12 research outputs found
Reliability of a progressive maximal cycle ergometer test to assess peak oxygen uptake in children with mild to moderate cerebral palsy
Rehabilitation research in children with cerebral palsy (CP) is increasingly addressing cardiorespiratory fitness testing. However, evidence on the reliability of peak oxygen uptake (Vo2peak) measurements, considered the best indicator of aerobic fitness, is not available in this population. The objective of this study was to establish the reliability of a progressive maximal cycle ergometer test when assessing Vo2peak in children with mild to moderate CP. Repeated measures were used to assess test-retest reliability. Eligible participants were ambulant, 6 to 14 years of age, and classified as level I, II, or III according to the Gross Motor Function Classification System (GMFCS). Two progressive maximal cycle ergometer tests were conducted (separated by 3 weeks), with the workload increasing every minute in steps of 3 to 11 W, dependent on height and GMFCS level. Reliability was determined by means of the intraclass correlation coefficient (ICC [2,1]) and smallest detectable change (SDC). Twenty-one children participated (GMFCS I: n=4; GMFCS II: n=12; and GMFCS III: n=5). Sixteen of them (9 boys, 7 girls; GMFCS I: n=3; GMFCS II: n=11; and GMFCS III: n=2) performed 2 successful tests, separated by 9.5 days on average. Reliability for Vo2peak was excellent (ICC=.94, 95% confidence interval=.83-.98). The SDC was 5.72 mL/kg/min, reflecting 14.6% of the mean. The small sample size did not allow separate analysis of reliability per GMFCS level. In children with CP of GMFCS levels I and II, a progressive maximal cycle ergometer test to assess Vo2peak is reliable and has the potential to detect change in cardiorespiratory fitness over time. Further study is needed to establish the reliability of Vo2peak in children of GMFCS level II
Physical Strain: A New Perspective on Walking in Cerebral Palsy
Objectives: To describe (1) physical strain of walking, (2) the proportion of participants walking above the anaerobic threshold, and (3) 4 phenotypes of physical strain of walking on the basis of deviations in aerobic capacity and walking energy cost (EC) in children and adolescents with cerebral palsy (CP). Design: Cohort study. Setting: Academic medical center. Participants: A sample (N=57) of participants (n=37; mean age, 13.5 +/- 4.0y) with CP (Gross Motor Function Classification System [GMFCS] levels I [n=13], II [n=17], and BI [n=7]) and typically developing (TD) participants (n=20; mean age, 11.8 +/- 3.5y). Interventions: Not applicable. Main Outcome Measures: Oxygen consumption (Vo(2)walk), speed, and EC were determined during walking at a comfortable speed. Peak oxygen consumption (Vo(2)peak) and anaerobic threshold were measured during a maximal cycling exercise test. Aerobic capacity was reduced if lower than the 10th percentile, and EC was increased if higher than 3SD. Physical strain was defined as follows: (Vo(2)walk/dVo(2)peak)x100. Results: Participants with CP had a higher physical strain (GMFCS level I, 55%+/- 12% GMFCS level II, 62%+/- 17%; GMFCS level III, 78%+/- 14%) than did TD participants (40%+/- 11%) (P <.001). Forty-three percent of participants with CP showed a Vo(2)walk at or above their anaerobic threshold as compared with 10% of TD participants (P=.007). Phenotypes showed that a reduced Vo(2)peak (n=9) or an increased EC (n=9) lead to an 18% to 20% higher physical strain, whereas a combination (n=12) leads to a 40% increase. Conclusions: Children and adolescents with CP walk at a high physical strain, approximating intense exercise and a considerable proportion walks close to or above their anaerobic threshold, probably explaining fatigue and reduced walking distance. Both an increased EC and a reduced Vo(2)peak contribute to high physical strain in children or adolescents with CP. The different causes of high physical strain in individuals with CP require different intervention strategies. (C) 2017 by the American Congress of Rehabilitation Medicin
Fatigue in Children and Young Adults with Physical Disabilities: Relation with Energy Demands of Walking and Physical Fitness
Purpose: To examine whether general fatigue and fatigue during or after walking are related to energy demands during walking and physical fitness in children and young adults with physical disabilities. Methods: Sixty-eight individuals with physical disabilities participated. General fatigue (Checklist Individual Strength [CIS8R] questionnaire), walking-induced fatigue (OMNI [OMNIwalk] scale after walking for 6 min), gross and net energy costs (ECs) of walking, physical strain of walking, and aerobic and anaerobic fitness were measured. Results: Regression analyses showed no relations with the CIS8R. For all participants, a higher net EC was weakly related to an increased OMNIwalk. For teenagers only, low anaerobic fitness and high physical strain of walking values were moderately related to high OMNIwalk scores. Conclusion: Low anaerobic fitness and high physical strain values partly explain fatigue after walking in teenagers with cerebral palsy, but not in younger children. General fatigue was not explained by low fitness levels or high energy demands of walking
Daily physical activity and exercise for people with long term neurological conditions; a delicate balance between training and recovery
Objective: In this paper we aim to provide insight, based on a comprehensive review of the literature, that may be helpful when enrolling people with long term neurological conditions (LTNC) into daily physical activity or exercise programs. We will discuss the following issues: 1) balance between training and recovery; 2) intensities of common physical activities like walking; and 3) consequences for daily physical activity and exercise prescription. Material and Methods: A comprehensive review of studies up to July 2017 was performed. Databases searched were PubMed, EMBASE, SPORTDiscus, and PsychINFO. Results: Five papers, that provided useful data on relative intensity of walking (i.e., the oxygen consumption of the activity relative to the maximal capacity) in people with LTNC, were identified and analysed. For stroke survivors the relative intensity of walking was between 62.3 and 69.8%. For children with cerebral palsy (CP) these values ranged from 52 to 78%, and for adults with CP an average relative intensity of 52% was found. These levels of relative intensity are considerably higher compared to those experienced by able-bodied children (36±8.4%) and adults (27±6%). Conclusion: For a group of people with LTNC, walking can be categorized as a moderate physical activity while for others it might be a vigorous physical activity. When developing a physical activity or exercise program, insight into the intensity of physical activities is important
Energy expenditure and muscle activity during lying, sitting, standing, and walking in people with motor-incomplete spinal cord injury
STUDY DESIGN: Cross-sectional exploratory study. OBJECTIVES: To determine oxygen uptake (VO2), energy expenditure (EE), and muscle activity (MA) during lying (rest), sitting, standing, and walking among ambulatory individuals with spinal cord Injury (SCI) and to compare VO2, EE, and MA between individuals with different levels of ambulation. SETTING: Rehabilitation institution with a spinal cord injury unit. METHODS: A total of 22 adults with motor-incomplete SCI, ten in a low-ambulation group (non-functional or household walker) and 12 in a high-ambulation group (community or normal walker). VO2 was measured using indirect calorimetry. EE was expressed in metabolic equivalent of task (MET). MA was measured using a wireless surface electromyography device. RESULTS: Mean VO2 was 3.19 ml/kg/min. During lying and sitting, EE was below 1.5 METs for all participants. During standing, three participants of the low-ambulation group and none in the high-ambulation group showed MET values of >1.5. In the walking condition, all participants showed MET values above 1.5. MA during stance was higher compared to the sitting condition and significantly higher in the low-ambulation group compared to the high-ambulation group. CONCLUSION: Lying, supported- and unsupported sitting, without moving, appear to be sedentary behaviors for ambulatory individuals with a motor-incomplete SCI (MET values of 1.5 METs), which can be used by all individuals with motor-incomplete SCI to interrupt sedentary behavior
Real-time daily fatigue, sleep, physical activity, and health-related fitness in adults with cerebral palsy
Aim To explore whether subgroups of adults with cerebral palsy (CP) with different fatigue diurnal profiles can be discerned, and to explore whether sleep, physical activity, or health-related fitness are associated with these profiles. Method Thirty-two adults (median age 29 years 8 months; range 20-54 years; 11 males, 21 females) with spastic CP (Gross Motor Function Classification System levels I-III) with physical activity-related fatigue complaints participated. Real-time fatigue and physical activity were assessed for 7 consecutive days by short message service text four times during the day and by wearing an accelerometer respectively. Sleep was assessed by the Pittsburgh Sleep Quality Index, and fitness by assessing body composition and aerobic capacity. Latent class growth modelling was used to classify subgroups according to their diurnal profiles of real-time fatigue. Univariable multinomial logistic regression analysis explored whether participant characteristics, sleep, physical activity, or health-related fitness were associated with diurnal profiles. Results Three distinct fatigue diurnal profiles were identified: stable low (n = 10), increasing (n = 14), and stable high (n = 8). Only aerobic capacity was associated with fatigue profiles (odds ratio 1.15, 95% confidence interval 1.00-1.34; p = 0.05). Interpretation Fatigue in adults with CP may be low or high stable or may increase during the day. These findings indicate the relevance of assessing fatigue variability
Systematic review of the clinimetric properties of laboratory- and field-based aerobic and anaerobic fitness measures in children with cerebral palsy
Objective: To systematically evaluate the level of evidence of the clinimetric properties of measures of aerobic and anaerobic capacity used for children with cerebral palsy (CP)
Walking activity of children with cerebral palsy and children developing typically: a comparison between the Netherlands and the United States
Multimorbidity risk assessment in adolescents and adults with cerebral palsy: a protocol for establishing a core outcome set for clinical research and practice
Background: Estimates of multimorbidity, defined as the presence of at least two chronic conditions, some of which attributable to modifiable behaviours, are high in adults with cerebral palsy (CP). An assessment protocol evaluating multimorbidity risk is needed in order to develop and evaluate effective interventions to optimize lifelong health in individuals with CP. The aim of this protocol paper is to describe the development of a core outcome set (COS) for assessing multimorbidity risk in adolescents and adults with CP, to be used in clinic and research.Methods: The expert consortium will first define the target population and outcomes to be measured. Through a process of literature review and an international Delphi survey with expert clinicians and researchers, we will then determine which outcome measurement instruments (OMIs) can best measure those outcomes. The resulting OMIs will be used in a feasibility study with adolescents and adults with CP from an international clinical research network. Finally, a face-to-face stakeholder meeting with adolescents and adults with CP, their families/caregivers and researchers and clinicians who are experts in CP, will be organized to reach final agreement on the COS.Discussion: This COS will guide clinicians and researchers in assessing multimorbidity risk in adolescents and adults with CP. The inclusion of experts and individuals with CP from international locations for establishing the COS lends strong support to its generalizability. Evidence of its feasibility and approval from all stakeholders will enable implementation in clinical practice, and guide future research using the COS in individuals with CP
Beneficial effect of BH4 treatment in a 15-year-old boy with biallelic mutations in DNAJC12
Background: Biallelic mutations in DNAJC12 were recently identified as a BH4-responsive cause of hyperphenylalaninemia (HPA). Outcome was only favorable when treatment was initiated early in life. We report on a 15-year-old boy with HPA due to a homozygous deletion in DNAJC12 in whom – despite his advanced age – treatment was initiated. Case: A boy with developmental delay, an extrapyramidal movement disorder, and persistently elevated plasma phenylalanine levels was diagnosed with DNAJC12 deficiency at the age of 15 years. Diagnosis was made upon exome reanalysis revealing a homozygous 6.9 kb deletion in DNAJC12 which had not been detected by the standard exome analysis pipeline. Treatment with the BH4 analog sapropterin dihydrochloride (10 mg/kg/day) was initiated and evoked a 50% reduction of the plasma phenylalanine levels. More strikingly, a marked improvement in daily functioning and improved exercise tolerance was noted. Additionally, gait analysis before and after treatment initiation revealed a partial normalization of his movement disorder. Conclusion: Patients with hyperphenylalaninemia due to DNAJC12 deficiency may benefit from treatment with a BH4 analog – even when introduced at a later age