Surveillance Of Cerebral Palsy In Europe Research Articles

Classification of events contributing to postneonatal cerebral palsy: Development, reliability, and recommendations for use.

This paper introduces the Surveillance of Cerebral Palsy in Europe (SCPE) classification of events contributing to postneonatally acquired cerebral palsy, presents its interrater reliability, and describes the cases identified in the SCPE database. The development of the classification, based on literature review and expert discussions, resulted in six main categories and 19 subcategories. The first chronological event designated as the primary event was mainly reported. Interrater reliability was assessed through online exercise providing 24 clinical vignettes representing single/complex pathways. Percent agreement and Gwet's AC1 index of reliability were estimated. Primary events were described using data of 221 children born between 2008 and 2012. Thirty-nine professionals (21 registries) participated in the reliability exercise. Substantial overall agreement was reached (0.75), with some contrast between complex (0.48, moderate agreement) and single events involved (0.89, almost perfect). The distribution of primary events showed that 32.1% were infections (category A), 23.1% head injuries (B), 15.4% related to surgery or medical interventions (C), 13.1% cerebrovascular accidents (D), 9.1% hypoxic brain damaging events of other origins (E), and 7.2% miscellaneous (F). This classification allows all the events involved to be recorded while consistently reporting the primary event, and may be used in different settings. A standardized classification enables the description of the events contributing to postneonatal cerebral palsy (CP). The first chronological event in complex pathway leading to CP is coded. Category choice and coding of the primary event identify preventable situations. The detailed 2-level classification is easy to use in various settings. Substantial overall interrater reliability shows that main categories can be consistently differentiated.

Public health indicators for cerebral palsy: A European collaborative study of the Surveillance of Cerebral Palsy in Europe network.

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.

Relationship among four functional classification systems and parent interpredicted intelligence level in children with different clinical types of cerebral palsy

ABSTRACT Objective To investigate the relationships between four functional classification systems in children with cerebral palsy (CP) and parent-interpredicted intelligence level, and the functional status in clinical types of CP. Methods Two hundred and twenty-five children with CP ages between 2 and 18 (mean age 6.5 ± 4.4) years included using the Surveillance of CP in Europe (SCPE) database in Turkey. Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Communication Function Classification System (CFCS) and Eating and Drinking Classification System (EDACS) levels were classified by clinical observation, and intelligence quotient (IQ) was determined by parent reports. Results Correlations were found between all functional levels; the strongest were between GMFCS-MACS (r = 0.784, p < .001), CFCS-EDACS (r = 0.772, p < .001). Strong correlations were found for the IQ-CFCS (r = 0.762, p < .001) and IQ-EDACS (r = 0.634, p < .001). Correlations were stronger in children with bilateral CP and IQ level <70. Conclusions Taken together, these four classification systems and reported IQ levels can adequately describe overall functioning for children with CP. Our results can guide clinicians in the rehabilitation of children with CP.

Assessment of Sleep-Related Problems in Children with Cerebral Palsy Using the SNAKE Sleep Questionnaire.

Cerebral palsy (CP) represents the most common motor impairment in childhood. The presence of sleep problems has not yet been investigated with an instrument specifically designed for this population. In this hospital-based, prospective study, N = 100 children (M = 7.9, range: 2–18 years) with CP were included. All patients underwent pediatric neurologists’ screening incorporating instruments (Data Collection Form; Gross Motor Functions Classification System, GMFCS; Bimanual Fine Motor Function, BFMF) recommended by the “Surveillance of Cerebral Palsy in Europe (SCPE)”. Parents completed the “Sleep Questionnaire for Children with Severe Psychomotor Impairment (SNAKE)”. Children’s sleep behavior was increasingly conspicuous, with greater gross motor (SNAKE scales: disturbances remaining asleep, daytime sleepiness) and fine motor (additionally SNAKE scale arousal and breathing problems) functional impairment. Overall, a proportion of children showed sleep behavior outside the SNAKE’s normal range. No relevant sleep differences were identified between different CP subtypes and comorbidities. Applying a population-specific questionnaire, children’s functional impairment seems to be more relevant to their sleep behavior than the CP subtype or CP comorbidities.

The Role of Neuroimaging and Genetic Analysis in the Diagnosis of Children With Cerebral Palsy.

Cerebral magnetic resonance imaging (MRI) is considered an important tool in the assessment of a child with cerebral palsy (CP), as it is abnormal in more than 80% of children with CP, disclosing the pathogenic pattern responsible for the neurological condition. MRI, therefore, is recommended as the first diagnostic step after medical history taking and neurological examination. With the advances in genetic diagnostics, the genetic contribution to CP is increasingly discussed, and the question arises about the role of genetic testing in the diagnosis of cerebral palsy. The paper gives an overview on genetic findings reported in CP, which are discussed with respect to the underlying brain pathology according to neuroimaging findings. Surveillance of Cerebral Palsy in Europe (SCPE) classifies neuroimaging findings in CP into five categories, which help to stratify decisions concerning genetic testing. Predominant white and gray matter injuries are by far predominant (accounting for around 50 and 20% of the findings). They are considered to be acquired. Here, predisposing genetic factors may play a role to increase vulnerability (and should especially be considered, when family history is positive and/or causative external factors are missing). In maldevelopments and normal findings (around 11% each), monogenic causes are more likely, and thus, genetic testing is clearly recommended. In the miscellaneous category, the precise nature of the MRI finding has to be considered as it could indicate a genetic origin.

Neuroimaging Patterns and Function in Cerebral Palsy-Application of an MRI Classification.

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.

FUNCTIONAL CLASSIFICATION OF CHILDREN WITH CEREBRAL PALSY IN KRAPINA-ZAGORJE COUNTY.

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.

Definition and diagnosis of cerebral palsy in genetic studies: a systematic review.

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.

The Origin of the Cerebral Palsies: Contribution of Population-Based Neuroimaging Data

Surveillance of cerebral palsy in Europe (SCPE) presents the first population-based results on neuroimaging findings in children with cerebral palsy (CP) using a magnetic resonance imaging classification system (MRICS). MRIs of children with CP born between 1999 and 2009 from 18 European countries were analyzed. MRICS identifies patterns of brain pathology according to timing during brain development which was analyzed with respect to CP subtypes and gestational age. MRIs or written reports from 3,818 children were available. The main clinical characteristics were similar to the 5,415 without such data. Most frequent was predominant white matter injury (49%), followed by predominant gray matter injury (21%). Maldevelopments were found in 11% of cases. Miscellaneous findings were present in 8.5% and normal findings in 10.6%. MRI patterns of children with unilateral spastic, bilateral spastic, and dyskinetic CP were mainly lesional (77, 71, and 59%, respectively), whereas children with ataxic CP had more maldevelopments, miscellaneous, and normal findings (25, 21, and 32%, respectively). In children born preterm, predominant white matter injury was most prevalent (80% in children born <32 weeks of gestation). Analysis of MRI in the European CP database identified CP as a mainly lesional condition on a large population basis, maldevelopments were relatively uncommon. An exception was ataxic CP. Children born preterm mostly presented with a lesion typical for their gestational age (GA) at birth. The decreasing prevalence of CP in this group suggests that progress in perinatal and neonatal medicine may lead to a reduction of these lesions.

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

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.

Prognostic value of cranial ultrasonography in comparison with magnetic resonance imaging in children with cerebral palsy: a population-based study.

SUMMARYThe aim of this population-based study was to evaluate the characteristics of cerebral palsy (CP) in relation to the predominant pattern of the Magnetic Resonance Imaging Classification System (MRICS) that was analogously applied to the neonatal/early infant cranial ultrasound (CUS). The study included children born during the 2004-2007 period from the Croatian part (C28 RCP-HR) of the Surveillance of Cerebral Palsy in Europe (SCPE) CP register. Motor functions, accompanying impairments and brain MRI were evaluated in 227 children, 185 of which also had CUS. Concerning CP types, 56% of children had bilateral spastic, 34% unilateral spastic, 9% dyskinetic and 1% ataxic CP type. Gross Motor Function Classification System (GMFCS) revealed that 62.05% had mild (GMFCS I-III) and 37.85% had severe motor impairment (GMFCS IV-V). CUS showed white matter injury in 60%, gray matter injury in 12%, maldevelopments in 8%, miscellaneous changes in 14%, while 6% were normal; MRI showed significant agreement (κ=0.675, p<0.001). Neuroimaging findings of maldevelopments and predominant gray matter injury were associated with more severe CP, but 7% of children with CP had normal MRI. As we found very good agreement between CUS and MRI findings, CUS is recommended in children at an increased risk of CP if MRI is not available.

Visual impairment in children with cerebral palsy: Croatian population-based study for birth years 2003-2008

AimTo evaluate visual impairment (VI) in children with cerebral palsy (CP).MethodsThis population-based study included 419 children from the Surveillance of Cerebral Palsy in Europe (SCPE) C28 RCP-HR – Register of Cerebral Palsy of Croatia born 2003-2008. Vision in children with CP (according to SCPE) was classified as normal or impaired, with the subcategory of severe VI. The proportion of children with VI was assessed in groups with different CP type/subtype, gross and fine motor function, and gestational age (GA).ResultsA total of 266 children had some degree of VI (266/400; 66.5%), 134 had normal vision, and data on VI were unknown for 19 children. Severe VI was present in 44 children (44/400; 11%). The proportion of children with VI and severe VI increased with the Gross Motor Function Classification System and Bimanual Fine Motor Function levels. Children with bilateral spastic CP had the highest frequency of severe VI (14.9%). The percentage of severe VI in children with bilateral spastic CP was 53.8% in the group born <28 weeks of GA, 13.3% in the group born 28-31 weeks of GA, 11.1% in the group born 32-36 weeks of GA, and 24.4% in the group born >36 weeks of GA (λ2 = 4.95; df = 6; P < 0.001).ConclusionChildren with CP have a high prevalence of VI and severe VI, which is increasing with the level of motor impairment. Severe VI is significantly more common in children with bilateral spastic CP, especially among extremely premature infants.

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

To examine the prevalence, clinical characteristics, and risk factors of cerebral palsy (CP) in children in Bangladesh. 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. 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. 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. 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.

MRI classification system (MRICS) for children with cerebral palsy: development, reliability, and recommendations.

To develop and evaluate a classification system for magnetic resonance imaging (MRI) findings of children with cerebral palsy (CP) that can be used in CP registers. The classification system was based on pathogenic patterns occurring in different periods of brain development. The MRI classification system (MRICS) consists of five main groups: maldevelopments, predominant white matter injury, predominant grey matter injury, miscellaneous, and normal findings. A detailed manual for the descriptions of these patterns was developed, including test cases (www.scpenetwork.eu/en/my-scpe/rtm/neuroimaging/cp-neuroimaging/). A literature review was performed and MRICS was compared with other classification systems. An exercise was carried out to check applicability and interrater reliability. Professionals working with children with CP or in CP registers were invited to participate in the exercise and chose to classify either 18 MRIs or MRI reports of children with CP. Classification systems in the literature were compatible with MRICS and harmonization possible. Interrater reliability was found to be good overall (k=0.69; 0.54-0.82) among the 41 participants and very good (k=0.81; 0.74-0.92) using the classification based on imaging reports. Surveillance of Cerebral Palsy in Europe (SCPE) proposes the MRICS as a reliable tool. Together with its manual it is simple to apply for CP registers.

Cerebral palsy in preterm infants.

Cerebral palsy (CP) is one of the leading causes of neurological impairment in childhood. Preterm birth is a significant risk factor in the occurrence of CP. Clinical outcomes may include impairment of gross motor function and intellectual abilities, visual impairment and epilepsy. The aim of this study was to examine the relationships among gestational age, type of CP, functional ability and associated conditions. The sample size was 206 children with CP. The data were obtained from medical records and included gestational age at birth, clinical characteristics of CP and associated conditions. Clinical CP type was determined according to Surveillance of Cerebral Palsy in Europe (SCPE) and topographically. Gross motor function abilities were evaluated according to the Gross Motor Function Classification System (GMFCS). More than half of the children with CP were born prematurely (54.4%). Statistically significant difference was noted with respect to the distribution of various clinical types of CP in relation to gestational age (p < 0.001). In the group with spastic bilateral CP type, there is a greater proportion of children born preterm. Statistically significant difference was noted in the functional classification based on GMFCS in terms of gestational age (p = 0.049), children born at earlier gestational age are classified at a higher GMFCS level of functional limitation. The greatest percentage of children (70.0%) affected by two or more associated conditions was found in the group that had extremely preterm birth, and that number declined with increasing maturity at birth. Epilepsy was more prevalent in children born at greater gestational age, and this difference in distribution was statistically significant (p = 0.032). The application of antenatal and postnatal protection of preterm children should be a significant component of the CP prevention strategy.

Association between gross motor function and postural control in sitting in children with Cerebral Palsy: a correlational study in Spain.

BackgroundCerebral palsy (CP) is one of the causes of physical disability in children. Sitting abilities can be described using the Level of Sitting Scale (LSS) and the Gross Motor Function Classification System (GMFCS). There is growing interest in the sitting posture of children with CP owing to a stable sitting position allows for the development of eye-hand coordination, functions of the upper extremities and functional skills. Besides, in recent years researchers have tried to develop a new terminology to classify the CP as performed by the Surveillance of Cerebral Palsy in Europe (SCPE), in order to improve the monitoring of the frequency of the PC, providing a framework for research and service planning. The aim of this study was to analyse the relationship between GMFCS and LSS. The second purpose was to describe how the SCPE relates to sitting abilities with the GMFCS and LSS.MethodsThe study involved 139 children with CP (range 3–18 years) from 24 educational centres. Age, gender, CP classification according to SCPE, GMFCS and LSS levels were recorded by an experienced physiotherapist.ResultsA significant inverse relationship between GMFCS and LSS score levels was found (rs = −0.86, p = 0.00). 45.3 % of the children capable of leaning in any direction and of re-erecting the trunk (level VIII on the LSS) could walk without limitation (level I on the GMFCS). There were differences in the distribution of the GMFCS (χ2(4):50.78) and LSS (χ2(7): 37.15) levels and CP according to the distribution of the spasticity (p <0.01).ConclusionsThere was a negative correlation between both scales and a relation between sitting ability and the capacity to walk with or without technical devices. GMFCS and the LSS are useful tools for describing the functional abilities and limitations of children with CP, specially sitting and mobility. Classification based on the distribution of spasticity and the gross motor function provides clinical information on the prognosis and development of children with CP.

Survival with cerebral palsy over five decades in western Sweden.

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.

Bimanual Fine Motor Function (BFMF) Classification in Children with Cerebral Palsy: Aspects of Construct and Content Validity

ABSTRACTThe Bimanual Fine Motor Function (BFMF) is currently the principal classification of hand function recorded by the Surveillance of Cerebral Palsy in Europe (SCPE) register. The BFMF is used in a number of epidemiological studies, but has not yet been validated. Aims: To examine aspects of construct and content validity of the BFMF. Methods and Results: Construct validity of the BFMF was assessed by comparison with the Manual Ability Classification System (MACS) using register-based data from 539 children born 1999–2003 (304 boys; 4–12 years). The high correlation with the MACS (Spearman's rho = 0.89, CI: 0.86–0.91, p<.001) supports construct validity of the BFMF. The content of the BFMF was appraised through literature review, and by using the ICF-CY as a framework to compare the BFMF and MACS. The items hold, grasp and manipulate were found to be relevant to describe increasingly advanced fine motor abilities in children with CP, but the description of the BFMF does not state whether it is a classification of fine motor capacity or performance. Conclusion: Our results suggest that the BFMF may provide complementary information to the MACS regarding fine motor function and actual use of the hands, particularly if used as a classification of fine motor capacity.

PS-247 Association Of Motor Function With Basal Ganglia And Thalamus Volumes In Dyskinetic Cerebral Palsy

<h3>Background and aims</h3> Dyskinetic Cerebral Palsy (CP) is mainly characterised by basal ganglia and thalamus injury as well as worse motor functioning compared to other CP types. Associations of higher levels in Gross Motor Function Classification System (GMFCS) and in Bimanual Fine Motor Function (BFMF) with basal ganglia and thalamus lesions have only been described qualitatively. This study aims to analyse the quantitative relationship of basal ganglia and thalamus volumes with motor status measured by means of the three Surveillance of Cerebral Palsy in Europe (SCPE) recommended scales: GMFCS, BFMF and Manual Ability Classification System (MACS). <h3>Methods</h3> Gross and fine motor status of 15 dyskinetic CP participants with signs of perinatal asphyxia (age range: 12–34) were assessed. 3T-MRI was obtained and Free Surfer software was used to estimate basal ganglia and thalamus volumes. Partial correlations controlling for age were performed. <h3>Results</h3> Caudate (r = -.70; p &lt; 0.01), putamen (r = -.59; p &lt; 0.05) and pallidum (r = -.60; p &lt; 0.05) volumes correlated with GMFCS. Moreover, caudate (r = -.68; p &lt; 0.01), putamen (r = -.63; p &lt; 0.05) and thalamus (r = -.55; p &lt; 0.05) volumes correlated with MACS. BFMF was not related with basal ganglia and thalamus measures. <h3>Conclusions</h3> Greater gross and fine motor impairment severity is associated with lower basal ganglia and thalamus volumes, which is consistent with qualitative results of previous studies. This is the first evidence of a quantitative relationship between two widely used scales of motor functioning and deep brain grey matter in dyskinetic CP. GMFCS and MACS, but maybe not BFMF scale, are sensitive to basal ganglia and thalamus injury in this CP type.

Clinical features of cerebral palsy in children with symptomatic congenital cytomegalovirus infection

BackgroundHuman cytomegalovirus is the most common cause of vertically transmitted viral infection, affecting around 1% of liveborns. Infection is symptomatic in nearly 10% of infected children who are at higher risk of development of severe neurological disorders, including cerebral palsy. AimsTo study the clinical profile of children with cerebral palsy caused by symptomatic congenital cytomegalovirus infection in a multicenter study involving six countries from the Surveillance of Cerebral Palsy in Europe (SCPE) Network. MethodsData on 35 children (13 males, 22 females; mean age at last assessment 12y 6mo, age range 14y 6mo, min 4y, max 18y 6mo) on pre/peri/neonatal history and last clinical assessment were collected. Classification of cerebral palsy and associated impairments was performed according to SCPE criteria. ResultsThe majority of children had bilateral spastic cerebral palsy, 85.7%, with a confidence interval (CI) [69.7–95.2], and 71.4% [CI 53.7–85.4] were unable to walk (GMFCS levels IV–V) while fine motor function was severely affected in 62.8% [CI 44.9–78.5] (BFMF levels IV and V). Most of the children with severe CP had severe associated impairments. 11.4% of children had severe visual and 42.8% severe hearing impairment, 77.1% [CI 59.9–89.6] suffered from epilepsy, also 77.1% had severe intellectual impairment, and speech was undeveloped in 71.4%. Female:male ratio was 1.69:1 and 80% of children were term born. ConclusionsCerebral palsy following symptomatic congenital cytomegalovirus infection seems to be in most cases a severe condition and associated impairments are overrepresented.