Use of primary health care services among children and adolescents with cerebral palsy.

AIM: To investigate the use of general practitioners and urgent care centres (UCC) among children and adolescents with cerebral palsy (CP) compared to a control group, and per gross motor function level. METHOD: Data on children with CP born 1996 to 2014 were collected from the Norwegian Quality and Surveillance Registry for Cerebral Palsy. A control group was extracted from Statistics Norway. The date and diagnosis codes for general practitioner and UCC contacts from 2006 to 2015 were collected from the Norwegian Control and Payment of Health Reimbursement Database. Incidence rate ratios (IRR) for the number of contacts per person-year with 95% confidence intervals (CI) were calculated using Poisson regression. Risk differences with 95% CI were used to compare cumulative diagnosis incidences between children with CP and the control group. RESULTS: The study included 2510 children (1457 males; 58.1%) with CP and 12 041 (7003 males; 58.2%) without CP (mean age in both groups 7 years 2 months, SD 4 years 8 months, range 0-19 years), with 336 250 contacts. Children with CP had more general practitioner (IRR 1.47; 95% CI 1.29-1.67) and UCC (IRR 1.30; 95% CI 1.13-1.50) contacts than children without CP, for all ages. IRRs remained unchanged when comparing children with CP in Gross Motor Function Classification System (GMFCS) levels I and II to children without CP. Among children with CP, contact increased as GMFCS levels increased, and they were in contact most often for respiratory and general and unspecified diagnoses. The risk for epilepsy was highest for those in contact with general practitioners. INTERPRETATION: Children with CP, including those with less severe motor impairments, contacted general practitioners and UCCs more than children without CP. However, contact increased as gross motor impairment increased. They had contact for many diagnoses, mostly respiratory.

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.

Prevalence, Clinical Features, Neuroimaging, and Genetic Findings in Children With Ataxic Cerebral Palsy in Europe.

BACKGROUND AND OBJECTIVES: To report on prevalence, associated impairments, severity, and neuroimaging findings in children with ataxic cerebral palsy (CP). METHODS: In children coded as having ataxic CP in the Central database of Joint Research Center-Surveillance of Cerebral Palsy in Europe (JRC-SCPE) and born during 1980-2010, birth characteristics, severity profiles including associated impairments, neuroimaging patterns, and the presence of syndromes were analyzed. Definitions were according to validated SCPE guidelines. Prevalence over time was estimated using Poisson regression. RESULTS: In total, 679 children with ataxic CP were identified in 20 European CP registers. The proportion with ataxic CP was 3.8% and varied from 0% to 12.9%. Prevalence over time showed no significant trend. Approximately 70% of children with ataxic CP were able to walk, and 40% had severe intellectual impairment and a high impairment index. Children with ataxic CP were mostly born at term (79%) and with normal birth weight (77%). Neuroimaging patterns revealed normal findings in 29%, brain maldevelopments in 28.5%, miscellaneous findings in 23.5%, and brain injuries in 19%, according to the SCPE classification. Genetic syndromes were described in 9%. DISCUSSION: This register-based multicenter study on children with ataxic CP provides a large sample size for the analysis of prevalence, severity, and origin of this rare CP subtype. Even with strict inclusion and classification criteria, there is variation between registers on how to deal with this subtype, and diagnosis of ataxic CP remains a challenge. Ataxic cerebral palsy differs from other CP subtypes: children with ataxic CP have a disability profile that is more pronounced in terms of cognitive than gross motor dysfunction. They are mostly term born and the origin rarely suggests acquired injuries. In addition to neuroimaging, a comprehensive genetic workup is particularly recommended for children with this CP type.

Association between gestational levels of toxic metals and essential elements and cerebral palsy in children.

Introduction: Cerebral palsy (CP) is the most common motor disability in childhood, but its causes are only partly known. Early-life exposure to toxic metals and inadequate or excess amounts of essential elements can adversely affect brain and nervous system development. However, little is still known about these as perinatal risk factors for CP. This study aims to investigate the associations between second trimester maternal blood levels of toxic metals, essential elements, and mixtures thereof, with CP diagnoses in children. Methods: In a large, population-based prospective birth cohort (The Norwegian Mother, Father, and Child Cohort Study), children with CP diagnoses were identified through The Norwegian Patient Registry and Cerebral Palsy Registry of Norway. One hundred forty-four children with CP and 1,082 controls were included. The relationship between maternal blood concentrations of five toxic metals and six essential elements and CP diagnoses were investigated using mixture approaches: elastic net with stability selection to identify important metals/elements in the mixture in relation to CP; then logistic regressions of the selected metals/elements to estimate odds ratio (OR) of CP and two-way interactions among metals/elements and with child sex and maternal education. Finally, the joint effects of the mixtures on CP diagnoses were estimated using quantile-based g-computation analyses. Results: The essential elements manganese and copper, as well as the toxic metal Hg, were the most important in relation to CP. Elevated maternal levels of copper (OR = 1.40) and manganese (OR = 1.20) were associated with increased risk of CP, while Hg levels were, counterintuitively, inversely related to CP. Metal/element interactions that were associated with CP were observed, and that sex and maternal education influenced the relationships between metals/elements and CP. In the joint mixture approach no significant association between the mixture of metals/elements and CP (OR = 1.00, 95% CI = [0.67, 1.50]) was identified. Conclusion: Using mixture approaches, elevated levels of copper and manganese measured in maternal blood during the second trimester could be related to increased risk of CP in children. The inverse associations between maternal Hg and CP could reflect Hg as a marker of maternal fish intake and thus nutrients beneficial for foetal brain development.

Working Memory Training in Norwegian Children with Cerebral Palsy (CP) Show Minimal Evidence of Near and No Far Transfer Effects.

In children with cerebral palsy (CP), learning disabilities are well documented, and impairments in executive functions, such as attention, inhibition, shifting and working memory, represent significant burdens on patients, their families and the society. The aim of this study was to evaluate whether Cogmed RM working memory training could improve working memory in children with CP and investigate whether increased working memory capacity would generalize to other cognitive functions. Twenty-eight children completed the training and the results were compared to a waitlist control group (n = 32). The results yielded three main findings. First, children with CP improved with practice on trained working memory tasks. Second, the intervention group showed minimal near transfer effects to non-trained working memory tasks. Third, no effects on cognitive and behavioral far transfer measures were found.

Cerebral palsy in children born after assisted reproductive technology in Norway: Risk, prevalence, and clinical characteristics.

INTRODUCTION: The aim was to investigate the risk, prevalence, and clinical characteristics of cerebral palsy among children born after assisted reproductive technology (ART) in Norway. MATERIAL AND METHODS: All liveborn children from 2002 to 2015 were included. Information was collected from the Medical Birth Registry of Norway, linked to the Norwegian Quality and Surveillance Registry for Cerebral Palsy as of December 31, 2022. Logistic regression analyses were used to calculate the prevalence of cerebral palsy per 1000 live births after ART and natural conception with birth year as covariate, crude odds ratios (OR) for cerebral palsy among children born after ART using children born after natural conception as reference, and OR adjusted for potential confounders, with 95% confidence intervals (CI). Potential mediators of the association were studied in stratified analyses. Descriptive statistics were used to compare proportions in clinical characteristics among children with cerebral palsy born after ART and natural conception. RESULTS: Among 833 645 livebirths, 23 645 children were born after ART and of the latter 97 were diagnosed with cerebral palsy. The overall prevalence of cerebral palsy after ART was 4.10 per 1000 live births (95% CI 3.36-5.00), decreasing from 7.79 per 1000 in 2002 to 3.55 in 2015. Compared with children born after natural conception, the OR for cerebral palsy was 2.01 (95% CI 1.63-2.47) adjusted for mother's age at birth, parity, and pre-pregnancy health. When restricted to singletons born at term, the adjusted OR for cerebral palsy was 1.13 (95% CI 0.76-1.69). The distribution of cerebral palsy subtypes and the severity of gross and fine motor function and associated impairments did not differ significantly between children with cerebral palsy born after ART and natural conception. CONCLUSIONS: Children born after ART had a risk of cerebral palsy that was twice that of children born after natural conception. The increased risk of cerebral palsy after ART is likely attributed to multiple pregnancies and preterm births. The prevalence of cerebral palsy after ART decreased significantly during the study period, despite an increased use of ART in the population. The distribution of clinical characteristics did not differ between children with cerebral palsy born after ART and those born after a natural conception, suggesting that the risk factors for, and causes of cerebral palsy were similar.

Pain and labor outcomes: A longitudinal study of adults with cerebral palsy in Sweden.

BACKGROUND: Pain is a global health concern with substantial societal costs and limits the activity participation of individuals. The prevalence of pain is estimated to be high among individuals with cerebral palsy (CP). OBJECTIVES: To estimate the association between pain and labor outcomes for adults with CP in Sweden. METHODS: A longitudinal cohort study based on data from Swedish population-based administrative registers of 6899 individuals (53,657 person-years) with CP aged 20-64 years. Individual fixed effects regression models were used to analyze the association between pain and labor outcomes (employment and earnings from employment), as well as potential pathways through which pain might affect employment and earnings. RESULTS: Pain was associated with adverse outcomes varying across severity, corresponding to a reduction of 7-12% in employment and 2-8% in earnings if employed. Pain might affect employment and earnings through increased likelihood of both sickness leave and early retirement. CONCLUSION: Pain management could potentially be important to improve labor outcomes for adults with CP, in addition to improving the quality of life.

Prevalence, birth, and clinical characteristics of dyskinetic cerebral palsy compared with spastic cerebral palsy subtypes: A Norwegian register-based study.

AIM: To study the prevalence, birth, and clinical characteristics of children with dyskinetic cerebral palsy (CP) in Norway compared with spastic quadriplegic CP and other spastic CP subtypes. METHOD: Data on children born from 1996 to 2015 were collected from the Norwegian Quality and Surveillance Registry for Cerebral Palsy and the Medical Birth Registry of Norway. RESULTS: One hundred and seventy (6.8%) children had dyskinetic CP. The birth prevalence decreased during 1996 to 2015 from 0.21 to 0.07 per 1000 livebirths (p < 0.001). Dyskinetic CP was more often associated with term/post-term birth, and motor and associated impairments were more severe compared with spastic bilateral and unilateral CP, but less severe than spastic quadriplegic CP. On neuroimaging, grey matter injuries were most prevalent in dyskinetic CP (mainly basal ganglia/thalamus) and spastic quadriplegic CP (mainly cortico-subcortical), white matter injuries in spastic bilateral, and white and grey matter injuries were equally common in spastic unilateral CP. Normal neuroimaging and brain maldevelopment were present in 25% of children with dyskinetic CP. INTERPRETATION: The decrease in birth prevalence of dyskinetic CP was probably due to improved antenatal and perinatal care. Potential sentinel events at term were more common in dyskinetic CP than other spastic CP subtypes. However, probable antenatal aetiologies were most prevalent. Motor and associated impairments were less severe in children with dyskinetic CP compared with spastic quadriplegic CP. WHAT THIS PAPER ADDS: Birth prevalence of those with dyskinetic and spastic bilateral cerebral palsy (CP) in Norway decreased between 1996 and 2015. Potential sentinel events at term were more common in dyskinetic CP. Nonetheless, probable antenatal aetiologies were most prevalent in dyskinetic CP. Basal ganglia/thalamus lesions were more common in dyskinetic than spastic quadriplegic CP. Motor and associated impairments were milder in dyskinetic than spastic quadriplegic CP.

The implementation of systematic monitoring of cognition in children with cerebral palsy in Sweden and Norway.

PURPOSE: Children with cerebral palsy (CP) are at risk of cognitive impairments and need to be cognitively assessed to allow for individualized interventions, if applicable. Therefore, a systematic protocol for the follow-up of cognition in children with CP, CPCog, with assessments offered at five/six and 12/13 years of age, was developed. This report presents and discusses assessment practices in Sweden and Norway following the introduction of CPCog and a quality improvement project in Norway aimed at increasing the number of children offered cognitive assessments. MATERIALS AND METHODS: A questionnaire investigating assessment practices was sent to pediatric habilitation centers in Sweden and Norway. In Norway, the habilitation centers also participated in a quality improvement project aimed at increasing adherence to the CPCog protocol. RESULTS: Of the respondents, 64-70% report that they assess cognition in children with all degrees of motor impairment, and 70-80% assess at the ages recommended in CPCog. Following the quality improvement project in Norway, the percentage of children assessed increased from 34 to 62%. CONCLUSIONS: The findings illustrate that the provision of information is not sufficient to change practice. Implementation of new re/habilitation procedures is aided by targeting health care practices individually.Implications for rehabilitationChildren with cerebral palsy (CP) have increased risk of cognitive impairments that require intervention.Assessments of cognition should be offered to all children with CP because the nature of cognitive impairments may vary.Introducing a follow-up protocol of how and when to perform cognitive assessments is a step towards ensuring equal access to the services for all children with CP.A quality improvement project might be a viable method for implementing a protocol into everyday clinical practice.

Pain burden in children with cerebral palsy (CPPain) survey: Study protocol.

Pain is a significant health concern for children living with cerebral palsy (CP). There are no population-level or large-scale multi-national datasets using common measures characterizing pain experience and interference (ie, pain burden) and management practices for children with CP. The aim of the CPPain survey is to generate a comprehensive understanding of pain burden and current management of pain to change clinical practice in CP. The CPPain survey is a comprehensive cross-sectional study. Researchers plan to recruit approximately 1400 children with CP (primary participants) across several countries over 6-12 months using multimodal recruitment strategies. Data will be collected from parents or guardians of children with CP (0-17 years) and from children with CP (8-17 years) who are able to self-report. Siblings (12-17 years) will be invited to participate as controls. The CPPain survey consists of previously validated and study-specific questionnaires addressing demographic and diagnostic information, pain experience, pain management, pain interference, pain coping, activity and participation in everyday life, nutritional status, mental health, health-related quality of life, and the effect of the COVID-19 pandemic on pain and access to pain care. The survey will be distributed primarily online. Data will be analyzed using appropriate statistical methods for comparing groups. Stratification will be used to investigate subgroups, and analyses will be adjusted for appropriate sociodemographic variables. The Norwegian Regional Committee for Medical and Health Research Ethics and the Research Ethics Board at the University of Minnesota in USA have approved the study. Ethics approval in Canada, Sweden, and Finland is pending. In addition to dissemination through peer-reviewed journals and conferences, findings will be communicated through the CPPain Web site (www.sthf.no/cppain), Web sites directed toward users or clinicians, social media, special interest groups, stakeholder engagement activities, articles in user organization journals, and presentations in public media.

High occurrence of perinatal risk factors and more severe impairments in children with postneonatal cerebral palsy in Norway.

AIM: To describe causal events, perinatal risk factors and clinical characteristics in children with postneonatal cerebral palsy (PNCP). METHODS: Population-based registry study of Norwegian children born 1999-2013. Prevalence, causal events and clinical characteristics of PNCP were described. The occurrence of perinatal risk factors for CP was compared with the general population. RESULTS: Among 1710 children with CP, 67 had PNCP (3.9%; 0.75 per 10,000 livebirths [95%CI: 0.59-0.96]). The prevalence of PNCP decreased during the study period. Leading causal events were cerebrovascular events (32.8%), head injuries/other accidents (22.4%), infections (19.4%) and hypoxic events (14.9%). Spastic hemiplegic (53.7%) or spastic quadriplegic/dyskinetic CP (31.3%) was more common in children with PNCP than non-PNCP (42.3% and 20.1%, respectively; p < 0.001). Children with PNCP had more severe motor and associated impairments. Perinatal risk factors for CP were more common in children with PNCP than in the general population. CONCLUSION: The prevalence of PNCP among Norwegian children was low and decreasing. The main causes were cerebrovascular events and head injuries/other accidents. Although spastic hemiplegic CP was the dominating subtype, children with PNCP had more severe motor and associated impairments than children with non-PNCP, as well as a higher occurrence of perinatal risk factors than in the general population.

Systematic Monitoring of Cognition for Adults With Cerebral Palsy-The Rationale Behind the Development of the CPCog-Adult Follow-Up Protocol.

Cerebral palsy (CP) comprises a heterogeneous group of conditions recognized by disturbances of movement and posture and is caused by a non-progressive injury to the developing brain. Birth prevalence of CP is about 2-2.5 per 1,000 live births. Although the motor impairment is the hallmark of the diagnosis, individuals with CP often have other impairments, including cognitive ones. Cognitive impairments may affect communication, education, vocational opportunities, participation, and mental health. For many years, CP has been considered a "childhood disability," but the challenges continue through the life course, and health issues may worsen and new challenges may arise with age. This is particularly true for cognitive impairments, which may become more pronounced as the demands of life increase. For individuals with CP, there is no one-to-one correlation between cognition and functioning in other areas, and therefore, cognition must be individually assessed to determine what targeted interventions might be beneficial. To facilitate this for children with CP, a systematic follow-up protocol of cognition, the CPCog, has been implemented in Norway and Sweden. However, no such protocol currently exists for adults with CP. Such discontinuity in healthcare services that results from lack of follow-up of cognitive functioning and subsequent needs for adjustments and interventions makes transition from pediatric to adult healthcare services challenging. As a result, a protocol for the surveillance of cognition in adults with CP, the CPCog-Adult, has been developed. It includes assessment of verbal skills, non-verbal reasoning, visual-spatial perception, and executive functioning. It is recommended to perform these assessments at least once in young adulthood and once in the mid-fifties. This report describes the process of developing the CPCog-Adult, which has a three-fold purpose: (1) to provide equal access to healthcare services to enable the detection of cognitive impairments; (2) to provide interventions that increase educational and vocational participation, enhance quality of life, and prevent secondary impairments; and (3) to collect systematic data for research purposes. The consent-based registration of data in the well-established Swedish and Norwegian national CP registries will secure longitudinal data from childhood into adulthood.

Treatment of spasticity in children and adolescents with cerebral palsy in Northern Europe: a CP-North registry study.

BACKGROUND: Spasticity is present in more than 80% of the population with cerebral palsy (CP). The aim of this study was to describe and compare the use of three spasticity reducing methods; Botulinum toxin-A therapy (BTX-A), Selective dorsal rhizotomy (SDR) and Intrathecal baclofen therapy (ITB) among children and adolescents with CP in six northern European countries. METHODS: This registry-based study included population-based data in children and adolescents with CP born 2002 to 2017 and recorded in the follow-up programs for CP in Sweden, Norway, Denmark, Iceland and Scotland, and a defined cohort in Finland. RESULTS: A total of 8,817 individuals were included. The proportion of individuals treated with SDR and ITB was significantly different between the countries. SDR treatment ranged from 0% ( Finland and Iceland) to 3.4% (Scotland) and ITB treatment from 2.2% (Sweden) to 3.7% (Denmark and Scotland). BTX-A treatment in the lower extremities reported 2017-2018 ranged from 8.6% in Denmark to 20% in Norway (p < 0.01). Mean age for undergoing SDR ranged from 4.5 years in Norway to 7.3 years in Denmark (p < 0.01). Mean age at ITB surgery ranged from 6.3 years in Norway to 10.1 years in Finland (p < 0.01). Mean age for BTX-A treatment ranged from 7.1 years in Denmark to 10.3 years in Iceland (p < 0.01). Treatment with SDR was most common in Gross Motor Function Classification System (GMFCS) level III, ITB in level V, and BTX-A in level I. The most common muscle treated with BTX-A was the calf muscle, with the highest proportion in GMFCS level I. BTX-A treatment of hamstring and hip muscles was most common in GMFCS levels IV-V in all countries. CONCLUSION: There were statistically significant differences between countries regarding the proportion of children and adolescents with CP treated with the three spasticity reducing methods, mean age for treatment and treatment related to GMFCS level. This is likely due to differences in the availability of these treatment methods and/or differences in preferences of treatment methods among professionals and possibly patients across countries.

Risk Factors for Cerebral Palsy in Moldova.

Background and Objectives: This is the first study assessing risk factors for cerebral palsy (CP) among children born in Moldova. The aim of this study was to identify and describe risk factors for cerebral palsy (CP) among children born in Moldova, which is one of the low-middle income countries in Europe. Materials and Methods: We identified 351 children with CP born during 2009 and 2010 in Moldova. Detailed information on 417 children without CP served as a reference group. Logistic regression analyses were applied to the calculate crude and adjusted odds ratios (OR) for CP with 95% confidence intervals (CI) in addition to attributable fraction (AF). Results: Among children with CP (40.5% girls), 26% had spastic unilateral, 54% bilateral, 13% dyskinetic, 5% ataxic and 2% unclassified CP. Significant risk factors for CP included maternal alcohol consumption during pregnancy (OR 1.7, p = 0.002), maternal hypertension (OR 2.0, p < 0.001), children born to mothers from the rural areas (OR 1.6, p < 0.001), maternal age ≥35 years (OR 0.6, p = 0.018), maternal epilepsy (OR 4.3, p < 0.001), breech delivery (OR 3.1, p = 0.001), home births (OR 6.3, p = 0.001), umbilical cord around neck (OR 2.2, p < 0.001), AVD (OR 3.1, p < 0.001), male gender (OR 1.3, p < 0.001), SGA (OR 1.3, p = 0.027), multiple gestations (OR 1.7, p < 0.001) and hyperbilirubinemia (OR 4.5, p < 0.001). Multivariable analyses showed that the AF of CP was 64% for rural residence (OR 2.8, p = 0.002), 87% for home birth (7.6, p = 0.005), 79% for pre-labor rupture of membrane (OR 4.9, p = 0.001), 66% for breech delivery (OR 2.9, p = 0.002) and 81% for hyperbilirubinemia (OR 5.4, p < 0.001). Conclusions: A combination of factors related to the mother, the delivery and the child were risk factors for CP in Moldova, many of them possibly avoidable. Improved pregnancy and maternity care would potentially reduce the risk of CP. A national CP registry in Moldova is suggested as an opportunity to follow up on these findings.

Trends in Prevalence and Severity of Pre/Perinatal Cerebral Palsy Among Children Born Preterm From 2004 to 2010: A SCPE Collaboration Study.

Aim: To report on prevalence of cerebral palsy (CP), severity rates, and types of brain lesions in children born preterm 2004 to 2010 by gestational age groups. Methods: Data from 12 population-based registries of the Surveillance of Cerebral Palsy in Europe network were used. Children with CP were eligible if they were born preterm (<37 weeks of gestational age) between 2004 and 2010, and were at least 4 years at time of registration. Severity was assessed using the impairment index. The findings of postnatal brain imaging were classified according to the predominant pathogenic pattern. Prevalences were estimated per 1,000 live births with exact 95% confidence intervals within each stratum of gestational age: ≤27, 28-31, 32-36 weeks. Time trends of both overall prevalence and prevalence of severe CP were investigated using multilevel negative binomial regression models. Results: The sample comprised 2,273 children. 25.8% were born from multiple pregnancies. About 2-thirds had a bilateral spastic CP. 43.5% of children born ≤27 weeks had a high impairment index compared to 37.0 and 38.5% in the two other groups. Overall prevalence significantly decreased (incidence rate ratio per year: 0.96 [0.92-1.00[) in children born 32-36 weeks. We showed a decrease until 2009 for children born 28-31 weeks but an increase in 2010 again, and a steady prevalence (incidence rate ratio per year = 0.97 [0.92-1.02] for those born ≤27 weeks. The prevalence of the most severely affected children with CP revealed a similar but not significant trend to the overall prevalence in the corresponding GA groups. Predominant white matter injuries were more frequent in children born <32 weeks: 81.5% (≤27 weeks) and 86.4% (28-31 weeks), compared to 63.6% for children born 32-36 weeks. Conclusion: Prevalence of CP in preterm born children continues to decrease in Europe excepting the extremely immature children, with the most severely affected children showing a similar trend.

Point prevalence and motor function of children and adolescents with cerebral palsy in Scandinavia and Scotland: a CP-North study.

AIM: To describe the point prevalence of cerebral palsy (CP) and distribution of gross and fine motor function in individuals registered in a CP-North surveillance programme. METHOD: Aggregate data of individuals with CP aged 6 to 19 years, sex, CP subtype, and gross and fine motor function levels were collected from each programme. Overall and age-specific point prevalence of CP was calculated for each programme using 95% confidence intervals. Logistic regression was used to estimate prevalence and CP subtypes with age as the covariate variable. Pearson χ2 tests were used to compare the distributions of CP subtypes, Gross Motor Function Classification System (GMFCS) levels, and Manual Ability Classification System (MACS) levels by age and between programmes. RESULTS: Among 3 759 138 individuals residing in Scandinavia and Scotland, 8278 had a diagnosis of CP (57-59% were males). The overall point prevalence of CP ranged from 2.13 to 2.32 per 1000 residents. Age-specific prevalence in each programme varied with the exception of Denmark. While the proportions of bilateral spastic CP were similar between programmes, there were variations in all other CP subtypes and in GMFCS and MACS levels. INTERPRETATION: While the results of this study may reflect real differences in CP populations between countries, they may not be clinically relevant. The variations may be attributable to differences in the year when each programme was first established, different data collection methods, and country-specific governmental policies.

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.

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).