Cerebral Palsy and Motor Impairment After Extreme Prematurity: Prediction of Diagnoses at Ages 2 and 10 Years.

OBJECTIVE: To identify perinatal factors in children born extremely preterm (EP) that were associated with motor impairment (MI) at 2 and 10 years of age and develop a predictive algorithm to estimate the risk of MI during childhood. STUDY DESIGN: Participants of the Extremely Low Gestational Age Newborns Study (ELGANS) were classified as: no MI, MI only at 2 years, MI only at 10 years, and MI at both 2 and 10 years, based on a standardized neurological examination at 2 and the Gross Motor Function Classification System (GMFCS) at 10 years of age. Least Absolute Shrinkage and Selection Operator (LASSO) regression was used to develop the final predictive model. RESULTS: Of the 849 study participants, 64 (7.5%) had a diagnosis of MI at both 2 and 10 years and 63 (7.4%) had a diagnosis of MI at 1 visit but not the other. Of 22 total risk factors queried, 4 variables most reliably and accurately predicted MI: gestational age, weight z-score growth trajectory during neonatal intensive care unit (NICU) stay, ventriculomegaly, and cerebral echolucency on head ultrasound. By selecting probability thresholds of 3.5% and 7.0% at ages 2 and 10, respectively, likelihood of developing MI can be predicted with a sensitivity and specificity of 71.2%/72.1% at age 2 and 70.7%/70.7% at age 10. CONCLUSION: In our cohort, the diagnosis of MI at 2 years did not always predict a diagnosis of MI at 10 years. Specific risk factors are predictive of MI and can estimate an individual infant's risk at NICU discharge of MI at age 10 years.

Increased Upper Extremity Muscle Mass in Ambulatory Children with Cerebral Palsy.

AIM: To compare muscle mass in the upper and lower extremities between ambulatory children with cerebral palsy (CP) and typically developing (TD) children. MATERIALS AND METHODS: A total of 21 children aged 2 to 12 years with CP and a Gross Motor Function Classification System (GMFCS) level of I, II, or III were matched with 21 TD children for age, sex, and body mass index. The lean body mass (LBM) of each extremity was calculated from whole-body dual-energy X-ray absorptiometry. RESULTS: The LBM of the upper extremities was greater in children with CP compared to TD children, and the difference was significant in the GMFCS level II group (1340.6 g vs. 1004.2 g, p = 0.027). There was no significant difference in the LBM of the lower extremities between the CP and TD groups (p = 0.190). The ratio of lower extremity LBM to total extremity LBM was lower in children with CP, while the ratio of upper extremity LBM to total extremity LBM was higher in children with CP (73.2% vs. 78.5% [p < 0.001] and 26.7% vs. 21.5% [p < 0.001], respectively). CONCLUSIONS: Ambulatory children with CP, especially in the GMFCS level II group, exhibit greater muscle mass in the upper extremities compared to TD children.

Prevalence of Sensory Processing Deficits in Children with Spastic Cerebral Palsy - An Indian Caregiver's Perspective.

This study aims to analyze the prevalence and patterns of sensory processing deficits (SPD) in Indian children with spastic cerebral palsy (CP) using child sensory profile-2 (CSP-2) caregiver questionnaire. The authors surveyed 230 caregivers of children aged 3 to 14 y with spastic CP, using CSP-2. The difference in prevalence and distribution of SPDs among the CP subtypes and Gross Motor Function Classification System (GMFCS) levels was done. Overall prevalence of "Definite" (>2 SD) SPDs was 83%. Forty-seven percent had definite SPDs in more than one sensory subsection. Prevalence of definite SPDs was similar among the spastic CP subtypes. "Conduct" domain had more affection among hemiplegics and quadriplegics. "Avoiding" pattern was observed more in quadriplegics and "Seeking" pattern was observed less in diplegics. Severe GMFCS levels had more definite sensory processing deficits. SPDs are highly prevalent in children with spastic CP with unique patterns of affection among the spastic CP subtypes.

Psychometric Evaluation of the Persian Version of the Functional Mobility Scale: Assessing Validity and Reliability.

AIM: This study aimed to assess the content, concurrent validity, test-retest, and intra-rater reliability of the Persian version of the Functional Mobility Scale (FMS) for evaluating functional mobility in children and adolescents with cerebral palsy (CP). METHODS: Following international standards for measurement translation, we developed a Persian version of the FMS. A total of 160 participants took part in this study. Concurrent validity was assessed using Spearman's coefficient to correlate with the Gross Motor Function Classification System (GMFCS). Test-retest (n = 30) and intra-rater (n = 30) reliability of the FMS were also examined by Cohen's weighted kappa coefficient. RESULTS: Concurrent validity ranged from -0.912 to -0.941 for children and -0.912 to -0.936 for adolescents. Test-retest ranged from 0.902 to 1. Intra-rater ranged from 0.933 to 0.987. CONCLUSION: The Persian version of the FMS demonstrates very strong validity and almost perfect reliability for assessing and classifying functional mobility in children and adolescents with CP. These findings suggest that this tool could be a useful addition to clinical practice and research for the Persian-speaking population.

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

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

Can developmental trajectories in gait variability provide prognostic clues in motor adaptation among children with mild cerebral palsy? A retrospective observational cohort study.

Aim: 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). Methods: 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. Results: 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. Interpretation: 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.

Scoliosis in children with severe cerebral palsy: a population-based study of 206 children at GMFCS levels III-V.

PURPOSE: To evaluate the prevalence of scoliosis and the rate of scoliosis progression in children with severe cerebral palsy (CP) at GMFCS levels III-V. METHODS: Two hundred and six children (86 girls, 120 boys), born 2002-2008, were recruited from The Norwegian Quality and Surveillance Registry for Cerebral Palsy (NorCP). Inclusion criteria were bilateral CP and GMFCS levels III-V. Scoliosis was evaluated annually by examination of the spine by a physiotherapist. Radiographic examination was performed in children with moderate or severe scoliosis at clinical evaluation. The Cobb angle was used as a measure of curve magnitude. RESULTS: Scoliosis, defined as Cobb angle ≥ 10°, occurred in 121 children (59%). Severe scoliosis (Cobb angle ≥ 40°) developed in 80 of the 206 patients (39%) at a mean age of 10.9 years (range 5-16) and was more prevalent in children at GMFCS level V (62%) than at levels IV (19%) and III (6%). Initial Cobb angle, Cobb angle ≥ 30° at age 10 years, and GMFCS level V were independent risk factors for severe scoliosis. In children at GMFCS level V, the rate of scoliosis progression decreased with age from a mean of 9.7° per year at age 3-5 years to 2-3° per year in children ≥ 11 years. CONCLUSIONS: The prevalence of scoliosis among children with CP increased with decreasing motor functional level. The most important risk factors for progression of scoliosis were high initial Cobb angle, Cobb angle ≥ 30° at age 10 years, and GMFCS level V.

The association between isometric strength and cognitive function in adults with cerebral palsy.

Background: The literature supports quantifying the maximum force/tension generated by one's forearm muscles such as the hand grip strength (HGS) to screen for physical and cognitive frailty in older adults. Thus, we postulate that individuals with cerebral palsy (CP), who are at higher risk for premature aging, could benefit from tools that objectively measure muscle strength as a functional biomarker to detect frailty and cognitive decline. This study assesses the clinical relevancy of the former and quantifies isometric muscle strength to determine its association with cognitive function in adults with CP. Methods: Ambulatory adults with CP were identified from a patient registry and were enrolled into this study. Peak rate of force development (RFD) and maximum voluntary isometric contraction of the quadriceps were measured using a commercial isokinetic machine, while HGS was collected with a clinical dynamometer. Dominant and non-dominant side were identified. Standardized cognitive assessments, including the Wechsler Memory and Adult Intelligence Scales IV, Short Test of Mental Status, and the Patient-Reported Outcomes Measurement Information System (PROMIS®) were used to evaluate cognitive function. Results: A total of 57 participants (32 females; mean age 24.3 [SD 5.3]; GMFCS levels I-IV) were included in the analysis. Although dominant and non-dominant RFD and HGS measures were associated with cognitive function, non-dominant peak RFD showed the strongest associations with cognitive function. Conclusion: RFD capacity may reflect age-related neural and physical health and could be a better health indicator than HGS in the CP population.

Stability of the Gross Motor Function Classification System in children with cerebral palsy in the two to four year age band.

PURPOSE: This study aimed to investigate the stability of the Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) in the two to four year age band, the frequency at which children were reclassified, and trends of reclassification to higher or lower ability. METHODS: This retrospective study included 164 children with CP between 24 to 48 months old with two or more GMFCS ratings at least 12 months apart between their second and fourth birthdays. GMFCS ratings were captured closest to 24, 36, and 48 months. Stability and reclassification trends were analyzed using inferential statistics. Frequency of reclassification, age at ratings, duration between ratings, and corresponding change rate were analyzed using descriptive statistics. RESULTS: When comparing ratings closest to second and fourth birthdays, a linear weighted kappa of 0.726 was found. Of the total population, 46.95% changed GMFCS levels at any time between two to four years of age, the majority of which were reclassified to a higher level of ability. CONCLUSION: Findings suggest the GMFCS is less stable in the two to four year age band compared to older age bands. Due to the importance of providing accurate guidance for caregivers and high rate of reclassification, it is recommended GMFCS levels be reassessed every six months during this period of time.

Relationship Between Epileptic Activity and Developmental Outcome in KCNQ2-Related Epilepsy.

BACKGROUND: We aim to describe a cohort of patients with KCNQ2-related epilepsy and evaluate the relationship between epileptic activity and developmental outcome. This topic is relevant for the selection of clinical end points in future clinical trials, since cessation of seizures may or may not be the most important outcome. METHODS: This retrospective cohort study of children with self-limited (familial) neonatal epilepsy and developmental and epileptic encephalopathy due to pathogenic variants in KCNQ2 was conducted between 2019 and 2021. We collected clinical, therapeutic, and genetic information. Available electroencephalographic recordings were reviewed by a neurophysiologist. Gross motor function was determined using the Gross Motor Function Classification System (GMFCS). The Vineland Adaptive Behavior Composite standard score (ABC SS) was used to measure adaptive functioning. RESULTS: Among 44 children (mean age 8.1 ± 4.0 years, 45.5% were male), 15 of 44 had S(F)NE, and 29 of 44 had DEE. Delayed seizure freedom was more frequent in DEE than in S(F)NE (P = 0.025), but no correlation was observed between age at seizure freedom and developmental outcome in patients with DEE. Multifocal interictal epileptiform abnormalities at epilepsy onset were more frequent in DEE than in S(F)NE (P = 0.014), and were associated with higher GMFCS (P = 0.027) and lower ABC SS (P = 0.048) in patients with DEE. Disorganized background activity at follow-up was more frequent in DEE than in S(F)NE (P = 0.001), and was associated with higher GMFCS levels (P = 0.009) and lower ABC SS (P = 0.005) in patients with DEE. CONCLUSIONS: This study shows a partial correlation between epileptic activity and developmental outcome in KCNQ2-related epilepsy.

[Relationship between the gross motor function classification system and hip and lumbar spine development in children with spastic cerebral palsy].

OBJECTIVE: To investigate the relationship among the gross motor function classification system (GMFCS)and the development of hip joint and lumbar spine in children with spastic cerebral palsy. METHODS: The clinical data of 125 children with spastic cerebral palsy admitted from January 2018 to July 2021 were retrospectively analyzed. There were 85 males and 40 females, aged from 4 to 12 years old with an average of (8.4±2.9) years. According to GMFCS, the patients were divided into gradeⅠ, Ⅱ, Ⅲ and Ⅳ groups. There were 27 cases in gradeⅠgroup, 40 cases in gradeⅡgroup, 35 cases in grade Ⅲ group and 23 cases in grade Ⅳ group. The migration percentage(MP), central edge angle(CE), neck-shaft angle(NSA), acetabular index(AI) were measured by the radiograph of pelvis, abnormal parameters were selected to evaluate the relationship between different GMFCS grades and hip joint development. Lumbar sagittal Cobb angle, lumbar sacral angle, lumbar lordosis index and apical distance were measured by lateral lumbar radiographs to evaluate the relationship between different GMFCS grades and lumbar spine development. RESULTS: ①Among the 125 spastic cerebral palsy children, there were 119 cases of pelvic radiographs that met the measurement standards. In the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, MP was (22.72±3.88), (26.53±4.36), (33.84±4.99), and (49.54±7.87)%, CE was(30.10±6.99) °, ( 22.92±4.19) °, ( 17.91±5.50) °, and (-0.70±17.33)°, AI was (16.41±2.77) °, (20.46±4.63) °, (23.76±5.10) °, and ( 29.15±7.35)°, respectively, there were significant differences between the two comparisons (P<0.05). And the higher GMFCS grade, the greater MP and AI, and the smaller CE.The NSA was(142.74±10.03) °, (148.66±9.09) °, (151.66±10.52) °, and (153.70±8.05)° in four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, respectively. The differences between the two comparisons of the GMFCS gradeⅠgroup and the other three groups were statistically significant (P<0.05). NSA of GMFCSⅠgroup was significantly lower than that of the others, there was no significant difference among other groups(P>0.05). ② Among the 125 spastic cerebral palsy children, there were 88 cases of lumbar spine radiographs that met the measurement standards. ③The lumbar sagittal Cobb angle was(32.62±11.10) °, (29.86±9.90) °, (31.70±11.84) °, and (39.69±6.80)° in the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, respectively;GMFSS of grade Ⅳ group was significantly higher than that of other three groups, there was significant difference between the two comparisons (P<0.05);there were no significant differences between other groups (P>0.05). In the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, the lumbosacral angle was (31.02±9.91) °, ( 26.57±9.41) °, (28.08±8.56) °, and ( 27.31±11.50)°, the lumbar lordosis index was (4.14±12.89), (8.83±13.53), (13.00±11.78), and (10.76±9.97) mm, the arch apex distance was (9.50±6.80), (6.68±3.20), (7.16±4.94), and (6.62±4.13) mm, respectively, there were no significant differences between the two comparisons(P>0.05). CONCLUSION: ①In children with GMFCS gradeⅠ-Ⅳ, the higher the GMFCS grade, the worse the hip develops. ② Children with GMFCS grade Ⅲ-Ⅳ may be at greater risk for lumbar kyphosis.

Relationship between the gross motor function classification system and hip and lumbar spine development in children with spastic cerebral palsy / 中国骨伤

OBJECTIVE@#To investigate the relationship among the gross motor function classification system (GMFCS)and the development of hip joint and lumbar spine in children with spastic cerebral palsy.@*METHODS@#The clinical data of 125 children with spastic cerebral palsy admitted from January 2018 to July 2021 were retrospectively analyzed. There were 85 males and 40 females, aged from 4 to 12 years old with an average of (8.4±2.9) years. According to GMFCS, the patients were divided into gradeⅠ, Ⅱ, Ⅲ and Ⅳ groups. There were 27 cases in gradeⅠgroup, 40 cases in gradeⅡgroup, 35 cases in grade Ⅲ group and 23 cases in grade Ⅳ group. The migration percentage(MP), central edge angle(CE), neck-shaft angle(NSA), acetabular index(AI) were measured by the radiograph of pelvis, abnormal parameters were selected to evaluate the relationship between different GMFCS grades and hip joint development. Lumbar sagittal Cobb angle, lumbar sacral angle, lumbar lordosis index and apical distance were measured by lateral lumbar radiographs to evaluate the relationship between different GMFCS grades and lumbar spine development.@*RESULTS@#①Among the 125 spastic cerebral palsy children, there were 119 cases of pelvic radiographs that met the measurement standards. In the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, MP was (22.72±3.88), (26.53±4.36), (33.84±4.99), and (49.54±7.87)%, CE was(30.10±6.99) °, ( 22.92±4.19) °, ( 17.91±5.50) °, and (-0.70±17.33)°, AI was (16.41±2.77) °, (20.46±4.63) °, (23.76±5.10) °, and ( 29.15±7.35)°, respectively, there were significant differences between the two comparisons (P<0.05). And the higher GMFCS grade, the greater MP and AI, and the smaller CE.The NSA was(142.74±10.03) °, (148.66±9.09) °, (151.66±10.52) °, and (153.70±8.05)° in four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, respectively. The differences between the two comparisons of the GMFCS gradeⅠgroup and the other three groups were statistically significant (P<0.05). NSA of GMFCSⅠgroup was significantly lower than that of the others, there was no significant difference among other groups(P>0.05). ② Among the 125 spastic cerebral palsy children, there were 88 cases of lumbar spine radiographs that met the measurement standards. ③The lumbar sagittal Cobb angle was(32.62±11.10) °, (29.86±9.90) °, (31.70±11.84) °, and (39.69±6.80)° in the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, respectively;GMFSS of grade Ⅳ group was significantly higher than that of other three groups, there was significant difference between the two comparisons (P<0.05);there were no significant differences between other groups (P>0.05). In the four groups with gradeⅠ, Ⅱ, Ⅲ, Ⅳ, the lumbosacral angle was (31.02±9.91) °, ( 26.57±9.41) °, (28.08±8.56) °, and ( 27.31±11.50)°, the lumbar lordosis index was (4.14±12.89), (8.83±13.53), (13.00±11.78), and (10.76±9.97) mm, the arch apex distance was (9.50±6.80), (6.68±3.20), (7.16±4.94), and (6.62±4.13) mm, respectively, there were no significant differences between the two comparisons(P>0.05).@*CONCLUSION@#①In children with GMFCS gradeⅠ-Ⅳ, the higher the GMFCS grade, the worse the hip develops. ② Children with GMFCS grade Ⅲ-Ⅳ may be at greater risk for lumbar kyphosis.

A clinical cross-sectional study of resting energy expenditure in children with cerebral palsy / 中华实用儿科临床杂志

Objective:To investigate the characteristics of resting energy expenditure (REE) in children with cerebral palsy (CP) graded with different levels of Gross Motor Function Classification System (GMFCS), and to evaluate the accuracy and association of commonly used REE prediction formulas in children with CP.Methods:It was a retrospective study involving 36 children with CP aged 24-144 months who visited the Third Affiliated Hospital of Zhengzhou University between September 2021 and August 2022.REE was measured by the indirect calorimetry.Based on the GMFCS, children with CP were divided into grade Ⅰ-Ⅱ group (20 cases), grade Ⅲ group (6 cases) and grade Ⅳ-Ⅴ group(10 cases). During the same period, 11 age-matched healthy children were included in control group.The measured REE (MREE) between children with CP and healthy controls was compared.Predicted REE (PREE) calculated by the Harris-Benedict, WHO, Schofield-W, Schofield-WH and Oxford prediction formulas were compared with MREE in children for their consistency and correlation.Independent samples were analyzed using t-test or Mann- Whitney U test, and categorical data were analyzed using Chi- square test.Using paired t-test and Pearson linear correlation analysis to analyze the correlation between MREE and PREE.The accuracy of PREE values calculated by different formulas was assessed using the root mean square error. Results:The MREE in control group and children with CP were (952.18±270.56) kcal/d and (801.81±201.89) kcal/d, respectively.There was no significant difference in the MREE between grade Ⅰ-Ⅱ group versus control group[(868.30±194.81) kcal/d vs.(952.18±270.56) kcal/d, P>0.05], and grade Ⅲ group versus control group [(813.17±192.48) kcal/d vs.(952.18±270.56) kcal/d, P>0.05]. The MREE was significantly lower in grade Ⅳ-Ⅴ group than that of control group [666.00(513.50, 775.50) kcal/d vs.(952.18±270.56) kcal/d, P=0.011]. There were no significant difference between MREE and PREEs calculated by Harris-Benedict, WHO, Schofield-W, Schofield-WH, and Oxford (all P>0.05). The correct classification fraction calculated by the 5 formulas were 33.3%, 47.2%, 41.7%, 47.2%, and 41.7%, respectively.The r values of the consistency of PREE calculated by the 5 formulas were 0.585, 0.700, 0.703, 0.712, and 0.701, respectively.The Blande-Altman Limits of Agreement were (-297.77, 359.22), (-245.60, 326.94), (-250.62, 316.05), (-242.22, 177.36) and (-241.28, 325.81), respectively.The clinically acceptable range was -80.18 to 80.18 kcal/d.The root mean square error were 168.09 kcal/d, 149.64 kcal/d, 146.24 kcal/d, 144.23 kcal/d and 148.77 kcal/d, respectively. Conclusions:The MREE values decreased significantly in children with CP classified as CMFCS grade Ⅳ and Ⅴ.When REE cannot be regularly monitored by indirect calorimetry to develop nutritional support programs, children with CP may be prioritized to estimate REE using the prediction formula of Schofield-WH.

Applicability of Modified Pediatric Nutritional Risk Screening Tool in children with cerebral palsy / 中华实用儿科临床杂志

Objective:To explore the scheme of assigning rational scores to the Modified Pediatric Nutritional Risk Screening Tool for children with cerebral palsy(CP) at different Gross Motor Function Classification System(GMFCS) levels.Methods:The clinical data of 360 children with CP hospitalized in the Department of Children′s Rehabilitation, the Third Affiliated Hospital of Zhengzhou University from January to October 2019 were analyzed retrospectively.All the CP children at different GMFCS levels who met the inclusion criteria were subject to nutrition screening and assessment by using the Modified Pediatric Nutritional Risk Screening Tool and the Subjective Global Nutritional Assessment(SGNA) scale.The distribution of malnutrition rates assessed by the SGNA scale among the children at different GMFCS levels was examined.Data between groups were compared by the χ2 test.Children at different GMFCS levels were divided into different subgroups according to the statistical difference.Then, 0 or 1 score was assigned to the Modified Pediatric Nutritional Risk Screening Tool in different subgroups, and different combinations were formed.The nutritional risk screening results of different combinations were evaluated by using the SGNA scale assessment results as a reference. Results:In children with CP, the risk detection rate and incidence rate of malnutrition were 58.1%(209/360) and 36.9%(133/360), respectively.There was no significant difference in the incidence rate of malnutrition between GMFCS Ⅱ and GMFCS Ⅲ, as well as between GMFCS Ⅳ and GMFCS Ⅴ(all P>0.05). Therefore, children with CP were divided into 3 subgroups, namely, group Ⅰ, group Ⅱ to Ⅲ, and group Ⅳ to Ⅴ.Different CP disease scores were given to the Modified Pediatric Nutritional Risk Screening Tool in 3 subgroups, forming 3 different protocols[protocol 1 (0, 0, 1 point); protocol 2(0, 1, 1 point); current protocol (1, 1, 1 point)]. Taking the SGNA scale assessment results as a reference, the sensitivity of protocol 1, protocol 2 and current protocol were 85.7%, 92.5%, and 93.2% respectively.The specificity protocol 1, protocol 2 and current protocol were 81.1%, 78.0%, and 62.6%, respectively.And the Youden indexes of above three protocols were 0.668, 0.705, and 0.558, respectively.The Youden index of protocol 2 was relatively high. Conclusions:The Modified Pediatric Nutritional Risk Screening Tool can effectively identify the risk of malnutrition in children with CP.The scheme of assigning 0 points to children with GMFCS grade Ⅰ and 1 point to children with GMFCS grade Ⅱ to Ⅴ is more reasonable.

Motor Responses in Pediatric Pompe Disease in the ADVANCE Participant Cohort.

BACKGROUND: ADVANCE (NCT01526785) presented an opportunity to obtain a more nuanced understanding of motor function changes in treatment-experienced children with Pompe disease receiving 4000L-production-scale alglucosidase alfa for 52 weeks. OBJECTIVE: To estimate minimal detectable change (MDC) and effect size on Gross Motor Function Measure-88 (GMFM-88) after 52 weeks of 4000L alglucosidase alfa (complete data N =  90). METHODS: The GMFM-88 mean total % score changes, MDC, and effect size were analyzed post hoc by Pompe Motor Function Level at enrollment, age groups at enrollment, and fraction of life on pre-study 160L-production-scale alglucosidase alfa. RESULTS: Overall, participants aged < 2 years surpassed MDC at Week 52 (change [mean±standard deviation] 21.1±14.1, MDC range 5.7-13.3, effect size 1.1), whereas participants aged≥2 years did not attain this (change -0.9±15.3, MDC range 10.8-25.2, effect size -0.03). In participants aged < 2 years, improvements surpassed the MDC for walkers (change 17.1±13.3, MDC range 3.0-6.9, effect size 1.7), supported standers (change 35.2±18.0, MDC range 5.9-13.7, effect size 1.8) and sitters (change 24.1±12.1, MDC range 2.6-6.2, effect size 2.7). Age-independent MDC ranges were only attained by walkers (change 7.7±12.3, MDC range 6.4-15.0, effect size 0.4) and sitters (change 9.9±17.2, MDC range 3.3-7.7, effect size 0.9). CONCLUSIONS: These first GMFM-88 minimal-detectable-change estimates for alglucosidase alfa-treated Pompe disease offer utility for monitoring motor skills. TRIAL REGISTRATION: ClinicalTrials.gov; NCT01526785; Registered 6 February 2012; https://clinicaltrials.gov/ct2/show/NCT01526785.

The Profile of Epilepsy and its characteristics in Children with Cerebral Palsy.

PURPOSE: To determine the characteristics of epilepsy in children with Cerebral Palsy (CP) visiting the Pediatric Outpatient Department (OPD) of a tertiary care hospital. METHODS: This cross-sectional study was carried out at a tertiary care pediatric hospital. All children with CP aged between 1 and 12 years seen at this hospital during an 18 months period (January 2018 to July 2019) were included. Children with CP who had seizures were studied in detail. Seizure semiologies were classified according to the International League Against Epilepsy (ILAE) 1981 and 2017 classifications. The severity of seizures was assessed with the Early Childhood Epilepsy Severity Scale (E-Chess). Functional impairment was characterised using the Gross Motor Function Classification System (GMFCS) score. RESULTS: Of 300 children with CP, 207 (69%) were male and 93 (31%) female. The mean age was 45.17±31.12 months. Seizures were present in 79 (26%) children. 89.9% of children had drug-responsive epilepsy, and 10.1% had refractory epilepsy. Seizures were present in 30.4% of children with a spastic hemiplegia CP subtype, 28.7% with spastic quadriplegia, 26.3% with spastic diplegia, 24% with mixed type CP, and 6.3% with dyskinetic CP. On E-Chess assessment, the median score was 8 (4-14). The majority had poor Gross Motor Function Classification System (GMFCS) scores (>III). CONCLUSION: The prevalence of epilepsy in the studied population of children with CP was 26%. The highest incidence of seizures was in the spastic hemiplegia subtype (30.4%). The severity of cortical damage is positively correlated with the risk of having epilepsy. The primary determinant of severity of the GMFCS score was the type of CP and not the presence or absence of epilepsy.

Comparison of intrathecal baclofen pump insertion and selective dorsal rhizotomy for nonambulatory children with predominantly spastic cerebral palsy.

OBJECTIVE: In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options. METHODS: The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes. RESULTS: Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified. CONCLUSIONS: Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.

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

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

Modified desk height helps children with cerebral palsy perform sit-to-stand.

AIM: Children with cerebral palsy (CP) have difficulties performing activities that require the use of fundamental motor skills such as sit-to-stand (STS). In this study, we used a height adjustable chair and desk to investigate the role of desk support in STS and how it might benefit children with CP. METHODS: Seventeen typically developing children (TDC), average age = 9.7 years, and 28 children with CP (Gross Motor Function Classification System [GMFCS] I and II), average age = 10.3 years, participated in the test. Elapsed time and body sway were measured using a pressure mat and load cell while each child performed a STS task. Two different desk heights were tested for time consumption and sway under the condition of hands-on-desk and arms-crossed. RESULTS: We found that the elapsed time of hands-on-desk with the elbow flexion height was the shortest (p < 0.05). Sway amount was also significantly reduced for all children when they used the table for STS (p < 0.05). CONCLUSION: Results of this study may be a useful reference in rehabilitation training and designing a desk height beneficial for children with CP.Implications for RehabilitationBetter performance of sit-to-stand for a child with cerebral palsy could be made by a desk supportElapsed time during preparation period for sit-to-stand could be reduced by desk support for all CP childrenSway amount during sit-to-stand could be reduced by desk support, especially for the CP children with GMFCS level IIDesk height is an important parameter and should be studied in detail.

Evaluating Completeness of Discrete Data on Physical Functioning for Children With Cerebral Palsy in a Pediatric Rehabilitation Learning Health System.

OBJECTIVE: The purpose of this study was to determine the extent that physical function discrete data elements (DDE) documented in electronic health records (EHR) are complete within pediatric rehabilitation settings. METHODS: A descriptive analysis on completeness of EHR-based DDEs detailing physical functioning for children with cerebral palsy was conducted. Data from an existing pediatric rehabilitation research learning health system data network, consisting of EHR data from 20 care sites in a pediatric specialty health care system, were leveraged. Completeness was calculated for unique data elements, unique outpatient visits, and unique outpatient records. RESULTS: Completeness of physical function DDEs was low across 5766 outpatient records (10.5%, approximately 2 DDEs documented). The DDE for Gross Motor Function Classification System level was available for 21% (n = 3746) outpatient visits and 38% of patient records. Ambulation level was the most frequently documented DDE. Intercept only mixed effects models demonstrated that 21.4% and 45% of the variance in completeness for DDEs and the Gross Motor Function Classification System, respectively, across unique patient records could be attributed to factors at the individual care site level. CONCLUSION: Values of physical function DDEs are missing in designated fields of the EHR infrastructure for pediatric rehabilitation providers. Although completeness appears limited for these DDEs, our observations indicate that data are not missing at random and may be influenced by system-level standards in clinical documentation practices between providers and factors specific to individual care sites. The extent of missing data has significant implications for pediatric rehabilitation quality measurement. More research is needed to understand why discrete data are missing in EHRs and to further elucidate the professional and system-level factors that influence completeness and missingness. IMPACT: Completeness of DDEs reported in this study is limited and presents a significant opportunity to improve documentation and standards to optimize EHR data for learning health system research and quality measurement in pediatric rehabilitation settings.