A scoping review of scientific concepts concerning motor recovery after stroke as employed in clinical trials.

The scientific literature on poststroke rehabilitation is remarkably vast. Over the last decades, dozens of rehabilitation approaches have been investigated. However, sometimes it is challenging to trace new experimental interventions back to some of the known models of motor control and sensorimotor learning. This scoping review aimed to investigate motor control models' diffusion among the literature on motor recovery after stroke. We performed a literature search on Medline, Cochrane, Web of Science, Embase, and Scopus databases. The last search was conducted in September 2023. This scoping review included full-text articles published in English in peer-reviewed journals that provided rehabilitation interventions based on motor control or motor learning frameworks for at least one individual with stroke. For each study, we identified the theoretical framework the authors used to design the experimental treatment. To this aim, we used a previously proposed classification of the known models of motor control, dividing them into the following categories: neuroanatomy, robotics, self-organization, and ecological context. In total, 2,185 studies were originally considered in this scoping review. After the screening process, we included and analyzed 45 studies: 20 studies were randomized controlled trials, 12 were case series, 4 were case reports, 8 were observational longitudinal pilot studies, and 1 was an uncontrolled trial. Only 10 studies explicitly declared the reference theoretical model. Considering their classification, 21 studies referred to the robotics motor control model, 12 to the self-organization model, 8 to the neuroanatomy model, and 4 to the ecological model. Our results showed that most of the rehabilitative interventions purposed in stroke rehabilitation have no clear theoretical bases on motor control and motor learning models. We suggest this is an issue that deserves attention when designing new experimental interventions in stroke rehabilitation.

Individualized Remotely Supervised Motor Activity Programs Promote Rehabilitation Goal Achievement, Motor Functioning, and Physical Activity of People with Rett Syndrome-A Single-Cohort Study.

BACKGROUND: Gross motor function in Rett syndrome (RTT) is always limited. The complex clinical picture typical of most people with RTT requires intensive and specific rehabilitation programs. Previous reports on remotely supervised motor activity programs suggested positive outcomes for this population. The current article describes the impact of a remote-supervised motor activity program carried out by family members of individuals with RTT on achieving rehabilitation goals and improving gross and fine motor functioning and daily physical activity. METHODS: Forty subjects with RTT followed a three-month remotely supervised motor activity program carried out by their family members at home after a three-month baseline period. After the end of the intervention, a three-month wash-out period was implemented. Rehabilitation goal achievement, motor functioning, and level of daily physical activity were measured. RESULTS: 82.4% of rehabilitation goals were achieved or overachieved. Participants' motor functioning and physical activity significantly increased after the intervention (p ˂ 0.001). Improvements were maintained after the wash-out phase. CONCLUSIONS: The proposed intervention was effective for people with RTT of various ages and severity levels. The results highlight the need for lifelong, individualized, daily based, and professionally supervised rehabilitation possibilities for individuals with RTT.

Impact of a Remotely Supervised Motor Rehabilitation Program on Maternal Well-Being During the COVID-19 Italian Lockdown.

COVID-19 Lockdown was particularly challenging for most mothers of people with intellectual disabilities, including those with Rett syndrome (RTT), leading to feelings of abandonment from healthcare services of their children. Within those days, telerehabilitation has represented a valid alternative to support physical activity and treatment, supporting parents in structuring their children's daily routine at home. This article aims to describe the well-being level of two groups of mothers of girls and women with RTT who were involved in a home-based remotely supervised motor rehabilitation program, respectively, before and during the COVID-19 Italian lockdown. Forty participants with classic RTT were recruited before the lockdown and randomly assigned to two groups that performed the intervention immediately before (Group 1) and during (Group 2) the lockdown, respectively. The intervention included an individualized daily physical activity program carried out for 12 weeks by participants' parents and fortnightly supervised throughout Skype contacts to plan, monitor, and accommodate individual activities in the participant's life at home. The short form Caregivers Well-Being Scale was collected for the mothers in each group 12 weeks before intervention (T1), at intervention initiation (T2), immediately after intervention termination (T3), as well as at 12 weeks after intervention termination (T4). Mothers of participants in the Group 1 showed a stable level of well-being across all four evaluations with a slight improvement during the lockdown, without significant change. Similarly, the well-being level of mothers in the Group 2 showed a statistically significant increase in their well-being between T2 and T3 (during the lockdown) and its reduction to the pre-intervention level between T3 and T4 (after the lockdown). The results suggest that the lockdown did not negatively affect the participants' mothers' well-being, leading to its improvement. Moreover, the proposed intervention could have supported the mothers in managing the new daily routine at home, positively affecting maternal well-being.

Influence of sagittal pelvic attitude on gait pattern in normally developed people and interactions with neurological pathologies: A pilot study.

Background: Gait Analysis of healthy people, imitating pathological conditions while walking, has increased our understanding of biomechanical factors. The influence of the pelvis as a biomechanical constraint during gait is not specifically studied. How could mimicking a pelvic attitude influence the dynamic mechanical interaction of the body segments? We proposed an investigation of the pelvic attitude role on the gait pattern of typically developed people when they mimicked pelvic anteversion and posteroversion. Materials and methods: Seventeen healthy volunteers were enrolled in this study (mean age 24.4 ± 5.5). They simulated a pelvic anteversion and posteroversion during walking, exaggerating these postures as much as possible. 3D gait analysis was conducted using an optoelectronic system with eight cameras (Vicon MX, Oxford, United Kingdom) and two force plates (AMTI, Or-6, Watertown, MA, United States). The kinematic, kinetic, and spatio-temporal parameters were compared between the three walking conditions (anteversion, posteroversion, and normal gait). Results: In Pelvic Anteversion gait (PA) we found: increased hip flexion (p < 0.0001), increased knee flexion during stance (p = 0.02), and reduction of ankle flexion-extension Range of Motion (RoM) compared with Pelvic Normal gait (PN). In Pelvic Posteroversion gait (PP) compared with PN, we found: decreased hip flexion-extension RoM (p < 0.01) with a tendency to hip extension, decreased knee maximum extension in stance (p = 0.033), and increased ankle maximum dorsiflexion in stance (p = 0.002). Conclusion: The configuration of PA contains gait similarities and differences when compared with pathologic gait where there is an anteversion as seen in children with Cerebral Palsy (CP) or Duchenne Muscular Dystrophy (DMD). Similarly, attitudes of PP have been described in patients with Charcot-Marie-Tooth Syndrome (CMT) or patients who have undergone Pelvic Osteotomy (PO). Understanding the dynamic biomechanical constraints is essential to the assessment of pathological behavior. The central nervous system adapts motor behavior in interaction with body constraints and available resources.

Comparison of the Gait Biomechanical Constraints in Three Different Type of Neuromotor Damages.

Background and Objective: Absolute angle represents the inclination of a body segment relative to a fixed reference in space. This work compares the absolute and relative angles for exploring biomechanical gait constraints. Methods: Gait patterns of different neuromotor conditions were analyzed using 3D gait analysis: normal gait (healthy, H), Cerebral Palsy (CP), Charcot Marie Tooth (CMT) and Duchenne Muscular Dystrophy (DMD), representing central and peripheral nervous system and muscular disorders, respectively. Forty-two children underwent gait analysis: 10 children affected by CP, 10 children by CMT, 10 children by DMD and 12 healthy children. The kinematic and kinetic parameters were collected to describe the biomechanical pattern of participants' lower limbs. The absolute angles of thigh, leg and foot were calculated using the trigonometric relationship of the tangent. For each absolute series, the mean, range, maximum, minimum and initial contact were calculated. Kinematic and kinetic gait data were studied, and the results were compared with the literature. Results: Statistical analysis of the absolute angles showed how, at the local level, the single segments (thigh, leg and foot) behave differently depending on the pathology. However, if the lower limb is studied globally (sum of the kinematics of the three segments: thigh, leg and foot), a biomechanical constraint emerges. Conclusion: Each segment compensates separately for the disease deficit so as to maintain a global biomechanical invariance. Using a model of inter-joint co-variation could improve the interpretation of the clinical gait pattern.

Upper Body Physical Rehabilitation for Children with Ataxia through IMU-Based Exergame.

BACKGROUND: Children with ataxia experience balance and movement coordination difficulties and needs intensive physical intervention to maintain functional abilities and counteract the disorder. Exergaming represents a valuable strategy to provide engaging physical intervention to children with ataxia, sustaining their motivation to perform the intervention. This paper aims to describe the effect of a home-conducted exergame-based exercise training for upper body movements control of children with ataxia on their ataxic symptoms, walking ability, and hand dexterity. METHODS: Eighteen children with ataxia were randomly divided into intervention and control groups. Participants in the intervention group were asked to follow a 12-week motor activity program at home using the Niurion® exergame. Blind assessments of participants' ataxic symptoms, dominant and non-dominant hand dexterity, and walking ability were conducted. RESULTS: On average, the participants performed the intervention for 61.5% of the expected time. At the end of the training, participants in the intervention group showed improved hand dexterity that worsened in the control group. CONCLUSION: The presented exergame enhanced the participants' hand dexterity. However, there is a need for exergames capable of maintaining a high level of players' motivation in playing. It is advisable to plan a mixed intervention to take care of the multiple aspects of the disorder.

Intensive Postural and Motor Activity Program Reduces Scoliosis Progression in People with Rett Syndrome.

BACKGROUND: A scoliosis prevalence of 94% was reported in the population with Rett syndrome (RTT), with an annual progression rate of 14 to 21° Cobb which may result in pain, loss of sitting balance, deterioration of motor skills, and lung disfunction. This paper describes the efficacy of an intensive conservative individualized physical and postural activity program in preventing scoliosis curvature progression in patients with RTT. METHODS: Twenty subjects diagnosed with RTT and scoliosis were recruited, and an individualized intensive daily physical activity program was developed for each participant. Each program was conducted for six months by participants' primary caregivers in their daily living environment. Fortnightly remote supervision of the program implementation was provided by an expert therapist. Pre- and post-intervention radiographs and motor functioning were analyzed. RESULTS: An averaged progression of +1.7° ± 8.7° Cobb, over one year (12.3 ± 3.5 months) was observed in our group, together with motor function improvements. A relation between curve progression and motor skill improvement was observed. CONCLUSIONS: The intervention prevented scoliosis progression in our group. The achievement of functional motor improvements could enable better body segment control and muscle balancing, with a protective effect on scoliosis progression. The intervention was effective for individuals with RTT across various ages and severity levels. Individual characteristics of each participant and the details of their activity program are described.

Skype Supervised, Individualized, Home-Based Rehabilitation Programs for Individuals With Rett Syndrome and Their Families - Parental Satisfaction and Point of View.

Individuals with Rett syndrome (RTT) experience impaired gross motor skills limiting their capacity. Therefore, they need support to participate in physical activities, and it is crucial to work with primary caregivers when developing appropriate strategies, thereby leading to an active lifestyle. There is limited evidence supporting the effectiveness of remotely supported physical activity interventions. This project aimed to evaluate the effects of a skype-based, telehealth-delivered physical activity program carried out by participants' parents at home. This article will focus on parental points of view. A mixed-methods design evaluating parental satisfaction was conducted. Forty participants with a confirmed genetic diagnosis of RTT and their families were recruited. The intervention included a 12-week individualized daily physical activity program carried out by participants' parents and bi-weekly supervised by expert therapists. Parents' impressions and feelings related to the program implementation were collected throughout semi-structured interviews, and an ad hoc developed questionnaire and discussed. The current project results suggest that a remote physical rehabilitation program, supported fortnightly by video calls, represents an effective way of conducting a remote physical therapy intervention for this population and that it can be easily carried out at home by primary caregivers, promoting positive functional changes, without bringing feelings of frustration due to the required workload. The strategies that families have learned during the program to support the motor activities of their daughters represent an easily performed set of tools that they can maintain and use in everyday life even after the cessation of the program.

Development of SaraHome: A novel, well-accepted, technology-based assessment tool for patients with ataxia.

BACKGROUND AND OBJECTIVE: Early onset ataxias (EOAs) are a heterogeneous group of neurological conditions, responsible for severe motor disability in paediatric age, which still lack reliable outcome measures. Available scales to assess ataxia, such as the Scale for Assessment and Rating of Ataxia (SARA), are based on subjective assessment of specific motor and language tasks by an examiner, and therefore is age dependent and lacks accuracy in detecting small variations in disease severity. In last years, novel technologies, including computer interfaces and videogames, have emerged for clinical applications and the advent of Internet of Medical Things and of Information Communication Technology have allowed the remote control of such technologies. This pilot study describes a newly developed tool (SaraHome) for the assessment at home of EOA evaluating its feasibility and acceptability on a small sample of children. METHODS: Ten EOA children and ten caregivers have been enrolled for a preliminary outpatient evaluation. The Microsoft Kinect 2.0 and Leap Motion Controller (LMC) connected to a personal computer with an ad hoc software have been set-up, for the acquisition of standardized motor tasks performed by the patients with the caregivers' assistance. Acceptance and practicability have been tested by QUEST 2.0 and IMI questionnaires in caregivers and patients respectively. RESULTS: The SaraHome software was developed, based on a collection of services provided by a complex architecture that consists of a Restful interface, which enables to access a series of plugins for the execution of different tasks. A graphical user interface allows the acquisition of the patient movements while performing a motor task. A protocol of standard tasks inspired by SARA was established, and a system of video-assisted instruction provided. The set-up for the optimal acquisition of such protocol by Kinect and LMC has been defined. Both patients and caregivers accomplished the SaraHome assessment with good feedback at the technology acceptance questionnaires. CONCLUSIONS: SaraHome represents a newly developed tool for the assessment of ataxia in patients, resulting from the integration of low-cost and easy-accessible technologies. This pilot application highlighted the feasibility and the acceptability of the system, suggesting the potential use in clinical practice.

A wearable video-oculography based evaluation of saccades and respective clinical correlates in patients with early onset ataxia.

BACKGROUND: Friedreich Ataxia (FRDA) and other inherited chronic ataxias (CAs) are common causes of early onset ataxias (EOA), a group of conditions still lacking effective therapies and biomarkers. Ocular saccades are considered a reliable paradigm of motor control, useful to track the functioning of underlying neural networks and serving as potential markers for neurological diseases. NEW METHOD: A non-invasive video-oculography device (EyeSeeCam) was used to test saccadic parameters (latency, amplitude, duration, velocity) and peak velocity/amplitude ratio ("main sequence") in pediatric patients with FRDA, CAs and healthy controls, providing correlations with standard clinical scores. RESULTS: Pattern of saccadic features differed between CA and FRDA. The main sequence analysis was impaired respectively in vertical saccades in CA, and in horizontal saccades in FRDA. In CA, the amplitude of vertical saccades was reduced, and the size inversely correlated with the Scale for the assessment and rating of ataxia (SARA) score. In FRDA the amplitude of horizontal saccades directly correlated with SARA score. COMPARISON WITH EXISTING METHOD: EyeSeeCam allowed testing saccades easily and quickly even in pediatric patients with EOA. CONCLUSIONS: The pattern of saccadic impairment differed between FRDA and CAs, resulting a prominent involvement of vertical saccades in CA and of horizontal ones in FRDA, which respectively correlated with SARA score. Since such differences may reflect distinct pathophysiological substrates, saccades emerged as a potential source of biomarkers in EOAs. Availability of handy tools, such as EyeSeeCam, may facilitate future research in this field.

One-year outcome of coenzyme Q10 supplementation in ADCK3 ataxia (ARCA2).

BACKGROUND: The recessive ataxia ARCA2 is a rare disorder characterized by Coenzyme Q10 (CoQ10) deficiency due to biallelic mutations in ADCK3 gene. Despite the pathophysiological role, available data are not univocal on clinical efficacy of CoQ10 supplementation in ARCA2. Here we described the long-term motor outcome of 4 untreated ARCA2 patients prospectively followed-up for one year after starting CoQ10 oral supplementation (15 mg/kg/day). METHODS: Clinical rating scales (SARA; 9 holes peg test; 6 min walking test; Timed 25-Foot Walk) and videoelectronic gait analysis were performed at baseline and every 6 months (T0, T1, T2) to evaluate the motor performances. Since two patients discontinued the treatment at the 7th month, we could provide comparative analysis between longer and shorter supplementation. RESULTS: At T2, the gait speed (Timed 25-Foot Walk test) significantly differed between patients with long and short treatment; overall, the clinical condition tended to be better in patients continuing CoQ10. CONCLUSIONS: Although preliminarily, this observation suggests that only prolonged and continuous CoQ10 supplementation may induce mild clinical effects on general motor features of ARCA2. Dedicated trials are now necessary to extend and validate such observation.

Evaluation of gait in Duchenne Muscular Dystrophy: Relation of 3D gait analysis to clinical assessment.

Walking ability in Duchenne Muscular Dystrophy (DMD) deteriorates progressively until complete loss of the function. Interventions aimed at maintaining ambulatory ability relies on accurate clinical-based scores and evaluations of walking. This kind of assessment has intrinsic limitations. A 3D optoelectronic system could provide elements useful for the functional evaluation of patients with DMD. Nineteen boys with DMD were evaluated using the 6-Minutes Walking Test, North Star Ambulatory Assessment and 3D gait analysis. Participants' gait parameters were compared to those of an age-matched control group and correlated with standard clinical scores. Seventeen kinematic variables differed between DMD and control groups. Strong correlations with North Star Ambulatory Assessment were found for stride width, gait velocity and ankle angles on the sagittal plane. The 6-Minutes Walking test did not correlate with investigated kinematic variables but showed a correlation with North Star Ambulatory Assessment. Our data support the reported DMD gait pattern characterized by increased anterior pelvic tilt and ankle plantar flexion. The stride width and ankle kinematics emerged as the main representative gait parameters of DMD global ambulatory status. Although preliminary, our findings suggest that 3D gait analysis may provide useful objective and accurate parameters reflecting the functional ability of individuals with DMD.

Non-invasive Focal Mechanical Vibrations Delivered by Wearable Devices: An Open-Label Pilot Study in Childhood Ataxia.

Non-invasive focal mechanical vibrations (NIFMV) now represent a strategy of increasing interest to improve motor control in different neurological diseases. Nanotechnology allowed the creation of wearable devices transforming thermal variations into mechanical energy with focal vibrations. This kind of wearable stimulators (WS) has produced encouraging preliminary results when used in the treatment of movement disorders and ataxia in adults. In this open label pilot study we first evaluated the feasibility, safety and effectiveness of NIFMV by WS in a cohort of 10 patients with childhood ataxia, a phenomenological category including different conditions still lacking of effective symptomatic therapies. Through the assessment of both clinical rating scales and spatio-temporal gait parameters via standardized gait analysis, we observed that a 4 weeks long treatment with WS Equistasi® was safe and provided significantly different effects in stride features of patients with slow/non-progressive cerebellar ataxia and Friedreich's Ataxia. Although limited by the sample size, the absence of a placebo-controlled group, the poor compliance of enrolled population to the original experimental design and the partial accuracy of outcome measures in pediatric subjects, we suggest that NIFMV by WS could support locomotion of patients with childhood slow/non-progressive cerebellar ataxia with preserved sensory system and no signs of peripheral neuropathy. Future studies are definitely necessary to confirm these preliminary results and define criteria for successful NIFMV-based treatment.

Longitudinal gait assessment in a stiff person syndrome.

Stiff person syndrome (SPS) is an autoimmune disorder with multiple clinical presentations, all characterized by generalized or focal muscular stiffness leading to abnormal postures and movements. To date, no standardized treatments are available; also, the outcome measures are mainly clinical based and unstandardized, limiting the reliability of clinical trials. In this case study, we used the eight-camera motion capture system for gait analysis (GA) to outline the gait features and track the clinical evolution of a young patient with SPS receiving a personalized multimodal therapy. GA was accurate in reflecting clinical changes over a 7-week-long period, thus representing a potential source for objective biomarkers in SPS. Therefore, future studies focusing on either the natural history or the treatment of SPS could adopt GA for reliable outocome measures, confirming this preliminary observation.