenital anomaly (83%). The odds of severe functional motor limitations (OR=2.4, 95% CI=1.9-2.9), epilepsy (OR=1.6, 95% CI=1.1-2.1), visual impairment (OR=2.6, 95% CI=2.0-3.2), presence of strabismus (OR=3.9, 95% CI=3.8-4.4), hearing (OR=1.2, 95% CI=0.6-1.9), speech (OR=5.4, 95% CI=4.6-6.2), and intellectual impairments (OR=2.3, 95% CI=1.8-2.8) were higher in children with congenital anomalies compared to children without. INTERPRETATION: The proportion of children with major congenital anomalies in the BCPR (11%) was lower than that identified in higher-income countries. This may be because of differences in how congenital anomalies are diagnosed as well as the impact of survival bias. In Bangladesh, children with CP and major congenital anomalies are more likely to have severe functional motor limitations and associated comorbidities. WHAT THIS PAPER ADDS: Eleven per cent of children with cerebral palsy (CP) in Bangladesh had major congenital anomalies. Neurological and musculoskeletal congenital anomalies were the most common. Severe functional motor limitations and associated comorbidities were more common in children presenting with CP and major congenital anomalies.

Epidemiology of cerebral palsy in Sumba Island, Indonesia.

AIM: To define the epidemiology, clinical characteristics, and rehabilitation status of children with cerebral palsy (CP) in Sumba Island, Indonesia. METHOD: A community-based key informant method survey among children (aged <18y) with CP was conducted between March and August 2017. Children with suspected CP underwent detailed neurodevelopmental assessment by a multidisciplinary medical team. Socio-demographic characteristics, aetiology, motor type, motor severity, associated impairments, educational, and rehabilitation status were documented. RESULTS: There were 130 children with clinically confirmed CP. The mean age at assessment was 8 years 11 months and 43.8% (n=57) of the children were female. The mean age at CP diagnosis was 6 years 5 months. Of these children, 46.9% (n=61) had post-neonatally acquired CP, most frequently because of vaccine-preventable infectious encephalopathy (73.8%, n=45). In total, 80.8% (n=105) had a predominantly spastic motor type of CP and 83.8% (n=109) were classified in Gross Motor Functional Classification System levels III to V. A total of 77.7% (n=101) had at least one associated impairment (speech 77.5%, intellectual 29.2%, visual 13.8%, hearing 20.0%, and epilepsy 13.5%). And 66.2% (n=86) had never received rehabilitation services. INTERPRETATION: Post-neonatally acquired CP was common in this setting. Addressing preventable post-neonatally acquired risk factors for CP should be a public health priority. Earlier identification and diagnosis of CP would also provide new opportunities for early intervention and targeted rehabilitation services.

Linking data from a large clinical trial with the Australian Cerebral Palsy Register.

AIM: To link data from a large maternal perinatal trial with the Australian Cerebral Palsy Register (ACPR) to identify children with cerebral palsy (CP). METHOD: Deidentified data from the Australasian Collaborative Trial of Magnesium Sulphate (ACTOMgSO4 ) and the ACPR were linked. Children born from 1996 to 2000 at Australian hospitals who survived and had 2-year paediatric assessments were included. Children identified with CP in: (1) both the ACTOMgSO4 (2y) and the ACPR (5y), (2) the ACTOMgSO4 only, and (3) the ACPR only were compared. RESULTS: We included 913 children (492 males, 421 females; mean gestational age at birth 27.8wks [standard deviation 2.1wks]; range 23.0-40.0wks). Eighty-four children received a CP diagnosis: 35 by the ACTOMgSO4 and the ACPR, 29 by the ACTOMgSO4 only, and 20 by the ACPR only. The ACTOMgSO4 diagnosed 76.2% (95% confidence interval [CI] 65.9-84.1) and the ACPR identified 65.5% (95% CI 54.7-74.9). Children born in states/territories with long-standing versus more recently established registers were more likely to be included on the ACPR (p<0.05). INTERPRETATION: Linking deidentified perinatal trial data with the ACPR was achieved. Limitations of both strategies for identifying children with CP in this era (late 1990s and early 2000s) probably explain many of the differences observed, and inform future linkage studies and evaluations of CP-preventive interventions. WHAT THIS PAPER ADDS: Randomized trial data were linked with the Australian Cerebral Palsy Register. Trial (2y) and register (up to 5y) diagnoses of cerebral palsy (CP) differed. States with long-standing registers were more likely to include children with CP.

Epidemiology of cerebral palsy in Bangladesh: a population-based surveillance study.

AIM: To examine the prevalence, clinical characteristics, and risk factors of cerebral palsy (CP) in children in Bangladesh. METHOD: The Bangladesh CP Register is an ongoing population-based surveillance database of children with CP from a geographically defined area in Bangladesh. Cases were defined based on Surveillance of CP in Europe and Australian CP Register criteria after clinical assessments and identification by the key informant's method. RESULTS: In total, 726 children with CP were identified between January 2015 and December 2016. Mean age was 7 years 7 months (standard deviation [SD] 4y 6mo; range: 4.8mo-18y; median 7y 1.2mo; 61.8% male, 38.2% female). Mean age at CP diagnosis was 5 years 2 months (SD 3.8). Observed prevalence was 3.4 per 1000 children (95% confidence interval [CI]: 3.2-3.7), resulting in an estimated 233 514 children (95% CI: 219 778-254 118) with CP in Bangladesh. The majority (79.6%) had spastic CP. Altogether, 79.6% of the children with CP had at least one associated impairment (speech 67.6%, intellectual 39.0%, epilepsy 23.7%, visual 10.2%, and hearing 10.2%). In total, 78.2% never received rehabilitation. INTERPRETATION: In Bangladesh, the burden of CP is high, and diagnosis is substantially delayed, limiting opportunities for early intervention. There is a lack of available services and the majority of the children had preventable risk factors. WHAT THIS PAPER ADDS: Prevalence of cerebral palsy (CP) is 3.4 per 1000 children in rural Bangladesh. There are an estimated 233 514 children with CP in Bangladesh. The majority have potentially preventable risk factors. Diagnosis of CP is delayed, limiting opportunities for early intervention. There is a lack of available services for children with CP in rural Bangladesh.

EPIDEMIOLOGÍA DE LA PARÁLISIS CEREBRAL EN BANGLADESH: UN ESTUDIO DE VIGILANCIA BASADO EN LA POBLACIÓN: OBJETIVO: Examinar la prevalencia, las características clínicas y los factores de riesgo de la parálisis cerebral (PC) en niños en Bangladesh. MÉTODO: El Registro de PC (BCPR siglas en ingles) de Bangladesh es una base de datos de vigilancia basada en la población de niños con PC, de un área geográficamente definida, en Bangladesh. Los casos se definieron siguiendo los criterios de vigilancia de PC en Europa y los criterios del registro de PC australiano, teniendo en cuenta las evaluaciones clínicas y la identificación por el método del informante clave. RESULTADOS: En total, se identificaron 726 niños con PC entre enero del 2015 y diciembre del 2016. La edad media fue de 7 años y 7 meses (desviación estándar [DE] 4ª 6m; rango: 4,8m-18a; mediana 7a 1,2m; 61,8% masculino, 38,2 % femenino). La edad promedio en el diagnóstico de PC fue de 5 años y 2 meses (DE 3.8). La prevalencia observada fue de 3,4 por cada 1.000 niños (intervalo de confianza [IC] del 95%: 3,2-3,7), resultando en un número estimado de 233.514 niños con PC (IC del 95% 219 778-254 118) en Bangladesh. La mayoría (79.6%) tenía PC espástica. En total, el 79.6% de los niños con PC tenían al menos un impedimento asociado (habla 67.6%, intelectual 39.0%, epilepsia 23.7%, visual 10.2% y audición 10.2%). En total, el 78.2% nunca recibió rehabilitación. INTERPRETACIÓN: En Bangladesh, el impacto de PC es alto y el diagnóstico se retrasa sustancialmente, lo que limita las oportunidades de intervención temprana. Hay una falta de servicios disponibles y la mayoría de los niños tenían factores de riesgo prevenibles.

EPIDEMIOLOGIA DA PARALISIA CEREBRAL EM BANGLADESH: UM ESTUDO DE VIGILÂNCIA COM BASE POPULACIONAL: OBJETIVO: Examinar a prevalência, características clínicas, e fatores de risco para paralisia cerebral (PC) em crianças de Bangladesh. MÉTODO: O Registro de PC de Bangladesh (RPCB) é uma base de dados populacional em andamento para vigilância de crianças com PC de uma área geograficamente definida de Bangladesh. Os casos foram definidos com base nos critérios do Vigilância da PC na Europa e do Registro Australiano de PC após avaliação clínica e identificação pelo método pelo informante principal RESULTADOS: No total, 726 crianças com PC foram identificadas entre Janeiro 2015 e Dezembro 2016. A média de idade foi 7 anos e 7 meses (desvio padrão [DP] 4a 6m; variação: 4,8m-18a; mediana 7a 1,2m; 61,8% do sexo masculino, 38,2% do sexo feminino). A média de idade no momento do diagnóstic de PC foi de 5 anos e 2 meses (DP 3,8). A prevalência observada foi 3,4 a cada 1000 crianças (intervalo de confiança [IC] a 95% 3,2-3,7), resultando em uma estimativa de 233.514 crianças (IC 95% 219.778-254.118) com PC em Bangladesh. A maioria (79,6%) tinha PC espástica. No total, 79,6% das crianças com PC tinha pelo menos uma deficiência associada (linguagem 67,6%, intelectual 39,0%, epilepsia 23,7%, visual 10,2%, e auditiva 10,2%). No total, 78,2% nunca recebeu reabilitação. INTERPRETAÇÃO: Em Bangladesh, o peso da PC é alto, e o diagnóstico é substancialmente tardio, limitando as oportunidades de intervenção precoce. Há carência de serviços disponíveis, e a maioria das crianças apresentou fatores de risco preveníveis.

Mortality in children with cerebral palsy in rural Bangladesh: a population-based surveillance study.

AIM: To determine the mortality rate, immediate cause of death (CoD), and predictors of death in children with cerebral palsy (CP) in rural Bangladesh. METHOD: We carried out a prospective population-based surveillance study of children with CP aged 0 to 18 years registered with the Bangladesh Cerebral Palsy Register (BCPR) between January 2015 and December 2016, with subsequent follow-up until December 2017. Verbal autopsy was applied to assign immediate CoD. Crude mortality rates, hazard ratios of death, and survival probabilities were estimated. RESULTS: Twenty-nine of the 678 children in the BCPR died during the study period, resulting in a crude mortality rate of 19.5 per 1000 person-years of observation (total follow-up duration 1486.8 person-years; mean 2y [standard deviation 6mo]). The leading immediate CoD was meningitis (n=9) and pneumonia (n=8). Survival probability and hazard ratio of death was significantly associated with age, Gross Motor Functional Classification System level, and associated impairments. Severe underweight and/or severe stunting was significantly overrepresented among deceased children than others in the cohort (p<0.05) when compared with the World Health Organization reference population. INTERPRETATION: The majority of deaths were due to potentially preventable causes. The life expectancy of these children could have been extended by ensuring primary healthcare and nutritional supplementation. WHAT THIS PAPER ADDS: Mortality rate in children with cerebral palsy (CP) in rural Bangladesh is 19.5 per 1000 person-years. The majority of children with CP died from potentially preventable and treatable conditions. Motor severity, associated impairments, and malnutrition make children with CP vulnerable to premature death in rural Bangladesh.