Rehabilitation interventions in Rett syndrome: a scoping review

Abstract

Aim To summarize existing interventions and their outcomes in Rett syndrome (RTT) rehabilitation and identify gaps in the literature. Method Five databases (Ovid MEDLINE, Ovid Embase Classic, Ovid PsycINFO, EBSCO CINAHL Plus, and ProQuest ERIC) were systematically searched up to 23rd July 2018 for studies describing rehabilitation interventions. Data on study participants, design, and outcomes were extracted. Results Sixty‐two articles were included in the final review. Evidence consistently demonstrated that females with RTT can improve their gross motor, fine motor, and communicative skills with rehabilitation. All 11 interventions targeting gross motor function, namely ambulation, achieved functional improvements. Twenty of 24 articles describing fine motor rehabilitation studies succeeded in decreasing stereotypies, improving functional hand use, and/or reducing self‐injurious behaviors. Twenty‐one of 22 studies describing communication interventions succeeded in training choice‐making, communicative language, or socialization behavior. Other key findings include the positive interplay between physical and communicative rehabilitation outcomes, and the ability of females with RTT to improve their cognitive abilities during intervention. Interpretation Rehabilitation can impact the daily lives of females with RTT and their caregivers in clinically meaningful ways. What this paper adds Females with Rett syndrome (RTT) can make developmental gains across their life span. Long‐term intervention can improve the physical abilities of individuals with RTT. Low‐, medium‐, and high‐technology interventions can improve the communicative abilities of individuals with RTT.

Implementing telehealth support to increase physical activity in girls and women with Rett syndrome—ActivRett: protocol for a waitlist randomised controlled trial

“…► Activities will be determined by goals that are set, modified and developed in collaboration with caregivers, 42 and based on contemporary models for participation 37 and quality of life. 20 38 ► Recruitment will be non-probabilistic, and the sample may not be representative of the population of RTT with some walking skills. ► We acknowledge that our findings will not inform how to increase activity in individuals who are unable to walk.…”

“…17 Recent systematic reviews of physical therapy interventions for individuals with RTT have reported mostly positive effects on gross motor skills, motor endurance and fitness within therapy settings. [18][19][20] Opportunities for training have been observed as enjoyable for the child when accompanied with a microswitch that delivered a light and sound sensory response to walking. 21 We note that the majority of studies were case studies, case series and single-case design studies, and only one study used randomisation in a stepped wedge design.…”

Comparison of the presence and absence of an intervention to reduce hand stereotypies in individuals with Rett syndrome

“…In this study, 72.1% of participants had received an intervention at some point in their lives. Effective interventions for decreasing stereotypies have been reported (Amoako & Hare, 2020; Lim et al, 2020; Yang et al, 2020). In the future, it is hoped that an appropriate intervention will be administered to all individuals with Rett syndrome for problems such as stereotypic hand movements and joint contractures at the right time because the symptoms, associated problems and parental stress change over time.…”

“…In this study, 72.1% of participants had received an intervention at some point in their lives. Effective interventions for decreasing stereotypies have been reported (Amoako & Hare, 2020;Lim et al, 2020;Yang et al, 2020). In the future, it is hoped that an…”

“…Interventions, including various communication, behavioural, ambulation and independence therapies and orthoses to improve fine motor skills help to decrease stereotypies, improve functional hand use, and/or reduce self‐injurious behaviour (Amoako & Hare, 2020; Lim et al, 2020; Yang et al, 2020). Various upper extremity splints have been used as effective interventions, with 18 out of 21 participants across seven splinting studies experiencing a decrease in their stereotypic hand movements (Aron, 1990; Bumin et al, 2002; Kubas, 1992; Naganuma & Billingsley, 1988; Sharpe, 1992; Sharpe & Ottenbacher, 1990; Tuten & Miedaner, 1989).…”