Body mass index is not suitable for assessing body composition in children with spastic cerebral palsy.

PURPOSE: To measure body composition by using bioelectrical impedance analysis (BIA) and body mass index (BMI) and to investigate the correlation and agreement between BMI and fat mass percentage in children with spastic Cerebral Palsy. MATERIALS AND METHODS: BIA was used to assess fat mass percentage and BMI was determined from body weight and height. BMI and fat mass percentage were both categorized into five categories. The association between fat mass percentage and BMI was assessed using Pearson's correlation coefficient. Agreement between BMI and fat mass percentage was investigated with weighted Cohen's kappa coefficient. RESULTS: One hundred and three children with CP across all Gross Motor Function Classification Levels (61% boys, mean age 9 years) were included. Mean BMI was 18.3 kg/m2 and mean fat mass was 24.9%. A large inter-subject variability was found with a weak correlation between BMI and fat mass percentage in children with a BMI < 20 kg/m2. Little agreement (k = 0.299, CI 0.16-0.44) between the categorization of children based on BMI and based on fat mass percentage was found. INTERPRETATION: The large inter-subject variability in fat mass percentage combined with little agreement between the BMI and BIA categories suggests that BMI is not a suitable measure of fat mass in children with CP.IMPLICATIONS FOR REHABILITATIONUsing body mass index (BMI) and instead of fat mass percentage increases the risk of misclassifying body composition in children with spastic Cerebral Palsy.Children with a BMI < 20 kg/m2 are more at risk to be misclassified for body composition.

The PURPLE N study: objective and perceived nutritional status in children and adolescents with cerebral palsy.

PURPOSE: To obtain information on characteristics, management, current objective nutritional status and perception of nutritional status of children with cerebral palsy (CP) from healthcare professionals (HCPs) and caregivers. MATERIALS AND METHODS: A detailed survey of several items on eight main topics (general characteristics, motor function, comorbidities, therapies, anthropometry, feeding mode and problems and perceived nutritional status) was developed and tested for the study. Correlation between nutritional status and Gross Motor Function Classification System (GMFCS) levels was assessed using continuous variables (Z-scores for weight-for-age, height-for-age, weight-for-height, and body mass index-for-age), and categorical variables (being malnourished, stunted, or wasted). HCP and caregiver perceptions of the child's nutritional status as well as agreement between perceived and objective nutritional status and agreement between perceived nutritional status and concerns about the nutritional status were analyzed. RESULTS: Data were available for 497 participants from eight European countries. Poorer nutritional status was associated with higher (more severe) GMFCS levels. There was minimal agreement between perceived and objective nutritional status, both for HCPs and caregivers. Agreement between HCP and caregiver perceptions of the child's nutritional status was weak (weighted kappa 0.56). However, the concerns about the nutritional status of the child were in line with the perceived nutritional status. CONCLUSIONS: The risk of poor nutritional status is associated with more severe disability in children and adolescents with CP. There is a mismatch between HCP and caregiver perceptions of participants' nutritional status as well as between subjective and objective nutritional status. Our data warrant the use of a simple and objective screening tool in daily practice to determine nutritional status in children and adolescents with CP. Clinical trial registration: ClinicalTrials.gov Identifier: NCT03499288 (https://clinicaltrials.gov/ct2/show/NCT03499288). IMPLICATIONS FOR REHABILITATIONUse of the ESPGHAN recommendations and simple screening tools in daily practice is needed to improve nutritional care for individuals with CP.Attention should be paid to the differences in the perception of nutritional status of individuals with CP between professionals and caregivers to improve appropriate referral for nutritional support.Objective measures rather than the professional's perception need to be used to define the nutritional status of individuals with CP.

Improvements in Upper Extremity Function Following Intensive Training Are Independent of Corticospinal Tract Organization in Children With Unilateral Spastic Cerebral Palsy: A Clinical Randomized Trial.

Background/Objectives: Intensive training of the more affected upper extremity (UE) has been shown to be effective for children with unilateral spastic cerebral palsy (USCP). Two types of UE training have been particularly successful: Constraint-Induced Movement Therapy (CIMT) and Bimanual training. Reorganization of the corticospinal tract (CST) early during development often occurs in USCP. Prior studies have suggested that children with an ipsilateral CST controlling the affected UE may improve less following CIMT than children with a contralateral CST. We tested the hypothesis that improvements in UE function after intensive training depend on CST laterality. Study Participants and Setting: Eighty-two children with USCP, age 5 years 10 months to 17 years, University laboratory setting. Materials/Methods: Single-pulse transcranial magnetic stimulation (TMS) was used to determine each child's CST connectivity pattern. Children were stratified by age, sex, baseline hand function and CST connectivity pattern, and randomized to receive either CIMT or Bimanual training, each of which were provided in a day-camp setting (90 h). Hand function was tested before, immediately and 6 months after the intervention with the Jebsen-Taylor Test of Hand Function, the Assisting Hand Assessment, the Box and Block Test, and ABILHAND-Kids. The Canadian Occupational Performance Measure was used to track goal achievement and the Pediatric Evaluation of Disability Inventory was used to assess functioning in daily living activities at home. Results: In contrast to our hypothesis, participants had statistically similar improvements for both CIMT and Bimanual training for all measures independent of their CST connectivity pattern (contralateral, ipsilateral, or bilateral) (p < 0.05 in all cases). Conclusions/Significance: The efficacy of CIMT and Bimanual training is independent of CST connectivity pattern. Children with an ipsilateral CST, previously thought to be maladaptive, have the capacity to improve as well as children with a contralateral or bilateral CST following intensive CIMT or Bimanual training. Clinical Trial Registration: www.ClinicalTrials.gov, identifier NCT02918890.

Dose, timing, and source of protein intake of young people with spastic cerebral palsy.

Purpose: Since the dose, timing and source of dietary protein intake are important for muscle growth and development, the aim of this study was to examine the dose, timing and source of protein intake of young people with cerebral palsy.Materials and methods: Dietary intake was assessed in 19 children with spastic cerebral palsy (Gross Motor Function Classification System levels I-V; Eating and Drinking Classification System levels I-V; 10 males, 9 females; mean [SD] age 11 years 2 months [3 years 3 months]) using a 3-day food diary. The data were analyzed for three age categories (4-8, 9-13, and 14-17 years).Results: Average 3-day protein intake (62.1 g [27.9 g]) was within the recommended boundaries with a minimum of 1.0 g/kg body weight/day and a maximum of 4.1 g/kg body weight/day. However, dinner was the only mealtime that provided at least 25 g of protein, which is needed for optimal muscle maintenance. The main food groups that contributed to protein intake were 'milk and milk products', 'meat, meat products and poultry', and 'bread'.Conclusions: These observations suggest timing of protein intake can be improved with higher intakes during breakfast and lunch to better support skeletal muscle growth and development.IMPLICATIONS FOR REHABILITATIONRecent studies have shown that smaller muscles and early atrophy are already present at young age in individuals with cerebral palsy.Besides physical training, adequate protein intake (with optimal dose, timing and source of protein) may be a key factor in the prevention and treatment of loss of muscle mass in children with cerebral palsy.In a relatively small sample this study shows that overall protein intake (dose) was in line with recommendations and also that the source of the protein seemed sufficient to contain all essential amino acids.Improvement of the timing of protein intake throughout the day, with higher intakes during breakfast and lunch, seems important to better support skeletal muscle growth and development.

Determinants of muscle preservation in individuals with cerebral palsy across the lifespan: a narrative review of the literature.

In individuals with cerebral palsy (CP), smaller muscle and atrophy are present at young age. Many people with CP also experience a decline in gross motor function as they age, which might be explained by the loss of muscle mass. The clinical observation of muscle wasting has prompted a comparison with sarcopenia in older adults, and the term accelerated musculoskeletal ageing is often used to describe the hallmark phenotype of CP through the lifespan. However, there has been very little research emphasis on the natural history of ageing with CP and even less with respect to the determinants or prevention of muscle loss with CP. Considering the burgeoning interest in the science of muscle preservation, this paper aims to (i) describe the characteristics of accelerated musculoskeletal ageing in people with CP, (ii) describe the pathophysiology of sarcopenia and parallels with CP, and (iii) discuss possible therapeutic approaches, based on established approaches for sarcopenia.

The Relationship Between Hand Function and Overlapping Motor Representations of the Hands in the Contralesional Hemisphere in Unilateral Spastic Cerebral Palsy.

BACKGROUND: In many children with unilateral spastic cerebral palsy (USCP), the corticospinal tract to the affected hand atypically originates in the hemisphere ipsilateral to the affected hand. Such ipsilateral connectivity is on average a predictor of poor hand function. However, there is high variability in hand function in these children, which might be explained by the complexity of motor representations of both hands in the contralesional hemisphere. OBJECTIVE: To measure the link between hand function and the size and excitability of motor representations of both hands, and their overlap, in the contralesional hemisphere of children with USCP. METHODS: We used single-pulse transcranial magnetic stimulation to measure the size and excitability of motor representations of both hands, and their overlap, in the contralesional hemisphere of 50 children with USCP. We correlated these measures with manual dexterity of the affected hand, bimanual performance, and mirror movement strength. RESULTS: The main and novel findings were (1) the large overlap in contralesional motor representations of the 2 hands and (2) the moderate positive associations of the size and excitability of such shared-site representations with hand function. Such functional associations were not present for overall size and excitability of representations of the affected hand. CONCLUSIONS: Greater relative overlap of the affected hand representation with the less-affected hand representation within the contralesional hemisphere was associated with better hand function. This association suggests that overlapping representations might be adaptively "yoked," such that cortical control of the child's less-affected hand supports that of the affected hand.

Combined transcranial direct current stimulation and robotic upper limb therapy improves upper limb function in an adult with cerebral palsy.

BACKGROUND: Robotic therapy can improve upper limb function in hemiparesis. Excitatory transcranial direct current stimulation (tDCS) can prime brain motor circuits before therapy. OBJECTIVE: We tested safety and efficacy of tDCS plus robotic therapy in an adult with unilateral spastic cerebral palsy (USCP). METHODS: In each of 36 sessions, anodal tDCS (2 mA, 20 min) was applied over the motor map of the affected hand. Immediately after tDCS, the participant completed robotic therapy, using the shoulder, elbow, and wrist (MIT Manus). The participant sat in a padded chair with affected arm abducted, forearm supported, and hand grasping the robot handle. The participant controlled the robot arm with his affected arm to move a cursor from the center of a circle to each of eight targets (960 movements). Motor function was tested before, after, and six months after therapy with the Wolf Motor Function Test (WMFT) and Fugl-Meyer (FM). RESULTS: Reaching accuracy on the robot task improved significantly after therapy. The WMFT and FM improved clinically meaningful amounts after therapy. The motor map of the affected hand expanded after therapy. Improvements were maintained six months after therapy. CONCLUSIONS: Combined tDCS and robotics safely improved upper limb function in an adult with USCP.

Motor learning paradigm and contextual interference in manual computer tasks in individuals with cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is a group of disorders of movement and posture that cause activity limitations. Due to the different motor problems these individuals encounter there is a need to offer rehabilitation programs that promote motor learning. Additionally, the understanding of the learning patterns of these individuals can help us attend to their learning needs to maximize their learning efficiency. AIMS: The present study aimed to add to the knowledge base in regards to motor learning and the contextual interference (CI) effect. METHODS AND PROCEDURES: The study included 40 individuals with CP and 40 typically developing (TD) participants matched for age and gender with the CP group. Both groups were divided into 2 subgroups regarding the practice schedule (random or constant practice) of a manual maze test on the computer. The participants who performed in the constant practice schedule performed the same standard maze 30 times, while participants in the random practice schedule performed a total of 30 trials on 5 mazes with a different spatial layout including the standard maze. After 5min of rest, retention was studied with a task in which all participants performed the standard maze. To examine the transfer effect, all participants also performed a maze with a new layout. Time of completion was registered in seconds for each trial. OUTCOMES AND RESULTS: The results showed that the performance was lower in individuals with CP compared to typically developing individuals. In addition, only the participants with CP showed a contextual interference effect, with performance after the random practice schedule being superior compared to participants who practiced with a constant practice schedule. CONCLUSIONS AND IMPLICATIONS: Overall performance was lower in individuals with CP compared to individuals with TD. Additionally, both TD individuals and individuals with CP showed the contextual interference effect in the transfer phase, with the execution of random practice leading to better performance than constant practice. These findings provide important information to assist clinicians in developing rehabilitation programs for children with CP.

Does Corticospinal Tract Connectivity Influence the Response to Intensive Bimanual Therapy in Children With Unilateral Cerebral Palsy?

BACKGROUND: Reorganization of the corticospinal tract (CST) can occur in unilateral spastic cerebral palsy (USCP). The affected hand can be controlled via (1) typical contralateral projections from the lesioned hemisphere, (2) ipsilateral projections from the nonlesioned hemisphere, and (3) a combination of contralateral and ipsilateral projections (ie, bilateral). Intensive bimanual therapy and constraint-induced movement therapy (CIMT) improve hand function of children with USCP. Earlier it was suggested that the CST connectivity pattern may influence the efficacy of CIMT. OBJECTIVE: To examine whether CST projection pattern influences the efficacy of intensive bimanual therapy in children with USCP. PARTICIPANTS: Thirty-three children with USCP (age 8.9 ± 2.6 years, 16 females). METHODS: Bimanual therapy was provided in a day-camp setting (90 hours). Participants were involved in different bimanual play and functional activities actively engaging both hands. Hand function was tested before and after the intervention with the Jebsen-Taylor Test of Hand Function, Assisting Hand Assessment, ABILHAND-Kids, and the Canadian Occupational Performance Measure. Single-pulse transcranial magnetic stimulation (TMS) was used to determine each child's CST projection pattern (ie, ipsilateral, contralateral, or bilateral). RESULTS: Children whose affected hand was controlled only by ipsilateral CST projections had worse Jebsen-Taylor Test of Hand Function and Assisting Hand Assessment scores than children in the contralateral group at baseline. Bimanual hand use and functional hand use was independent of CST projection pattern. After bimanual therapy, improvements on all outcome measures were observed, and these improvements were independent of the CST connectivity pattern. CONCLUSION: The efficacy of bimanual therapy on hand function in children with USCP appears to be independent of CST connectivity pattern.

Reflections on mirror therapy: a systematic review of the effect of mirror visual feedback on the brain.

BACKGROUND: Mirror visual feedback (MVF), a phenomenon where movement of one limb is perceived as movement of the other limb, has the capacity to alleviate phantom limb pain or promote motor recovery of the upper limbs after stroke. The tool has received great interest from health professionals; however, a clear understanding of the mechanisms underlying the neural recovery owing to MVF is lacking. OBJECTIVE: We performed a systematic review to assess the effect of MVF on brain activation during a motor task. METHODS: We searched PubMed, CINAHL, and EMBASE databases for neuroimaging studies investigating the effect of MVF on the brain. Key details for each study regarding participants, imaging methods, and results were extracted. RESULTS: The database search yielded 347 article, of which we identified 33 suitable for inclusion. Compared with a control condition, MVF increases neural activity in areas involved with allocation of attention and cognitive control (dorsolateral prefrontal cortex, posterior cingulate cortex, S1 and S2, precuneus). Apart from activation in the superior temporal gyrus and premotor cortex, there is little evidence that MVF activates the mirror neuron system. MVF increases the excitability of the ipsilateral primary motor cortex (M1) that projects to the "untrained" hand/arm. There is also evidence for ipsilateral projections from the contralateral M1 to the untrained/affected hand as a consequence of training with MVF. CONCLUSION: MVF can exert a strong influence on the motor network, mainly through increased cognitive penetration in action control, though the variance in methodology and the lack of studies that shed light on the functional connectivity between areas still limit insight into the actual underlying mechanisms.

Quantification of functional abilities in Rett syndrome: a comparison between stages III and IV.

We aimed to evaluate the functional abilities of persons with Rett syndrome (RTT) in stages III and IV. The group consisted of 60 females who had been diagnosed with RTT: 38 in stage III, mean age (years) of 9.14, with a standard deviation of 5.84 (minimum 2.2/maximum 26.4); and 22 in stage IV, mean age of 12.45, with a standard deviation of 6.17 (minimum 5.3/maximum 26.9). The evaluation was made using the Pediatric Evaluation of Disability Inventory, which has 197 items in the areas of self-care, mobility, and social function. The results showed that in the area of self-care, stage III and stage IV RTT persons had a level of 24.12 and 18.36 (P=0.002), respectively. In the area of mobility, stage III had 37.22 and stage IV had 14.64 (P<0.001), while in the area of social function, stage III had 17.72 and stage IV had 12.14 (P=0.016). In conclusion, although persons with stage III RTT have better functional abilities when compared with stage IV, the areas of mobility, self-care, and social function are quite affected, which shows a great functional dependency and need for help in basic activities of daily life.

Upper limb activity in children with unilateral spastic cerebral palsy: the role of vision in movement strategies.

This article reviews the capacity of children with unilateral spastic cerebral palsy (USCP) to (re)organize the available degrees of freedom and to use visual information in interceptive actions during motion with either the impaired or the less-impaired hand. Atypical reaching movements, such as increased trunk movement or slower wrist velocity, are considered adaptive coordination patterns that are the result of a change in the constraints. It is argued that manipulation of the task context facilitates children with USCP to enhance performance. For example, when reducing the time available to intercept a ball, the children are found to exceed their usual maximum walking speed and to increase range of motion of the elbow. In addition, the children appear to rely on a visual information strategy similar to typically developing children ('bearing angle'), although more variability is observed when using the impaired arm. The implications for interventions are, it should be recognized, that these children adapt to the impairment by reorganizing the movement system and that this process can be influenced by changing the task context. Attention should be paid to the importance of using correct visual cues for initiation and guidance of interceptive actions, which may be provoked by using external visual triggers.

Practicing a matching movement with a mirror in individuals with spastic hemiplegia.

This experimental study aimed to determine the effect of practicing a position matching task with (mirror) visual feedback of the less-impaired arm on the matching accuracy of the impaired arm in children and adolescents with spastic hemiparetic cerebral palsy. Practice consisted of 40 trials of bimanual target matching, where one group received regular visual feedback and a second group received mirror visual feedback of the less-impaired arm. On three occasions (pre, post, and after a 1-week-retention) position sense (matching accuracy measured as the endpoint error in cm) of the impaired arm was tested with a unimanual and bimanual matching task, performed without any visual information of either hand. Matching accuracy of the impaired arm was better in the post-test than in the pre-test, but this improvement was similar for both practice groups. In the retention-test, accuracy had returned to pre-test-level, which might be ascribed to the short duration of the practice period. These outcomes suggest that practicing a matching task with visual feedback of the less-impaired arm might help to improve the matching accuracy of the impaired arm in individuals with spastic hemiparetic cerebral palsy.

Matching accuracy in hemiparetic cerebral palsy during unimanual and bimanual movements with (mirror) visual feedback.

In the present study participants with Spastic Hemiparetic Cerebral Palsy (SHCP) were asked to match the position of a target either with the impaired arm only (unimanual condition) or with both arms at the same time (bimanual condition). The target was placed at 4 different locations scaled to the individual maximum reaching distance. To test the effect of mirror visual feedback of the less-impaired arm on the matching accuracy, an opaque screen or a mirror was placed in between the arms which masked vision of the impaired arm. Absolute endpoint error was smaller in the bimanual condition compared to the unimanual condition, but there was no effect of mirror visual feedback. Inspection of the individual data, however, showed that 13 out of 23 participants did experience a positive effect of mirror visual feedback. A positive correlation between the baseline error (screen) and the improvement in accuracy with mirror visual feedback seems to suggest that individuals with lower proprioceptive accuracy in the baseline condition may benefit more from mirror visual feedback. Together these findings indicate that bimanual therapy and therapy with mirror visual feedback might be valuable approaches for rehabilitation for a subset of the individuals with SHCP.

Deficits in upper limb position sense of children with Spastic Hemiparetic Cerebral Palsy are distance-dependent.

This study examined the arm position sense in children with Spastic Hemiparetic Cerebral Palsy (SHCP) and typically developing children (TD) by means of a contralateral matching task. This task required participants to match the position of one arm with the position of the other arm for different target distances and from different starting positions. Results showed that children with SHCP exhibited with both arms larger matching errors than the TD group, but only when the distance between the arms at the start of the movement was large. In addition, the difference in errors between the less-impaired and the impaired limb changed as a function of the distance in the SHCP group whereas no interlimb differences were found in the TD group. Finally, spasticity and restricted range of motion in children with SHCP were not related to the proportion of undershoot and size of absolute error. This suggests that SHCP could be associated with sensory problems in conjunction with their motor problems. In conclusion, the current study showed that accurate matching of the arms is greatly impaired in SHCP when compared to TD children, irrespective of which arm is used. Moreover, this deficit is particularly present for large movement amplitudes.

The positive effect of mirror visual feedback on arm control in children with spastic hemiparetic cerebral palsy is dependent on which arm is viewed.

Mirror visual feedback has previously been found to reduce disproportionate interlimb variability and neuromuscular activity in the arm muscles in children with Spastic Hemiparetic Cerebral Palsy (SHCP). The aim of the current study was to determine whether these positive effects are generated by the mirror per se (i.e. the illusory perception of two symmetrically moving limbs, irrespective of which arm generates the mirror visual feedback) or by the visual illusion that the impaired arm has been substituted and appears to move with less jerk and in synchrony with the less-impaired arm (i.e. by mirror visual feedback of the less-impaired arm only). Therefore, we compared the effect of mirror visual feedback from the impaired and the less-impaired upper limb on the bimanual coupling and neuromuscular activity during a bimanual coordination task. Children with SHCP were asked to perform a bimanual symmetrical circular movement in three different visual feedback conditions (i.e. viewing the two arms, viewing only one arm, and viewing one arm and its mirror image), combined with two head orientation conditions (i.e. looking from the impaired and looking from the less-impaired body side). It was found that mirror visual feedback resulted in a reduction in the eccentric activity of the Biceps Brachii Brevis in the impaired limb compared to the condition with actual visual feedback from the two arms. More specifically, this effect was exclusive to mirror visual feedback from the less-impaired arm and absent when mirror visual feedback from the impaired arm was provided. Across conditions, the less-impaired arm was the leading limb, and the nature of this coupling was independent from visual condition or head orientation. Also, mirror visual feedback did not affect the intensity of the mean neuromuscular activity or the muscle activity of the Triceps Brachii Longus. It was concluded that the positive effects of mirror visual feedback in children with SHCP are not just the result of the perception of two symmetrically moving limbs. Instead, in order to induce a decrease in eccentric neuromuscular activity in the impaired limb, mirror visual feedback from the 'unaffected' less-impaired limb is required.

Visual feedback of the non-moving limb improves active joint-position sense of the impaired limb in Spastic Hemiparetic Cerebral Palsy.

This study examined the active joint-position sense in children with Spastic Hemiparetic Cerebral Palsy (SHCP) and the effect of static visual feedback and static mirror visual feedback, of the non-moving limb, on the joint-position sense. Participants were asked to match the position of one upper limb with that of the contralateral limb. The task was performed in three visual conditions: without visual feedback (no vision); with visual feedback of the non-moving limb (screen); and with visual feedback of the non-moving limb and its mirror reflection (mirror). In addition to the proprioceptive measure, a functional test [Quality of Upper Extremity Skills Test (QUEST)] was performed and the amount of spasticity was determined in order to examine their relation with proprioceptive ability. The accuracy of matching was significantly influenced by the distance that had to be covered by the matching limb; a larger distance resulted in a lower matching accuracy. Moreover it was demonstrated that static (mirror) visual feedback improved the matching accuracy. A clear relation between functionality, as measured by the QUEST, and active joint-position sense was not found. This might be explained by the availability of visual information during the performance of the QUEST. It is concluded that static visual feedback improves matching accuracy in children with SHCP and that the initial distance between the limbs is an influential factor which has to be taken into account when measuring joint-position sense.