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

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. 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. 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. 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.

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Evaluating the Effectiveness of Accommodations Given to Students With Learning Impairments: Ordinal and Interpretable Machine-Learning-Based Methodology

In most academic institutions, students with learning impairments (LIs) are entitled to various accommodations as a means of compensating for their impairment. Ensuring that the appropriate accommodations were selected requires an intelligent support tool to track their effectiveness. In this article, we regard the effectiveness of such accommodations in terms of their quality and reliability. High-quality accommodations allow students with LIs equal access and equal opportunity to demonstrate their knowledge compared to their peers who do not have LIs. Highly reliable accommodations mean a significant performance difference between students with LIs who actually use the accommodations compared with students with LIs who do not use the accommodations, given similar student characteristics. Previous literature is inconclusive regarding the evidence of the effectiveness of such accommodations since different accommodations may have a different effect on different subgroups of exams and students. This article proposes a methodology, based on ordinal interpretable models, that produce practical insights to professionals who are responsible for students with LIs, to address the problem of exploring the effectiveness of learning accommodations. The suggested models use the ordinal information of the target variables for evaluating student performance and yield practical insights for designing the most suitable accommodation for a student with LIs based on their characteristics. The ordinal interpretable models are evaluated using a database of tens of thousands of engineering students. The results demonstrate that the suggested interpretable models perform significantly better than the compared algorithms on all measures.

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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.

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Parent and therapist perspectives on "uptime" activities and participation in Rett syndrome

Purpose People with a disability may spend more time sitting and lying (“downtime”) and less time standing and walking (“uptime”). Caregivers and therapists supporting individuals with Rett syndrome were surveyed, aiming to gather insights on how to support participation in “uptime” activities. Method An anonymous online survey including open ended questions about the enablers and barriers to “uptime” was administered to parent/caregivers and therapists/health professionals in an international sample. Responses were coded to the International Classification of Functioning, Health and Disability (ICF) framework identifying barriers, enablers, and strategies for increasing uptime activities. Results Parents (N = 115) and therapists (N = 49) completed the survey. Barriers and enablers to “uptime” were identified for all ICF domains and additional data coded to enabling access to the physical environment. Strategies to promote “uptime” activities and participation particularly related to the individual’s physical capacity and personal factors as well as social and physical environmental factors. Conclusions Findings can inform the design of interventions aiming to increase “uptime” in individuals with Rett syndrome. Strategies should create individualised support by considering how to build fitness using activities that are motivating, at the same time creating opportunities for social interactions within a range of environments. IMPLICATIONS FOR REHABILITATION “Uptime” participation comprised a dynamic interaction of “doing” the standing or walking activity, with a sense of self-engagement with the activities and interaction with others. Strategies to promote “uptime” participation should consider how to create support for person-related attributes, including building physical capacity for a greater volume of “uptime” in activities that are enjoyable and motivating. Strategies to promote “uptime participation should also include creating a supportive environment, comprising opportunities for social interaction within a range of environments”.

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