The Stability of the Gross Motor Function Classification System in Children with Cerebral Palsy Living in Stockholm and Factors Associated with Change.

AIM: To determine the degree of stability in the Gross Motor Function Classification System (GMFCS) for children with cerebral pares (CP) and to analyze factors associated with changes of the over time, in Stockholm between the year 2000 and 2019. METHOD: A register study on 768 children with at least two GMFCS ratings, linear regression analysis was used to study factors associated to a change in GMFCS level. RESULTS: 72% of the children kept the same GMFCS level. A change in GMFCS level was most common for children in GMFCS level II (68%). The first change in GMFCS level happened most commonly between the ages 2 and 4. Initial GMFCS level (Beta 0.127; p < 0.001) and one or more intensive training periods with a physiotherapist (Beta 0.097; p = 0.018) were associated with a change in GMFCS level. INTERPRETATION: Most children with CP remain stable in their GMFCS level, but for those classified in level II, a change occurs for almost seven out of ten. Classifications made before the child turns four are less certain than those made later. Further studies are needed to clarify if occurrence of intensive training is the cause or result of the change in GMFCS level.

Development and Psychometric Properties of the Arabic Parent Nutritional Assessment Scale (A-PNAS) for Children with Developmental Disabilities.

Aims: To describe the development of the Arabic-Parent Nutritional Assessment Scale (A-PNAS), and to examine construct and known-group discriminant validity, internal consistency, and test-retest reliability of the A-PNAS.Methods: A cross-sectional design was used. Participants were 130-children with CP (mean age = 4.26 ± 3.29 years) who were registered in the national CP registry of Jordan with a matching group of 130-children with typical development (mean age = 4.65 ± 3.54 years). Parents completed the developed A-PNAS through a structured phone interview. Parents of children with CP confirmed their child's level of gross motor function classification system.Results: Exploratory factor analysis identified three subscales in the A-PNAS: Food Intake Problems, Health Problems, and Behavioral Problems which explained 31.6% of the variance in nutritional problems of children with CP. Cronbach's alpha indicated acceptable internal consistency for Food Intake (α = 0.61) and Health Problems (α = 0.67)subscales. Parents of children with CP reported that their children had more food intake, health, and behavioral problems compared to children with typical development (p<.001). Test-retest reliability was excellent for the subscales of the A-PNAS (ICCs = 0.96, 0.98, 0.96).Conclusions: The findings provide support for the face validity, construct validity, internal consistency, Known-Groups discriminant validity, and test-retest reliability of the A-PNAS.

Inequity in physiotherapeutic interventions for children with Cerebral Palsy in Sweden-A national registry study.

AIM: The aim of this study was to investigate the distribution of physiotherapeutic interventions for children with Cerebral Palsy in Sweden from an equity perspective, considering sex, country of birth and geographical region. METHOD: This national cross-sectional registry study includes children with Cerebral Palsy aged 0-18 years who participated in 2015 in the Swedish national quality registry, the Cerebral Palsy follow-up programme, CPUP. Comparisons and associations between physiotherapeutic interventions and sex, country of birth and geographical regions were conducted using chi-square and logistic regression analysis, controlling for cognitive level, level of motor function, age group and dominating symptom. RESULTS: Of the 2855 participants, 2201 (79%) had received physiotherapy. Children born in Sweden had 1.60 times higher odds (95% CI 1.10-2.33) of receiving physiotherapy compared with children born in foreign countries. Distribution of physiotherapeutic interventions differed significantly between geographical regions. No associations between sex and physiotherapeutic interventions were observed. CONCLUSION: The results of this study indicate inequity in care in Sweden towards children with Cerebral Palsy born in other counties. Further, physiotherapeutic interventions were not equally distributed in different geographical regions of Sweden. Knowledge of inequity is crucial in order to address the problem.

Development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan).

AIMS: This study aims to describe the development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan) and to provide a baseline child and parent demographic information, birth history of the child participants, and distribution of the participants based on topographical distribution of cerebral palsy (CP) and functional classification systems. METHODS: The CPUP-Jordan was developed using a similar framework of a follow-up surveillance programme for persons with CP in Sweden (CPUP). Standard assessment forms were utilized to collect data related to child and family demographics, child birth history, and functional classifications and physiotherapy and occupational therapy assessments and interventions. Research assistants were trained to conduct the assessments. A secured web-based system was developed to store data and disseminate knowledge maintained in the registry. Children with CP were included in the registry if they have confirmed diagnosis of CP. The ascertainment age of inclusion and the minimum age of survival required are 4 years. RESULTS: One hundred sixty-seven children were registered between 2013 and 2015 (mean age is 3.6 ± 3.0 years). Forty-two percent were born premature, and 48% were less than the normal birthweight. Perinatal causes were reported for 54% of the participants. The most common type of CP based on tone disturbance was spastic type, and the most common topographical distributions of motor dysfunction were quadriplegia followed by diplegia. Fifty-six percent of the participants had severe limitation in ambulation; 48% had restricted manual abilities, and 47% had limited communication abilities even with familiar family members and partners. CONCLUSIONS: The development of CPUP-Jordan registry for children with CP proved to be both feasible and informative. The registry baseline descriptive data were similar to those reported in previous research in Jordan supporting validity of the data. The implementation of CPUP-Jordan at national level is expected to have a positive impact on children with CP, clinicians, policymakers, and researchers.

Treatment with Botulinum toxin A in a total population of children with cerebral palsy - a retrospective cohort registry study.

BACKGROUND: Botulinum toxin A (BTX-A) has been used to reduce spasticity in children with cerebral palsy (CP) for decades. The purpose of this study was to analyze to what extent BTX-A treatment was used to treat spasticity in a total population of children with CP. We investigated 1) the use of BTX-A in relation to age, sex, and Gross Motor Function Classification System (GMFCS) level, 2) the most common muscle groups treated with BTX-A in relation to the same variables, and 3) changes in the proportions of children treated with BTX-A between two time points (2010 and 2015). METHODS: The study was based on data from CPUP, a combined Swedish follow-up program and national healthcare registry, comprising >95% of all children with CP in Sweden. The participants (N = 3028) were born in 2000 or later. Potential BTX-A treatment and treated muscle groups were included from all CPUP assessments recorded in the registry in 2014-2015. In Aim 3, BTX-A administration in 3-5 year-olds at two time points was assessed. Crosstabs and 95% confidence intervals (CIs) for binominal proportions were calculated and logistic regression was used to regress age, sex, and GMFCS level on BTX-A treatment. Muscle groups treated with BTX-A were assessed using crosstabs and 95% CIs. Proportional change in BTX-A treatment over a 5-year period was analyzed using chi-square. RESULTS: We included 3028 children (57% boys; median age 7 years) of whom 26% received BTX-A. Significantly more boys (28%) than girls (23%) received BTX-A (OR = 1.25, [95% CI 1.05-1.48]). Significant differences were found for age and GMFCS levels; 4-6 year-olds and those at GMFCS III-IV were more likely to receive BTX-A. BTX-A treatment in the gastrocnemius muscle was most common in the 4-6 year-olds and at GMFCS I-III, whereas treatment of the hamstring and adductor muscles was more common in older children and at GMFCS IV-V. No significant change in the proportion of BTX-A administered in 2010 and 2015 was demonstrated. CONCLUSIONS: BTX-A treatment differed based on age, sex, and GMFCS level. Proportion of BTX-A treatment in Sweden has remained stable during the past five years.

Limited associations between passive range of motion and gross motor function in ambulant/semi-ambulant children and adolescents with cerebral palsy: A cross-sectional study.

BACKGROUND AND AIM: Cerebral palsy (CP) is the most common childhood motor disability, and the Cerebral Palsy Follow-Up Program (CPUP) in Nordic countries uses a traffic light system for passive range of motion (ROM) assessment to aid interpretation and guide decisions regarding interventions. However, the arbitrary chosen ROM threshold values and their potential clinical impact are uncertain. We investigated whether lower extremity ROM values were positively associated with gross motor function and whether gross motor function scores differ between the CPUP ROM thresholds. METHODS: This was a cross-sectional analysis of CPUP data for 841 ambulatory children and adolescents with CP, at a mean (SD) age of 9 (3). Regression analyses were employed to explore the relationship between gross motor capacity and performance (using the Gross Motor Function Measure (GMFM-66) and the Functional Mobility Scale (FMS) 5/50/500 m, respectively) and lower extremity ROM, measured with a goniometer. ROM was assessed both as continuous and categorical variables. RESULTS: We found that two out of ten continuous ROM measures were positively associated with gross motor function. Limited differences in gross motor function between the ROM thresholds were seen for seven out of ten ROM measures. The CPUP traffic light thresholds primarily differentiated gross motor function between the red and green categories, predominantly for the subgroup of participants with bilateral spastic CP. CONCLUSION: Limited associations between passive ROM and gross motor function in children and adolescents with CP were observed, indicating that there is more to consider than ROM when identifying whether interventions are needed.

The CPUP Hip Score predicts displacement of the hip in children with cerebral palsy.

AIMS: The Uppföljningsprogram för cerebral pares (CPUP) Hip Score distinguishes between children with cerebral palsy (CP) at different levels of risk for displacement of the hip. The score was constructed using data from Swedish children with CP, but has not been confirmed in any other population. The aim of this study was to determine the calibration and discriminatory accuracy of this score in children with CP in Scotland. METHODS: This was a total population-based study of children registered with the Cerebral Palsy Integrated Pathway Scotland. Displacement of the hip was defined as a migration percentage (MP) of > 40%. Inclusion criteria were children in Gross Motor Function Classification System (GMFCS) levels III to V. The calibration slope was estimated and Kaplan-Meier curves produced for five strata of CPUP scores to compare the observed with the predicted risk of displacement of the hip at five years. For discriminatory accuracy, the time-dependent area under the receiver operating characteristic curve (AUC) was estimated. In order to analyze differences in the performance of the score between cohorts, score weights, and subsequently the AUC, were re-estimated using the variables of the original score: the child's age at the first examination, GMFCS level, head shaft angle, and MP of the worst hip in a logistic regression with imputation of outcomes for those with incomplete follow-up. RESULTS: The discriminatory accuracy of the score in the new population of 367 children was high (AUC 0.78 (95% confidence interval (CI) 0.71 to 0.86)). The calibration of the score was insufficient (slope 0.48 (95% CI 0.31 to 0.65)), and the absolute risks of displacement of the hip in this population were overestimated. The AUC increased with re-estimated weights (0.85 (95% CI 0.79 to 0.91)). CONCLUSION: The CPUP Hip Score had a high ability to discriminate between children at different levels of risk for displacement of the hip. The score overestimated the absolute risks of displacement in this population, which may have resulted from differences in the way children were initially registered in the two programmes. The results are promising, but the score weights may need re-estimation before its clinical application in Scotland. Cite this article: Bone Joint J 2022;104-B(5):640-644.

Inter- and intra-rater reliability of the head-shaft angle in children with cerebral palsy.

PURPOSE: Children with cerebral palsy (CP) are at increased risk for hip dislocation. This can be prevented in most cases using surveillance programmes that include radiographic examinations. Known risk factors for hip dislocation include young age, high Gross Motor Function Classification System (GMFCS) level and high migration percentage (MP). The head-shaft angle (HSA) has recently been described as an additional risk factor. The study aim was to determine inter- and intra-rater reliability of the HSA in a surveillance programme for children with CP. METHODS: We included hip radiographs from the CP surveillance programme CPUP in southern Sweden during the first half of 2016. Fifty radiographs were included from children at GMFCS levels II-V, with a mean age of 6.6 (SD 3.2) years. Three raters measured the HSA of one hip (left or right) at baseline and four weeks later; intraclass correlation coefficient (ICC) was used to estimate inter- and intra-rater reliability. RESULTS: Inter- and intra-rater reliability were excellent for the HSA, with ICC 0.92 (95% CI 0.87-0.96) and ICC 0.99 (95% CI 0.98-0.99), respectively. CONCLUSION: The HSA showed excellent inter- and intra-rater reliability for children with CP, providing further evidence for use of the HSA as an additional factor for identifying risk for further hip displacement or dislocation.

A combined surveillance program and quality register improves management of childhood disability.

PURPOSE: To describe a concept for prevention of secondary conditions in individuals with chronic neuromuscular disabilities by using two Swedish developed follow-up-programmes for cerebral palsy (CP; CPUP) and myelomeningocele (MMC; MMCUP) respectively as examples. METHOD: This paper describes and outlines the rationale, development and implementation of CPUP and MMCUP. RESULTS: Both programmes are multidisciplinary longitudinal follow-up programmes that simultaneously serve as national registries. The programmes are population-based and set in Swedish habilitation clinics. Most children (95%) born 2000 or later with CP are enrolled in CPUP and the recruitment of adults is underway. CPUP has also been implemented in Norway, Denmark, Iceland, Scotland and parts of Australia. In MMCUP, almost all children with MMC born 2007 or later participate and individuals of all ages are now invited. The registries provide epidemiological profiles associated with CP and MMC and platforms for population-based research and quality of care improvement. CONCLUSIONS: Through multidisciplinary follow-up and early detection of emerging complications individuals with CP or MMC can receive less complex and more effective interventions than if treatment is implemented at a later stage. Possibilities and challenges to design, implement and continuously run multidisciplinary secondary prevention follow-up programmes and quality registries for individuals with CP or MMC are described and discussed. Implications for rehabilitation Individuals with disabilities such as cerebral palsy or myelomeningocele are at risk of developing secondary conditions. Multidisciplinary population-based longitudinal follow-up programmes seem effective in preventing certain types of secondary conditions.

Physiotherapeutic interventions and physical activity for children in Northern Sweden with cerebral palsy: a register study from equity and gender perspectives.

BACKGROUND: Young people with disabilities, especially physical disabilities, report worse health than others. This may be because of the disability, lower levels of physical activity, and discrimination. For children with cerebral palsy, access to physiotherapy and physical activity is a crucial prerequisite for good health and function. To date, there is limited knowledge regarding potential gender bias and inequity in habilitation services. OBJECTIVES: To map how physiotherapeutic interventions (PTI), physical leisure activity, and physical education are allocated for children with cerebral palsy regarding sex, age, level of gross motor function, and county council affiliation. This was done from a gender and equity perspective. METHODS: A register study using data from the Cerebral Palsy follow-Up Program (CPUP). Data included 313 children ≤18 years with cerebral palsy from the five northern counties in Sweden during 2013. Motor impairment of the children was classified according to the expanded and revised Gross Motor Function Classification System (GMFCS). RESULTS: In three county councils, boys received more physiotherapy interventions and received them more frequently than girls did. Differences between county councils were seen for frequency and reasons for physiotherapy interventions (p < 0.001). The physiotherapist was involved more often with children who had lower motor function and with children who had low physical leisure activity. Children with lower motor function level participated in physical leisure activity less often than children with less motor impairment (p < 0.001). Boys participated more frequently in physical education than did girls (p = 0.028). CONCLUSION: Gender and county council affiliation affect the distribution of physiotherapy interventions for children with cerebral palsy, and there are associations between gender and physical activity. Thus, the intervention is not always determined by the needs of the child or the degree of impairment. A gender-bias is indicated. Further studies are needed to ensure fair interventions.

Prediction of hip displacement in children with cerebral palsy: development of the CPUP hip score.

Hip displacement, defined in this study as a migration percentage (MP) of more than 40%, is a common, debilitating complication of cerebral palsy (CP). In this prospective study we analysed the risk of developing hip displacement within five years of the first pelvic radiograph. All children with CP in southern and western Sweden are invited to register in the hip surveillance programme CPUP. Inclusion criteria for the two groups in this study were children from the CPUP database born between 1994 and 2009 with Gross Motor Function Classification System (GMFCS) III to V. Group 1 included children who developed hip displacement, group 2 included children who did not develop hip displacement over a minimum follow-up of five years. A total of 145 children were included with a mean age at their initial pelvic radiograph of 3.5 years (0.6 to 9.7). The odds ratio for hip displacement was calculated for GMFCS-level, age and initial MP and head-shaft angle. A risk score was constructed with these variables using multiple logistic regression analysis. The predictive ability of the risk score was evaluated using the area under the receiver operating characteristics curve (AUC). All variables had a significant effect on the risk of a MP > 40%. The discriminatory accuracy of the CPUP hip score is high (AUC = 0.87), indicating a high ability to differentiate between high- and low-risk individuals for hip displacement. The CPUP hip score may be useful in deciding on further follow-up and treatment in children with CP.