Selective dorsal rhizotomy in non-ambulant children with cerebral palsy: a multi-center prospective study.

PURPOSE: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

The effect of selective dorsal rhizotomy on the improvement of the quality of life of children with stage GMFCS IV and V cerebral palsy: Pain, nursing, positioning, and dressing.

AIM: The objective of this study is to evaluate the benefit of selective dorsal rhizotomy on the quality of life of patients with severe spasticity with significant impairment of gross motor functions (GMFCS stages IV and V) according to 4 items: pain, nursing care, positioning, and dressing. MATERIALS AND METHODS: We conducted a monocentric retrospective cohort study including patients who underwent selective dorsal rhizotomy between March 2008 and May 2022 at the University Hospital of Marseille. RESULTS: Seventy percent of patients showed an improvement in quality of life criteria: dressing, nursing, positioning, and pain at the last follow-up. A small proportion of patients still showed a worsening between the first 2 follow-ups and the last follow-up. Postoperatively, 27.3% of patients were free of joint spasticity treatment, and we have shown that there was a significant decrease in the number of children who received botulinum toxin postoperatively. However, there was no significant reduction in the number of drug treatments or orthopaedic procedures following RDS. For the CPCHILD© scores, an overall gain is reported for GMFCS IV and V patients in postoperative care. The gain of points is more important for GMFCS IV patients. Improvement was mainly observed in 2 domains, "comfort and emotions" and "hygiene and dressing". For the "quality of life" item, only 3 parents out of the 8 noted a positive change. CONCLUSION: Our study shows an improvement in nursing care, positioning, and dressing which are associated with a reduction in pain in children with a major polyhandicap GMFCS IV and V who have benefited from a selective dorsal rhizotomy.

Palliative single-level selective dorsal rhizotomy for children with spastic cerebral palsy Gross Motor Function Classification System level IV and V: a case series and systematic review of the literature.

OBJECTIVE: Single-level selective dorsal rhizotomy (SDR), typically indicated for ambulatory patients, is a controversial topic for severe spastic cerebral palsy (CP) with Gross Motor Function Classification System (GMFCS) level IV or V. The objective of this case series and systematic literature review was to outline the indication and outcome of palliative SDR for nonambulatory patients with CP and GMFCS level IV and V, focusing on improvement of spasticity and of patient and caregiver reported quality of life assessment. METHODS: A retrospective case series of patients with CP and GMFCS level IV or V who underwent single-level SDR at the authors' institution is presented. Furthermore, two databases (PubMed and Embase) were searched and a systematic review with a search string based on the terms "selective dorsal rhizotomy," "cerebral palsy," and "outcome" was conducted. The primary outcome was the reduction of spasticity based on the modified Ashworth scale (MAS). Secondary outcomes were change on the Gross Motor Function Measure-66 (GMFM-66), evaluation of patient-reported outcome measures (PROMs), surgical morbidity, and mortality. RESULTS: Eleven consecutive children under the age of 25 years undergoing palliative single-level SDR were included. All patients showed a reduction in MAS score (mean 1.09 ± 0.66 points) and no surgical morbidity and mortality occurred. For the systematic review results from our case series, in addition to 4 reports, 274 total patients were included. Reduction of spasticity based on MAS score was noted in all studies (mean range 1.09-3.2 points). Furthermore, in 2 studies spasticity of the upper extremities showed a MAS score reduction as well (range 1.7-2.8 points). The GMFM-66 score improved in 72% of the patients, while bladder function improved in 78% of the patients. Based on the PROMs, 92% of the patients/caregivers were satisfied with the outcome and their quality of life after the procedure. Two wound infections (2.7%) and one CSF leak (1.3%) occurred, while no surgery-related deaths were described. CONCLUSIONS: This analysis showed an improvement in spasticity, daily care, and comfort for patients with CP and GMFCS levels IV and V. Larger cohorts analyzing the outcome of palliative single-level SDR, based on the MAS, GMFM-66, and PROMs, are still needed and should be the focus of future studies. Systematic review registration no.: CRD42024495762 (https://www.crd.york.ac.uk/prospero/).

Pain in children with cerebral palsy as reported by parents.

BACKGROUND: Pain in children with cerebral palsy (CP) is a problem that has not received adequate attention in developing countries. The aim of this study was to explore the presence of pain and common sites of pain in children with CP based on age, gender and ambulatory status as determined by the Gross Motor Function Classification System (GMFCS) level. METHODS: This was a cross-sectional study based on data from a CP registry (CPUP-Jordan). Participants were 310 children with CP (mean age: 3.3 ± 2.9 years, range: 5 months to 15.9 years), 77.1% were below 5 years of age, 56.8% were boys and 49% were classified as Levels IV and V of the GMFCS. Parents were asked to indicate whether their child is currently experiencing pain (yes/no); if yes, they were asked to report the sites of pain. RESULTS: Seventy-nine (25.5%) children experienced pain (34.1% had pain in the stomach, 15.2% at the hips and knees and 12.7% in their feet). The presence of pain did not significantly differ by age or gender. More children in GMFCS Levels V (34.7%) and IV (31.6%) experienced pain. Compared with Level V on the GMFCS (non-ambulant children), children in Level I and children in Level III experienced less pain (odds ratio [OR] = 0.25, 95% confidence interval [CI] = 0.087-0.727, p = 0.011; OR = 0.42, 95% CI = 0.18-0.98, p = 0.045, respectively). Pain sites differed with age and GMFCS. CONCLUSIONS: Pain was reported to be less than previous studies. Non-ambulant children experienced more pain. Pain monitoring and management should be a regular practice in rehabilitation for this population, with special attention for non-ambulant children.

Interpretable classification for multivariate gait analysis of cerebral palsy.

BACKGROUND: The Gross Motor Function Classification System (GMFCS) is a widely used tool for assessing the mobility of people with Cerebral Palsy (CP). It classifies patients into different levels based on their gross motor function and its level is typically determined through visual evaluation by a trained expert. Although gait analysis is commonly used in CP research, the functional aspects of gait patterns has yet to be fully exploited. By utilizing the gait patterns to predict GMFCS, we can gain a more comprehensive understanding of how CP affects mobility and develop more effective interventions for CP patients. RESULT: In this study, we propose a multivariate functional classification method to examine the relationship between kinematic gait measures and GMFCS levels in both normal individuals and CP patients with varying GMFCS levels. A sparse linear functional discrimination framework is utilized to achieve an interpretable prediction model. The method is generalized to handle multivariate functional data and multi-class classification. Our method offers competitive or improved prediction accuracy compared to state-of-the-art functional classification approaches and provides interpretable discriminant functions that can characterize the kinesiological progression of gait corresponding to higher GMFCS levels. CONCLUSION: We generalize the sparse functional linear discrimination framework to achieve interpretable classification of GMFCS levels using kinematic gait measures. The findings of this research will aid clinicians in diagnosing CP and assigning appropriate GMFCS levels in a more consistent, systematic, and scientifically supported manner.

Epidemiology of scoliosis in cerebral palsy: A population-based study at skeletal maturity.

AIM: This study investigated the prevalence of scoliosis in a large, population-based cohort of individuals with cerebral palsy (CP) at skeletal maturity to identify associated risk factors that may inform scoliosis surveillance. METHODS: Young people with CP born between 1990 and 1992 were reviewed through routine orthopaedic review or a transition clinic. Classification of CP was recorded by movement disorder, distribution, gross and fine motor function. Clinical examination was undertaken and those with clinical evidence of scoliosis or risk factors had radiographs of the spine. Scoliosis severity was measured and categorised by Cobb angle. RESULTS: Two hundred and ninety-two individuals were evaluated (78% of the birth cohort) at a mean age of 21 years, 4 months (range 16-29 years). Scoliosis (Cobb angle >10°) was found in 41%, with strong associations to the Gross Motor Function Classification System (GMFCS), Manual Abilities Classification System (MACS) and dystonic/mixed movement disorders. Those at GMFCS V were 23.4 times (95%CI 9.9-55.6) more likely to develop scoliosis than those at GMFCS I. Severe curves (Cobb >40°, 13% of the cohort) were found almost exclusively in those functioning at GMFCS IV and V, and were 18.2 times (95%CI 6.9-48.5) more likely to occur in those with dystonia than those with spasticity. CONCLUSIONS: Scoliosis was very common in young people with CP, with prevalence and severity strongly associated with GMFCS and MACS level and dystonic movement disorder. Severe curves were almost exclusively found in non-ambulant children. Clinical screening for scoliosis should occur for all children with CP, with radiographic surveillance focusing on those functioning at GMFCS IV and V.

Live births with cerebral palsy at a tertiary maternity hospital: incidence and associated risk factors over a 17-year period.

The aim of this retrospective study was to establish the incidence and associated risk factors for cerebral palsy (CP) at a tertiary maternity hospital in the UK between 2000-2016. We identified CP patients from our electronic coding system using ICD codes. Multiple independent variables for all live births born during this period were included in a univariate and multivariate logistic regression (LR) to identify associations between these and CP. We identified 130 CP children out of 87318 live births. Univariate LR determined male sex, birth weight <2500 g, gestational age of ≤36 weeks, Small-for-gestational-age, 1-and 5-minute Apgar score <9, neonatal intensive care unit (NICU) admission, multiple births, breech, emergency Caesarean section and delivery between 16.00-20.00 as significant risk factors. In the multivariate LG male sex, 1-minute Apgar <9, 5-minute Apgar <5 and admission to NICU remained as significant risk factors. The risk for delivery between 16.00-19.59 was nearly significant. There was a significant association between NICU admission and moderate-severe CP. Our CP incidence of 0.149% is at the lower end of the incidence spectrum of international comparisons.Impact StatementWhat is already known on this subject? The historic reported incidence of cerebral palsy (CP) ranges from 1.1 to 3.6 cases per 1000 live births, with birth weight <2500g, birth <28 weeks of gestation, Apgar scores ≤4 and male sex having been associated with an increased incidence.What do the results of this study add? This is a large series of live births from a tertiary maternity hospital with a comparative low CP incidence of 0.149%, despite the hospital dealing with many complex pregnancies and deliveries. We identified that already an Apgar score of <9 at 1 minute (significant) and births between 16.00-20.00 (non-significant) were associated with an increased risk to develop CP but not with a specific day of the week.What are the implications of these findings for clinical practice and/or future research? Our significant association between a 1-minute Apgar score of <9 and CP stresses the importance of immediate efficient resuscitation already for babies with a 1-minute score as high as 8. The increased CP risk for deliveries between 16.00-19.59 may be linked to staffing issues and needs further exploration.What this paper addsNew data from a single maternity hospitalAnalysis of risk factorsGMFCS distribution.

The effect of combined transcranial pulsed current stimulation and transcutaneous electrical nerve stimulation on lower limb spasticity in children with spastic cerebral palsy: a randomized and controlled clinical study.

BACKGROUND: In the current study, we applied a combination of non-invasive neuromodulation modalities concurrently with multiple stimulating electrodes. Specifically, we used transcranial pulsed current stimulation (tPCS) and transcutaneous electrical nerve stimulation (TENS) as a novel strategy for improving lower limb spasticity in children with spastic cerebral palsy (SCP) categorized on levels III-V of the Gross Motor Function Classification System (GMFCS) with minimal side effects. METHODS: Sixty-three SCP children aged 2-12 years, who were classified on levels III-V of the GMFCS were randomly assigned to one of two groups, resulting in 32 children in the experimental group and 31 children in the control group. The experimental group underwent a combination therapy of tPCS (400 Hz, 1 mA cerebello-cerebral stimulation) and TENS (400 Hz, max 10 mA) for 30 min, followed by 30 min of physiotherapy five times per week for 12 weeks. The control group underwent physiotherapy only 30 mins per day five times per week for 12 weeks. In total, all groups underwent 60 treatment sessions. The primary outcome measures were the Modified Ashworth Scale (MAS) and Modified Tardieu Scale (MTS). Evaluations were performed 3 days before and after treatment. RESULTS: We found a significant improvement in MAS and MTS scores of the lower limbs in the experimental group compared to the control group in the hip adductors (Left: p = 0.002; Right: p = 0.002), hamstrings (Left: p = 0.001; Right: p < 0.001, and gastrocnemius (Left: p = 0.001; Right: p = 0.000). Moreover, MTS scores of R1, R2 and R2-R1 in left and right hip adduction, knee joint, and ankle joint all showed significant improvements (p ≤ 0.05). Analysis of MAS and MTS scores compared to baseline scores showed significant improvements in the experimental group but declines in the control group. CONCLUSION: These results are among the first to demonstrate that a combination of tPCS and TENS can significantly improve lower limb spasticity in SCP children classified on GMFCS levels III-V with minimal side effects, presenting a novel strategy for addressing spasticity challenges in children with severe SCP. TRIAL REGISTRATION: ChiCTR.org, ChiCTR1800020283, Registration: 22 December 2018 (URL: http://www.chictr.org.cn/showproj.aspx?proj=33953 ).

The effect of GMFCS level, age, sex, and dystonia on multi-dimensional outcomes after selective dorsal rhizotomy: prospective observational study.

PURPOSE: Investigate the effect of age category (1-9 years vs 10-18 years), sex, Gross Motor Function Classification System (GMFCS) level, and presence of dystonia on changes in eight function test parameters 24 months after selective dorsal rhizotomy (SDR). METHODS: Prospective, single-center study of all children aged 3-18 years with bilateral cerebral palsy with spasticity who underwent SDR at a tertiary pediatric neurosurgery center between 2012 and 2019. A linear mixed effects model was used to assess longitudinal changes. RESULTS: From 2012 to 2019, 42 children had follow-up available at 24 months. Mean GMFM-66 scores increased after SDR (mean difference 5.1 units: 95% CI 3.05-7.13, p < 0.001). Statistically significant improvements were observed in CPQoL, PEDI Self-care and Mobility, 6MWT, Gillette, and MAS scores. There was no significant difference in the improvements seen for age category, sex, GMFCS level, and presence of dystonia for most of the parameters tested (5/8, 6/8, 5/8, and 6/8 respectively). CONCLUSION: SDR may improve gross and fine motor function, mobility and self-care, quality of life, and overall outcome based on extensive scoring parameter testing at 24 months. Atypical patient populations may benefit from SDR if appropriately selected. Multi-center, prospective registries investigating the effect of SDR are required.

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

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

Stability of the gross motor function classification system in children with cerebral palsy for two years.

BACKGROUND: The prognosis of gross motor function is a major concern for therapy and intervention in children with cerebral palsy (CP). The classification system for gross motor function, the Gross Motor Function Classification System (GMFCS), is actively studied because it could be useful in the communication between professionals and families. This study aimed to verify the stability of GMFCS over 2 years in children with CP aged 2-12 years. METHODS: The GMFCS level of 100 children with CP who underwent rehabilitation therapy in hospitals or who attended special elementary schools in South Korea were collected in the study. The agreements across three measurement points were analyzed in these children. RESULTS: The weighted kappa coefficients were statistically significant (p < .05). The coefficients ranged from 0.690 to 0.789 in children with CP aged 2-12 years. The lowest coefficient of 0.557 was observed in children with CP aged 2-4 years between the first and third measurements points. CONCLUSIONS: The results provided evidence of GMFCS stability for the first year and change of the GMFCS during the two-year study period in children aged 2-4 years. Moreover, the findings indicate that the stability of GMFCS varies with time, duration, and age. It is recommended that GMFCS assessments be performed periodically, which are even more necessary for children with CP aged 2-4 years.

Frequency distribution in intraoperative stimulation-evoked EMG responses during selective dorsal rhizotomy in children with cerebral palsy-part 2: gender differences and left-biased asymmetry.

INTRODUCTION: Spinal reflexes reorganize in cerebral palsy (CP), producing hyperreflexia and spasticity. CP is more common among male infants, and gender might also influence brain and spinal-cord reorganization. This retrospective study investigated the frequency of higher-graded EMG responses elicited by electrical nerve-root stimulation during selective dorsal rhizotomy (SDR), prior to partial nerve- root deafferentation, considering not only segmental level and body side, but also gender. METHODS: Intraoperative neuromonitoring (IOM) was used in SDR to pinpoint the rootlets most responsible for exacerbated stimulation-evoked EMG patterns recorded from lower-limb muscle groups. Responses were graded according to an objective response-classification system, ranging from no abnormalities (grade 0) to highly abnormal (grade 4+), based on ipsilateral spread and contralateral involvement. Non-parametric analysis of data with repeated measures was primarily used in investigating the frequency distribution of these various EMG response grades. Over 7000 rootlets were stimulated, and the results for 65 girls and 81 boys were evaluated, taking changes in the composition of patient groups into account when considering GMFCS levels. RESULTS: The distribution of graded EMG responses varied according to gender, laterality, and level. Higher-graded EMG responses were markedly more frequent in the boys and at lower segmental levels (L5, S1). Left-biased asymmetry in higher-graded rootlets was also more noticeable in the boys and in patients with GMFCS level I. A close link was observed between higher-grade assessments and left-biased asymmetry. CONCLUSIONS: Detailed insight into the patient's initial spinal-neurofunctional state prior to deafferentation suggests that differences in asymmetrical spinal reorganization might be attributable to a hemispheric imbalance.

Machine Learning to Quantify Physical Activity in Children with Cerebral Palsy: Comparison of Group, Group-Personalized, and Fully-Personalized Activity Classification Models.

Pattern recognition methodologies, such as those utilizing machine learning (ML) approaches, have the potential to improve the accuracy and versatility of accelerometer-based assessments of physical activity (PA). Children with cerebral palsy (CP) exhibit significant heterogeneity in relation to impairment and activity limitations; however, studies conducted to date have implemented "one-size fits all" group (G) models. Group-personalized (GP) models specific to the Gross Motor Function Classification (GMFCS) level and fully-personalized (FP) models trained on individual data may provide more accurate assessments of PA; however, these approaches have not been investigated in children with CP. In this study, 38 children classified at GMFCS I to III completed laboratory trials and a simulated free-living protocol while wearing an ActiGraph GT3X+ on the wrist, hip, and ankle. Activities were classified as sedentary, standing utilitarian movements, or walking. In the cross-validation, FP random forest classifiers (99.0-99.3%) exhibited a significantly higher accuracy than G (80.9-94.7%) and GP classifiers (78.7-94.1%), with the largest differential observed in children at GMFCS III. When evaluated under free-living conditions, all model types exhibited significant declines in accuracy, with FP models outperforming G and GP models in GMFCS levels I and II, but not III. Future studies should evaluate the comparative accuracy of personalized models trained on free-living accelerometer data.

The relationship between motor abilities and quality of life in children with severe multiple disabilities.

BACKGROUND: This study aimed to determine the relationship between motor abilities and quality of life in children with severe multiple disabilities. METHODS: In this cross-sectional study, motor abilities of 29 children (mean age 9.8 years; 45% girls) with severe multiple disabilities [IQ < 25; Gross Motor Function Motor Classification System level V] were measured with the MOtor eVAluation in Kids with Intellectual and Complex disabilities (Movakic) questionnaire (completed by the child's physical therapist). Quality of life was measured with the Quality of Life-Profound Multiple Disabilities (QoL-PMD) questionnaire (completed by the child's parents). RESULTS: A significantly moderate to high correlation was found between the total scores on the Movakic and the QoL-PMD (r = 0.40, P = 0.03), indicating that higher scores in motor abilities are associated with a higher level of quality of life. Furthermore, significantly moderate to high correlations were found between the total score on the Movakic and the dimension Physical Well-Being, Development and Activities of the Qol-PMD. In multiple linear regression models, all significant bivariate relationships between the Movakic total scores and QoL-PMD dimensions remained significant after controlling for the Gross Motor Function Motor Classification System level. CONCLUSIONS: In these children with severe multiple disabilities, motor abilities (as measured by Movakic) are moderately related to quality of life (as measured by the QoL-PMD).

Associations between impairments and activity limitations components of the international classification of functioning and the gross motor function and subtypes of children with cerebral palsy.

[Purpose] Cerebral palsy (CP) encompasses a group of disorders of movement and posture with wide ranges of impairments, activity limitations and participation restrictions. Guiding management of children with CP by the ICF model is important to deliver quality services. This study aimed to explore relationship between CP subtypes and the Gross Motor Function Classification System-Expanded and Revised (GMFCS-E&R) and to examine differences in distribution of impairments and activity limitations across CP subtypes and GMFCS-E&R levels. [Participants and Methods] 70 children with CP (mean age: 6.5 ± 2.9 years) were classified using CP subtypes and GMFCS-E&R. Research assistants examined impairments including: scoliosis, scissoring, and inability to bear weight. Parents described their children's transfers and functional mobility. [Results] CP subtypes and GMFCS-E&R levels were significantly associated. Scissoring and scoliosis were predominant in children in levels IV and V of the GMFCS-E&R. Only scoliosis was predominant in children with quadriplegia. Transfer activities and functional mobility were more limited in children with quadriplegia and in level V of the GMFCS-E&R. [Conclusion] Impairments and activity limitations components of the ICF can be differentiated by CP subtypes and GMFCS-E&R. Clinicians can use the two classification in providing comprehensive and individualized services for children with CP and their families.

Association of Gross Motor Function Classification System Level and School Attendance with Bone Mineral Density in Patients With Cerebral Palsy.

The present study aimed to evaluate bone mineral density (BMD) in children and adolescents with cerebral palsy (CP) and to critically analyze the effects of a variety of factors, particularly the Gross Motor Function Classification System (GMFCS) level, the Caregiver Priorities and Child Health Index of Life with Disabilities questionnaire, and the Pediatric Outcomes Data Collection Instrument (PODCI), on BMD. Fifty patients with CP who underwent dual-energy X-ray absorptiometry were included. Collected data included the extent of involvement, muscle tone, demographic data, factors determined through chart review, and laboratory results. Factors associated with BMD in this group were analyzed by performing multiple regression analysis. The mean Z-scores in male and female patients were -3.252 ± 1.822 and -3.789 ± 1.764, respectively, in the proximal part of the femur and -2.219 ± 1.323 and -2.451 ± 1.434, respectively, in the lumbar spine. In multiple regression analysis, the GMFCS level and the average frequency of missed school in the PODCI were significant factors associated with both femur and lumbar spine BMD. Both the GMFCS level and school attendance were independently associated with BMD and should be considered for the prevention and management of osteoporosis in patients with CP.

Selective dorsal rhizotomy for the treatment of severe spastic cerebral palsy: efficacy and therapeutic durability in GMFCS grade IV and V children.

BACKGROUND: Selective dorsal rhizotomy (SDR) has been established as an effective surgical treatment for spastic diplegia. The applicability of SDR to the full spectrum of spastic cerebral palsy and the durability of its therapeutic effects remain under investigation. There are currently limited data in the literature regarding efficacy and outcomes following SDR in Gross Motor Function Classification System (GMFCS) IV and V patients. Intrathecal baclofen has traditionally been the surgical treatment of choice for these patients. When utilised primarily as a treatment for the relief of spasticity, it is proposed that SDR represents a rational and effective treatment option for this patient group. We report our outcomes of SDR performed on children with severe cerebral palsy (GMFCS grade IV and V). The commensurate improvement in upper as well as lower limb spasticity is highlighted. Apparent benefit to urological function following SDR in this patient group is also discussed. METHOD: A retrospective review of prospectively collected data for 54 paediatric patients with severe cerebral palsy (GMFCS IV-V) who received SDR plus specialised physiotherapy. Mean age was 10.2 years (range, 3.0-19.5). SDR guided by electrophysiological monitoring was performed by a single experienced neurosurgeon. All subjects received equivalent physiotherapy. The primary outcome measure was change to the degree of spasticity following SDR. Spasticity of upper and lower limb muscle groups were quantified and standardised using the Ashworth score. Measures were collected at baseline and at 2-, 8- and 14-month postoperative intervals. In addition, baseline and 6-month postoperative urological function was also evaluated as a secondary outcome measure. RESULTS: The mean lower limb Ashworth score at baseline was 3.2 (range, 0-4). Following SDR, significant reduction in lower limb spasticity scores was observed at 2 months and maintained at 8 and 14 months postoperatively (Wilcoxon rank, p < 0.001). The mean reduction at 2, 8 and 14 months was 3.0, 3.2 and 3.2 points respectively (range, 1-4), confirming a sustained improvement of spasticity over a 1-year period of follow-up. Significant reduction in upper limb spasticity scores following SDR was also observed (mean, 2.9; Wilcoxon rank, p < 0.001). Overall, the improvement to upper and lower limb tone following SDR-generally to post-treatment Ashworth scores of 0-was clinically and statistically significant in GMFCS IV and V patients. Urological assessment identified pre-existing bladder dysfunction in 70% and 90% of GMFCS IV and V patients respectively. Following SDR, improvement in urinary continence was observed in 71% of affected GMFCS IV and 42.8% of GMFCS V patients. No serious postoperative complications were identified. CONCLUSIONS: We conclude that SDR is safe and-in combination with physiotherapy-effectively reduces spasticity in GMFCS grade IV and V patients. Our series suggests that spastic quadriplegia is effectively managed with significant improvements in upper limb spasticity that are commensurate with those observed in lower limb muscle groups. These gains are furthermore sustained more than a year postoperatively. In light of these findings, we propose that SDR constitutes an effective treatment option for GMFCS IV and V patients and a rational alternative to intrathecal baclofen.

Machine learning algorithms for activity recognition in ambulant children and adolescents with cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is the most common physical disability among children (2.5 to 3.6 cases per 1000 live births). Inadequate physical activity (PA) is a major problem effecting the health and well-being of children with CP. Practical, yet accurate measures of PA are needed to evaluate the effectiveness of surgical and therapy-based interventions to increase PA. Accelerometer-based motion sensors have become the standard for objectively measuring PA in children and adolescents; however, current methods for estimating physical activity intensity in children with CP are associated with significant error and may dramatically underestimate HPA in children with more severe mobility limitations. Machine learning (ML) models that first classify the PA type and then predict PA intensity or energy expenditure using activity specific regression equations may be more accurate than standalone regression models. However, the feasibility and validity of ML methods has not been explored in youth with CP. Therefore, the purpose of this study was to develop and test ML models for the automatic identification of PA type in ambulant children with CP. METHODS: Twenty two children and adolescents (mean age: 12.8 ± 2.9 y) with CP classified at GMFCS Levels I to III completed 7 activity trials while wearing an ActiGraph GT3X+ accelerometer on the hip and wrist. Trials were categorised as sedentary (SED), standing utilitarian movements (SUM), comfortable walking (CW), and brisk walking (BW). Random forest (RF), support vector machine (SVM), and binary decision tree (BDT) classifiers were trained with features extracted from the vector magnitude (VM) of the raw acceleration signal using 10 s non-overlapping windows. Performance was evaluated using leave-one-subject out cross validation. RESULTS: SVM (82.0-89.0%) and RF (82.6-88.8%) provided significantly better classification accuracy than BDT (76.1-86.2%). Hip (82.7-85.5%) and wrist (76.1-82.6%) classifiers exhibited comparable prediction accuracy, while the combined hip and wrist (86.2-89.0%) classifiers achieved the best overall performance. For all classifiers, recognition accuracy was excellent for SED (94.1-97.9%), good to excellent for SUM (74.0-96.6%) and brisk walking (71.5-86.0%), and modest for comfortable walking (47.6-70.4%). When comfortable and brisk walking were combined into a single walking class, recognition accuracy ranged from 90.3 to 96.5%. CONCLUSIONS: ML methods provided acceptable classification accuracy for detection of a range of activities commonly performed by ambulatory children with CP. The resultant models can help clinicians more effectively monitor bouts of brisk walking in the community. The results indicate that 2-step models that first classify PA type and then predict energy expenditure using activity specific regression equations are worthy of exploration in this patient group.

Differences in Body Composition According to Gross Motor Function in Children With Cerebral Palsy.

OBJECTIVE: To assess differences in body composition according to gross motor function in children with cerebral palsy (CP) compared with healthy controls. DESIGN: Retrospective case-control study. SETTING: Tertiary referral center for CP. PARTICIPANTS: Participants (N=146) comprised consecutive patients with CP (n=100; mean age, 11.5±4.2y) who were admitted for orthopedic surgery between May 2014 and March 2016 and typically developing children (TDC, n=46; control group). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Bioelectrical impedance analysis (BIA) was used to assess body composition, including body fat, soft lean mass (SLM), fat-free mass (FFM), skeletal muscle mass (SMM), body cell mass (BCM), bone mineral content (BMC), and basal metabolic rate. Body composition measures were compared according to Gross Motor Function Classification System (GMFCS) level, as well as between children with CP and TDC. RESULTS: Children with CP with GMFCS levels IV and V had a lower height, weight, and body mass index than those with GMFCS levels I to III. Children with CP with GMFCS levels IV and V had a significantly lower SLM, SLM index, FFM, FFM index, SMM, SMM index, BCM, BCM index, BMC, and BMC index than those with GMFCS levels I to III and TDC. GMFCS level significantly affected SLM and BMC. CONCLUSIONS: Body composition analysis using BIA showed that nonambulatory children with CP had significantly lower FFM, SLM, SMM, BCM, and BMC than ambulatory children with CP and TDC. However, further study is required to allow the use of BIA as a valid nutritional assessment tool in patients with CP.

Medication, rehabilitation and health care consumption in adults with cerebral palsy: a population based study.

OBJECTIVE: To evaluate medication, rehabilitation and healthcare consumption in adults with CP as a function of Gross Motor Function Classification System (GMFCS) level. DESIGN: Questionnaire-based cross-sectional study. SETTING: Brittany, a French county. SUBJECTS: Adults with cerebral palsy. INTERVENTIONS: Questionnaires relating to drugs, orthotic devices, mobility aids, rehabilitation and medical input were sent to 435 members of a unique regional French network dedicated to adults with cerebral palsy. The questionnaire was completed by the participant or a helper if necessary. RESULTS: Of the 282 responders, 7.8% had a GMFCS level of I, 14.2% II, 17.7% III, 29.1% IV and 31.2% V. Participants consumed a large amount of healthcare. Almost three-quarters took orally administered drugs, of which antispastic and antiepileptic drugs were among the most frequent. Nearly all patients had at least one type of rehabilitation, 87.2% had physiotherapy, 78% used at least one mobility aid and 69.5% used at least one orthotic device. The frequency of numerous inputs increased with GMFCS level. Specificities were found for each GMFCS level, e.g. participants with GMFCS level IV and V had a high level of medical input and a greater use of trunk-supporting devices, antireflux and laxative. Profiles could be established based on GMFCS levels. CONCLUSIONS: Adults with cerebral palsy use a large amount of drugs, mobility aids, orthotic devices, rehabilitation and medical input. Healthcare is targeted at cerebral palsy-related issues. GMFCS is a determinant of healthcare consumption and thus a useful tool for clinical practice to target care appropriately.