Common data elements of cerebral palsy registries in Arabic-speaking countries: A scoping review.

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). 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. 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. 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.

To conduct a systematic review of phenotypic definition and case ascertainment in published genetic studies of cerebral palsy (CP) to inform guidelines for the reporting of such studies. Inclusion criteria comprised genetic studies of candidate genes, with CP as the outcome, published between 1990 and 2019 in the PubMed, Embase, and BIOSIS Citation Index databases. Fifty-seven studies met the inclusion criteria. We appraised how CP was defined, the quality of information on case ascertainment, and compliance with international consensus guidelines. Seven studies (12%) were poorly described, 33 studies (58%) gave incomplete information, and 17 studies (30%) were well described. Missing key information precluded determining how many studies complied with the definition by Rosenbaum et al. Only 18 out of 57 studies (32%) were compliant with the Surveillance of Cerebral Palsy in Europe (SCPE) international guidelines on defining CP. Limited compliance with international consensus guidelines on phenotypic definition and mediocre reporting of CP case ascertainment hinders the comparison of results among genetic studies of CP (including meta-analyses), thereby limiting the quality, interpretability, and generalizability of study findings. Compliance with the SCPE guidelines is important for ongoing gene discovery efforts in CP, given the potential for misclassification of unrelated neurological conditions as CP.

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. 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. 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. 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. 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.

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. 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. 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. 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. 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).

Cerebral palsy (CP) research has broadened its horizons in the past decades. In addition to the long-term, population-based studies of prevalence of the different CP types, gestational-age groups, and accompanying impairments, data on several aspects of function have emerged. The well-accepted Gross Motor Function Classification System1 has given us a common language to compare gross motor function in different populations and at various ages. Gross motor classification has been followed by classifications of hand function, such as the Bimanual Fine Motor Function2 and the Manual Ability Classification System.3 However, differences in CP classification around the world still exist. This is well illustrated by Rice et al. in their report from the South Australian CP Register in the current issue of the journal. Although a method of classification was proposed along with the most recent definition of CP,4 the traditional systems tend to predominate. This makes comparison between CP registers difficult and important knowledge about trends, differences, and similarities may be overlooked or lost. An essential aspect of the diagnosis of CP is the neurological signs. It is now recommended that classification should be made according to the dominant type of tone or movement abnormality. This has not been the case before in all countries, and has made any attempt to pool data difficult. However, such comparisons have been made possible in Europe after the introduction of the Reference and Training Manual, produced by the Surveillance of Cerebral Palsy in Europe (SCPE). It contains descriptions and video illustrations of the different CP types. It is an excellent tool in the endeavor to harmonize classification of CP in Europe and elsewhere.5 The paper by Rice et al. is a commendable attempt to carry out a comparison between CP registers on two different continents, despite differences in classification systems. The fact that this is feasible is very promising, but the paper also depicts the difficulties of such a comparison. This is especially apparent when comparing the quadriplegia and dyskinetic groups in the Australian and Swedish registers. Although very similar groups are described the outcome regarding classification differed, because of the different traditions in the two countries on classifying dyskinetic symptoms. The prevalence of dyskinetic CP may be different from what we find in most studies today if, or when, classification according to the dominating symptom is fully accepted. As we strive to describe the symptoms and function of children with CP, we have to accept that there are not yet valid and reliable instruments for every measurement. When there is such an instrument is at hand, it should be used to compare studies, and also to make it possible for other researchers to relate to the data. The specific differentiation between dyskinetic and spastic features may indeed be a challenge, as will be, in some cases, deciding which is in fact the dominating symptom. Children with dyskinetic CP, classified according to dominating movement abnormality, have in a majority of cases also signs of spasticity.6 Very few instruments exist to describe dyskinesia. As dyskinetic movements may severely hamper motor function, there is a need for valid and reliable methods to assess this aspect of motor disability. The Barry-Albright Dystonia Scale, referred to by Rice et al., deals with the dystonic, but not the hyperkinetic symptoms.7 This scale is seldom used in CP research, but might be considered a useful tool.6 Also, in children with predominantly spastic CP there may be involuntary movements of a choreo-athetotic type that interfere with activities, and we have little means to describe this phenomenon in a structured way. Despite all this, Rice et al. take on the challenge to describe and discuss the dyskinetic features in children with CP and find this in almost every fifth child! CP research is carried out in various ways around the world. Many CP registers rely on reports from local physicians on what CP type, gross and fine motor function, and accompanying impairments a child might have, while others, such as the South Australian CP Register, has the advantage of a clinical examination in addition to the data from the records. Here is an opportunity to apply a variety of assessment instruments to ascertain information that is not possible to assess from records, but that is required to investigate the child. This may be one way to develop further population-based CP research. Future studies from the South Australian CP register will surely benefit from the access to clinical investigation using valid and reliable instruments to gather more population-based knowledge on CP.

The purpose of this work was to describe walking ability in children with cerebral palsy from the Surveillance of Cerebral Palsy in Europe common database through 21 years and to examine the association between walking ability and predicting factors. Anonymous data on 10042 children with cerebral palsy born between 1976 and 1996 were gathered from 14 European centers; 9012 patients were eligible for the analyses. Unaided walking as the primary way of walking at 5 years of age was reported for 54%, walking with assistive devices was reported for 16%, and no walking ability was reported for 30%. The proportion of children who were unable to walk was rather stable over time in all of the centers, with a mean proportion of 28%. Walking ability related significantly to cerebral palsy types, that is, spastic unilateral, spastic bilateral, dyskinetic, and ataxic cerebral palsy, as well as to IQ level, active epilepsy, and severe visual and hearing impairment. Severe cerebral palsy, defined as both the inability to walk and an IQ of <50, was present in 20% of the subjects. Logistic regression revealed that intellectual capacity was the variable most associated with walking ability in all 4 of the cerebral palsy types. The presence of a severe intellectual impairment increased the risk of being unable to walk 56 times if the child had unilateral spastic cerebral palsy type and 9 times if the child had bilateral spastic cerebral palsy type. The collaboration Surveillance of Cerebral Palsy in Europe provides a powerful means of monitoring trends in cerebral palsy and its functional consequences. The proportion of nonwalking in children with cerebral palsy seems to be rather stable over years and across centers despite the changes that have occurred in neonatal care across Europe. As is well known and also shown in this study, walking ability varied strongly with cerebral palsy type. Additional impairments, as well as the presence of epilepsy, correlated significantly with walking ability and, thus, the walking ability can be an indicator of total disability load.

To describe the prevalence of cerebral palsy (CP), subtype distribution, motor and intellectual impairment, and epilepsy in adults with CP compared with children with CP. CP subtype and impairment data from the population-based CP register of western Sweden and population data from Statistics Sweden were used to compare surviving adults (n=581; 244 females, 337 males) born between 1959 and 1978, with the same cohort as children (n=723; 307 females, 416 males), andwiththe most recent cohort, born from 2007 to 2010 (n=205; 84 females, 121 males). Prevalence of CP in adults born between 1959 and 1978 was 1.14 per 1000. The occurrence of impairments differed between CP subtypes. Motor and intellectual impairment were closely related, regardless of subtype. Subtype distribution among survivors differed significantly from the original cohorts (p=0.002), and the most recent cohort (p<0.01), tetraplegia and dyskinetic CP being less common in survivors. Severe motor impairment, intellectual disability, and epilepsy were less common among survivors than in the original cohorts (p=0.004, p=0.002, p=0.037) and the most recent cohort (p=0.004, p=0.008, p<0.01). Data on prevalence, subtype distribution, and impairments in children with CP are not applicable to adults with CP. Population-based studies of adults with CP are needed. Cerebral palsy (CP) subtypes are differently distributed in adults compared to children. The prevalence of impairments in adults with CP is related to CP subtype. Spastic tetraplegia and dyskinetic CP are less common in adults than children. Severe motor impairment, intellectual disability, and epilepsy are less common in adults.

Incidence, subtypes and severity of cerebral palsy in infants born extremely preterm in Switzerland: A retrospective study comparing two time periods.

Public health indicators (PHIs) play an increasingly important role in health policy decision-making. Although cerebral palsy (CP) is the commonest physical disability in children, its impact at population level has not been systematically measured so far. We aimed to propose six PHIs for CP designed to annually document the extent of CP and effectiveness of perinatal organisation, the burden of this condition, access to health services and preventive health strategies in the post-neonatal period and to report on the latest updated estimations using population-based data routinely collected by European CP registries. The study included children with CP born between 2002 and 2011. Harmonised data (number of cases, functional profile, imaging) were extracted from the Surveillance of Cerebral Palsy in Europe (SCPE) database. Eligibility criteria for analyses were applied separately for each indicator by selecting registries, birth years and CP cases. Current estimates were based on the last 3 birth years, while trends were reported over a 10-year period. All analyses were descriptive. Sensitivity analyses were carried out to examine the stability of the results using various thresholds of percentages of missing values. Analyses were performed on a total of 8621 children with CP from 12 to 17 SCPE registries. A decreasing prevalence of pre/perinatal CP overall, as well as in preterm and full-term-born children, was observed. The burden of the condition was strongly dependent on CP subtype and the presence of associated impairments. Access to brain imaging ranged from 80% to 100% depending on registries. The overall prevalence of post-neonatally acquired CP was approximately 0.8 per 10,000 live births over the study period. Population-based CP registries can provide data that are relevant for generating key outcomes of interest at the population level, thus potentially contributing to improving public health policies for children with disabilities.

Background. A European multicenter study (Surveillance of Cerebral Palsy in Europe, SCPE) was used to describe changes over time in multiple birth rates and cerebral palsy (CP) rates among multiple born infants, to compare CP rates and clinical types between multiples and singletons, and to analyse the influence of birth order in twins. Methods. Data were collected from 12 European population-based CP registers on 6613 children born in 1975–90, as well as demographic data. Results. The rate of multiple birth in the populations increased from 1.9% in 1980 to 2.4% in 1990, and the proportion of multiples among CP infants increased from 4.6% in 1976 to 10% in 1990. Multiples have a four times higher rate of CP than singletons [7.6 vs. 1.8 per 1000 live births, relative risk (RR) 4.36; 95% confidence interval (CI) 3.76–4.97] overall. The risk is marginally higher in multiples with birthweight > 2500 g (RR 1.60; 95% CI 0.95–2.28) and born at term (RR 1.65; 95% CI 0.91–2.40), and there is no difference in the risk for the low-birthweight and preterm groups. Correcting for differences in gestational age and birthweight, the clinical type of CP was the same in multiples and singletons. Twin CP infants are more often second than first born (56% vs. 44%, p < 0.05). Conclusions. Multiple born infants have a four times higher risk of developing cerebral palsy than singletons, mainly related to the higher risk of preterm birth in multiples. As the rate of multiples doubled through the 1980s, cerebral palsy cases in multiples increased in the same period.

A European multicenter study (Surveillance of Cerebral Palsy in Europe, SCPE) was used to describe changes over time in multiple birth rates and cerebral palsy (CP) rates among multiple born infants, to compare CP rates and clinical types between multiples and singletons, and to analyse the influence of birth order in twins. Data were collected from 12 European population-based CP registers on 6613 children born in 1975-90, as well as demographic data. The rate of multiple birth in the populations increased from 1.9% in 1980 to 2.4% in 1990, and the proportion of multiples among CP infants increased from 4.6% in 1976 to 10% in 1990. Multiples have a four times higher rate of CP than singletons [7.6 vs. 1.8 per 1000 live births, relative risk (RR) 4.36; 95% confidence interval (CI) 3.76-4.97] overall. The risk is marginally higher in multiples with birthweight > 2500 g (RR 1.60; 95% CI 0.95-2.28) and born at term (RR 1.65; 95% CI 0.91-2.40), and there is no difference in the risk for the low-birthweight and preterm groups. Correcting for differences in gestational age and birthweight, the clinical type of CP was the same in multiples and singletons. Twin CP infants are more often second than first born (56% vs. 44%, p < 0.05). Multiple born infants have a four times higher risk of developing cerebral palsy than singletons, mainly related to the higher risk of preterm birth in multiples. As the rate of multiples doubled through the 1980s, cerebral palsy cases in multiples increased in the same period.

SUMMARYThe aim was to study functional abilities and to create functional classification of children with cerebral palsy (CP) in Krapina-Zagorje County, based on the classification of gross and fine motor skills and associated impairments. Classification was performed according to the SCPE (Surveillance of Cerebral Palsy in Europe) criteria. We used standardized and complementary functional classification systems for cerebral palsy to create a functional profile. Research included 44 children with CP in the age range of 4 to 18 years. The results showed that the majority of children had bilateral spastic CP (63.6%), followed by unilateral spastic (22.7%) while the representation of dyskinetic CP was 9.09% and ataxic CP 4.55%. Based on the classification of gross and fine motor skills, 43.2% of children had the ability to walk, 11% of children could walk with assistive mobility devices, while 45.4% of children had a low functional level. The study also analyzed the associated impairments where higher classification score of motor impairment correlated with the severity of impairment. The results showed that children with dyskinetic CP and severe motor impairment could have mild cognitive impairment. We systematically present the neuropsychological and functional profile according to the CP type.

Background: Cerebral palsy (CP) is a disorder of movement and posture and every child with CP has a unique composition of neurological symptoms, motor severity, and associated impairments, constituting the functional profile. Although not part of the CP definition, magnetic resonance imaging (MRI) sheds light on the localization, nature, and severity of brain compromise. The MRI classification system (MRICS), developed by the Surveillance of Cerebral Palsy in Europe (SCPE), describes typical MRI patterns associated with specific timing of vulnerability in different areas of the brain. The classification has proven to be reliable and easy to use.Aims: The aim of this study is to apply the MRICS on a large dataset and describe the functional profile associated with the different MRI patterns of the MRICS.Materials and Methods: Data on children with CP born in 1999–2009 with a post-neonatal MRI from 20 European registers in the JRC-SCPE Central Registry was included. The CP classification and the MRICS was applied, and The Gross Motor Function Classification (GMFCS) and the Bimanual Fine Motor Function (BFMF) classification were used. The following associated impairments were documented: intellectual impairment, active epilepsy, visual impairment, and hearing impairment. An impairment index was used to characterize severity of impairment load.Results: The study included 3,818 children with post-neonatal MRI. Distribution of CP type, motor, and associated impairments differed by neuroimaging patterns. Functional profiles associated with neuroimaging patterns were described, and the impairment index showed that bilateral findings were associated with a more severe outcome both regarding motor impairment and associated impairments than unilateral compromise. The results from this study, particularly the differences in functional severity regarding uni- and bilateral brain compromise, may support counseling and service planning of support of children with CP.

The life expectancy of individuals with cerebral palsy (CP) is often reduced compared with the general population. Long-term survival with CP is rarely reported. The aim of this study was to investigate survival and the causes of death in relation to CP type and motor and accompanying impairments documented in the CP register of western Sweden over five decades. All individuals born between 1959 and 2002 were included in the study. CP was classified according to Hagberg and the Surveillance of Cerebral Palsy in Europe (SCPE). Motor and accompanying impairments were documented. Causes of death were derived from the National Board of Health and Welfare, and population data were obtained from Statistics Sweden. Log-rank tests with Kaplan-Meier plots were used for statistical analyses. Of the 1856 individuals (1033 males, 823 females) with CP included in the study, 180 (9.6%) had died by 31 December 2009. Tetraplegia, dyskinetic CP, severe cognitive impairment, and epilepsy were associated with decreased survival rates. At Gross Motor Function Classification System (GMFCS) level V, survival rates among individuals with spastic CP were lower than among those with dyskinetic CP. However, compared with the general population, there was an elevated death rate among individuals with CP for all age groups and CP types. At all ages, females with CP had a larger excess risk of death than males. Respiratory failure caused 53% of deaths. For individuals with hemiplegia, as in the general population, 20% of deaths were accidental. Survival rates are influenced by CP type but there is an elevated risk of death for individuals with any type of CP, compared with those without CP.

Objective:To describe the trends for and severity of dyskinetic cerebral palsy in a European collaborative study between cerebral palsy registers, the Surveillance of Cerebral Palsy in Europe (SCPE).Methods:The prevalence of...