Motor Development in Children with Cerebral Palsy in Sweden-A Population-Based Longitudinal Register Study.

The aim was to explore longitudinal motor development in children with cerebral palsy (CP) in Sweden with respect to the Gross Motor Function Classification System (GMFCS). In this national CP registry-based study, 2138 children aged 0.5-19 years participated (42% girls). The distribution with respect to GMFCS was I: 49%, II: 16%, III: 10%, IV: 14%, and V: 11%. In total, 5538 assessments (mean 2.7, min-max: 1-9) with the Gross Motor Function Measure-66 were included. Data were analysed using non-linear mixed-effects regression models, and the Stable Limit Model was selected to fit data. Five distinct curves of predicted gross motor development with respect to GMFCS levels were obtained. The achieved motor development was maintained over time. The estimated average GMFM-66 limit and the average age when 90% of the expected limits were reached were at GMFCS I: 88 at age 4.5; GMFCS II: 71 at age 4.2; GMFCS III: 54 at age 3.1; GMFCS IV: 38 at age 2.6, and at GMFCS V: 18 at age 0.9. In conclusion, this is the first national population-based study following motor development in CP. Five distinct curves reported in previous controlled research studies were confirmed. Our study adds knowledge about motor development captured in children's everyday context.

Long-term motor development after hypothermia-treated hypoxic-ischaemic encephalopathy.

AIMS: To describe longitudinal motor development in children treated with therapeutic- hypothermia (TH) due to neonatal hypoxic-ischaemic encephalopathy (HIE) and to explore motor functioning in early adolescence. MATERIAL AND METHODS: Children treated with TH due to HIE during 2007-2009, in Stockholm, participated in a prospective follow-up study. Motor development was assessed on four occasions, reported as percentiles and at mean ages. Alberta Infant Motor Scale was used at 0.35 years of age, Bayley Scales of Infant and Toddler Development-III at 2.1 years and Movement Assessment Battery for Children (MABC-2) at 7.3 and 11.1 years of age. MABC-2 Checklist was completed by parents at 7.3 and 11.1 years of age. General cognition was assessed using Wechsler Intelligence Scales for Children Fifth Edition (WISC-V). RESULTS: Thirty-one percent (14/45) of the children had a motor score ≤ 15th percentile, indicating risk of motor difficulties at 11.1 years of age, and simultaneously the scores from parents of 52% (23/44), indicating risk of motor difficulties in the everyday context. These children had significantly lower motor percentile at 2.1 years of age, but within the normal range. Longitudinal motor development displayed a weak association with WISC-V Full Scale IQ (rs0.38, p = 0.013). CONCLUSION: Among survivors of hypothermia-treated HIE free of moderate/severe cerebral palsy, a third had MABC-2 scores indicating risk of motor difficulties at 11.1 years of age. As motor difficulties became more apparent over time, we suggest that children treated with TH due to neonatal HIE should be followed into at least middle school age.

Dystonia during hand activity in children with spastic unilateral cerebral palsy, an observational study.

BACKGROUND: Spasticity and dyskinesia are motor signs that co-exist in cerebral palsy (CP). It is well accepted that, in spastic bilateral CP, dystonia can be present in addition to spasticity, and equally that spasticity is often present in individuals with dyskinetic CP. In unilateral spastic CP, dystonia of the upper extremity is only rarely identified or addressed. The aim of this study was to investigate if dystonia was present in the hand of children with unilateral spastic CP, and, if present, to what extent, and when it was first noticeable. METHOD: Ninety-seven children with unilateral spastic CP, born 1999-2014, with standardized digital films of hand function from Assisting Hand Assessments (AHA), were included. Films were reviewed, and presence or absence, of dystonia and choreoathetosis were scored by three experienced raters. RESULTS: Dystonia in the hand was present during activities in 70% (68/97) of the children at a mean age of 12 years (SD 4,4). In 74% (50/68) of these children, dystonia was present more than 50% of the evaluated time. For 63% (43/68) more than one digital recording at younger ages were available. Dystonia could first clearly be observed at a mean age of 3,8 years. Choreoathetosis was observed in 7% (5/68) of the children with dystonia. Children without dystonia had significantly higher (corresponding to better function) AHA units (median: 75, 25th - 75th: 45-82) in comparison to children with dystonia (median: 57, 25th - 75th: 52-63) (p = 0.01). CONCLUSION: Dystonia in the hand is common in unilateral CP and correlates to lower hand functioning.

Exploring pain interference and self-perceived health status in children with osteogenesis imperfecta - a cross-sectional study.

BACKGROUND: Chronic pain may affect and interfere in children's everyday life and can be present in children with Osteogenesis Imperfecta (OI). However, the knowledge is still sparse to what extent pain is present, how pain interfere in children's everyday life and affect their self-perceived health status. The purpose of the study was therefore to explore presence of chronic pain, pain interference in daily life, and self-perceived health status in children with OI. METHODS: Children with OI, aged 6-18 years, were recruited consecutively to this cross-sectional study. Participants answered a standardised interview including five pre-structured questions, and the Numeric Pain Rating Scale (NPRS), the Pain Interference Index, and a questionnaire concerning self-perceived health status the Patient Reported Outcomes Measurement Information System Pediatric-25 Profile v1.1 (PROMIS-25). RESULTS: Twenty-eight children (median: 11 years, IQR 6) with OI type I, III, or IV participated. Pain was present in 27 of 28 children and interfered in their everyday life regardless of OI-type, sex, and age. The median NPRS for average pain intensity was 4 (IQR 2), the median for pain frequency was 2-3 times/week, and the median frequency of school absence due to pain was 2-3 times per month. The most common pain locations were back and feet. Pain in the feet was more frequently reported in children with type I (p = 0.032), and pain in the hip was more often reported in children ≥13 years (p = 0.011). The children were asked what they thought to be the cause of pain and the most frequent response was "walking long distances". Self-perceived health status for mobility was lower than the general population, and lowest for children with type III (p = 0.016). Pain interference was associated with children's self-perceived health status (rs = 0.84, p < 0.001). CONCLUSION: Almost all children experienced pain, which interfered in children's everyday lives, affected participation in various activities and was associated with reduced self-perceived health status. If children avoid physical activities because of pain, it might cause a vicious circle of inactivity, which further decreases bone density and increase the risk of fractures. The results emphasize the importance to offer adequate pain reducing interventions.

A First Clinical Trial on Botulinum Toxin-A for Chronic Muscle-Related Pain in Cerebral Palsy.

Objective: To test if botulinum toxin-A (BoNT-A) is effective in reducing chronic muscle-related pain in adults with spastic cerebral palsy (CP), as compared to placebo. Design: A single-center, double-blind, parallel, randomized placebo-controlled trial. The design included an interim analysis to allow for confirmatory analysis, as well as pilot study outcomes. Setting: Tertiary university hospital. Participants: Adults with spastic CP and chronic pain associated with spastic muscle(s). Intervention: Treatment was one session of electromyographically guided intramuscular injections of either BoNT-A or placebo normosaline. Main Study Outcomes: The primary outcome was the proportion who achieved a reduction of pain intensity of two or more steps on the Numerical Rating Scale 6 weeks after treatment. Results: Fifty individuals were screened for eligibility, of whom 16 were included (10 female, 6 male, mean age = 32 years, SD = 13.3 years). The randomization yielded eight participants per treatment arm, and all completed the study as randomized. The study was stopped at the interim analysis due to a low probability, under a preset threshold, of a positive primary outcome. Four individuals were treatment responders in the BoNT-A group for the primary outcome compared to five responders in the placebo group (p = 1.000). Adverse events were mild to moderate. In exploratory analysis, the BoNT-A group had a trend of continuing reduction of pain at the last follow-up, after the primary endpoint. Conclusions: This study did not find evidence that BoNT-A was superior to placebo at the desired effect size (number needed to treat of 2.5) at 6 weeks after treatment. Trial registration:ClinicalTrials.gov: NCT02434549.

Prevalence, identification, and interference of pain in young children with cerebral palsy: a population-based study.

OBJECTIVE: To explore the presence of pain, how pain was addressed by physicians and parents, and how pain affected everyday life in young children with cerebral palsy (CP). METHODS: Children with CP, aged 5-10 years, participated in this cross-sectional study. Data were collected from medical records spanning a period of two years and by a standardized parental interview that included six structured questions and the Pain Interference Index. RESULTS: A total of 118 children, with a mean age of 7.4 years (SD 1.5), participated in the study. The parents of 81% of these children were interviewed. Pain was reported in 52% of the children, and pain was present at all severity levels. The prescription of analgesics was documented in 25% of these children's medical records. Fifty-nine percent of the children with pain received analgesics from their parents. Pain restricted the children's everyday lives particularly concerning sleep, school work and being with friends. CONCLUSIONS: Half of this group of young children with CP were reported to have pain. Pain restricted the children's everyday lives and seemed to be under-treated. If pain can be addressed early, the children's everyday lives are likely to be improved.Implications for rehabilitationThere is a need to early identify and treat pain in young children.Important to discuss pain with parents irrespectively of the child's age and severity level.Pain interference assessment gives valuable information.Early treatment of pain might improve children's everyday life.

Parents' Experiences of the First Year at Home with an Infant Born Extremely Preterm with and without Post-Discharge Intervention: Ambivalence, Loneliness, and Relationship Impact.

With increasing survival rates of children born extremely preterm (EPT), before gestational week 28, the post-discharge life of these families has gained significant research interest. Quantitative studies of parental experiences post-discharge have previously reported elevated levels depressive symptoms, posttraumatic stress-disorder and anxiety among the parents. The current investigation aims to qualitatively explore the situation for parents of children born EPT in Sweden during the first year at home. Semi-structured interviews were performed with 17 parents of 14 children born EPT; eight parents were from an early intervention group and nine parents from a group that received treatment as usual, with extended follow-up procedures. Three main themes were identified using a thematic analytic approach: child-related concerns, the inner state of the parent, and changed family dynamics. Parents in the intervention group also expressed themes related to the intervention, as a sense of security and knowledgeable interventionists. The results are discussed in relation to different concepts of health, parent-child interaction and attachment, and models of the recovery processes. In conclusion, parents describe the first year at home as a time of prolonged parental worries for the child as well as concerns regarding the parent's own emotional state.

Exploring sleep problems in young children with cerebral palsy - A population-based study.

OBJECTIVE: To describe and explore sleep problems in a population-based cohort of young children with cerebral palsy (CP) in Stockholm, Sweden. METHODS: All children with CP, aged 5-10 years, and living in the Northern Karolinska University Hospital's catchment area were invited to participate in a cross-sectional study. Medical records obtained in the previous two-year period were reviewed, and a pre-planned parental telephone interview that included five structured questions and the Insomnia Severity Index (ISI) was conducted. RESULTS: In total, 118 children, with a mean age of 7.4 years (SD 1.5), were included. Bilateral CP was present in 45%, unilateral in 37%, dyskinetic in 15%, and ataxic CP in 3%. Parents of 81% of the children participated in the interview. They reported sleep problems in 41% of their children, and in 80% of these children, night-time sleep was negatively affected by pain. Differences between the ISI total score in relation to CP subtypes (p < 0.025) and levels in GMFCS-E&R (p < 0.001) were detected, with increasing sleep problems for children with dyskinetic CP and children in GMFCS-E&R V. Sleep problems affected by pain were associated to the total score at ISI (rs = 0.83, p < 0.001, n = 95). CONCLUSION: The results identified that sleep problems were present in more than 40% of children with CP. Sleep problems were more frequently and extensively present in children with dyskinetic CP and children in GMFCS-E&R level V. Sleep problems were associated with the presence of pain and, in particular, in the most severely affected children.

Do Infants at Risk of Developing Cerebral Palsy or Other Neurodevelopmental Disorders Learn What They Practice?

Through secondary analyses of the Small Step. Randomized Control Trial, we tested the hypothesis that children at risk of developing cerebral palsy (CP) or other neurodevelopmental disorders would learn what they practice, i.e., that they would have a more rapid development within the specifically trained foci (hand use or mobility) of each time period compared to the development rate within the foci not trained at that time. Nineteen infants (6.3 (1.62) months corrected age) included in the Small Step program were assessed at six time points during the intervention. For statistical analysis, general and mixed linear models were used, and the independent variables were the Peabody Developmental Motor scale (stationary, locomotion, grasping and visuomotor sub scales), the Gross Motor Function Measure-66 and the Hand Assessment for Infants. Outcomes related to gross motor function improved significantly more after mobility training than after hand use training, while fine motor function was improved to the same extent following both training types. Significantly higher improvements after the first training period were seen in one out of three outcome measures in both gross and fine motor assessments. The improvements observed were all independent of diagnosis at two years. The concept "you learn what you practice" was most clearly confirmed in the case of gross motor development.

Stockholm preterm interaction-based intervention (SPIBI) - study protocol for an RCT of a 12-month parallel-group post-discharge program for extremely preterm infants and their parents.

BACKGROUND: Improved neonatal care has resulted in increased survival rates among infants born after only 22 gestational weeks, but extremely preterm children still have an increased risk of neurodevelopmental delays, learning disabilities and reduced cognitive capacity, particularly executive function deficits. Parent-child interaction and parental mental health are associated with infant development, regardless of preterm birth. There is a need for further early interventions directed towards extremely preterm (EPT) children as well as their parents. The purpose of this paper is to describe the Stockholm Preterm Interaction-Based Intervention (SPIBI), the arrangements of the SPIBI trial and the chosen outcome measurements. METHODS: The SPIBI is a randomized clinical trial that includes EPT infants and their parents upon discharge from four neonatal units in Stockholm, Sweden. Inclusion criteria are EPT infants soon to be discharged from a neonatal intensive care unit (NICU), with parents speaking Swedish or English. Both groups receive three initial visits at the neonatal unit before discharge during the recruitment process, with a strengths-based and development-supportive approach. The intervention group receives ten home visits and two telephone calls during the first year from a trained interventionist from a multi-professional team. The SPIBI intervention is a strengths-based early intervention programme focusing on parental sensitivity to infant cues, enhancing positive parent-child interaction, improving self-regulating skills and supporting the infant's next small developmental step through a scaffolding process and parent-infant co-regulation. The control group receives standard follow-up and care plus extended assessment. The outcomes of interest are parent-child interaction, child development, parental mental health and preschool teacher evaluation of child participation, with assessments at 3, 12, 24 and 36 months corrected age (CA). The primary outcome is emotional availability at 12 months CA. DISCUSSION: If the SPIBI shows positive results, it could be considered for clinical implementation for child-support, ethical and health-economic purposes. Regardless of the outcome, the trial will provide valuable information about extremely preterm children and their parents during infancy and toddlerhood after regional hospital care in Sweden. TRIAL REGISTRATION: The study was registered in ClinicalTrials.gov in October 2018 (NCT03714633).

Health-related quality of life, pain, and fatigue in young adults with cerebral palsy.

AIM: To describe health-related quality of life (HRQoL), pain, fatigue, and other health variables in young adults with cerebral palsy (CP), and to explore associations with the Gross Motor Function Classification System - Expanded and Revised (GMFCS-ER) and physical activity. METHOD: This was a cross-sectional study of 61 young adults at a mean age of 21 years 2 months (standard deviation 8mo, range 20-22y) with CP, from a geographically defined area. Data collection included: Short Form 36 version 2 for HRQoL, Brief Pain Inventory - Short Form, Fatigue Severity Scale, level of physical activity, medical history, and physical examination. RESULTS: Overall HRQoL equalled that of population norms; however self-reported physical health was lower in GMFCS-ER levels III to V compared to GMFCS-ER levels I to II. Self-reported mental health was, inversely, lower in GMFCS-ER levels I to II compared to GMFCS-ER levels III to V. Pain prevalence was 49%, and pain was present across all GMFCS-ER levels. Fatigue, as well as sleep problems, had 41% prevalence, with fatigue severity decreasing with increasing level of physical activity. INTERPRETATION: General HRQoL in young adults with CP was comparable to population norms. Pain and fatigue are important to address in high motor-functioning individuals also. Physical activity could be a possible protective factor against fatigue. WHAT THIS PAPER ADDS: Health-related quality of life in young adults with cerebral palsy (CP) was comparable to population norms. Pain, fatigue, and sleep problems occurred at all Gross Motor Function Classification System levels. There is a possible protective effect of physical activity on fatigue.

Efficacy of the Small Step Program in a Randomized Controlled Trial for Infants under 12 Months Old at Risk of Cerebral Palsy (CP) and Other Neurological Disorders.

The objective was to evaluate the effects of the Small Step Program on general development in children at risk of cerebral palsy (CP) or other neurodevelopmental disorders. A randomized controlled trial compared Small Step with Standard Care in infants recruited at 4-9 months of corrected age (CA). The 35-week intervention targeted mobility, hand use, and communication during distinct periods. The Peabody Developmental Motor Scales2ed (PDMS-2) was the primary outcome measure. For statistical analysis, a general linear model used PDMS-2 as the main outcome variable, together with a set of independent variables. Thirty-nine infants were randomized to Small Step (n = 19, age 6.3 months CA (1.62 SD)) or Standard Care (n = 20, age 6.7 months CA (1.96 SD)). Administering PDMS-2 at end of treatment identified no group effect, but an interaction between group and PDMS-2 at baseline was found (p < 0.02). Development was associated with baseline assessments in the Standard Care group, while infants in the Small Step group developed independent of the baseline level, implying that Small Step helped the most affected children to catch up by the end of treatment. This result was sustained at 2 years of age for PDMS-2 and the PEDI mobility scale.

A qualitative study examining the validity and comprehensibility of physical activity items: developed and tested in children with juvenile idiopathic arthritis.

BACKGROUND: Not all physical activity (PA) questionnaires (PAQ) gather information regarding PA intensity, duration, and modes and only a few were developed specifically for children. We assessed children's comprehensibility of items derived from two published PAQs used in children along with three items designed to ascertain PA intensity in order to assess comprehensibility of items and identify response errors. We modified items to create a new PAQ for children (ASCeND). We hypothesized that children would have comprehension difficulties with some original PAQ items and that ASCeND would be easier to comprehend, and would improve recall and reporting of PA. METHODS: For this qualitative study, we recruited 30 Swedish children [ages 10-16 years; mean age = 13.0 (SD = 1.8)]; median disease activity score = 4.5 (IQR 2.2-9.0); median disease duration = 5.0 (IQR 2.6-10.8) with juvenile idiopathic arthritis (JIA) from a children's hospital-based rheumatology clinic. We conducted cognitive interviews to identify children's comprehension of PAQ items. Interviews were audiotaped, transcribed, and independently analyzed. In phase one, 10 children were interviewed and items modified based on feedback. In phase two, an additional 20 children were interviewed to gather more feedback and further refine the modified items, to create the ASCeND. RESULTS: The median interview time was 41 min (IQR 36-56). In phase one, 219 comments were generated regarding directions for recording PA duration, and transportation use, walking, dancing, weight-bearing exercise and cardio fitness. Based on feedback we modified the survey layout, clarified directions and collapsed or defined items to reduce redundancy. In phase two, 95 comments were generated. Most comments related to aerobic fitness and strenuous PA. Children had difficulty recalling total walking and other activities per day. Children used the weather on a particular day, sports practice, or gym schedules to recall time performing activities. The most comments regarding comprehension were generated about the 3-item PA intensity survey, suggesting children had problems responding to intensity items. CONCLUSIONS: The newer layout facilitated recall of directions or efficiency in answering items. The 3-item intensity survey was difficult to answer. Sports-specific items helped children more accurately recall the amount of daily PA. The ASCeND appeared to be easy to answer and to comprehend.

Exploring social participation in young adults with cerebral palsy.

OBJECTIVES: To describe social outcomes for young adults with cerebral palsy, and to explore associations of social outcomes with their classification levels within the Gross Motor Function, Manual Ability and Communication Function Classification Systems, and with the presence of intellectual disability. DESIGN: A cross-sectional study with a population-based inclusion approach at a neuropaediatric referral centre in Sweden. SUBJECTS: Sixty-one young adults with cerebral palsy, age 20-22 years. METHODS: Physical examination and questionnaires on social outcomes including living arrangements, relationships, occupation, personal finances, extent of family support with personal care, and physical examination. RESULTS: Twenty percent of the young adults with cerebral palsy had moved out of the parental home. Forty-three percent were dependent on family support for basic activities of daily living. Seventy-nine percent of those without intellectual disability were employed or studying. The Communication Function Classification Systems, and presence of intellectual disability, demonstrated associations with most social outcomes, followed in significance by Manual Ability Classification System. CONCLUSION: In this study young adults with cerebral palsy to a high extent lived in the parental home, and more often without employment, compared with their peers. Many were dependent on parental support, financially, and with activities of daily living. Intellectual disability and communication function were important determinants of social participation. Interventions aimed at alleviating the impact of these particular disabilities should be prioritized.

Quality of goal setting in pediatric rehabilitation-A SMART approach.

BACKGROUND: Setting goals for treatment is often the core of the rehabilitation process. The quality of the set goals has however rarely been evaluated. The aims of this study were therefore to assess the quality of goals set in clinical practice of pediatric rehabilitation using SMART criteria (Specific, Measurable, Achievable, Relevant, and Timed) and to assess if the goals were considered relevant from both a client perspective and expertise perspective. METHODS: In a retrospective multicase study, a total of 161 goals from 42 children with disabilities (cerebral palsy, n = 22; Down syndrome, n = 16; and developmental disability, n = 4) were assessed. The children were 1.5-5.5 years and had previously participated in goal-directed, activity-focused therapy at four pediatric rehabilitation centers. Collaborative goal setting had been used to define the desired treatment outcome. The quality of the goals was assessed using defined SMART criteria. RESULTS: Specific: All goals could be reliably linked to International Classification of Functioning, Disability and Health-Children and Youth version chapters within the Activity/Participation domain. Measurable: A total of 75% of the goals were rated as having a well-defined scaling; in 20%, the scaling was less clear, and in 5%, a scaling could not be determined. Achievable: A total of 80% of the goals were attained. Relevant: All goals were set in collaboration with the family and could therefore be considered relevant from a client perspective. Relevancy judged from a professional perspective was strengthened by the fact that age, baseline status, and diagnosis had an influence on the choice of goals. Timed: All goals were set within a specific time frame. CONCLUSIONS: The goals set in clinical practice showed high quality with respect to the SMART criteria. The most difficult part was the construction of the goal attainment scale. The goals settled in clinical practice were considered relevant from both a client perspective and expertise perspective.

Effects of Botulinum Toxin-A and Goal-Directed Physiotherapy in Children with Cerebral Palsy GMFCS Levels I & II.

AIMS: To evaluate short and long-term effects of botulinum toxin-A combined with goal-directed physiotherapy in children with cerebral palsy (CP). METHOD: A consecutive selection of 40 children, ages 4-12 years, diagnosed with unilateral or bilateral CP, and classified in GMFCS levels I-II. During the 24 months, 9 children received one BoNT-A injection, 10 children two injections, 11 children three injections, and 10 children received four injections. 3D gait analysis, goal-attainment scaling, and body function assessments were performed before and at 3, 12, and 24 months after initial injections. RESULTS: A significant but clinically small long-term improvement in gait was observed. Plantarflexor spasticity was reduced after three months and remained stable, while passive ankle dorsiflexion increased after 3 months but decreased slightly after 12 months. Goal-attainment gradually increased, reached the highest levels at 12 months, and levels were maintained at 24 months. CONCLUSION: The treatments' positive effect on spasticity reduction was identified, but did not relate to improvement in gait or goal-attainment. No long-term positive change in passive ankle dorsiflexion was observed. Goal attainment was achieved in all except four children. The clinical significance of the improved gait is unclear. Further studies are recommended to identify predictors for positive treatment outcome.

Efficacy of the small step program in a randomised controlled trial for infants below age 12 months with clinical signs of CP; a study protocol.

BACKGROUND: Children with cerebral palsy (CP) have life-long motor disorders, and they are typically subjected to extensive treatment throughout their childhood. Despite this, there is a lack of evidence supporting the effectiveness of early interventions aiming at improving motor function, activity, and participation in daily life. The study will evaluate the effectiveness of the newly developed Small Step Program, which is introduced to children at risk of developing CP during their first year of life. The intervention is based upon theories of early learning-induced brain plasticity and comprises important components of evidence-based intervention approaches used with older children with CP. METHOD AND DESIGN: A two-group randomised control trial will be conducted. Invited infants at risk of developing CP due to a neonatal event affecting the brain will be randomised to either the Small Step Program or to usual care. They will be recruited from Astrid Lindgren Children's Hospital at regular check-up and included at age 3-8 months. The Small Step Program was designed to provide individualized, goal directed, and intensive intervention focusing on hand use, mobility, and communication in the child's own home environment and carried out by their parents who have been trained and coached by therapists. The primary endpoint will be approximately 35 weeks after the start of the intervention, and the secondary endpoint will be at 2 years of age. The primary outcome measure will be the Peabody Developmental Motor Scale (second edition). Secondary assessments will measure and describe the children's general and specific development and brain pathology. In addition, the parents' perspective of the program will be evaluated. General linear models will be used to compare outcomes between groups. DISCUSSION: This paper presents the background and rationale for developing the Small-Step Program and the design and protocol of a randomized controlled trial. The aim of the Small Step Program is to influence development by enabling children to function on a higher level than if not treated by the program and to evaluate whether the program will affect parent's ability to cope with stress and anxiety related to having a child at risk of developing CP. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT02166801 . Registered June 12, 2014.

Introduction of the gross motor function classification system in Venezuela--a model for knowledge dissemination.

BACKGROUND: A current worldwide common goal is to optimize the health and well-being of children with cerebral palsy (CP). In order to reach that goal, for this heterogeneous group, a common language and classification systems are required to predict development and offer evidence based interventions. In most countries in Africa, South America, Asia and Eastern Europe the classification systems for CP are unfamiliar and rarely used. Education and implementation are required. The specific aims of this study were to examine a model in order to introduce the Gross Motor Function Classification System (GMFCS-E&R) in Venezuela, and to examine the validity and the reliability. METHODS: Children with CP, registered at a National child rehabilitation centre in Venezuela, were invited to participate. The Spanish version of GMFCS-E&R was used. The Wilson mobility scale was translated and used to examine the concurrent validity. A structured questionnaire, comprising aspects of mobility and gross motor function, was constructed. In addition, each child was filmed. A paediatrician in Venezuela received supervised self-education in GMFCS-E&R and the Wilson mobility scale. A Swedish student was educated in GMFCS-E&R and the Wilson mobility scale prior to visiting Venezuela. In Venezuela, all children were classified and scored by the paediatrician and student independently. An experienced paediatric physiotherapist (PT) in Sweden made independent GMFCS-E&R classifications and Wilson mobility scale scorings, accomplished through merging data from the structured questionnaire with observations of the films. Descriptive statistics were used and reliability was presented with weighted Kappa (Kw). Spearman's correlation coefficient was calculated to explore the concurrent validity between GMFCS-E&R and Wilson mobility scale. RESULTS: Eighty-eight children (56 boys), mean age 10 years (3-18), with CP participated. The inter-rater reliability of GMFCS-E&R between; the paediatrician and the PT was Kw = 0.85 (95% CI: 0.75-0.88), the PT and student was Kw = 0.91 (95% CI: 0.86-0.95) and the paediatrician and student was Kw = 0.85 (95 % CI: 0.79-0.90). The correlations between GMFCS-E&R and Wilson mobility scale were high rs =0.94-0.95 (p < 0.001). CONCLUSIONS: In a setting with no previous knowledge of GMFCS-E&R, the model with education, supervised self-education and practice was efficient and resulted in very good reliability and validity.

A prospective cohort study investigating gross motor function, pain, and health-related quality of life 17 years after selective dorsal rhizotomy in cerebral palsy.

AIM: The aim of this study is to evaluate the long-term effects of selective dorsal rhizotomy (SDR), 15 to 20 years after surgery in patients with cerebral palsy. METHOD: Eighteen children (four females, 14 males; mean age at SDR 4y 7mo, SD 1y 7mo) with bilateral spastic cerebral palsy (CP), were prospectively assessed after SDR. This study focuses on the outcome 15 to 20 years after the procedure. The assessments include the Modified Ashworth Scale for spasticity, the Gross Motor Function Measure (GMFM-88), the Wilson Mobility Scale, The Health-Related Quality of Life Health Survey, SF-36v2, and the Brief Pain Inventory. RESULTS: The effect of normalized muscle tone in lower extremities after SDR was sustained after a median of 17 years. The best gross motor function capacity, according to the GMFM score, was seen at the 3-year follow-up, thereafter a gradual decline followed. Half of the individuals reported low intensity pain and interference. Compared to a norm sample the physical health component of SF-36v2 was slightly lower and the mental health component slightly higher. INTERPRETATION: The spasticity-reducing effect of SDR does not improve long-term functioning, nor prevent contractures, but it can possibly reduce the pain often experienced by individuals with CP.