The relationship between manual ability, dystonia and choreoathetosis severity and upper limb movement patterns during reaching and grasping in children and young adults with dyskinetic cerebral palsy.

INTRODUCTION: Impaired upper limb movements are a key feature in dyskinetic cerebral palsy (CP). However, information on how specific movement patterns relate to manual ability, performance and underlying movement disorders is lacking. Insight in these associations may contribute to targeted upper limb management in dyskinetic CP. This study aimed to explore associations between deviant upper limb movement patterns and (1) manual ability, (2) severity of dystonia/choreoathetosis, and (3) movement time/trajectory deviation during reaching and grasping. PARTICIPANTS/METHODS: Participants underwent three-dimensional upper limb analysis during reaching forwards (RF), reaching sideways (RS) and reach-and-grasp vertical (RGV) as well as clinical assessment. Canonical correlation and regression analysis with statistical parametric mapping were used to explore associations between clinical/performance parameters and movement patterns (mean and variability). RESULTS: Thirty individuals with dyskinetic CP participated (mean age 16±5 y; 20 girls). Lower manual ability was related to higher variability in wrist flexion/extension during RF and RS early in the reaching cycle (p < 0.05). Higher dystonia severity was associated with higher mean wrist flexion (40-82 % of the reaching cycle; p = 0.004) and higher variability in wrist flexion/extension (31-75 %; p < 0.001) and deviation (2-14 %; p = 0.007/60-73 %; p = 0.006) during RF. Choreoathetosis severity was associated with higher elbow pro/supination variability (12-19 %; p = 0.009) during RGV. Trajectory deviation was associated with wrist and elbow movement variability (p < 0.05). CONCLUSION: Current novel analysis of upper limb movement patterns and respective timings allows to detect joint angles and periods in the movement cycle wherein associations with clinical parameters occur. These associations are not present at each joint level, nor during the full movement cycle. This knowledge should be considered for individualized treatment strategies.

Exploring harmonic walking development in children with unilateral cerebral palsy and typically developing toddlers: Insights from walking experience.

This longitudinal study investigated the impact of the first independent steps on harmonic gait development in unilateral cerebral palsy (CP) and typically developing (TD) children. We analysed the gait ratio values (GR) by comparing the duration of stride/stance, stance/swing and swing/double support phases. Our investigation focused on identifying a potential trend towards the golden ratio value of 1.618, which has been observed in the locomotion of healthy adults as a characteristic of harmonic walking. Locomotor ability was assessed in both groups at different developmental stages: before and after the emergence of independent walking. Results revealed that an exponential fit was observed only after the first unsupported steps were taken. TD children achieved harmonic walking within a relatively short period (approximately one month) compared to children with CP, who took about seven months to develop harmonic walking. Converging values for stride/stance and stance/swing gait ratios, averaged on the two legs, closely approached the golden ratio in TD children (R2 = 0.9) with no difference in the analysis of the left vs right leg separately. In contrast, children with CP exhibited a trend for stride/stance and stance/swing (R2 = 0.7), with distinct trends observed for the most affected leg which did not reach the golden ratio value for the stride/stance ratio (GR = 1.5), while the least affected leg exceeded it (GR = 1.7). On the contrary, the opposite trend was observed for the stance/swing ratio. These findings indicate an overall harmonic walking in children with CP despite the presence of asymmetry between the two legs. These results underscore the crucial role of the first independent steps in the progressive development of harmonic gait over time.

Efficacy of methylphenidate treatment in childhood myotonic dystrophy type 1 and comorbid attention deficit hyperactivity disorder: A case report using eye tracking assessment.

INTRODUCTION: Despite the increased prevalence of comorbid attention deficit hyperactivity disorder (ADHD) in children with myotonic dystrophy type 1, the effects of methylphenidate treatment on associated cognitive deficits in this population is not yet investigated. CASE: We describe a case study of an eleven-year-old male patient with myotonic dystrophy type 1 and comorbid ADHD that was treated with methylphenidate in a twice daily regime (0.60 mg/kg/day). Positive effects on learning and cognition were reported by the parents and teachers. No negative side effects were reported. Sequential neuropsychological assessments before and 45 minutes after methylphenidate intake were conducted to quantify the cognitive effects of methylphenidate treatment. Significant improvements in regulation of attention were behaviorally observed and were quantified using eye tracking technology. CONCLUSION: We conclude that methylphenidate may be an effective treatment for ADHD-related cognitive deficits and learning difficulties in children with myotonic dystrophy type 1 which merits further research.

Functional electrical stimulation during walking in children with unilateral spastic cerebral palsy: A randomized cross-over trial.

AIM: To study if functional electrical stimulation (FES) of the peroneal nerve, which activates dorsiflexion, can improve body functions, activities, and participation and could be an effective alternative treatment in individuals with unilateral spastic cerebral palsy (CP). METHOD: A randomized cross-over trial was performed in 25 children with unilateral spastic CP (classified in Gross Motor Function Classification System levels I and II) aged 4 to 18 years (median age at inclusion 9 years 8 months, interquartile range = 7 years-13 years 8 months), 15 patients were male. The study consisted of two 12-week blocks of treatment, that is, conventional treatment (ankle foot orthosis [AFO] or adapted shoes) and FES, separated by a 6-week washout period. Outcome measures included the Goal Attainment Scale (GAS), the Cerebral Palsy Quality of Life questionnaire, and a three-dimensional gait analysis. RESULTS: Eighteen patients completed the trial. The proportion of GAS goals achieved was not significantly higher in the FES versus the conventional treatment phase (goal 1 p = 0.065; goal 2 p = 1.00). When walking while stimulated with FES, ankle dorsiflexion during mid-swing decreased over time (p = 0.006, average decrease of 4.8° with FES), with a preserved increased ankle range of motion compared to conventional treatment (p < 0.001, mean range of motion with FES +10.1° compared to AFO). No changes were found in the standard physical examination or regarding satisfaction with orthoses and feelings about the ability to dress yourself. In four patients, FES therapy failed; in 12 patients FES therapy continued after the trial. INTERPRETATION: FES is not significantly worse than AFO; however, patient selection is critical, and a testing period and thorough follow-up are needed.

Exploring the core network of the structural covariance network in childhood absence epilepsy.

Childhood absence epilepsy (CAE) is a generalized pediatric epilepsy, which is generally considered to be a benign condition since most children become seizure-free before reaching adulthood. However, cognitive deficits and changes of brain morphological have been previously reported in CAE. These morphological changes, even if they might be very subtle, are not independent due to the underlying network structure and can be captured by the structural covariance network (SCN). In this study, SCNs were used to quantify the structural brain network for children with CAE as well as controls. Seventeen children with CAE (6-12y) and fifteen controls (6-12y) were included. To estimate the SCN, T1-weighted images were acquired and parcellated into 68 cortical regions. Graph measures characterizing the core network architecture, i.e. the assortativity and rich-club coefficient, were calculated for all individuals. Multivariable linear regression models, including age and sex as covariates, were used to assess differences between children with CAE and controls. Additionally, potential relations between the core network and cognitive performance was investigated. A lower assortativity (i.e. less efficiently organized core network organization) was found for children with CAE compared to controls. Moreover, better cognitive performance was found to relate to stronger assortative mixing pattern (i.e. more efficient core network structure). Rich-club coefficients did not differ between groups, nor relate to cognitions. The core network organization of the SCN in children with CAE tend to be less efficient organized compared to controls, and relates to cognitive performance, and therefore this study provides novel insights into the SCN organization in relation to CAE and cognition.

Long-term occupational exposures on disability-free survival and mortality in older adults.

BACKGROUND: The impact of long-term occupational exposures on health in older adults is increasingly relevant as populations age. To date, no studies have reported their impact on survival free of disability in older adults. AIMS: We aimed to investigate the association between long-term occupational exposure and disability-free survival (DFS), all-cause mortality and cause-specific mortality in initially healthy older adults. METHODS: We analysed data from 12 215 healthy participants in the ASPirin in Reducing Events in the Elderly (ASPREE) study whose mean age was 75 years. Their work history was collated with the 'ALOHA-plus JEM' (Job Exposure Matrix) to assign occupational exposures. The primary endpoint, DFS, was a composite measure of death, dementia or persistent physical disability. The secondary endpoint, mortality, was classified according to the underlying cause. Cox proportional hazard models were used to calculate hazard ratios and 95% confidence intervals, adjusted for confounders. RESULTS: A total of 1835 individuals reached the DFS endpoint during the median 4.7 years follow-up period. Both ever-high and cumulative exposure to all dusts and all pesticides during a person's working years were associated with reduced DFS. Compared to no exposure, men with high exposure to dusts and pesticides had a reduced DFS. Neither of these exposures were significantly associated with all-cause mortality. Men with high occupational exposure to solvents and women exposed to dusts experienced higher all-cause and cancer-related mortality. CONCLUSIONS: Long-term occupational exposure to all dusts and pesticides was associated with a reduced DFS and increased mortality in community-dwelling healthy older adults.

Urinary pesticide mixture patterns and exposure determinants in the adult population from the Netherlands and Switzerland: Application of a suspect screening approach.

INTRODUCTION: Non-occupational sources of pesticide exposure may include domestic pesticide usage, diet, occupational exposure of household members, and agricultural activities in the residential area. We conducted a study with the ambition to characterize pesticide mixture patterns in a sample of the adult population of the Netherlands and Switzerland, using a suspect screening approach and to identify related exposure determinants. METHODS: A total of 105 and 295 adults participated in the Dutch and Swiss studies, respectively. First morning void urine samples were collected and analyzed in the same laboratory. Harmonized questionnaires about personal characteristics, pesticide-related activities, and diet were administered. Detection rates and co-occurrence patterns were calculated to explore internal pesticide exposure patterns. Censored linear and logistic regression models were constructed to investigate the association between exposure and domestic pesticide usage, consumption of homegrown and organic foods, household members' exposure, and distance to agricultural and forest areas. RESULTS: From the 37 detected biomarkers, 3 (acetamiprid (-CH2), chlorpropham (4-HSA), and flonicamid (-C2HN)) were detected in ≥40% of samples. The most frequent combination of biomarkers (acetamiprid-flonicamid) was detected in 22 (5.5%) samples. Regression models revealed an inverse association between high organic vegetable and fruit consumption and exposure to acetamiprid, chlorpropham, propamocarb (+O), and pyrimethanil (+O + SO3). Within-individual correlations in repeated samples (summer/winter) from the Netherlands were low (≤0.3), and no seasonal differences in average exposures were observed in Switzerland. CONCLUSION: High consumption of organic fruit and vegetables was associated with lower pesticide exposure. In the two countries, detection rates and co-occurrence were typically low, and within-person variability was high. Our study results provide an indication for target biomarkers to include in future studies aimed at quantifying urinary exposure levels in European adult populations.

Ambient Air Pollution and All-Cause and Cause-Specific Mortality in an Analysis of Asian Cohorts.

INTRODUCTION: Much of what is currently known about the adverse effects of ambient air pollution comes from studies conducted in high-income regions, with relatively low air pollution levels. The aim of the current project is to examine the relationship between exposure to ambient air pollution (as predicted from satellite-based models) and all-cause and cause-specific mortality in several Asian cohorts. METHODS: Cohorts were recruited from the Asia Cohort Consortium (ACC). The geocoded residences of participants were assigned levels of ambient particulate material with aerodynamic diameter of 2.5 µm or less (PM2.5) and nitrogen dioxide (NO2) utilizing global satellite-derived models and assigned for the year of enrollment (or closest available year). The association between ambient exposure and mortality was established with Cox proportional hazard models, after adjustment for common confounders. Both single- and two-pollutant models were generated. Model robustness was evaluated, and hazard ratios were calculated for each cohort separately and combined via random effect meta-analysis for pooled risk estimates. RESULTS: Six cohort studies from the ACC participated: the Community-based Cancer Screening Program (CBCSCP, Taiwan), the Golestan Cohort Study (Iran), the Health Effects for Arsenic Longitudinal Study (HEALS, Bangladesh), the Japan Public Health Center-based Prospective Study (JPHC), the Korean Multi-center Cancer Cohort Study (KMCC), and the Mumbai Cohort Study (MCS, India). The cohorts represented over 340,000 participants.Mean exposures to PM2.5 ranged from 8 to 58 µg/m3. Mean exposures to NO2 ranged from 7 to 23 ppb. For PM2.5, a positive, borderline nonsignificant relationship was observed between PM2.5 and cardiovascular mortality. Other relationships with PM2.5 tended toward the null in meta-analysis. For NO2, an overall positive relationship was observed between exposure to NO2 and all cancers and lung cancer. A borderline association between NO2 and nonmalignant lung disease was also observed. The findings within individual cohorts remained consistent across a variety of subgroups and alternative analyses, including two-pollutant models. CONCLUSIONS: In a pooled examination of cohort studies across Asia, ambient PM2.5 exposure appears to be associated with an increased risk of cardiovascular mortality and ambient NO2 exposure is associated with an increased cancer (and lung cancer) mortality. This project has shown that satellite-derived models of pollution can be used in examinations of mortality risk in areas with either incomplete or missing air pollution monitoring.

Psychopharmaceutical treatment for neurobehavioral problems in Duchenne muscular dystrophy: a descriptive study using real-world data.

Patients with Duchenne muscular dystrophy (DMD) are at risk to develop neurobehavioral problems. Evidence on how to treat these difficulties is scarce. This descriptive study reports the clinical experience with psychopharmaceutical treatment in 52 patients with DMD. Electronic patient files were searched for patients with DMD that had been treated with psychopharmaceuticals between 2008 and 2022. Information about neurobehavioral symptoms, type of medication, side effects, and behavioral changes were collected. Two independent clinicians used the clinical global impression scale (CGI) to assess severity of the neurobehavioral problems before and the change in symptoms after treatment. Descriptive statistics were used. Our results include 52 males with DMD (mean age 11 years) treated with psychopharmaceuticals of which 55.8% had four or more comorbid neurobehavioral symptoms. The clinical condition was much improved on the GCI in 54.2% treated with methylphenidate, in 38.9% of the patients treated with fluoxetine, and in 22.2% treated with risperidone. Minimal effects and side effects were also reported. In conclusion, patients with DMD may experience severe neurobehavioral symptoms interfering with learning and/or development. Treatment with psychopharmaceuticals can improve these neurobehavioral symptoms, but further research is needed to gain better insights in psychopharmaceutical treatment in patients with DMD.

Impact of risk-reducing salpingo-oophorectomy on lipid determinants, HbA1c and CRP.

OBJECTIVE: Risk-reducing salpingo-oophorectomy (RRSO) is advised before 40-45 years of age for BRCA1/2 mutation carriers. This study describes the effect of RRSO on lipid determinants, hemoglobin A1c (HbA1c) and C-reactive protein (CRP). METHODS: A total of 142 women with increased risk of ovarian cancer were included, 92 premenopausal and 50 postmenopausal. Serum levels of low-density lipoprotein (LDL)-cholesterol, high-density lipoprotein (HDL)-cholesterol and total cholesterol, triglycerides, HbA1c and CRP were determined at three points in time: before (T0) and 6 weeks (T1) and 7 months (T2) following RRSO. The Hot Flush Rating Scale was administered at the same time points. RESULTS: In premenopausal women, levels of HDL-cholesterol, the cholesterol ratio and HBA1c increased significantly over time, although still staying within the reference range. In this group, hot flushes increased over time (p < 0.001). In postmenopausal women, no significant changes were observed following RRSO. At T2, serum LDL-cholesterol, triglycerides, HbA1c and CRP were significantly lower in premenopausal women compared to postmenopausal women, whereas HDL was increased. CONCLUSIONS: Seven months after RRSO, the lipid profile in premenopausal women had changed, although still staying within the reference range. For postmenopausal women, we did not observe any significant changes. Our results do not suggest a worsening of cardiovascular risk within 7 months of RRSO.

ANK2 loss-of-function variants are associated with epilepsy, and lead to impaired axon initial segment plasticity and hyperactive network activity in hiPSC-derived neuronal networks.

PURPOSE: To characterize a novel neurodevelopmental syndrome due to loss-of-function (LoF) variants in Ankyrin 2 (ANK2), and to explore the effects on neuronal network dynamics and homeostatic plasticity in human-induced pluripotent stem cell-derived neurons. METHODS: We collected clinical and molecular data of 12 individuals with heterozygous de novo LoF variants in ANK2. We generated a heterozygous LoF allele of ANK2 using CRISPR/Cas9 in human-induced pluripotent stem cells (hiPSCs). HiPSCs were differentiated into excitatory neurons, and we measured their spontaneous electrophysiological responses using micro-electrode arrays (MEAs). We also characterized their somatodendritic morphology and axon initial segment (AIS) structure and plasticity. RESULTS: We found a broad neurodevelopmental disorder (NDD), comprising intellectual disability, autism spectrum disorders and early onset epilepsy. Using MEAs, we found that hiPSC-derived neurons with heterozygous LoF of ANK2 show a hyperactive and desynchronized neuronal network. ANK2-deficient neurons also showed increased somatodendritic structures and altered AIS structure of which its plasticity is impaired upon activity-dependent modulation. CONCLUSIONS: Phenotypic characterization of patients with de novo ANK2 LoF variants defines a novel NDD with early onset epilepsy. Our functional in vitro data of ANK2-deficient human neurons show a specific neuronal phenotype in which reduced ANKB expression leads to hyperactive and desynchronized neuronal network activity, increased somatodendritic complexity and AIS structure and impaired activity-dependent plasticity of the AIS.

Neuro-imaging characteristics of sensory impairment in cerebral palsy; a systematic review.

Background: Objective: To identify and examine neural reorganization of the sensory network in terms of lesion type, somatotopic organization of the primary somatosensory area, and functional connectivity in relation to sensory function in children and young adults with cerebral palsy (CP). Methods: Design: systematic review, Prospero registration ID 342570. Data sources: PubMed; Cochrane; Web of Science; Embase; CINAHL and PEDro from inception to March 13, 2021. Eligibility criteria: All types of original studies, concerning sensory connectivity in relation to sensory outcome in patients with spastic CP, <30 years of age. No publication status or date restrictions were applied. Data extraction and synthesis: Two authors independently determined the eligibility of studies. Quality assessment was performed by a third author. Neuro-imaging/neurophysiological techniques, sensory outcomes and patient characteristics were extracted. Results: Children and young adults with periventricular leucomalacia (PVL) lesions have significantly better hand function and sensation scores than patients with cortical-subcortical/middle cerebral artery (MCA) lesions. Ipsilesional reorganization of the S1 (primary somatosensory cortex) area appears to be the primary compensation mechanism after a unilateral early brain lesion, regardless of the timing of the lesion. Interhemispheric reorganization of the sensory system after early brain lesions is rare and, when it occurs, poorly effective. Diffusion tractography shows a positive correlation between the ascending sensory tract (AST) diffusivity metrics of the more affected hemisphere and sensory test outcomes. Discussion and conclusions: Because of the large variability in study design, patient characteristics, neuroimaging/neurophysiological techniques and parameters as well as sensory assessment methods used, it is difficult to draw definite inferences on the relationship between the reorganization of the sensory network following early brain damage and sensory function in children and young adults with CP. In general, sensory function seems to be worse in cortical as opposed to white matter tract (PVL) lesions. International consensus on a clinically relevant sensory test battery is needed to enhance understanding of the intriguing compensatory mechanisms of sensory network following early brain damage and potential consequences for rehabilitation strategies. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/.

A physiologically-based kinetic (PBK) model for work-related diisocyanate exposure: Relevance for the design and reporting of biomonitoring studies.

Diisocyanates are highly reactive substances and known causes of occupational asthma. Exposure occurs mainly in the occupational setting and can be assessed through biomonitoring which accounts for inhalation and dermal exposure and potential effects of protective equipment. However the interpretation of biomonitoring data can be challenging for chemicals with complex kinetic behavior and multiple exposure routes, as is the case for diisocyanates. To better understand the relation between external exposure and urinary concentrations of metabolites of diisocyanates, we developed a physiologically based kinetic (PBK) model for methylene bisphenyl isocyanate (MDI) and toluene di-isocyanate (TDI). The PBK model covers both inhalation and dermal exposure, and can be used to estimate biomarker levels after either single or chronic exposures. Key parameters such as absorption and elimination rates of diisocyanates were based on results from human controlled exposure studies. A global sensitivity analysis was performed on model predictions after assigning distributions reflecting a mixture of parameter uncertainty and population variability. Although model-based predictions of urinary concentrations of the degradation products of MDI and TDI for longer-term exposure scenarios compared relatively well to empirical results for a limited set of biomonitoring studies in the peer-reviewed literature, validation of model predictions was difficult because of the many uncertainties regarding the precise exposure scenarios that were used. Sensitivity analyses indicated that parameters with a relatively large impact on model estimates included the fraction of diisocyanates absorbed and the binding rate of diisocyanates to albumin relative to other macro molecules.We additionally investigated the effects of timing of exposure and intermittent urination, and found that both had a considerable impact on estimated urinary biomarker levels. This suggests that these factors should be taken into account when interpreting biomonitoring data and included in the standard reporting of isocyanate biomonitoring studies.

Dietary fatty acids and endometrial cancer risk within the European Prospective Investigation into Cancer and Nutrition.

BACKGROUND: Diet may impact important risk factors for endometrial cancer such as obesity and inflammation. However, evidence on the role of specific dietary factors is limited. We investigated associations between dietary fatty acids and endometrial cancer risk in the European Prospective Investigation into Cancer and Nutrition (EPIC). METHODS: This analysis includes 1,886 incident endometrial cancer cases and 297,432 non-cases. All participants were followed up for a mean of 8.8 years. Multivariable Cox proportional hazard models were used to estimate hazard ratios (HR) and 95% confidence intervals (CI) of endometrial cancer across quintiles of individual fatty acids estimated from various food sources quantified through food frequency questionnaires in the entire EPIC cohort. The false discovery rate (q-values) was computed to control for multiple comparisons. RESULTS: Consumption of n-6 γ-linolenic acid was inversely associated with endometrial cancer risk (HR comparing 5th with 1st quintileQ5-Q1=0.77, 95% CI = 0.64; 0.92, ptrend=0.01, q-value = 0.15). This association was mainly driven by γ-linolenic acid derived from plant sources (HRper unit increment=0.94, 95%CI= (0.90;0.98), p = 0.01) but not from animal sources (HRper unit increment= 1.00, 95%CI = (0.92; 1.07), p = 0.92). In addition, an inverse association was found between consumption of n-3 α-linolenic acid from vegetable sources and endometrial cancer risk (HRper unit increment= 0.93, 95%CI = (0.87; 0.99), p = 0.04). No significant association was found between any other fatty acids (individual or grouped) and endometrial cancer risk. CONCLUSION: Our results suggest that higher consumption of γ-linolenic acid and α-linoleic acid from plant sources may be associated with lower risk of endometrial cancer.

Effectiveness of functional intensive therapy on mobility and self-care activities in children and adolescents with cerebral palsy - a prospective clinical study.

PURPOSE: Cerebral palsy (CP) is a major cause of childhood disability. Children with CP often lack motor skills to effectively perform activities of daily living. The aim is to assess the effectiveness of a functional intensive therapy program focused on improving individual goals in the domain of mobility and self-care in children and adolescents with CP. MATERIAL AND METHODS: Thirty-five CP patients, aged 11-19 years, GMFCS I-IV, received daily 6-7 h of functional therapy for 15 days. Outcomes were assessed at baseline, immediately after the program and at three months follow-up. RESULTS: Significant post-intervention improvement was seen on all primary and secondary outcome measures; personal goals (GAS score; COPM performance and COPM satisfaction), daily activities (ACTIVLIM), hand function (ABILHAND-Kids), mobility (ABILOCO-Kids; GMFM-66-IS score). There was no loss to follow up during the program and after three months. At follow-up, improvements were retained except for ABILOCO and GMFM-66-IS. CONCLUSIONS: Functional intensive therapy appears feasible and seems to be effective in improving treatment goals focused on mobility and self-care, even in older and more severely affected children and adolescents with CP. After three months, these possible effects were still present.Implications for rehabilitationShort intensive functional training is feasible and showing no loss to follow up in the older and more severely affected children and adolescents with cerebral palsy (CP).Short intensive functional training appears effective in improving individual goals in children and adolescents with CP and improvements endorse three months.Short intensive functional training seems to be effective on both mobility and self-care domains of the ICF-CY.

Attainment of personal goals in the first year of intrathecal baclofen treatment in dyskinetic cerebral palsy: a prospective cohort study.

PURPOSE: To assess attainment of individual treatment goals one year after intrathecal baclofen (ITB) pump implantation in individuals with dyskinetic cerebral palsy (CP). MATERIALS AND METHODS: A multi-center prospective cohort study was conducted including 34 non-walking individuals with severe dyskinetic CP, classified as Gross Motor Function Classification System (GMFCS) IV/V, aged 4-24 years, 12 months after pump implantation. The main outcome measure was Goal Attainment Scaling (GAS). Predictors of GAS results were analyzed. Complications were registered systematically. RESULTS: Seventy-one percent of individuals with dyskinetic CP fully achieved one or more treatment goals. One or more treatment goals were partially achieved in 97% of individuals. Two factors were found to be associated with attainment of goals: Dyskinesia Impairment Scale (DIS) score at baseline and the difference in pain score between baseline and follow-up. These two variables explain 30% of the variance in the outcome. CONCLUSIONS: Intrathecal baclofen is effective in achieving individual treatment goals in children and young adults with dyskinetic CP after nine to 12 months of ITB treatment. A positive outcome on treatment goals is, for a small part, associated with higher severity of dystonia at baseline and with improvement of pain during treatment. CLINICAL TRIAL REGISTRATION NUMBER: Dutch Trial Register, number NTR3642.Implications for rehabilitationIntrathecal baclofen treatment is effective in attainment of personal treatment goals, one year after pump implantation in patients with dyskinetic cerebral palsy.A positive outcome on treatment goals is, for a small part, related to higher severity of dystonia at the start and on improvement of pain during treatment.

Determinants of spoken language comprehension in children with cerebral palsy.

PURPOSE: To identify determinants of spoken language comprehension (SLC) in children with cerebral palsy (CP). MATERIALS AND METHODS: Cross-sectional data of 207 children with CP were included: 82 toddlers (18 months-3;11 years), 59 preschool children (4;0-5;11 years), and 66 schoolchildren (6;0-8;11 years), across all Gross Motor Function Classification System (GMFCS) levels. SLC was assessed using the Computer-Based instrument for Low motor Language Testing (C-BiLLT). Potential determinants were retrieved from medical files and through parental questionnaires. Per developmental stage, multivariable regression analyses were used to assess determinants of SLC. RESULTS: Fifty-nine percent of the children showed below average SLC. Significant determinants for SLC differed per developmental stage. In toddlers: age, motor type, functional communication and speech function (R2 = 0.637); in preschool children: functional communication, speech function and language activities (R2 = 0.820), and in schoolchildren: functional communication, intellectual functioning and arm-hand functioning (R2 = 0.807). For all developmental stages, functional mobility was not a significant determinant. CONCLUSIONS: A large proportion of children with CP across all GMFCS levels have SLC impairments. Findings indicate that SLC is strongly determined by functional communication classified with CFCS. We recommend standardized assessment and monitoring of SLC in all children with CP. IMPLICATIONS FOR REHABILITATIONChildren across all GMFCS levels can experience difficulties in spoken language comprehension.At all developmental stages, functional communication (classified with CFCS) is an important determinant of spoken language comprehension.Standardized assessment and monitoring of spoken language comprehension, language production, speech, and communication of all children with CP, is strongly recommended.When children show below average performances, especially in spoken language comprehension, intervening with speech and language therapy and guidance for parents, is advised.

Lower limb muscle fatigue after uphill walking in children with unilateral spastic cerebral palsy.

Fatigue during walking is a common complaint in cerebral palsy (CP). The primary purpose of this study is to investigate muscle fatigue from surface electromyography (sEMG) measurements after a treadmill-based fatigue protocol with increasing incline and speed in children with CP with drop foot. The secondary purpose is to investigate whether changes in sagittal kinematics of hip, knee and ankle occur after fatigue. Eighteen subjects with unilateral spastic CP performed the protocol while wearing their ankle-foot orthosis and scored their fatigue on the OMNI scale of perceived exertion. The median frequency (MF) and root mean square (RMS) were used as sEMG measures for fatigue and linear mixed effects model were applied. The MF was significantly decreased in fatigued condition, especially in the affected leg and in the tibialis anterior and peroneus longus muscle. The RMS did not change significantly in fatigued condition, while the OMNI fatigue score indicated patients felt really fatigued. No changes in sagittal kinematics of hip, knee and ankle were found using statistical non-parametric mapping. In conclusion, the current fatigue protocol seems promising in inducing fatigue in a population with CP with drop foot and it could be used to expand knowledge on muscle fatigue during walking in CP.