Feasibility and Effectiveness of an Individualized 12-Week "Uptime" Participation (U-PART) Intervention in Girls and Women With Rett Syndrome.

BACKGROUND: Girls and women with Rett Syndrome (RTT) have low levels of daily physical activity and high levels of sedentary time. Reducing sedentary time and enhancing "uptime" activities, such as standing and walking, could be an important focus for interventions to address long-term health and quality of life in RTT. OBJECTIVE: The aim of the study was to evaluate the feasibility and health-related effects of an individualized 12-week uptime participation (U-PART) intervention in girls and women with RTT. DESIGN: The study used a single-group pretest-posttest design with 4 assessments (2 baseline, postintervention, and follow-up). METHODS: A participation-based intervention employing a whole-day approach was used. During a 12-week intervention period, individualized programs focused on participation in enjoyable uptime activities in home, school/day center, and community settings. Feasibility was assessed with a study-specific questionnaire. Primary outcome measures were sedentary time and daily step count. Secondary outcomes were gross motor skills, walking capacity, quality of life, and goal attainment scaling. RESULTS: Fourteen girls and women who were 5 to 48 years old and had RTT participated. The U-PART intervention was perceived as feasible by caregivers. Similar scores were observed at baseline assessments in all outcomes. Positive effects with small to medium effect sizes (0.27-0.54) were seen in sedentary time (- 4%), daily step count (+ 689 steps/d), walking capacity (+ 18.8 m), quality of life (+ 2.75 points), and goal attainment scaling after the intervention. Positive effects were maintained in sedentary time (- 3.2%) and walking capacity (+ 12.1 m) at short-term follow-up. LIMITATIONS: This study was limited by the lack of a control group. However, participants acted as their own control, and the stable baseline period partially mitigated this issue. CONCLUSIONS: The U-PART intervention was found to be feasible and effective in the short term in girls and women with RTT.

Patterns of sedentary time and ambulatory physical activity in a Danish population of girls and women with Rett syndrome.

BACKGROUND: Rett syndrome (RTT) is a rare neurodevelopmental disorder leading to multiple disabilities and high dependency on caregivers. This study aimed to: (1) describe the patterns of sedentary time and daily steps and (2) identify the association of individual and environmental characteristics with sedentary time. METHODS: All Danish females with RTT older than 5 years of age and with a MECP2 mutation were invited to participate. The activPAL and StepWatch Activity Monitor (SAM) were worn by participants for at least four days. Sedentary time and step counts were plotted by time to examine daily activity patterns. Associations between sedentary time and individual and environmental covariates were assessed with linear regression models. RESULTS: The median (interquartile range) age of participants was 22.0 (14.3-36.5) years. On average 83.3% (standard deviation 13.9%) of waking hours were spent in sedentary behaviours (n = 48) and the median (interquartile range) daily step count was 5128 (2829-7704) (n = 28). Females older than 33.5 years, and those unable to walk independently were more sedentary. CONCLUSIONS: This study demonstrated high levels of sedentary time and low daily step counts in a Danish population of females with RTT. Advancing age and lower walking skills were associated with higher levels of sedentary time. Implications for Rehabilitation Sedentary lifestyles in individuals with disabilities have a negative impact on health and quality of life. High levels of sedentary time and low daily step counts were demonstrated in a Danish population of females with Rett syndrome. Advancing age and inability to walk independently were strongly associated with higher levels of sedentary time in females with Rett syndrome. Understanding patterns of sedentary behaviour and physical activity can aid health care professionals in developing health-promoting physical activity interventions.

Facilitators and Barriers of Participation in "Uptime" Activities in Girls and Women With Rett Syndrome: Perspectives From Parents and Professionals.

Rett syndrome (RTT) is a rare neurodevelopmental disorder usually affecting females. It is associated with intellectual and multiple disabilities leading to a high level of dependency in all aspects of daily living including participation in physical activities. This study explored facilitators and barriers to "uptime" (non-sedentary) activities in Danish girls and women with RTT as perceived by parents and professionals using focus groups. Through thematic analysis, one central theme emerged: a constant balance to do the best thing for the girl or woman. Within the central theme, five subthemes of facilitators and barriers were identified relating to the individual and the physical, organizational, social, and attitudinal environments. Environmental barriers can be reduced through policy and management-level changes in health promotion and strong advocacy of physical activity by health professionals. Targeting both facilitators and barriers of "uptime" activities enables the planning and implementing of health-promoting interventions in individuals with RTT.

Functional profiles of children with cerebral palsy in Jordan based on the association between gross motor function and manual ability.

BACKGROUND: Cerebral palsy (CP) is the most common cause of physical disability in childhood. A major challenge for delivering effective services for children with CP is the heterogeneity of the medical condition. Categorizing children into homogeneous groups based on functional profiles is expected to improve service planning. The aims of this study were to (1) to describe functional profiles of children with CP based on the Gross Motor Function Classification System-Expanded & Revised (GMFCS-E & R) and the Manual Ability Classification System (MACS); and (2) to examine associations and agreements between the GMFCS-E & R and the MACS for all participants then for subgroups based on subtypes of CP and chronological age of children. METHODS: A convenience sample of 124 children with CP (mean age 4.5, SD 2.9 years, 56% male) participated in the study. Children were classified into the GMFCS-E & R and the MACS levels by research assistants based on parents input. Research assistants determined the subtypes of CP. RESULTS: Thirty six percent of the participants were able to ambulate independently (GMFCS-E & R levels I-II) and 64% were able to handle objects independently (MACS levels I-II). The most common functional profile of children with CP in our study is the "manual abilities better than gross motor function". An overall strong correlation was found between the GMFCS-E & R and the MACS (rs = .73, p < .001), the correlations vary significantly based on subtypes of CP and chronological age of children. A very strong correlation was found in children with spastic quadriplegia (rs = .81, p < .001), moderate with spastic diplegia (rs = .64, p < .001), and weak with spastic hemiplegia (rs = .37, p < .001). CONCLUSIONS: The GMFCS- E & R and the MACS provide complementary but distinctive information related to mobility and manual abilities of children with CP. Subtypes of CP and chronological age differentiated functional profiles. Functional abilities of children with CP in Jordan have similar patterns to children with CP in other countries. Functional profiles can inform clinicians, researchers, and policy makers.

Assessments of pain in children and adolescents with cerebral palsy: a retrospective population-based registry study.

AIM: To explore pain screening in CPUP, a follow-up surveillance programme for people with cerebral palsy (CP), specifically to describe reported pain prevalence, localizations, patterns of distribution; to compare with studies using psychometrically sound assessment instruments; and to assess agreement between pain documented in CPUP and medical records. METHOD: Registry study of a population with CP, born 1993 to 2008, living in Skåne, Sweden in 2013. Descriptive data, cross-tabulations, and chi-square tests to characterize and compare the study groups. Kappa analysis to test the concordance between register and medical record reports on pain. RESULTS: Pain was reported by 185 out of 497 children (37%; females 40%, males 35%). Level V in both Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) was associated with highest prevalence of pain (50% and 54%), and level I with lowest prevalence of pain (30% and 32%). Pain was most frequent in dyskinetic CP (46%) and least frequent in unilateral spastic CP (33%). Feet and knees were the dominant localizations. Fair-moderate agreement (kappa 0.37, prevalence-adjusted bias-adjusted kappa [PABAK] 0.44) was found between documented pain in CPUP and medical records, although more seldom recognized in medical records. INTERPRETATION: The distribution of pain between CP subtypes, functional levels, sex, and age in CPUP is concordant with previous population-based studies, indicating the validity of the CPUP pain screening. Despite this, further clinical evaluation with extended pain assessments and pain management were largely neglected in children reporting chronic pain.

Parents' experiences of participation in physical activities for children with cerebral palsy - protecting and pushing towards independence.

PURPOSE: To explore how parents of children with cerebral palsy (CP) experience their child's participation in physical activities and to identify facilitators and barriers for being physically active and reducing sedentary behaviour. METHODS: Twenty-five parents of sixteen children, aged 8-11 years old with CP, with varying gross motor, cognitive and communicative functions and with different cultural backgrounds, participated in focus group or individual interviews. Content analysis was used for analysis. RESULTS: Five subcategories addressing children's participation in physical activity were found: "Belonging and taking space in the family", "Important persons facilitating and hindering", "Friends important but hard to get", "Good for the body but challenging" and "Availability and opting out possibilities". The subcategories built the main category "Protecting and pushing towards independence", expressing the challenges parents experienced when their child wanted to be physically active. CONCLUSIONS: Parents desire competent persons to be available for support in participation in physical activities. They want support in finding friends for their child to be physically active with. Family culture and attitudes affect their child's motivation for being physically active and should be taken into account when designing interventions for increased participation in physical activities and for reduced sedentary behaviour in children with disabilities. Implications for Rehabilitation Friends and competent adults facilitate participation in physical activities and reduce sedentary behaviour. Information on accessible and tailored physical activities is an important facilitator for participation in physical activities. Service planning and design of interventions may be facilitated by taking the individual family culture into account.

Stability of the Gross Motor Function Classification System in children and adolescents with cerebral palsy: a retrospective cohort registry study.

AIM: To investigate the stability and to determine factors that affect change in the Gross Motor Function Classification System (GMFCS) in a sample from the total population with cerebral palsy (CP) in two regions of Sweden. METHOD: Retrospective cohort registry study based on the follow-up programme for CP. Children with CP and a minimum of two GMFCS ratings were included. Subtype, sex, ages at GMFCS ratings, time between ratings, number of ratings, assessor change, and birth cohort were analysed in relation to initial GMFCS levels, with descriptive statistics and logistic regression models. RESULTS: Ninety-three per cent (n=736) of children with CP born between 1990 and 2007 were included, resulting in 7922 assessments between 1995 and 2014. Fifty-six per cent of the children received the same GMFCS rating at all assessments, with a median of 11 individual GMFCS ratings (range 2-21) and a median of three different assessors (range 1-10). Changes were often transient; downward change (higher performance) was more likely in GMFCS levels II and III than in the other levels. The probability of upward change (lower performance) was lowest in unilateral spastic CP. INTERPRETATION: The results support the stability of the GMFCS shown previously and add new information on the properties of the classification.

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.

Participation in physical activities for children with cerebral palsy: feasibility and effectiveness of physical activity on prescription.

BACKGROUND: Children with cerebral palsy (CP) are less physically active and more sedentary than other children which implies risk factors for their physical and mental health. Physical activity on prescription (PAP) is an effective intervention to promote a lifestyle change towards increased physical activity in adults in general. Knowledge is lacking about the use of PAP in children with CP. Therefore, the aim of this study was to evaluate the feasibility of PAP for children with CP and its effectiveness on participation in physical activity and sedentary behaviour. METHODS: Eleven children with CP, aged 7-11 years, participated in PAP, consisting of a written agreement between each child, their parents and the physiotherapist and based on Motivational Interviewing (MI), Canadian Occupational Performance Measure (COPM) and Goal Attainment Scaling (GAS). Individual goals, gross motor function and physical activity were assessed at baseline, at 8 and/or 11 months using COPM, GAS, logbooks, Gross Motor Function Measure (GMFM-66), physical activity questionnaires, physical activity and heart rate monitors and time-use diaries. At 8 and 11 months the feasibility of the intervention and costs and time spent for the families and the physiotherapist were evaluated by questionnaires. RESULTS: The intervention was feasible according to the feasibility questionnaire. Each child participated in 1-3 self-selected physical activities during 3-6 months with support from the physiotherapist, and clinically meaningful increases from baseline of COPM and GAS scores were recorded. Being physically active at moderate-vigorous levels varied between less than 30 and more than 240 minutes/day, and the median for the whole group was 84 minutes/day at baseline and 106 minutes/day at 8 months. CONCLUSIONS: The intervention PAP seems to be feasible and effective for children with CP, involving both every day and organised physical activities to promote an active lifestyle through increased participation, motivation, and engagement in physical activities. Further research of PAP is needed, preferably in a long term randomised controlled trial and including health economic analysis to show costs and benefits. TRIAL REGISTRATION: ISRCTN76366356, retrospectively registered.

Building the repertoire of measures of walking in Rett syndrome.

BACKGROUND: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome. METHODS: Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4-60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n = 42) on two occasions approximately one week apart. RESULTS: There were negative correlations between clinical severity and 2MWT (r = -0.48) and FMS-RS (r = -0.60-0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r = 0.51, 0.43) and FMS-RS (r = 0.71-0.93, 0.74-0.94), respectively. Test-retest reliability for the 2MWT was good with high intraday and interday correlations (ICC = 0.86-0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test-retest reliability for all three distances on the FMS-RS (ICC = 0.94-0.99). CONCLUSIONS: Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test-retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research. Implications for Rehabilitation Walking is one of the commonest daily physical activities in ambulant girls and women with RTT. Comprehensive knowledge about the walking abilities in this population is limited. Evidence of validity and test-retest reliability have been demonstrated for the modified two-minute walk test (2MWT) and the Rett syndrome-specific functional mobility scale (FMS-RS). The 2MWT and FMS-RS offer detailed information of the capacity and performance of walking, respectively, in girls and women with RTT.

Functional performance in self-care and mobility after selective dorsal rhizotomy: a 10-year practice-based follow-up study.

AIM: To explore changes in performance in daily activities (self-care and mobility) 10 years after selective dorsal rhizotomy (SDR). METHOD: Twenty-four children with bilateral spastic cerebral palsy were followed; the median age at SDR was 4 years 1 month (range 2y 5mo-6y 4mo) and at 10-year follow-up was 14 years 6 months (range 12y 3mo-16y 9mo). The preoperative Gross Motor Function Classification System (GMFCS) levels were: I (n=1), II (n=7), III (n=4), IV (n=11), and V (n=1). The Pediatric Evaluation of Disability Inventory (PEDI) was used to assess performance in functional skills, caregiver assistance, and frequency of modifications and adaptive equipment (MAE) in self-care and mobility domains. Changes were analysed in relation to preoperative GMFCS levels, PEDI scores, and age at operation. RESULTS: All scores improved significantly (p<0.01) during the first 5 years in patients assigned to GMFCS levels I-III and IV-V. Between 5 years and 10 years, changes were seen in patients grouped in GMFCS levels I-III in the functional skills, mobility (p=0.04), caregiver assistance self-care (p=0.03), and caregiver assistance mobility (p=0.03) domains. Those grouped in GMFCS levels IV-V showed small changes between 5 years and 10 years after surgery. Changes were dependent on the preoperative GMFCS levels in all domains; caregiver assistance, self-care and mobility changes were dependent on preoperative values. The use of MAE increased in participants in GMFCS levels IV-V. INTERPRETATION: Children who underwent SDR and physiotherapy improved in functional performance in self-care and mobility and were more independent 10 years postoperatively.

"It's fun, but " Children with cerebral palsy and their experiences of participation in physical activities.

PURPOSE: To explore the experiences of children with cerebral palsy (CP) regarding participation in physical activities, and to describe facilitators and barriers. METHODS: Sixteen children with CP 8-11 years old who varied in gross motor, cognitive and communicative function participated in either an individual interview or a focus group. RESULTS: Two categories and 10 sub-categories emerged from the content analysis. The category "Being physically active, because …" describes facilitators for being physically active divided into the sub-categories "Enjoying the feeling", "Being capable", "Feeling of togetherness", "Being aware it is good for me", and "Using available opportunities". The second category "Being physically active, but …" describes barriers to being physically active, divided into the sub-categories "Getting tired and experiencing pain", "Something being wrong with my body", "Being dependent on others", "Not being good enough" and "Missing available opportunities". CONCLUSIONS: Asking children with CP about the physical activities they enjoy, and giving them the opportunity of trying self-selected activities with the right support is important for facilitating an increased participation in physical activities. Having fun with family and friends when being physically active, and enjoying the sensation of speed should be taken into consideration when designing interventions. When supporting children to become and remain physically active, attention should be paid to pain, fatigue and the accessibility of activities and locations. Implications for Rehabilitation Children want to be physically active together with friends or others. Children want to have fun and enjoy the sensation of speed when being physically active. Self-selected physical activities and the opportunity of trying new activities with the right support is essential for facilitating an increased participation in physical activities. Service planning and design may be facilitated by asking children about the physical activities they enjoy.

Fractures in children with cerebral palsy: a total population study.

AIM: To analyse factors associated with fractures in children with cerebral palsy (CP) in different levels of Gross Motor Function Classification System (GMFCS). METHOD: This was an epidemiological retrospective study of a total population of 536 children (214 females, 322 males) with CP born between 1990 and 2005. CP type was unilateral spastic (n=159), bilateral spastic (n=225), ataxic (n=60), dyskinetic (n=80), and mixed type (n=12); 384 children were in Gross Motor Function Classification Scale (GMFCS) levels I-III and 152 children were in GMFCS levels IV-V. Data were collected for a 9-year period on sex, CP-type, GMFCS level, gastrostomy, height, weight, the use of a standing device, antiepileptic drug (AED) therapy, and fractures. RESULTS: The risk of fracture in the total population of children with CP was similar to that for typically developing children. The risk for fractures of those in GMFCS levels I-III was not significantly associated with any of the studied risk factors. The risk of fractures for those in GMFCS levels IV-V on AED therapy was a twofold increase (p=0.004). The risk for fractures without trauma in children with stunted growth (height for age <-3 SD) and those who did not use standing devices was significantly increased: adjusted incidence rate ratio (AIRR) 4.16 (p=0.011) and 3.66 (p=0.010) respectively. Results regarding gastrostomy feeding for those in GMFCS levels IV-V were conflicting: a gastrostomy was associated with a reduced risk of fractures with trauma, but with increased risk of fractures without trauma (AIRR 0.10, p=0.003 and 4.36, p=0.012) respectively. INTERPRETATION: Children in GMFCS levels I-III had a similar incidence and pattern for fractures as normally developing children. Those in GMFCS levels IV-V had stunted growth, often a sign of longstanding undernourishment, and were associated with an increased risk of fractures. Children using standing devices had a fourfold reduction of fractures without trauma. Regular loading exercises and early adequate nutritional intake could prevent fractures in severe CP.

Physical activity in a total population of children and adolescents with cerebral palsy.

The aims of this study were to describe the participation in physical activity of children with cerebral palsy (CP) at school and during leisure time and to identify characteristics associated with physical activity. The frequency of receiving physiotherapeutic interventions were described as a variable of interest. A total population of 364 children with verified CP aged 7-17 years living in the Skåne region in Sweden was studied using cross-sectional data from the CP follow-up programme (CPUP). Proportional odds ratios showed the most severe gross motor limitations Gross Motor Function Classification System Expanded and Revised (GMFCS-E&R) to be a characteristic for low participation in physical education at school (PE) and GMFCS-E&R level III to be a characteristic for low participation in regular physical leisure activity. The age group of 7-11 years and obesity were characteristics associated with high participation in PE, whereas thinness was associated with low participation in regular physical leisure time activities. The highest proportion of children receiving physiotherapeutic interventions was found in GMFCS-E&R level III, while mental retardation, especially if moderate or severe, proved to be an independent characteristic associated with low frequency of physiotherapeutic interventions. Gender and epilepsy did not influence the odds for participation in physical activities. Special considerations are needed when planning interventions for increased physical activity in children with CP, as the individual prerequisites differ, even among children with the same gross motor function level according to the GMFCS-E&R.

Motor function after selective dorsal rhizotomy: a 10-year practice-based follow-up study.

AIM: The aim of this study was to explore changes in motor function up to 10 years after selective dorsal rhizotomy (SDR). METHOD: The participants comprised 29 children (20 males, nine females) with bilateral spastic diplegia who were consecutively operated on at a median age of 4 years and 3 months and followed until a median age of 15 years. SDR was combined with physiotherapy and regular follow-up visits. The distribution of preoperative Gross Motor Function Classification System (GMFCS) levels was as follows: I, n=1; II, n=7; III, n=8; IV, n=12; and V, n=1. Muscle tone in hip flexors, hip adductors, knee flexors, and plantar flexors was assessed with the modified Ashworth scale, passive range of motion in hip abduction, popliteal angle, maximum knee extension, dorsiflexion of the foot was measured with a goniometer, and gross motor function was assessed using the Gross Motor Function Measure (GMFM-66). The results were compared with preoperative values, taking into account age at the time of SDR. RESULTS: After 10 years, muscle tone in hip flexors, hip adductors, knee flexors and plantar flexors was normalized in 19, 24, 13 and 23 participants respectively; mean change in passive range of motion ranged from -2.0° to 8.6°, and the mean increase in GMFM-66 was 10.6. Changes in GMFM-66 were associated with preoperative GMFCS level and GMFM-66 scores. INTERPRETATION: Children who underwent SDR and physiotherapy and were regularly followed up by an experienced team showed improved gross motor function for up to 10 years postoperatively.

Survival at 19 years of age in a total population of children and young people with cerebral palsy.

AIM: The aims were to investigate survival of children with cerebral palsy (CP) and to search for modifiable factors that influence survival in CP. METHOD: The total population of children with CP in southern Sweden born between 1990 and 2005, and followed from 1994 to 2010 comprised 718 children. The study included 708 of these children (297 females, 411 males) participating in a secondary prevention programme. CP subtype, Gross Motor Function Classification System (GMFCS) levels, and comorbidities were described. Kaplan-Meier survival curves were plotted. The following factors were investigated using Cox regression analysis: GMFCS level (co-varies with overall health), size of health care catchment area, gastrostomy feeding, and sex. RESULTS: The estimated survival at 19 years of age was 60% in children with the most severe gross motor limitations (GMFCS level V). Death occurred throughout childhood. All children at GMFCS level I or II, and 96% of the whole CP population, survived. The mortality risk in childhood CP was three times higher in catchment areas that covered small populations than in areas with a large population. Gastrostomy feeding was associated with a ninefold increased risk of dying, regardless of GMFCS level and catchment area. INTERPRETATION: Fragile children with CP, as indicated by GMFCS level V and gastrostomy feeding, had the lowest chance of surviving childhood. Health care catchment area seemed to influence survival rate.

Growth in children with cerebral palsy during five years after selective dorsal rhizotomy: a practice-based study.

BACKGROUND: Overweight is reported as a side effect of SDR. The aims were to study the development of weight, height and body mass index (BMI) during five years after SDR. METHODS: This prospective, longitudinal and practice-based study included all 56 children with CP spastic diplegia undergoing SDR from the start in March 1993 to April 2003 in our hospital. The preoperative Gross Motor Function Classification System (GMFCS) levels were I-II in 17, III in 15, IV-V in 24 children. Median age at SDR was 4.3 years (range 2.4-7.4 years). Weight and height/recumbent length were measured. Swedish growth charts for typically developing children generated weight, height and BMI z-scores for age and gender. RESULTS: The preoperative median z-scores were for height -1.92 and for body mass index (BMI) -0.22. Five years later, the median BMI z-score was increased by + 0.57 (p < 0.05). The occurrence of thinness (BMI < -2 SD) was decreased (n.s.) and obesity (BMI > + 2 SD) increased (p < 0.05). Baseline BMI and age at the start of follow-up influenced the BMI change during the five years (p < 0.001 and p < 0.05 respectively).The individual growth was highly variable, but a tendency towards increasing stunting with age was seen in severe gross motor dysfunction (GMFCS levels IV-V) and the opposite, a slight catch-up of height in children with walking ability (GMFCS levels I-III). CONCLUSIONS: These are the first available subtype- and GMFCS-specific longitudinal growth data for children with CP spastic diplegia. Their growth potential according to these data should be regarded as a minimum, as some children were undernourished. It is unknown whether the spasticity reduction through SDR increased the weight gain velocity, or if the relative weight increase was part of the general "obesity epidemic".For some children the weight increase was highly desirable. In others, it resulted in overweight and obesity with risk of negative health effects. Weight and height should be monitored to enable early prevention of weight aberrations also causing problems with mobility, activity and participation.

Development of lower limb range of motion from early childhood to adolescence in cerebral palsy: a population-based study.

BACKGROUND: The decreasing range of joint motion caused by insufficient muscle length is a common problem in children with cerebral palsy (CP), often worsening with age. In 1994 a CP register and health care programme for children with CP was initiated in southern Sweden. The aim of this study was to analyse the development of the passive range of motion (ROM) in the lower limbs during all the growth periods in relation to gross motor function and CP subtype in the total population of children with CP. METHODS: In total, 359 children with CP born during 1990-1999, living in the southernmost part of Sweden in the year during which they reached their third birthday and still living in the area in the year of their seventh birthday were analysed. The programme includes a continuous standardized follow-up with goniometric measurements of ROM in the lower limbs. The assessments are made by each child's local physiotherapist twice a year until 6 years of age, then once a year. In total, 5075 assessments from the CPUP database from 1994 to 1 January 2007 were analysed. RESULTS: The study showed a decreasing mean range of motion over the period 2-14 years of age in all joints or muscles measured. The development of ROM varied according to GMFCS level and CP subtype. CONCLUSION: We found a decreasing ROM in children with CP from 2-14 years of age. This information is important for both the treatment and follow-up planning of the individual child as well as for the planning of health care programmes for all children with CP.

Longitudinal construct validity of the GMFM-88 total score and goal total score and the GMFM-66 score in a 5-year follow-up study.

BACKGROUND: The Gross Motor Function Measure (GMFM) is the instrument most commonly used to measure gross motor function in children with cerebral palsy (CP). Different scoring options have been developed, and their measurement properties have been assessed. Limited information is available regarding longitudinal construct validity. OBJECTIVE: The objective of this research was to study the longitudinal construct validity of 3 scoring options: the 88-item GMFM (GMFM-88) total, the GMFM-88 goal total, and the 66-item GMFM (GMFM-66). DESIGN: A clinical measurement design was used in this study. METHODS: Forty-one children with CP diplegia who were undergoing selective dorsal rhizotomy (SDR) were monitored with the GMFM for 5 years. The mean age at SDR was 4.4 years (range=2.5-6.6). Two subgroups for gross motor function before surgery were created according to the Gross Motor Function Classification System (GMFCS): GMFCS levels I to III and GMFCS levels IV and V. This study included results obtained before SDR and at 6, 12, and 18 months and 3 and 5 years after SDR. The effect size (ES) and the standardized response mean (SRM) were calculated. RESULTS: At 6 months postoperatively, ES and SRM values were small (

Long-term outcomes five years after selective dorsal rhizotomy.

BACKGROUND: Selective dorsal rhizotomy (SDR) is a well accepted neurosurgical procedure performed for the relief of spasticity interfering with motor function in children with spastic cerebral palsy (CP). The goal is to improve function, but long-term outcome studies are rare. The aims of this study were to evaluate long-term functional outcomes, safety and side effects during five postoperative years in all children with diplegia undergoing SDR combined with physiotherapy. METHODS: This study group consisted of 35 children, consecutively operated, with spastic diplegia, of which 26 were Gross Motor Function Classification System (GMFCS) levels III-V. Mean age was 4.5 years (range 2.5-6.6). They were all assessed by the same multidisciplinary team at pre- and at 6, 12, 18 months, 3 and 5 years postoperatively. Clinical and demographic data, complications and number of rootlets cut were prospectively registered. Deep tendon reflexes and muscle tone were examined, the latter graded with the modified Ashworth scale. Passive range of motion (PROM) was measured with a goniometer. Motor function was classified according to the GMFCS and measured with the Gross Motor Function Measure (GMFM-88) and derived into GMFM-66. Parent's opinions about the children's performance of skills and activities and the amount of caregiver assistance were measured with Pediatric Evaluation Disability Inventory (PEDI). RESULTS: The mean proportion of rootlets cut in S2-L2 was 40%. Muscle tone was immediately reduced in adductors, hamstrings and dorsiflexors (p < 0.001) with no recurrence of spasticity over the 5 years. For GMFCS-subgroups I-II, III and IV-V significant improvements during the five years were seen in PROM for hip abduction, popliteal angle and ankle dorsiflexion (p = 0.001), capacity of gross motor function (GMFM) (p = 0.001), performance of functional skills and independence in self-care and mobility (PEDI) (p = 0.001). CONCLUSION: SDR is a safe and effective method for reducing spasticity permanently without major negative side effects. In combination with physiotherapy, in a group of carefully selected and systematically followed young children with spastic diplegia, it provides lasting functional benefits over a period of at least five years postoperatively.