We report the effectiveness of task-specific walking training for a child with hereditary spastic paraplegia using various orthoses, assistive mobility aids, and tasks.

A 4-year-8-month-old boy, classified as Gross Motor Function Classification System level IV, had selective dorsal rhizotomy and single-event multilevel surgery. The child began walking training using knee-ankle-foot orthoses and a posterior walker, setting the tasks to be "just right" for improving the child's abilities.

At 6 years and 11 months of age, the child walked using bilateral ankle-foot orthoses and crutches at school, and scores improved on the Canadian Occupational Performance Measure, Gross Motor Function Measure-66, and Functional Mobility Scale.Recommendations for Clinical Practice: Physical therapists need to carefully select the factors involved in walking training based on an assessment and the difficulty level of each child in order to optimize improvements.

The Exopulse Mollii Suit is an external electrical stimulation garment that is designed to reduce spasticity through electrical stimulation of targeted muscles. Our aims were to study the impact of the garment in improving gait and function in children with cerebral palsy (CP).

Individuals aged 4-18 years with spastic CP, Gross Motor Function Classification System level I-III were included for a prospective single-arm study from January 2021 to January 2022. Participants wore the suit for 4 weeks 60 min a day. Outcome measures taken pre, post and 1-month-post intervention included: 3D gait analysis (gait profile score, gait deviation index and temporo-spatial parameters), gross motor function measure-88 (GMFM-88), EQ-5D-Y, compliance rate, adverse event and satisfaction. Paired t-test was used for data analysis to compare measurement time points.

Twenty children (median age 7 [range: 4-16; interquartile range: 3.1] years, 55% female, 45% male were recruited. Post-intervention results showed there was no improvement in the gait profile but there was an improving trend in temporo-spatial parameters GMFM Domain C crawling and kneeling improved significantly (p = 0.03). Improvement in EQ-5D- Y usual activity was significant (p = 0.04). Compliance rate was 95% and nil major adverse event was reported. The majority (75%, n = 15) of parents and participants perceived overall positive experience.

The positive changes in gait profile and function were no longer significant at 1-month post-intervention. Further studies with a longer intervention period and concurrent strengthening program are required.

Using the Molli Suit for 60 min a day for 4 weeks, may be useful in improving:(1) Gait cadence in children with CP. (2) Gross motor function in terms of crawling and kneeling in children with CP.

To evaluate the efficacy of the Akwenda Intervention Program on motor, self-care, and social function of children and young people with cerebral palsy (CP).

This was a cluster-randomized, controlled, single-blinded, intervention study of 100 participants with CP (2-23 years; 52 males) in rural eastern Uganda. Half were allocated to the intervention program, the remainder served as waitlist controls. Gross Motor Function Measure-66 (GMFM-66) and the Ugandan version of Pediatric Evaluation of Disability Inventory (PEDI-UG) were collected before group allocation and after intervention. General linear models and t-tests were used to compare changes within and between groups. Cohen's d estimated the effect size of group differences. Change scores were evaluated by age and mobility subgroups.

Significant group by time interactions were found for GMFM-66 (p =0.003) and PEDI-UG outcomes (p <0.001), except mobility, with the intervention group demonstrating greater changes. Both groups increased their scores on the GMFM-66 and child PEDI-UG, while only the intervention group had significant increases in caregiver assistance scores and across all age and mobility subgroups. Cohen's d showed large effect sizes (d >0.8) of differences for PEDI-UG outcomes except mobility.

The Akwenda Intervention Program had a large positive impact on functioning and activity across age and mobility levels.

To identify outcome predictors of selective dorsal rhizotomy (SDR) in children with spastic cerebral palsy (CP) using logistic regression models.

A retrospective single-center study was conducted on children with spastic CP who had undergone SDR. Two outcomes were defined, one representing children not improving in motor function and spasticity and one representing children improving in motor function two years after surgery. Baseline variables were chosen based on established regressors and clinical considerations and tested for being predictors using multivariate logistic regression.

We included 96 patients (mean age [SD] 6y 9 m [2y 5 m], range 2y to 17y) in the study. Significant predictors of non-improvement in motor function and spasticity two years after SDR were higher age at surgery and higher preoperative Gross Motor Function Measure-88 (GMFM-88) score. Significant predictors of motor function improvement after SDR were lower preoperative Modified Ashworth Scale (MAS) scores, higher preoperative passive range of motion (pROM) and lower age at surgery.

For the first time, pROM and preoperative spasticity were identified as predictors of SDR outcome. We confirmed motor function and age as predictors. These variables will be useful for future patient selection. Adjusting for the GMFM-88's ceiling effect, children with higher motor function can still profit significantly from SDR.

Background: Cerebral palsy is a complex lifespan disability caused by a lesion to the immature brain. Evaluation of interventions for children with cerebral palsy requires valid and reliable outcome measures. Motor development curves and reference percentiles for The Gross Motor Function Measure (GMFM-66) are valuable tools for following, predicting, comparing, and evaluating changes in gross motor skills. The aims of this study were to create motor development curves with reference percentiles based on Norwegian data and compare them with published counterparts for Canadian children aged 2-21 years. Method: Prospective population-based cohort data from the Norwegian Quality and Surveillance Registry for Cerebral Palsy (NorCP) for 1206 children with 3612 GMFM-66 tests between 0.5 and 17.3 years of age. Median development by Gross Motor Function Classification System (GMFCS) levels was estimated using a generalized additive regression model with smoothed parameters for location, scale, and shape (based on the R GAMLSS library). To adjust for repeated individual measurements, we report the median curve of 100 random samples with only one observation per observed child. Results: The Norwegian motor development curves for GMFCS levels I-IV increase up to 7 years of age before flattening off, while GMFCS level V curves are relatively flat. Overall, both motor development curves and GMFM-66 percentiles are very similar to Canadian counterparts. Conclusions: The existing Canadian reference curves are valid also for Norway, working well for both clinical and research applications. However, Norwegian percentiles can be used from an earlier age.

Reference centiles describing gross motor function in children with cerebral palsy (CP) are used in clinical and research settings to guide treatments and evaluate interventions. However, it is unknown how existing references generalize to populations in novel settings.

The aim of this study is to evaluate the cross-sectional and longitudinal performance of three reference centiles to describe the motor function of children with CP aged 2-12 years at a large urban US pediatric hospital through a retrospective observational study.

and Procedures: We assessed cross-sectional performance by ranking our clinical population by quartile distributions described by the references. We assessed longitudinal performance by analyzing the distribution of prediction errors and correlations between predictions and observed scores.

For cross-sectional distribution, the reference centiles by Hanna more closely described our population than those by Duran. For longitudinal progression, prediction error was less than 6 GMFM points for most records at 24-, 12-, and 6-month time scales for all three sets of reference centiles, but higher at a 48-month time scale. Prediction errors increased at younger ages and higher motor ability.

Despite differences in cross-sectional performance, all three reference centiles achieved similar longitudinal performance and are sufficient for most clinical and research uses. Caution should be used when applying these curves to locations with different standards of care.

This article describes how the Gross Motor Ability Estimator (GMAE) software can provide important information based on the Gross Motor Function Measure (GMFM)-66 score of a child with congenital Zika syndrome.A child was assessed at 9, 17, and 25 months of age through the GMFM-66. At 2 years, the child's gross motor ability was estimated and classified according to the Gross Motor Function Classification System (GMFCS).At 2 years of age, the child in this case required assistance to roll and was unable to maintain antigravity trunk posture in sitting position, typical abilities of GMFCS level V.GMAE can be useful to guide health professionals that care for children with lifelong physical and developmental care needs. This is the first study that demonstrated how to use the GMAE in this specific population.

Developmental trajectories are crucial for evidence-based prognostication, planning interventions, and monitoring progress in children with cerebral palsy (CP).

To describe gross motor development patterns of children with CP in rural South India for the five Gross Motor Function Classification System (GMFCS) levels.

Longitudinal cohort study of 302 children (176 males, 126 females) with CP aged 0 to 10 years, followed by a community-based early intervention program. GMFCS levels were 5.4 % level 1, 16.5 % level II, 22.8 % level III, 26.8 % level IV, and 28.5 % level V. Assessments were undertaken using the Gross Motor Function Measure (GMFM-66) at 6-month intervals between April 2017 and August 2020. Longitudinal analyses were performed using mixed-effects linear regression models.

Five distinct motor development curves were created for ages 0 to 10 years by GMFCS levels as a function of age and GMFM-66 with a stable limit model, variation in estimated limits and rates of development.

Motor development trajectories for CP in an LMIC differ from those reported in HICs. Consideration of how social determinants of health, environmental and personal factors impact motor development in low-resource contexts is crucial. Further work is needed to describe developmental trajectories of children for CP in LMICs.

Abnormal gait kinematics are common in youth with cerebral palsy (CP), but prior studies have not analyzed their longitudinal change throughout childhood. This study examines how age and orthopaedic surgery influence gait kinematics throughout childhood in those with ambulatory CP.

In this institutional review board-approved prospective cohort study, children with spastic CP (GMFCS I-III) were recruited at age 17-40 months. Instrumented gait analysis was performed at 3-year intervals from age 4 to 21 years, collecting longitudinal kinematic data in bare feet at a self-selected speed. The change in Gait Profile Score (ΔGPS) between each pair of gait analyses (intervals) was analyzed by age distribution (<10, 10-15, ≥15 years) and by presence/absence of orthopaedic surgery.

The study included 31 children (GMFCS: I [13], II [14], III [4]). A baseline instrumented gait analysis was performed at age 5.8 ± 1.6 years with subsequent analysis at 2.5 ± 1.3-year intervals. Examining ΔGPS from baseline to final outcome, 87% of limbs were improved/unchanged; 298 intervals of ΔGPS were analyzed and classified as nonsurgical or surgical. Analysis revealed greater GPS improvement in intervals with surgery versus intervals without (p = 0.0004). Surgical intervals had significantly greater GPS improvement in the <10- vs. >15-year-old groups, p = 0.0063.

Improvement in gait kinematics in children with CP is significantly influenced by age and timing of orthopaedic surgical intervention for gait correction, and was most pronounced for children <10 years old. Although surgery was associated with improved outcomes in all age groups, these improvements were significantly less for children >10 years old. These results reinforce the importance of considering the timing of orthopaedic surgery.

EEG plays an integral part in the diagnosis and management of children with genetic epilepsies. Nevertheless, how quantitative EEG features differ between genetic epilepsies and neurological outcomes remains largely unknown. Here, we aimed to identify quantitative EEG biomarkers in children with epilepsy and a genetic diagnosis in STXBP1, SCN1A, or SYNGAP1, and to assess how quantitative EEG features associate with neurological outcomes in genetic epilepsies more broadly. We analyzed individuals with pathogenic variants in STXBP1 (95 EEGs, n=20), SCN1A (154 EEGs, n=68), and SYNGAP1 (46 EEGs, n=21) and a control cohort of individuals without epilepsy or known cerebral disease (847 EEGs, n=806). After removing artifacts and epochs with excess noise or altered state from EEGs, we extracted spectral features. We validated our preprocessing pipeline by comparing automatically-detected posterior dominant rhythm (PDR) to annotations from clinical EEG reports. Next, as a coarse measure of pathological slowing, we compared the alpha-delta bandpower ratio between controls and the different genetic epilepsies. We then trained random forest models to predict a diagnosis of STXBP1, SCN1A, and SYNGAP1. Finally, to understand how EEG features vary with neurological outcomes, we trained random forest models to predict seizure frequency and motor function. There was strong agreement between the automatically-calculated PDR and clinical EEG reports (R 2=0.75). Individuals with STXBP1-related epilepsy have a significantly lower alpha-delta ratio than controls (P<0.001) across all age groups. Additionally, individuals with a missense variant in STXBP1 have a significantly lower alpha-delta ratio than those with a protein-truncating variant in toddlers (P<0.001), children (P=0.02), and adults (P<0.001). Models accurately predicted a diagnosis of STXBP1 (AUC=0.91), SYNGAP1 (AUC=0.82), and SCN1A (AUC=0.86) against controls and from each other in a three-class model (accuracy=0.74). From these models, we isolated highly correlated biomarkers for these respective genetic disorders, including alpha-theta ratio in frontal, occipital, and parietal electrodes with STXBP1, SYNGAP1, and SCN1A, respectively. Models were unable to predict seizure frequency (AUC=0.53). Random forest models predicted motor scores significantly better than age-based null models (P<0.001), suggesting spectral features contain information pertinent to gross motor function. In summary, we demonstrate that STXBP1-, SYNGAP1-, and SCN1A-related epilepsies have distinct quantitative EEG signatures. Furthermore, EEG spectral features are predictive of some functional outcome measures in patients with genetic epilepsies. Large-scale retrospective quantitative analysis of clinical EEG has the potential to discover novel biomarkers and to quantify and track individuals' disease progression across development.

Background/Objectives: Early interventions for infants at high risk of cerebral palsy (CP) are recommended, but limited evidence exists. Our objective was, therefore, to evaluate the effects of the family-centered and interprofessional Small Step early intervention program on motor development in infants at high risk of CP (ClinicalTrials.gov: NCT03264339). Methods: A single-subject research design was employed to investigate participant characteristics (motor dysfunction severity measured using the Hammersmith Infant Neurological Examination (HINE) and Alberta Infant Motor Scale (AIMS) at three months of corrected age (3mCA) related to intervention response. The repeated measures Peabody Developmental Motor Scales-2 fine and gross motor composite (PDMS2-FMC and -GMC) and Hand Assessment for Infants (HAI) were analyzed visually by cumulative line graphs, while the Gross Motor Function Measure-66 (GMFM-66) was plotted against reference percentiles for various Gross Motor Function Classification System (GMFCS) levels. Results: All infants (n = 12) received the Small Step program, and eight completed all five training steps. At two years of corrected age (2yCA), nine children were diagnosed with CP. The children with the lowest HINE < 25 and/or AIMS ≤ 6 at 3mCA (n = 4) showed minor improvements during the program and were classified at GMFCS V 2yCA. Children with HINE = 25-40 (n = 5) improved their fine motor skills during the program, and four children had larger GMFM-66 improvements than expected according to the reference curves but that did not always happen during the mobility training steps. Three children with HINE = 41-50 and AIMS > 7 showed the largest improvements and were not diagnosed with CP 2yCA. Conclusions: Our results indicate that the Small Step program contributed to the children's motor development, with better results for those with an initial higher HINE (>25). The specificity of training could not be confirmed.

An improved understanding of the current practice of standing frame use may have implications for supporting parents in managing standing frames. We aimed to investigate how parents of children with cerebral palsy perceive and manage standing frame use in home settings.

We conducted a mixed methods study with an explanatory sequential design, first collecting and analysing quantitative questionnaire data and then using these results to inform a qualitative follow-up phase to explain them. The questionnaire was answered by 103 parents of children with cerebral palsy across five countries, Denmark, Norway, Great Britain, Canada and the United States, and 12 Danish families participated in the subsequent interviews. A descriptive analysis was conducted using the questionnaire data. The qualitative data were analysed using a directed content analysis, enabling integration of the quantitative and qualitative data.

The quantitative analysis showed that 89% of the parents felt confident with their child's standing frame, and 82% felt they had sufficient knowledge about how their child's standing frame could/should be used. However, the qualitative analysis showed that even when feeling confident, the parents experienced insecurity regarding whether their child was positioned correctly, and being responsible for positioning was challenging.

Our study implies a need for providing educational materials to assist the parents in ensuring optimal positioning of their child in the standing frame to decrease insecurity. Additionally, our study suggests a need to provide more thorough information about the benefits of using a standing frame and ensure alignment of expectations in relation to the child's prognosis of functional independence.

Cerebral palsy (CP) is the most cause of motor dysfunction in children. Selective dorsal rhizotomy (SDR) plays a major role in long term spasticity control. However, limited data exists on the effect of SDR on postoperative spasticity treatment requirements and supraspinal effects, and the stimulation responses of dorsal nerve roots in those with CP.

The current study included the outcome for 35 individuals undergoing SDR for motor functional outcome, spasticity, baclofen dose changes, botulinum toxin injection frequency, and spasticity related orthopedic procedures. We also report on the stimulation responses in 112 individuals who underwent SDR at our institution.

There was a significant difference in gross motor function measures (GMFM)-66 scores at last follow up that remained present when considering only ambulatory children but not with non-ambulatory children. Ashworth scores were significantly decreased for both upper and lower extremities after SDR at all follow up points. There was a significant decrease in Baclofen dose and botulinum toxin injections requirements after SDR, but no significant difference in the need for orthopedic intervention. A total of 5502 dorsal nerve roots were tested showing a decrease in stimulation intensity and increase in grade on the right side and for descending lumbosacral levels.

SDR improves gross motor scores during short term follow up but has additional benefits in decreasing baclofen dosing and botulinum toxin injections requirements after surgery. They stimulation responses of sectioned dorsal nerve roots adds to the limited available data and our understanding of the pathological changes that occur in CP.

To provide a detailed description of the development of the first case of congenital Zika syndrome (CZS) to be reported in the literature worldwide.

This report describes the case of a child with CZS monitored from pregnancy until four years of age, with periodic evaluations of head circumference, weight, height, motor function according to the Gross Motor Function Measure (GMFM-88), and the occurrence of comorbidities.

The child's birth weight and length were normal (z-score = 1.1 and -1.95, respectively), while head circumference was below the expected value (z-score = -3.15). At 48 months, head circumference reached 43 cm (z-score = -4.48). During daily home physiotherapy sessions, the child achieved developmental milestones, standing unsupported at 17 months, with a GMFM-88x score of 137. With specialist therapy, the child walked independently at 36 months and a total GMFM-66 score of 214 was achieved by 42 months. In the four years of follow-up, the child was hospitalized four times for different reasons. No convulsive seizures occurred.

Despite severe neurological impairment, the child's weight and height are adequate for age, with motor and cognitive function improving over the first four years of life.

Despite the proven effectiveness of rehabilitation robots (RR) in the literature, they are still little used in clinical rehabilitation. The aim of this study was to analyse the factors influencing the use of RR and the perception of therapists who used RR.

In order to characterize the factors influencing the use of RR by therapists, a semi-structured interview was conducted with 18 therapists. These interviews are based on an interview guide inspired by the Unified Theory of Acceptance and Use of Technology model. The interviews were recorded and then transcribed, summarized and finally synthesized cross-sectionally. In addition and in parallel, the System Usability Scale (SUS) was also proposed to clinicians in order to collect quantitative data.

The interviews highlight the facilitators perceived by the therapists, such as the intensity of the movement, the complementarity with conventional rehabilitation. The results also showed the possible barriers perceived, these can be sometimes inconclusive (e.g., bugs). The SUS results show no effect, either on the gender of the users, their therapists, or the duration of use of the tool.

Better communication on the functionality of the robot and the construction of achievable goals would lead to more results that are conclusive but also better patient care. To date, and despite the evidence for the effectiveness of RRs, therapists believe that there are still many barriers to their use. They agree, however, that if changes are made, RRs will become an integral part of therapy.IMPLICATIONS FOR REHABILITATIONThe study idenfied and highlighted the factors influencing the use of the rehabilitation robot in the clinics through metric and ergonomic evaluations.The study allowed to quantify the level of acceptance of the Lokomat among therapists.This study allowed to identify negative factors that could be resolved through the implementation of a structured and generalized protocol for patients and thus improve their care.

This paper authentically represents the voices of non-verbal children with cerebral palsy using a case study design. Policy suggests that children should have the right to play and leisure opportunities, however non-verbal children with cerebral palsy have fewer choices. Additionally, children with communication, learning and mobility limitations are usually excluded from research. The aim of this research was to capture the voices of non-verbal children by exploring their well-being impact in terms of their experiences and choices about their level of participation in recreational activities.

A qualitative case series study included interviews, observations, photographs and diaries. Where possible, the diaries were completed by both caregivers and children. Data were analysed thematically, and the lens of positioning theory applied.

Seven children aged nine to sixteen years participated. The findings showed how equipment, people and environments enabled or hindered the children's participation. The children also advocated as champions for their own well-being. Positioning theory was applied across the data and was adapted offering a way to better understand the children's well-being responses.

The findings demonstrate how these children were able to self-advocate, demonstrating their well-being by their intentional behaviours from their level of participation in a recreational activity.

Physical exercise is known to have beneficial effects on psychosocial well-being and cognitive performance. Children with cerebral palsy (CP) showed lower levels of physical activity (PA) than healthy children; this fact, in addition to the basic clinical condition, increased the sedentary habit with a psychological impact and motor impairment of these children. Furthermore, children and adolescents with CP are less committed to sports activities than typically developing children of the same age. The aim of the present narrative review was to increase the amount of knowledge regarding the effectiveness and importance of specific and individualized sports in children with CP. A comprehensive search of MED-LINE and EMBASE databases was performed, including specific search terms such as "cerebral palsy" combined with "sport", "physical activity", and the names of different sports. No publication date limits were set. We included studies with an age range of 0-18 years. The main results pointed out that most of the sports improved motor function, quality of life, and coordination in children and adolescents with CP. Physicians, therapists, and parents should become aware of the benefits of sports activities for this population of patients. Specific sports activities could be included as a usual indication in clinical practice in addition to rehabilitation treatment.

The Gross Motor Function Measure is used in most studies measuring gross motor function in children with cerebral palsy. In many studies, including those evaluating the effect of hyperbaric treatment, the Gross Motor Function Measure variations were potentially misinterpreted because of the lack of control groups. The Gross Motor Function Measure Evolution Ratio (GMFMER) uses historical data from the Gross Motor Function Classification System curves and allows to re-analyze previous published studies which used the Gross Motor Function Measure by considering the natural expected evolution of the Gross Motor Function Measure. As the GMFMER is defined by the ratio between the recorded Gross Motor Function Measure score increase and the expected increase attributed to natural evolution during the duration of the study (natural evolution yields a GMFMER of 1), it becomes easy to assess and compare the efficacy of different treatments.

The objective of this study is to revisit studies done with different dosage of hyperbaric treatment and to compare the GMFMER measured in these studies with those assessing the effects of various recommended treatments in children with cerebral palsy.

PubMed Searches were conducted to included studies that used the Gross Motor Function Measure to evaluate the effect of physical therapy, selective dorsal rhizotomy, botulinum toxin injection, hippotherapy, stem cell, or hyperbaric treatment. The GMFMER were computed for each group of the included studies.

Forty-four studies were included, counting 4 studies evaluating the effects of various dosage of hyperbaric treatment in children with cerebral palsy. Since some studies had several arms, the GMFMER has been computed for 69 groups. The average GMFMER for the groups receiving less than 2 h/week of physical therapy was 2.5 ± 1.8 whereas in context of very intensive physical therapy it increased to 10.3 ± 6.1. The GMFMER of stem cell, selective dorsal rhizotomy, hippotherapy, and botulinum toxin treatment was, 6.0 ± 5.9, 6.5 ± 2.0, 13.3 ± 0.6, and 5.0 ± 2.9, respectively. The GMFMER of the groups of children receiving hyperbaric treatment were 28.1 ± 13.0 for hyperbaric oxygen therapy and 29.8 ± 6.8 for hyperbaric air.

The analysis of the included studies with the GMFMER showed that hyperbaric treatment can result in progress of gross motor function more than other recognized treatments in children with cerebral palsy.

Translate, investigate reliability, and construct validity of the Brazilian Early Activity Scale for Endurance (EASE).

Translation followed the international guidelines. Test-retest reliability was tested by 100 parents of children with cerebral palsy (CP): 18 months-5 years and 6-11 years. To determine construct validity, 94 parents of typically children completed the EASE. Statistical analysis included Bland-Altman, Intraclass Correlation Coefficient (ICC), Internal Consistency, and Floor and Ceiling Effect.

The majority of the sample consisted of children with CP in GMFCS (IV-V). EASE showed good test-retest reliability for younger (ICC = 0.8) and excellent test-retest reliability for older children with CP (ICC = 0.9), and good internal consistency of 0.7 and 0.8 for the young and older group, respectively. Bland-Altman showed the bias close to zero, with no ceiling or floor effect. Regarding construct validity, younger children showed lower scores when compared to the older children. Endurance differed significantly between children with CP who were walking and those who were not walking and also for age groups. Children with CP showed lower endurance compared to typically participants in the same age group.

Brazilian EASE is reliable and valid to estimate endurance in children with CP. Results provide evidence of construct validity.

To compare the 66-item Gross Motor Function Measure (GMFM-66) with the reduced version of the GMFM-66 (rGMFM-66) with respect to the detection of clinically relevant changes in gross motor function in children with cerebral palsy (CP).

The study was a retrospective single centre analysis of children with CP who participated in a rehabilitation programme. Overall, 1352 pairs of GMFM-66 and rGMFM66 measurements with a time interval of 5 to 7 months were available. To measure clinically relevant changes in gross motor function, the individual effect size (iES) was calculated.

The study population consisted of 1352 children (539 females), mean age 6 years 4 months (SD 2 years 4 months). The iES based on the GMFM-66 and the rGMFM-66 showed a significant correlation (r = 0.84, p < 0.001). The analysis of the area under the receiver operating characteristic curve showed an excellent agreement for clinically relevant gross motor improvement (Cohen's d ≥ 0.5; area under the curve = 0.90 [95% confidence interval 0.88-0.92]) or deterioration (Cohen's d ≤ -0.5; area under the curve = 0.95 [95% confidence interval 0.92-0.97]).

Performing the rGMFM-66 saves time compared to the full GMFM-66. The rGMFM-66 showed good agreement with the GMFM-66 with respect to the detection of clinically relevant changes in gross motor function in children with CP, so its use in everyday clinical practice seems justifiable.

The reduced version of the 66-item Gross Motor Function Measure (rGMFM-66) detects clinically relevant changes in gross motor function in children with cerebral palsy. The rGMFM-66 correlates highly with the full GMFM-66. The rGMFM-66 can be used in clinical practice when the time schedule is limited.

To compare the effect of iMOVE (Intensive Mobility training with Variability and Error) therapy with dose-matched conventional therapy on gross motor development and secondary outcomes in young children with cerebral palsy.

This single-blind, randomized controlled trial included repeated assessments of gross motor function (using the Gross Motor Function Measure) and secondary outcomes during a 12- to 24-week intervention phase and at three follow-up points after treatment. Treatment was delivered three times per week in both groups. Forty-two children aged 12 to 36 months were stratified by age and motor function to ensure equivalence between groups at baseline.

Thirty-six children completed treatment and follow-up phases. Treatment fidelity was high and adherence was equivalent between groups (77.3% conventional therapy, 76.2% iMOVE). There were no group differences on the primary (gross motor function after 12 weeks p = 0.18; after 24 weeks p = 0.94) or any secondary (postural control p = 0.88, caregiver satisfaction p = 0.52, child engagement p = 0.98) measure after treatment or at the follow-up points. However, one-third of total participants exceeded predicted change after 12 weeks and 77% exceeded predicted change after 24 weeks of treatment.

Our observations indicate a potential dose-response effect of rehabilitation therapy. We further demonstrated that individual therapeutic ingredients can be manipulated. When delivered consistently, both iMOVE and conventional therapy interventions might both be more effective than standard care.

Those receiving iMOVE therapy demonstrated more independent practice time, error, and child-initiation than those receiving the dose-matched control. iMOVE therapy was not superior to the control (conventional physical) therapy. Most participants exceeded predicted change after 24 weeks of treatment.

Regaining motor function in individuals with cerebral palsy (CP) has been predominantly studied in children, resulting in an underrepresentation of adults in research efforts. We tested the efficacy of noninvasive spinal neuromodulation with neurorehabilitation (Spinal Cord Innovation in Pediatrics; SCiP™ therapy). A 60-year-old CP participant underwent 8 weeks of SCiP™ therapy, resulting in significant motor recovery measured by 14.2-points increase in gross motor function measure (GMFM-88) score, ~ three times the Minimal Clinically Important Difference (MCID) of 5-points. This represented gains in kneeling, sitting, and walking functions. The improvement in GMFM-88 score was maintained above the MCID at the follow up visit (10.3 points above the baseline), twenty weeks following the last therapy session, indicating a persistent effect of the therapy. Our preliminary findings support the therapeutic promise of SCiP™ therapy for enhancing motor function in CP adults. Broader investigations are needed to establish its wider applicability.

Background Boys with Duchenne Muscular Dystrophy (DMD) display heterogeneous motor function trajectory in clinics, which represents a significant obstacle to monitoring.

In this paper, we present the UK centiles for the North Star Ambulatory Assessment (NSAA), the 10 m walk/run time (10MWR) and velocity (10MWRV), and the rise from floor time (RFF) and velocity (RFFV) created from a cohort of glucocorticoid treated DMD boys between the age of 5 and 16 years.

Participants were included from the UK NorthStar registry if they had initiated steroids (primarily deflazacorts/prednisolone, intermittent/daily) and were not enrolled in an interventional trial. Assessments were included if the participant had a complete NSAA, the timed tests had been completed or the corresponding items were 0, or the participant was recorded as non-ambulant, in which case the NSAA was assumed 0.

We analysed 3987 assessments of the NSAA collected from 826 participants. Of these, 1080, 1849 and 1199 were imputed as 0 for the NSAA, RFFV and 10MWRV respectively. The 10th, 25th, 50th, 75th and 90th centiles were presented. The NSAA centiles showed a peak score of 14, 20, 26, 30 and 32 respectively, with loss of ambulation at 10.7, 12.2 and 14.3 years for the 25th, 50th and 75th centiles, respectively. The centiles showed loss of rise from floor at 8.6, 10.1 and 11.9 years and a loss of 10MWR of 0 at 8.9, 10.3 and 13.8 years for the 25th, 50th and 75th centiles, respectively. The centiles were pairwise less correlated than the raw scores, suggesting an increased ability to detect variability in the DMD cohort.

The NSAA, 10MWR and RFF centiles may provide insights for clinical monitoring of DMD boys, particularly in late ambulatory participants who are uniformly declining. Future work will validate the centiles in national and international natural history cohorts.

Cerebral palsy (CP) is a group of neuromotor diseases that develops as a result of damage to the developing central nervous system during the perinatal period. The condition is usually accompanied by musculoskeletal problems resulting in movement disorders. Gait improvement therefore, is an important part of its treatment. Roboticassisted gait training (RAGT) is a new potential rehabilitation tool for CP patients, however there is no clear evidence for the effectiveness of this method.

Can robotic-assisted gait training improve walking function in children with CP?

A systematic search was performed in five databases: MEDLINE (via PubMed), Cochrane Central Register of Controlled Trials (CENTRAL), Embase, Scopus, and Web of Science. Eligible studies were randomized controlled trials (RCT) with CP patients under the age of 18. Gross motor function and kinematic gait parameters of patients were the main outcomes. Two authors determined the risk of bias of the RCTs independently using the revised Risk of Bias 2 (ROB 2) tool. Mean Differences (MDs) along with their 95% Confidence Interval (CI) were calculated when at least three studies were present for an outcome, subgroup analysis was performed based on the treatment of the control group.

Of the 7363 screened articles, 13 papers met our inclusion criteria and among them, 7 studies could be used in our meta-analyses. The results related to RAGT suggest nonsignificant improvement in standing and walking function (Gross Motor Function Measure D, E), moreover changes in gait speed, step length, and in cadence were also only comparable to controls.

The results indicate that there is a trend in some gait parameters where the improvement was higher in the intervention group than in control group. The therapeutic effect of RAGT was probably not superior to physiotherapy combined with treadmill training.

Assess the effects of selective dorsal rhizotomy (SDR) on motor function and quality of life in children with a Gross Motor Function Classification System (GMFCS) level of IV or V (non-ambulatory).

This is a prospective, observational study in three tertiary neurosurgery units in England, UK, performing SDR on children aged 3-18 with spastic diplegic cerebral palsy, and a GMFCS level of IV or V, between 2012 and 2019. The primary outcome measure was the change in the 66-item Gross Motor Function Measure (GMFM-66) from baseline to 24 months after SDR, using a linear mixed effects model. Secondary outcomes included spasticity, bladder function, quality of life, and pain scores.

Between 2012 and 2019, 144 children who satisfied these inclusion criteria underwent SDR. The mean age was 8.2 years. Fifty-two percent were female. Mean GMFM-66 score was available in 77 patients (53.5%) and in 39 patients (27.1%) at 24 months after SDR. The mean increase between baseline and 24 months post-SDR was 2.4 units (95% CI 1.7-3.1, p < 0.001, annual change 1.2 units). Of the 67 patients with a GMFM-66 measurement available, a documented increase in gross motor function was seen in 77.6% (n = 52). Of 101 patients with spasticity data available, mean Ashworth scale decreased after surgery (2.74 to 0.30). Of patients' pain scores, 60.7% (n = 34) improved, and 96.4% (n = 56) of patients' pain scores remained the same or improved. Bladder function improved in 30.9% of patients.

SDR improved gross motor function and reduced pain in most patients at 24 months after surgery, although the improvement is less pronounced than in children with GMFCS levels II and III. SDR should be considered in non-ambulant patients.

STXBP1-related disorders are among the most common genetic epilepsies and neurodevelopmental disorders. However, the longitudinal epilepsy course and developmental end points, have not yet been described in detail, which is a critical prerequisite for clinical trial readiness. Here, we assessed 1281 cumulative patient-years of seizure and developmental histories in 162 individuals with STXBP1-related disorders and established a natural history framework. STXBP1-related disorders are characterized by a dynamic pattern of seizures in the first year of life and high variability in neurodevelopmental trajectories in early childhood. Epilepsy onset differed across seizure types, with 90% cumulative onset for infantile spasms by 6 months and focal-onset seizures by 27 months of life. Epilepsy histories diverged between variant subgroups in the first 2 years of life, when individuals with protein-truncating variants and deletions in STXBP1 (n = 39) were more likely to have infantile spasms between 5 and 6 months followed by seizure remission, while individuals with missense variants (n = 30) had an increased risk for focal seizures and ongoing seizures after the first year. Developmental outcomes were mapped using milestone acquisition data in addition to standardized assessments including the Gross Motor Function Measure-66 Item Set and the Grasping and Visual-Motor Integration subsets of the Peabody Developmental Motor Scales. Quantification of end points revealed high variability during the first 5 years of life, with emerging stratification between clinical subgroups. An earlier epilepsy onset was associated with lower developmental abilities, most prominently when assessing gross motor development and expressive communication. We found that individuals with neonatal seizures or early infantile seizures followed by seizure offset by 12 months of life had more predictable seizure trajectories in early to late childhood compared to individuals with more severe seizure presentations, including individuals with refractory epilepsy throughout the first year. Characterization of anti-seizure medication response revealed age-dependent response over time, with phenobarbital, levetiracetam, topiramate and adrenocorticotropic hormone effective in reducing seizures in the first year of life, while clobazam and the ketogenic diet were effective in long-term seizure management. Virtual clinical trials using seizure frequency as the primary outcome resulted in wide range of trial success probabilities across the age span, with the highest probability in early childhood between 1 year and 3.5 years. In summary, we delineated epilepsy and developmental trajectories in STXBP1-related disorders using standardized measures, providing a foundation to interpret future therapeutic strategies and inform rational trial design.

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.

In evaluating treatment efficacy, there is an ongoing discussion about which endpoint is more efficient to represent the treatment effect. Absolute change (AC) is the difference between before and after treatment, while relative change (RC) is the AC relative to the baseline value. Principal investigators sometimes support the credibility of relative change, but the FDA is more likely to support absolute change. Therefore, whether these two endpoints can be translated or combined is worth investigating in order to satisfy both parties.

In this article, a motivating example is presented to show that the choice of endpoint will result in different conclusions. The compared relationship of AC and RC is discussed in terms of required sample size, power, and precision. A new type of responder endpoint that combines the concepts of AC and RC is proposed. The comparative relationship regarding sample size, power, and precision of the proposed responder endpoint and the original two endpoints are also investigated.

As a result, the performance of AC and RC is highly dependent on the choice of threshold that is often informed based on minimum clinically important difference or other clinical experience. Therefore, an absolute translation between them is hard to achieve. Inspired by the concept of responder analysis, three types of responder endpoints are proposed and discussed. The pattern of the third type of responder endpoint is having higher power, higher precision, and less required sample size in estimating the treatment effect compared to AC and RC within a range of thresholds. This advantage becomes more obvious when applying higher AC and RC thresholds and lower [Formula: see text] threshold.

The proposed endpoint incorporates the information from the AC and RC endpoints and could be another wise choice when designing clinical trials especially when there is no absolute preference between AC and RC.

The feasibility of ELEVATE with respect to adherence and preliminary efficacy was determined for children with spastic bilateral cerebral palsy (CP) from encephalopathy of prematurity.

A case series was used. Participants were randomized to receive ELEVATE immediately or delay the intervention by 3 months before receiving the intervention. The outcomes included feasibility measures of (1) number of children recruited, (2) percentage of sessions attended, (3) stride counts during the intervention, and preliminary efficacy measures of change over the intervention period in (4) Gross Motor Function Measure-66 (GMFM-66), and (5) kinematics and weight-bearing during treadmill walking.

Four boys under 3 years of age participated. All participants tolerated 60-minute intervention sessions four times/week for 12 weeks, and attended 75%-94% (min-max) of the targeted sessions. The median step count per session ranged from 833 to 2484 steps (min-max) during the final week of training. Participants showed an increase in GMFM-66 score of 2.4-7.5 points (min-max) over the 3-month intervention phase, as compared to a decrease of 1.7 for one participant and an increase of 1.3 for another over the delay period. Three participants demonstrated small improvements in their gait with the intervention.

Engaging young children with bilateral CP in intensive rehabilitation targeting gross motor function was feasible and demonstrated preliminary efficacy. The results have guided the design of a larger clinical trial to assess efficacy of early, active interventions for children with spastic bilateral CP.

Our aim in this study was to examine relationships between the motor activity ability, sensor-based kinematics and forward-backwards gait characteristics of children with cerebral palsy (CP). In this prospective cross-sectional study we studied 40 children with CP. We used the Pediatric Motor Activity Log Revised (PMAL-R) to assess motor activity, evaluated motion kinematics (acceleration and angular velocity) with a sensor-based application, applied the Edinburgh Visual Gait Score (EVGS) and the Timed Up and Go Test (TUG) to observe gait performance, and used the Three Meter Backward Walk Test (3MBWT) to assess backward gait. We found moderately positive significant correlations (r1 = 0.416, r2 = 0.418, p < 0.05) between the chilidren's minimum angular velocity on PMAL-R motor activity frequency (how often) and quality (how well) scores, respectively. We also found moderately negative significant correlations (r1 = -0.529, r2 = -0.521, p < 0.05) between PMAL-R frequency (how often) and quality (how well) scores with TUG, respectively. There were moderately high positive correlations (r1 = 0.415, r2 = 0.726, p < 0.05) between EVGS scores and 3MBWT and TUG scores, respectively. We concluded that angular velocity ability was moderately related to children's motor activity and showed that physical performance tests (TUG and 3MBWT) could monitor gait function and upper extremity motor activity level, including both forward and backward walking tasks, in children with CP.

To investigate whether multiple domains of gait variability change during motor maturation and if this change over time could differentiate children with a typical development (TDC) from those with cerebral palsy (CwCP).

This cross-sectional retrospective study included 42 TDC and 129 CwCP, of which 99 and 30 exhibited GMFCS level I and II, respectively. Participants underwent barefoot 3D gait analysis. Age and parameters of gait variability (coefficient of variation of stride-time, stride length, single limb support time, walking speed, and cadence; as well as meanSD for hip flexion, knee flexion, and ankle dorsiflexion) were used to fit linear models, where the slope of the models could differ between groups to test the hypotheses.

Motor-developmental trajectories of gait variability were able to distinguish between TDC and CwCP for all parameters, except the variability of joint angles. CwCP with GMFCS II also showed significantly higher levels of gait variability compared to those with GMFCS I, these levels were maintained across different ages.

This study showed the potential of gait variability to identify and detect the motor characteristics of high functioning CwCP. In future, such trajectories could provide functional biomarkers for identifying children with mild movement related disorders and support the management of expectations.

Gene replacement therapy is a rational therapeutic strategy and clinical intervention for neurodegenerative disorders like Canavan disease, a leukodystrophy caused by biallelic mutations in the aspartoacylase (ASPA) gene. We aimed to investigate whether simultaneous intravenous (i.v.) and intracerebroventricular (i.c.v.) administration of rAAV9-CB6-ASPA provides a safe and effective therapeutic strategy in an open-label, individual-patient, expanded-access trial for Canavan disease. Immunomodulation was given prophylactically prior to adeno-associated virus (AAV) treatment to prevent an immune response to ASPA or the vector capsid. The patient served as his own control, and change from baseline was assessed by clinical pathology tests, vector genomes in the blood, antibodies against ASPA and AAV capsids, levels of cerebrospinal fluid (CSF) N-acetylaspartate (NAA), brain water content and morphology, clinical status, and motor function tests. Two years post treatment, the patient's white matter myelination had increased, motor function was improved, and he remained free of typical severe epilepsy. NAA level was reduced at 3 months and remained stable up to 4 years post treatment. Immunomodulation prior to AAV exposure enables repeat dosing and has prevented an anti-transgene immune response. Dual-route administration of gene therapy may improve treatment outcomes.

Impaired selective motor control, weakness and spasticity represent the key characteristics of motor disability in the context of bilateral spastic cerebral palsy. Independent walking ability is an important goal and training of the gluteal muscles can improve endurance and gait stability. Combining conventional physical excercises with a neuromodulatory, non-invasive technique like repetitive neuromuscular magnetic stimulation probably enhances effects of the treatment. This prospective study aimed to assess the clinical effects of repetitive neuromuscular magnetic stimulation in combination with a personalized functional physical training offered to children and adolescents with bilateral spastic cerebral palsy.

Eight participants Gross Motor Function Classification System level II and III (10.4 ± 2y5m; 50% Gross Motor Function Classification System level II) received a personalized intervention applying functional repetitive neuromuscular magnetic stimulation (12 sessions within 3 weeks; 12,600 total stimuli during each session). At baseline and follow up the following assessments were performed: 10-m-walking-test, 6-min-walking-test, GMFM-66. Six weeks after the end of treatment the patient-reported outcome measure Gait Outcome Assessment List was completed.

GMFM-66 total score improved by 1.4% (p = 0.002), as did scoring in domain D for standing (1.9%, p = 0.109) and domain E for walking, jumping and running (2.6%, p = 0.021). Gait speed or distance walked during 6 min did not improve from baseline to follow up. Patient-reported outcome showed improvement in 4 patients in altogether 14 ratings. Caregiver-reported outcome reported benefits in 3 participants in altogether 10 ratings.

Repetitive neuromuscular magnetic stimulation promises to be a meaningful, non-invasive treatment approach for children and adolescents with bilateral spastic cerebral palsy that could be offered in a resource-efficient manner to a broad number of patients. To further investigate the promising effects of repetitive neuromuscular magnetic stimulation and its mechanisms of action, larger-scaled, controlled trials are needed as well as comprehensive neurophysiological investigations.

To identify and provide a descriptive overview of the development of children, adolescents, and young adults with cerebral palsy (CP) in longitudinal studies; and map areas of focus according to the components of the World Health Organization's International Classification of Functioning, Disability, and Health (ICF).

Longitudinal studies of the development of children, adolescents, and/or young adults with CP were included in this scoping review. A search for eligible studies was conducted in the databases MEDLINE, PubMed, LILACS, EMBASE, Cochrane, CINAHL, and Scopus, and was restricted to the years 2002 to 2022. All outcome measures of the studies were classified into ICF components.

In the 56 studies included, there were 19 438 participants, involving mainly children, followed by adolescents, and lastly young adults. All components of the ICF were investigated and many studies reported outcomes in more than one component. Activity was the most investigated (67.9%; n = 38 studies), followed by body functions and structures (42.9%; n = 24 studies). Participation (14.2%; n = 8 studies) and environmental factors (3.6%; n = 2 studies) were the least studied. None of the studies investigated personal factors as an outcome.

This scoping review provides an overview of studies on the development of children, adolescents, and young adults with CP, using the ICF framework, identifying current areas of focus and gaps in the research. Future studies should target participation, contextual factors, and the transition into adulthood.

The International Classification of Functioning, Disability, and Health can be used to map a range of outcomes through developmental studies. The main outcomes investigated in children with cerebral palsy were activity, and body functions and structures. Little has been explored in participation and contextual factors outcomes over time. The main classification used to stratify the participants was the Gross Motor Function Classification System.

STXBP1-related disorders are among the most common genetic epilepsies and neurodevelopmental disorders. However, the longitudinal epilepsy course and developmental endpoints have not yet been described in detail, which is a critical prerequisite for clinical trial readiness. Here, we assessed 1,281 cumulative patient-years of seizure and developmental histories in 162 individuals with STXBP1-related disorders and established a natural history framework. STXBP1-related disorders are characterized by a dynamic pattern of seizures in the first year of life and high variability in neurodevelopmental trajectories in early childhood. Epilepsy onset differed across seizure types, with 90% cumulative onset for infantile spasms by 6 months and focal-onset seizures by 27 months of life. Epilepsy histories diverged between variant subgroups in the first 2 years of life, when individuals with protein-truncating variants and deletions in STXBP1 (n=39) were more likely to have infantile spasms between 5 and 6 months followed by seizure remission, while individuals with missense variants (n=30) had an increased risk for focal seizures and ongoing seizures after the first year. Developmental outcomes were mapped using milestone acquisition data in addition to standardized assessments including the Gross Motor Function Measure-66 Item Set and the Grasping and Visual-Motor Integration subsets of the Peabody Developmental Motor Scales. Quantification of endpoints revealed high variability during the first five years of life, with emerging stratification between clinical subgroups, most prominently between individuals with and without infantile spasms. We found that individuals with neonatal seizures or early infantile seizures followed by seizure offset by 12 months of life had more predictable seizure trajectories in early to late childhood than compared to individuals with more severe seizure presentations, including individuals with refractory epilepsy throughout the first year. Characterization of anti-seizure medication response revealed age-dependent response over time, with phenobarbital, levetiracetam, topiramate, and adrenocorticotropic hormone effective in reducing seizures in the first year of life, while clobazam and the ketogenic diet were effective in long-term seizure management. Virtual clinical trials using seizure frequency as the primary outcome resulted in wide range of trial success probabilities across the age span, with the highest probability in early childhood between 1 year and 3.5 years. In summary, we delineated epilepsy and developmental trajectories in STXBP1-related disorders using standardized measures, providing a foundation to interpret future therapeutic strategies and inform rational trial design.

To determine the feasibility of an intensive interdisciplinary programme in improving goal and motor outcomes for preschool-aged children with non-progressive neurodisabilities. The primary hypothesis was that the intervention would be feasible.

A single group feasibility study.

An Australian paediatric community therapy provider.

Forty children were recruited. Inclusion criteria were age 2-5 years with a non-progressive neurodisability, Gross Motor Function Classification System (GMFCS) levels III-V or equivalent, and goals relating to mobility, communication and upper limb function. Exclusion criteria included orthopaedic surgery in the past 6 months, unstable hip subluxation, uncontrolled seizure disorder or treadmill training in the past month.

A goal-directed programme of three 2-hour sessions per week for 4 weeks (24 hours total). This consisted of treadmill and overground walking, communication practice, and upper limb tasks tailored by an interdisciplinary team.

Limited-efficacy measures from preintervention (T1) to postintervention (T2) and 4-week follow-up (T3) included the Goal Attainment Scaling (GAS), Canadian Occupational Performance Measure (COPM), Gross Motor Function Measure (GMFM-66) and 10-Metre Walk Test (10MWT). Acceptability, demand, implementation and practicality were also explored.

There were improvements at T2 compared with T1 for all limited-efficacy measures. The GAS improved at T2 (mean difference (MD) 27.7, 95% CI 25.8 to 29.5) as well as COPM performance (MD 3.2, 95% CI 2.8 to 3.6) and satisfaction (MD 3.3, 95% CI 2.8 to 3.8). The GMFM-66 (MD 2.3, 95% CI 1.0 to 3.5) and 10MWT (median difference -2.3, 95% CI -28.8 to 0.0) improved at T2. Almost all improvements were maintained at T3. Other feasibility components were also demonstrated. There were no adverse events.

An intensive interdisciplinary programme is feasible in improving goal and motor outcomes for preschool children with neurodisabilities (GMFCS III-V or equivalent). A randomised controlled trial is warranted to establish efficacy.

ACTRN12619000064101.

Objective, quantitative postural data is limited for individuals who are non-ambulatory, especially for those who have not yet developed trunk control for sitting. There are no gold standard measurements to monitor the emergence of upright trunk control. Quantification of intermediate levels of postural control is critically needed to improve research and intervention for these individuals. Accelerometers and video were used to record postural alignment and stability for eight children with severe cerebral palsy aged 2 to 13 years, under two conditions, seated on a bench with only pelvic support and with additional thoracic support. This study developed an algorithm to classify vertical alignment and states of upright control; Stable, Wobble, Collapse, Rise and Fall from accelerometer data. Next, a Markov chain model was created to calculate a normative score for postural state and transition for each participant with each level of support. This tool allowed quantification of behaviors previously not captured in adult-based postural sway measures. Histogram and video recordings were used to confirm the output of the algorithm. Together, this tool revealed that providing external support allowed all participants: (1) to increase their time spent in the Stable state, and (2) to reduce the frequency of transitions between states. Furthermore, all participants except one showed improved state and transition scores when given external support.