Effectiveness of action observation treatment based on pathological model in hemiplegic children: a randomized-controlled trial.

BACKGROUND: Action observation treatment (AOT) is an innovative therapeutic approach consisting in the observation of actions followed by their subsequent repetition. The standard version of AOT consists in the observation/imitation of a typically developed individual, which is proposed as model (TDM-AOT). AIM: This study aims to compare the effectiveness of AOT based on a pathological ameliorative model (PAM-AOT) versus TDM-AOT in improving upper limb ability in children with unilateral cerebral palsy (UCP). DESIGN: The study consists in a prospective randomized controlled, evaluator-blinded trial (RCT), with two active arms, designed to evaluate the effectiveness of AOT based on pathological model (PAM-AOT) as compared to a standard AOT based on TDM (TDM-AOT). SETTING: The 3-week AOT program was administered in a clinical setting. For some patients, the treatment was delivered at participant's home with the remote support of the physiotherapist (tele-rehabilitation). POPULATION: Twenty-six children with UCP (mean age 10.5±3.09 years; 14 females) participated in the study, with the experimental group observing a pathological model and the control group observing a typically developed model. METHODS: Motor assessments included unimanual and bimanual ability measures conducted at T0 (baseline, before the treatment), T1 (3 weeks after T0), T2 (8-12 weeks after treatment) and T3 (24-28 weeks after treatment); a subset of 16 patients also underwent fMRI motor assessment. Generalized Estimating Equations models were used for statistical analysis. RESULTS: Both groups showed significant improvement in bimanual function (GEE, Wald 106.16; P<0.001) at T1 (P<0.001), T2 (P<0.001), and T3 (P<0.001). Noteworthy, the experimental group showed greater improvement than the control group immediately after treatment (P<0.013). Both groups exhibited similar improvement in unimanual ability (GEE, Wald 25.49; P<0.001). The fMRI assessments revealed increased activation of ventral premotor cortex after treatment in the experimental compared with control group (GEE, Wald 6.26; P<0.012). CONCLUSIONS: Overall, this study highlights the effectiveness of PAM-AOT in achieving short-term improvement of upper limb ability in children with UCP. CLINICAL REHABILITATION IMPACT: These findings have significant implications for rehabilitative interventions based on AOT in hemiplegic children, by proposing a non-traditional approach focused on the most functional improvement achievable by imitating a pathological model.

Lesion mapping and functional characterization of hemiplegic children with different patterns of hand manipulation.

Brain damage in children with unilateral cerebral palsy (UCP) affects motor function, with varying severity, making it difficult the performance of daily actions. Recently, qualitative and semi-quantitative methods have been developed for lesion classification, but studies on mild to moderate hand impairment are lacking. The present study aimed to characterize lesion topography and preserved brain areas in UCP children with specific patterns of hand manipulation. A homogeneous sample of 16 UCP children, aged 9 to 14 years, was enrolled in the study. Motor assessment included the characterization of the specific pattern of hand manipulation, by means of unimanual and bimanual measures (Kinematic Hand Classification, KHC; Manual Ability Classification System, MACS; House Functional Classification System, HFCS; Melbourne Unilateral Upper Limb Assessment, MUUL; Assisting Hand Assessment, AHA). The MRI morphological study included multiple methods: (a) qualitative lesion classification, (b) semi-quantitative classification (sq-MRI), (c) voxel-based morphometry comparing UCP and typically developed children (VBM-DARTEL), and (d) quantitative brain tissue segmentation (q-BTS). In addition, functional MRI was used to assess spared functional activations and cluster lateralization in the ipsilesional and contralesional hemispheres of UCP children during the execution of simple movements and grasping actions with the more affected hand. Lesions most frequently involved the periventricular white matter, corpus callosum, posterior limb of the internal capsule, thalamus, basal ganglia and brainstem. VMB-DARTEL analysis allowed to detect mainly white matter lesions. Both sq-MRI classification and q-BTS identified lesions of thalamus, brainstem, and basal ganglia. In particular, UCP patients with synergic hand pattern showed larger involvement of subcortical structures, as compared to those with semi-functional hand. Furthermore, sparing of gray matter in basal ganglia and thalamus was positively correlated with MUUL and AHA scores. Concerning white matter, q-BTS revealed a larger damage of fronto-striatal connections in patients with synergic hand, as compared to those with semi-functional hand. The volume of these connections was correlated to unimanual function (MUUL score). The fMRI results showed that all patients, but one, including those with cortical lesions, had activation in ipsilesional areas, regardless of lesion timing. Children with synergic hand showed more lateralized activation in the ipsilesional hemisphere both during grasping and simple movements, while children with semi-functional hand exhibited more bilateral activation during grasping. The study demonstrates that lesion localization, rather than lesion type based on the timing of their occurrence, is more associated with the functional level of hand manipulation. Overall, the preservation of subcortical structures and white matter can predict a better functional outcome. Future studies integrating different techniques (structural and functional imaging, TMS) could provide further evidence on the relation between brain reorganization and specific pattern of manipulation in UCP children.

Signs of perceptual disorder during movement were reliably assessed in children with cerebral palsy in Sweden.

AIM: The aim of this Swedish study was to evaluate the assessment of clinical signs of perceptual disorder in children with cerebral palsy (CP). METHODS: Three experienced raters assessed 56 videos of 19 children from 1 to 18 years of age with bilateral spastic CP, which were recorded by colleagues at an Italian hospital. Six signs were evaluated for inter-rater reliability and criterion validity. Clinical applicability was evaluated by assessing inter-rater reliability between 47 Swedish clinicians, who examined 15 of the videos during face-to-face and online education seminars. There were 41 physiotherapists, two occupational therapists and four doctors, with 1-37 years of clinical experience and a median of 10 years. RESULTS: The experienced raters demonstrated moderate to almost perfect inter-rater reliability (kappa 0.54-0.81) and criterion validity (0.54-0.87) for startle reaction, upper limbs in startle position, averted eye gaze and eye blinking. The clinicians recognised these signs with at least moderate reliability (0.56-0.88). Grimacing and posture freezing were less reliable (0.22-0.35) and valid (0.09-0.50). CONCLUSION: Four of the six signs of perceptual disorder were reliably recognised by experienced raters and by clinicians after education seminars. Extended education and larger study samples are needed to recognise all the signs.

Effectiveness of the home-based training program Tele-UPCAT (Tele-monitored UPper Limb Children Action Observation Training) in unilateral cerebral palsy: a randomized controlled trial.

BACKGROUND: The effects of unilateral cerebral palsy (UCP) are largely observed in the upper limb (UL), which represents the main focus of rehabilitation for this disorder. Thanks to an increment in home training and progress in technology innovative systems have been created. The Tele-UPCAT (Tele-monitored UPper Limb Children Action Observation Training) platform is dedicated to the delivery at home of a program for UL rehabilitation, based on action observation therapy (AOT). AIM: This study aimed to investigate the immediate effectiveness of Tele-UPCAT for promoting UL skills in children with UCP and to determine if immediate effects were retained in the medium and long term. DESIGN: Tele-UPCAT was conducted on an intention-to-treat basis and was proposed as a randomized, allocation concealed (waitlist controlled) and evaluator-blinded clinical trial with two investigative arms: intensive in-home AOT program and standard care (SC). SETTING: This is a home-based AOT program delivered with a customized ICT platform. POPULATION: Thirty children (mean age 11.61±3.55 years) with confirmed diagnosis of spastic UCP with predominant UL involvement and cognitive level within or at normal limits were enrolled in this study. Orthopedic surgery or an intramuscular botulinum toxin A injection in the UL within 6 months prior to enrolment represented an exclusion criteria. METHODS: Participants were randomized using concealed random allocation. They were assessed according to the study design with the Assisting Hand Assessment (AHA), the Box and Block Test (BBT) and the Melbourne Assessment 2 (MA2). Linear mixed models were used for statistical analysis. RESULTS: A significant difference between the AOT and SC groups was identified immediately after the training on the AHA (6.406 [2.73] P=0.021) with an effect size (ES) of 1.99, and for the BBT of the less affected hand (9.826 [4.535] P=0.032) with an ES of 1.44. These effects were sustained at medium and long term. CONCLUSIONS: This study supports the effectiveness of AOT home training in promoting UL skills in children with UCP, with immediate effects lasting for 6 months. CLINICAL REHABILITATION IMPACT: This should encourage the use of technology for rehabilitative purposes and further applications of the AOT paradigm.

Evidence-based management and motor rehabilitation of cerebral palsy children and adolescents: a systematic review.

Background: Evidence regarding the management of several aspects of cerebral palsy improved in recent years. Still, discrepancies are reported in clinical practice. Italian professionals and stakeholders expressed the need of setting up updated, evidenced-based, shared statements, to address clinical practice in cerebral palsy rehabilitation. The objective of the present study was to provide an updated overview of the state of knowledge, regarding the management and motor rehabilitation of children and young people with cerebral palsy, as the framework to develop evidence-based recommendations on this topic. Methods: Guidelines and systematic reviews were searched, relative to evidence-based management and motor treatment, aimed at improving gross motor and manual function and activities, in subjects with cerebral palsy, aged 2-18 years. A systematic search according to the Patients Intervention Control Outcome framework was executed on multiple sites. Independent evaluators provided selection and quality assessment of the studies and extraction of data. Results: Four guidelines, 43 systematic reviews, and three primary studies were included. Agreement among guidelines was reported relative to the general requirements of management and motor treatment. Considering the subject's multidimensional profile, age and developmentally appropriate activities were recommended to set individual goals and interventions. Only a few approaches were supported by high-level evidence (i.e., bimanual therapy and constraint-induced movement therapy to enhance manual performance). Several task-specific active approaches, to improve gross motor function and gait, were reported (mobility and gait training, cycling, backward gait, and treadmill), based on low-level evidence. Increasing daily physical activity and countering sedentary behavior were advised. Based on the available evidence, non-invasive brain stimulation, virtual reality, action-observation therapy, hydrotherapy, and hippotherapy might be complementary to task or goal-oriented physical therapy programs. Conclusion: A multiple-disciplinary family-centered evidence-based management is recommended. All motor rehabilitation approaches to minors affected by cerebral palsy must share the following fundamental characteristics: engaging active involvement of the subject, individualized, age and developmentally appropriate, goal-directed, skill-based, and preferably intensive and time-limited, but suitable for the needs and preferences of the child or young person and their family, and feasible considering the implications for themselves and possible contextual limitations.

Prevalence and determinants of hip pain in non-ambulatory cerebral palsy children: a retrospective cohort study.

BACKGROUND: Hip pain is common in cerebral palsy children, particularly at Gross-Motor Function Classification System level IV-V. It is associated to hip displacement and relates to the migration percentage. Recent literature suggested early reconstructive bone surgery, as the best approach to prevent hip luxation, then hip pain. Still, high rates of hip pain are reported. AIM: To investigate prevalence and determinants of hip pain in an Italian cerebral palsy sample. DESIGN: Single-center retrospective cohort study. SETTING: Inpatient and outpatient. POPULATION: Patients with spastic or dyskinetic cerebral palsy, Gross-Motor Function Classification System level IV or V, age 0-18. METHODS: A chart review was implemented to report hip pain, as a dichotomous variable (pain/no pain), age, sex, cerebral palsy subtype, Gross-Motor Function level, lumbar scoliosis, migration percentage, previous orthopedic surgery, or botulinum injections, oral or intrathecal baclofen, drug-resistant epilepsy, assistive devices for standing or walking. Descriptive statistics and a multivariate logistic stepwise regression were performed. RESULTS: A total of 504 subjects were included: 302 level V, 209 females, 432 spastics. The mean length of follow-up was 6 years. The overall prevalence of hip pain was 8.9% (6.3% were at level V) and of hip dislocation was 19% (15.9% were at level V). Just 39% of dislocated hips were painful. Children at spastic subtype and level V were predominantly affected. Botulinum and soft tissue surgery related to lower rates of hip pain, without statistical significance. Age (OR 1.19, 95%CI 1.14-1.25, P value 0.000), sex (OR 1.72, 95%CI 1.18-2.52, P value 0.005), migration percentage (OR 1.02, 95%CI 1.02-1.03, P value 0.000) and lumbar scoliosis (OR 1.32, 95%CI 0.86-2.01, P value 0.200) resulted significant independent determinants of hip pain. CONCLUSIONS: Hip pain relates with the migration percentage, but not all dislocated hips become painful. Hip pain may be transient and requires a targeted and individualized approach. Children at spastic subtype and level V were predominantly affected. Age and sex are confirmed as determinants. Specific validated measures are to be implemented to assess hip pain. CLINICAL REHABILITATION IMPACT: Considering severe non-ambulatory cerebral palsy patients, pain and quality of life should be considered as outcomes, in the management of hip luxation.

Hip subluxation in Italian cerebral palsy children and its determinants: a retrospective cohort study.

The study's aim was two-fold: to describe the trend of hip subluxation in the largest sample of Italian nonambulatory cerebral palsy (CP) children ever published; to investigate its determinants. This single-centre retrospective cohort study included patients with spastic or dyskinetic CP, Gross Motor Function Classification System (GMFCS) level IV or V, age 0-18 years, having been referred to our unit before March 2020. The hip subluxation was measured by means of the migration percentage (MP). Other data were gathered such as sex, CP subtype, GMFCS level, presence of drug-resistant epilepsy, age, use of walkers with weight relief or standing devices, previous botulinum injection or hip surgery, oral or intrathecal baclofen and hip pain. Multiple linear stepwise regression was performed and descriptive statistics are provided. Spastic CP had MP maximum increase in early ages, with GMFCS level V values persistently higher than level IV. The dyskinetic subtype showed a slower increase of the MP, with GMFCS level IV presenting similar or higher values, compared to level V. Age, CP severity and spastic subtype are the main determinants. The stepwise multiple regression analysis demonstrated that weight relief walking and standing assistive devices, combined with botulinum contributed to reduce the MP progression. Dyskinetic CP showed overall lower MP values and a more variable behaviour relative to age and GMFCS level, compared to the spastic subtype. Standing and walking assistive devices, with partial or total weight relief, combined with individually targeted botulinum injections, should be considered in the management of bilateral nonambulatory CP patients, to prevent hip subluxation or its recurrence after surgery.

Evaluation of sense of position and agency in children with diplegic cerebral palsy: A pilot study.

PURPOSE: This study explores non-motor impairment of the upper dominant limb in children with diplegic cerebral palsy (CP). Specifically, it firstly investigates sense of position without visual control, ability to compare visual and proprioceptive information, and capacity to recognize effects of self-movement. Secondly, it explores the possible association between these items with cognitive function, perceptual disorder (PD), and manual ability (Manual Ability Classification System -MACS). METHODS: Ten subjects (7 female; 3 male) were tested with three protocols: the first one (A) explored sense of position, the second one (B) collimation between visual and proprioceptive information, and the last one (C) sense of agency with the use of videogames. RESULTS: All subjects executed Protocol A without making any mistakes, while in Protocol B the median percentage of mistakes was 4.8%. Mistakes were recorded more frequently in positions characterized by wrist extension. Data reported a significant correlation with Processing Speed Index (PSI). Sense of agency was positively associated with cognitive functioning, with a significant correlation with PSI.PD, MACS, and Video-Gaming Experience (VGE) showed no correlation with Protocol C. CONCLUSION: In the observed sample with diplegic CP, preliminary data support the hypothesis that there is an alteration of both sense of position and sense of agency. They were both associated to PSI, with a positive trend of correlation with cognitive functioning. PD seemed to have no influence. Further studies, with a larger sample size, a control group, and involving children without CP, are required to corroborate the results obtained.

Application of Virtual Reality Rehabilitation System for the assessment of postural control while standing in typical children and peers with neurodevelopmental disorders.

BACKGROUND: The assessment of postural control in children is crucial, due to its central role in their overall development. However, a tool that objectively quantifies the difference in postural control between typical and atypical developing children is lacking. In this study, we introduce a new technology (Virtual Reality Rehabilitation System, VRRS) for assessing children's postural control. RESEARCH QUESTION: Is this new assessment tool capable to highlight the differences between typical development (TD) and atypical development, (children with Developmental Coordination Disorder (DCD) and Cerebral Palsy (CP))? METHODS: 30 TD children, 20 children with DCD and 27 with CP (mean ages: 6.29 ± 2.74; 9.11 ± 2.65; 10.07 ± 3.89 years) were tested with the VRRS Tablet with stabilometric balance platform. Postural parameters, related to the movements of the Centre of Pressure (COP) were collected. A multivariate analysis of variance (MANOVA) followed by a post-hoc analysis has been carried out. Moreover, the influence of age, sex, clinical scores and sub-diagnoses on parameters of interest has been explored. RESULTS: COP distance and sway area in the three groups (TD: 7.35 ± 2.32 mm, 101.70 ± 64.16 mm2/s; DCD: 12.05 ± 8.19 mm, 188.46 ± 231.23 mm2/s; CP: 13.25 ± 8.09 mm, 239.13 ± 313.83 mm2/s, respectively) and all other VRRS parameters were significantly different among the three groups (p-values between 0.028 and <0.001). The TD group showed significantly different values than CP (p from < 0.03 to < 0.001) but not than DCD (p = n.s.). Clinical scores showed to correlate with the COP distances and Root Mean Square distances in all subgroups (p < 0.05). For age, only an influence was found within the TD group (p < 0.01); sex did not show to affect the outcomes (p = n.s.). SIGNIFICANCE: An objective tool for quantitative measurement of postural control in childhood is needed. Our proposed VRRS tool could support the traditional assessment tests, highlighting differences between typical and atypical development.

Effectiveness comparison between carbon spring and hinged ankle-foot orthoses in crouch gait treatment of children with diplegic cerebral palsy: a randomized crossover trial.

BACKGROUND: Children with cerebral palsy (CP) often present a loss of effectiveness of the plantarflexors/knee-extensors couple that leads to crouch gait. When treating a child with crouch gait by means of ankle foot orthoses, preserving or restoring push off power is a key issue. AIM: To compare carbon-fiber spring (Carbon Ankle Seven® [CAFO], Ottobock® HealthCare, Duderstadt, Germany) and hinged ankle-foot orthoses (HAFO) effectiveness in improving functionality and walking ability in children with diplegic CP and crouch gait. DESIGN: Randomized crossover trial. SETTING: Hospital center. POPULATION: Ten children with diplegic CP and crouch gait, 5 males and 5 females, aged 11 (4) years. METHODS: The gait of each child was evaluated by means of instrumental gait analysis with both CAFO and HAFO, in a randomized order and after a 4-week adaptation period. The primary outcome measure was the change in ankle power generation. As secondary outcome measures, knee joint kinematics, stride length, walking speed, Observational Gait Scale, and preferred orthosis were considered. RESULTS: The median of the energy produced in stance was superior with CAFO (+2.2 J/kg, IQR 4.7, P=0.006), and the energy absorbed inferior (-3.3 J/kg, IQR 4.3, P=0.011). No statistically significant difference was found for any other parameter. Preference of the children was equally distributed between the two orthoses. CONCLUSIONS: No evident superiority of CAFO with respect to HAFO was found in improving gait performance of children with CP and crouch gait. Nevertheless, the results suggest the possibility that CAFO permits an energy saving and reduction of the more compromising deficits. CLINICAL REHABILITATION IMPACT: The final choice of the participants indicates that CAFOs are preferred by older and heavier children, but the preference does not correlate with the performance of the orthoses during gait.

Reorganization of action observation and sensory-motor networks after action observation therapy in children with congenital hemiplegia: A pilot study.

New rehabilitation programs based on action observation therapy (AOT) are effective in improving motor function in children with congenital hemiplegia. In this pilot study we tested the potential effects of AOT on the reorganization of the motor system by functional magnetic resonance imaging (fMRI). As part of a randomized trial, eight subjects (age range: 6.2-14.5 years) with congenital hemiplegia were randomly assigned to an experimental (EG) or control (CG) group. All children underwent a clinical and neurophysiological assessment with Assisting Hand Assessment (AHA), MRI, and fMRI at baseline (T0), 1(T1), and 8(T2) weeks after the end of 3-week treatment. For the EG, AOT consisted in the observation of uni/bimanual goal-directed actions followed by their execution. CG watched same-duration computer games and then performed the same actions in the same order used in the EG. fMRI study was carried out using two different paradigms, for exploring sensory-motor network (SMN) localization and action observation network (AON). The pattern of brain activation was generally similar between T0 and T1 for both groups, while it was more widespread at T2, compared to T0 and T1, in the EG. This enlargement was coupled with functional improvement at AHA. Single-subject analysis shows a reduction of lateralization indexes both for the AON and the SMN. This pilot study, despite the small sample, showed the fMRI feasibility for providing relevant biomarkers of brain plasticity for monitoring the AOT response in children with congenital hemiplegia. The study was registered at http://www.clinicaltrials.gov (identifier NCT01016496).

Construct Validity of the Activities Scale for Kids Performance in Children with Cerebral Palsy: Brief Report.

Aims: This study collects evidence of construct convergent validity of the Activity Scale for Kids performance (ASKp), comparing its results with the 66-item Gross Motor Function Measure (GMFM-66) and with the Gross Motor Function Classification System (GMFCS) and testing the ASKp's ability to discriminate between individuals with different functional capabilities. Methods: This cross-sectional study involved 60 children with spastic cerebral palsy (CP) assessed with the GMFM-66 who self-administered the Italian version of the ASKp. Results: Children were 10.9 (±3) years old with GMFCS Level I-III. Moderate correlations were found between GMFM and ASKp scores (r = 0.577; p < .001), and between GMFCS levels and ASKp score (rs = -0.541, p < .001). The ASKp discriminated between children with different functional capabilities, determined by the GMFCS (F = 18.2, p < .001). Conclusions: ASKp is valid to assess physical functioning, a crucial domain in rehabilitation of children with spastic CP. Trial registration: ClinicalTrials.gov Identifier: NCT03325842.

Cross-cultural validation of Activities Scale for Kids: the performance of healthy Italian children.

BACKGROUND: The assessment of physical performance is a key element in the rehabilitation of individuals with disabilities, and understanding patients' perception of their abilities is of foremost importance for the success of the whole process. The Activities Scale for Kids performance (ASKp) is one of the few reliable and responsive outcome measures that allows children and adolescents to accurately report their physical functioning in typical activities for this age. The performance version of the ASKp has recently been translated and culturally adapted to the Italian context. METHODS: This cross-sectional study was implemented to describe the ASKp score distribution in a population of Italian school aged individuals. RESULTS: A population of 209 Italian children with a mean age of 10.96 years (SD 2.92, range 5-15) participated in this study during the academic year 2017/18. The ASKp score ranged from 52.58 to 100, with a mean value of 89.07 points (SD 10.57). Scores greater than 90 were attained for 57.4% of participants, confirming the ceiling effect of this assessment tool when administered in healthy children (95% CI: 50.4-64.2%). The ASKp was able to differentiate between different levels of physical performance according to different developmental ages (P<0.001), and it confirmed the ability to distinguish between children with typical development and those with disability (P<0.001). CONCLUSIONS: The results of this study provide the first evidence of construct validity of the Italian version of the ASKp. These results will help clinicians to interpret ASKp scores of children with musculoskeletal limitations.

Feasibility of a Home-Based Action Observation Training for Children With Unilateral Cerebral Palsy: An Explorative Study.

Unilateral Cerebral Palsy (UCP), the most frequent form of Cerebral Palsy, usually affects more the upper limb (UL) than the lower limb. Rehabilitation programs are addressed to improve manual abilities and UL use. In recent years, Information and Communication Technology (ICT) has been introduced in rehabilitation to increase treatment opportunities for patients, and also in home-based intervention. Moreover, the discovery of the Mirror Neuron System allowed to insert a new paradigm of treatment that is the Action Observation Training (AOT). The aim of the present study was to investigate the feasibility of a new rehabilitative home-based approach, called Tele-UPCAT (Tele-monitored UPper Limb Children Action Observation Training), based on the principles of AOT, in a group of Italian children and adolescents with UCP. This investigation was to provide information about the possibility of introducing ICT in telerehabilitation field. Twenty-nine children aged 11.73 ± 3.65 years (range 6.00-18.75) with a diagnosis of UCP participated in the study. They carried out 15 days of training based on the AOT paradigm with Tele-UPCAT system while wearing Actigraphs on both wrists. The feasibility of both training and study design and procedures was assessed through nine criteria taken from existent literature and from a questionnaire designed and realized ad hoc for the purpose, based on standard items of usability and acceptability. All feasibility criteria were met: 80% of training sessions were completed in the planned time and no significant technical issues were found. From the questionnaire, total scores were all above 82.15%, while the four sections obtained the following scores: (i) customization of exercises 80.00%; (ii) acceptability at home, 77.50%; (iii) required effort 80.00%; and (iv) suitability of manual and software 95.00%. No differences were found for age and sex. Tele-UPCAT demonstrated to be feasible as a home-based AOT for children and adolescents with UCP. Trial registration NCT03094455.

Are perceptual disorder signs in diplegic cerebral palsied children stable over time? A retrospective cohort analysis.

BACKGROUND: A group of diplegic cerebral palsied (CP) children presents six precise signs that can be easily observed during clinical examinations, physiotherapy sessions and everyday activities. These signs are: startle reaction, upper limbs in startle position, averted-eye gaze, grimaces, eye blinking and posture freezing. METHODS: In a population of 32 diplegic CP children (aged 1-8 years) perceptual signs were retrospectively identified through videos to verify their stability in the same child over time. RESULTS: Startle reaction, upper limb in startle position and posture freezing were the most frequently observed signs and the easiest to recognize with the highest agreement in both observations (P<0.001). Eye signs (eye blinking and averted-eye gaze) were more difficult to detect in our recordings. CONCLUSIONS: Signs of perceptual disorders were present in our sample of diplegic CP children from the second year of age and could still be observed after 1- to 3-year intervals, demonstrating they remain unaltered over time. Furthermore, if absent in the first observation, they did not appear later on. CP children with these perceptual signs could represent a new clinical entity, which we are currently describing and defining.

From movement to action: a new framework for cerebral palsy.

The interpretation of cerebral palsy (CP) is closely linked to points of view that are no longer acceptable: 1) the idea that it is primarily a motor problem (posture and movement disorder); 2) the idea that it is only a central (cerebral) pathology; 3) the idea that it is a non-progressive disease (fixed encephalopathy). Actually, the problems that contribute to producing the CP clinical picture are several and complex. First of all, building of the action, starting from subject motivation, through motor imagery and subsequent project elaboration. Sequentially, executive planning, disorder often hidden under the most remarkable alteration of motor patterns and muscle tone. Finally, realization, conditioned by the idea that the locomotor apparatus is only and always the victim of an incapable central nervous system. Little known and very neglected perceptive components can contribute to compromising subject motor control. The influences that primitive changes of musculoskeletal system, often depending on site, nature, size and time of the lesion, exert on the possible choices of the central nervous system are often overlooked. Peripheral structures can in fact modify considerably the expression of palsy (understood as the form of adaptive functions) primitively. At least six different sources of error can be identified in the cerebral palsied child. For a rehabilitative intervention with greater possibilities of effectiveness, it is necessary to recognize and evaluate each of them. Especially as regards the prevention of secondary deformities, the responsibility attributed to physiotherapy must be re-evaluated.

Explicit Motor Imagery for Grasping Actions in Children With Spastic Unilateral Cerebral Palsy.

Background: Motor Imagery (MI) refers to mental simulation of a motor action without producing any overt movement. Previous studies showed that children with Unilateral Cerebral Palsy (UCP) are impaired in implicit MI, as demonstrated by the performance of Hand Laterality Judgment tasks. The aim of this study was to examine the specificity of explicit MI deficits in UCP children. Methods: A group of UCP children (n = 10; aged 9-14) performed a mental chronometry task consisting in grasping an object and placing it into a container, or in imagining to perform the same action. As control, a group of typically developing (TD) children, matched by age, performed the same task. Movement durations for executed and imagined trials were recorded. A subgroup of 7 UCP children and 10 TD children also underwent a session of functional MRI to examine the activation of parieto-frontal areas typically associated to MI processes, during the imagination of reaching-grasping actions performed with the paretic hand. Results: Behavioral results revealed the existence of a correlation between executed and imagined movement durations both in TD and UCP groups. Moreover, the regression analysis in TD children showed that higher scores in mental chronometry tasks were positively correlated to increased bilateral activation of the intraparietal sulcus (IPS), superior parietal lobule (SPL), and dorsal premotor (PMd) cortex. A similar analysis revealed in the UCP group a positive correlation between a higher score in the mental chronometry task and bilateral activations of IPS, and to activation of contralesional, right PMd, and putamen during imagination of grasping movements. Conclusions: These results provide new insights on the relationship between MI capacity and motor deficits in UCP children, suggesting the possibility of the use of explicit MI training to improve patient's upper limb motor functions.

Mirror Neuron System Activation in Children With Unilateral Cerebral Palsy During Observation of Actions Performed by a Pathological Model.

Background. Recent evidence suggested that Action Observation Therapy (AOT), based on observation of actions followed by immediate reproduction, could be a useful rehabilitative strategy for promoting functional recovery of children affected by unilateral cerebral palsy (UCP). AOT most likely exploits properties of the parieto-premotor mirror neuron system (MNS). This is more intensely activated when participants observe actions belonging to their own motor repertoire. Objective. The aim of the present study was to investigate the issue of whether MNS of UCP children is better activated by actions performed by a paretic hand rather than a healthy one. Methods. Using functional magnetic resonance imaging, we assessed brain activation in a homogeneous group of 10 right UCP children compared with that of 10 right-handed typically developing (TD) children, during observation of grasping actions performed by a healthy or a paretic hand. Results. The results revealed a significant activation within the MNS in both UCP and TD children, more lateralized to the left hemisphere in the TD group. Most important, region of interest (ROI) analysis on parietal and premotor regions showed that, in UCP, the MNS was more strongly activated by observation of actions performed by the paretic hand, a motor model more similar to the observer's motor repertoire. Conclusions. This study shows that children affected by spastic UCP exhibit enhanced activation of the MNS during observation of goal-directed actions performed by a pathological model with respect to a healthy one.

Gait-Based Diplegia Classification Using LSMT Networks.

Diplegia is a specific subcategory of the wide spectrum of motion disorders gathered under the name of cerebral palsy. Recent works proposed to use gait analysis for diplegia classification paving the way for automated analysis. A clinically established gait-based classification system divides diplegic patients into 4 main forms, each one associated with a peculiar walking pattern. In this work, we apply two different deep learning techniques, namely, multilayer perceptron and recurrent neural networks, to automatically classify children into the 4 clinical forms. For the analysis, we used a dataset comprising gait data of 174 patients collected by means of an optoelectronic system. The measurements describing walking patterns have been processed to extract 27 angular parameters and then used to train both kinds of neural networks. Classification results are comparable with those provided by experts in 3 out of 4 forms.

Spinal bracing and lung function in type-2 spinal muscular atrophy.

BACKGROUND: Respiratory muscle weakness associated with scoliosis in type-2 spinal muscular atrophy (SMA) leads to respiratory impairment. Spinal brace, generally utilized to slow scoliosis progression and support sitting, could worsen lung function and hamper cough maneuvers. CASE SERIES: Six home-treated type-2 SMA children (aged 6-15 years, subtype 2.1-2.5) were assessed to evaluate time-dependent influence of "static-balanced brace" on pulmonary function. Lung function tests, including peak expiratory flow (PEF), peak cough flow (PCF), maximal static inspiratory pressure (MIP), maximal static expiratory pressure (MEP), forced vital capacity (FVC), were performed. PEF, MEP, FVC parameters were higher in tests after wearing braces three-hours, PCF slightly higher and MIP slightly lower compared to upon awakening values. CLINICAL REHABILITATION IMPACT: "Static-balanced brace" did not impair lung function in our sample of six type-2 SMA children; in addition, it seemed to support cough maneuvers. Double assessment is determinant for decisions concerning use/non-use of brace differently from the usual one time evaluation procedure (base level compared to level wearing brace).