Updated systematic-narrative review on communication intervention in Rett Syndrome: 2010-2022.

Due to loss of spoken language and resulting complex communication needs, people with Rett syndrome are obvious candidates for communication intervention. To advance evidence-based practice and guide future research efforts, we identified and summarized 16 communication intervention studies published since a previous 2009 review on this topic. Studies were summarized in terms of (a) participants, (b) dependent variables related to communication, (c) intervention characteristics, (d) outcomes, and (e) certainty of evidence. Across the 16 studies, intervention was provided to a total of 100 participants from 3 to 47 years of age. Half of the studies used systematic instruction to teach aided AAC. Other interventions and associated technologies included music therapy, eye tracking technology, and transcranial stimulation. Positive outcomes (e.g., using AAC devices to make requests and/or initiate social-communication interactions) were reported in 13 of the studies. These 16 new studies provide additional guidance on how to enhance the communicative functioning of people with Rett syndrome. Future research directions are highlighted.

A Behavioral Approach to Treatment and Assessment of People with Disorders of Consciousness: The Response-contingent Stimulation Strategy.

Response-contingent stimulation is a behavioral strategy used to improve the situation of patients with disorders of consciousness. Such strategy involves the presentation of brief periods (e.g., 10 to 15 s) of stimulation considered preferred by the patients, contingent on (immediately after) the emission of specific patients' responses. The aim is to help the patients learn the link between their responding and the preferred stimulation and thus learn to use their responding to access the stimulation in a self-determined/independent manner. Achieving these goals is considered important for the patients' recovery process and thus the response-contingent stimulation strategy that promotes such an achievement can be considered a valuable treatment approach. The same strategy combined with the use of periods of non-contingent stimulation (i.e., stimulation delivered independent of responding) may also serve as an assessment supplement with patients with apparent unresponsive wakefulness. The patients' increase in responding during the response-contingent stimulation and decline in responding during the non-contingent stimulation could be taken as a sign of discrimination between conditions, and possibly a sign of awareness of the immediate environmental situation, compatible with a diagnosis of minimally conscious state. This paper analyzes a number of studies aimed at using the response-contingent stimulation as a treatment strategy and a number of studies aimed at combining response-contingent stimulation with non-contingent stimulation for treatment and assessment purposes. The results of the studies are discussed in terms of the effectiveness, accessibility and affordability of the strategy. The need for new research (i.e., replication studies) is also pointed out.

Mainstream technology to support basic communication and leisure in people with neurological disorders, motor impairment and lack of speech.

OBJECTIVE: To assess a simple technology solution to support basic communication and leisure in people with neurological disorders, extensive motor impairment, and absence of speech. DESIGN: The design was a non-concurrent multiple baseline across participants. METHODS: The study included eight participants and assessed a technology setup including a Samsung Galaxy Tab S2 LTE tablet and a Samsung Galaxy A3 smartphone. The smartphone, automated via MacroDroid, presented the participant with leisure, messages, and caregiver options. Choosing leisure or messages (by activating the smartphone's proximity sensor) led the smartphone to present the alternatives available for that option and eventually verbalize the alternative selected. This verbalization triggered the tablet's Google Assistant and led the tablet to present a leisure event or start a message exchange. Choosing the caregiver led the smartphone to invite the caregiver to interact with the participant. RESULTS: During baseline (i.e., when a standard smartphone was available), the participants did not activate any of the options. During intervention and post-intervention (i.e., with the technology described above), participants activated all options and spent most of the session time positively engaged with them. CONCLUSIONS: The aforementioned technology seems to be a useful tool for individuals like those involved in this study.

Promoting physical activity in people with intellectual and multiple disabilities through a basic technology-aided program.

This study assessed a technology-aided program (monitoring responding, and ensuring preferred stimulation and encouragements) for promoting physical activity with 11 participants with severe/profound intellectual and multiple disabilities. Each participant was provided with an exercise device (e.g. a static bicycle and a stepper) and exposed to the program according to an ABAB design, in which A and B represented baseline and intervention phases, respectively. Data recording concerned (a) the participants' responses with the exercise device (e.g. pedaling) during baseline and intervention phases and (b) their heart rates during the last intervention phase. The results showed that all participants had significant increases in responding with the exercise devices during the intervention phases. Heart-rate values during the intervention sessions indicated that the participants' responding during those sessions mostly amounted to moderate-intensity physical activity, with potential benefits for their overall physical condition. Implications of the findings and questions for future research in the area were discussed.

A Novel Way to Measure and Predict Development: A Heuristic Approach to Facilitate the Early Detection of Neurodevelopmental Disorders.

PURPOSE OF REVIEW: Substantial research exists focusing on the various aspects and domains of early human development. However, there is a clear blind spot in early postnatal development when dealing with neurodevelopmental disorders, especially those that manifest themselves clinically only in late infancy or even in childhood. RECENT FINDINGS: This early developmental period may represent an important timeframe to study these disorders but has historically received far less research attention. We believe that only a comprehensive interdisciplinary approach will enable us to detect and delineate specific parameters for specific neurodevelopmental disorders at a very early age to improve early detection/diagnosis, enable prospective studies and eventually facilitate randomised trials of early intervention. In this article, we propose a dynamic framework for characterising neurofunctional biomarkers associated with specific disorders in the development of infants and children. We have named this automated detection 'Fingerprint Model', suggesting one possible approach to accurately and early identify neurodevelopmental disorders.

Same or different: Common pathways of behavioral biomarkers in infants and children with neurodevelopmental disorders?

The extent to which early motor patterns represent antecedents to later communicative functions, and the emergence of gesture and/or sign as potential communicative acts in neurodevelopmental disorders (NDDs), are research questions that have received recent attention. It is important to keep in mind that different NDDs have different neurological underpinnings, with correspondingly different implications for their conceptualization, detection, and treatment.

Parents' initial concerns about the development of their children later diagnosed with fragile X syndrome.

BACKGROUND: Retrospective parental reports have often been used to identify the early characteristics of children later diagnosed with a developmental disorder. METHOD: We applied this methodology to document 13 parents' initial concerns about the development of their 17 children later diagnosed with fragile X syndrome (FXS). Parents were additionally asked about when they noticed the emergence of behavioural signs related to FXS. RESULTS: More than half of the parents reported initial concerns prior to the child's first birthday and in most cases it was deviant motor behaviours that caused the first concerns. Behavioural signs related to the FXS phenotype were also reported to be perceptible in the first year of the child's life. CONCLUSIONS: Due to limitations of retrospective parental questionnaires, we suggest that other methodologies, such as home video analysis, are needed to complement our understanding of the pathways of developmental disorders with late clinical onsets.

Research note: attitudes of teachers and undergraduate students regarding three augmentative and alternative communication modalities.

The social validity of different communication modalities is a potentially important variable to consider when designing augmentative and alternative communication (AAC) interventions. To assess the social validity of three AAC modes (i.e., manual signing, picture exchange, and an iPad®-based speech-generating device), we asked 59 undergraduate students (pre-service teachers) and 43 teachers to watch a video explaining each mode. They were then asked to nominate the mode they perceived to be easiest to learn as well as the most intelligible, effective, and preferred. Participants were also asked to list the main reasons for their nominations and report on their experience with each modality. Most participants (68-86%) nominated the iPad-based speech-generating device (SGD) as easiest to learn, as well as the most intelligible, effective, and preferred. This device was perceived to be easy to understand and use and to have familiar and socially acceptable technology. Results suggest that iPad-based SGDs were perceived as more socially valid among this sample of teachers and undergraduate students. Information of this type may have some relevance to designing AAC supports for people who use AAC and their current and future potential communication partners.

Microswitch Technology for Enabling Self-Determined Responding in Children with Profound and Multiple Disabilities: A Systematic Review.

We reviewed 18 studies reporting on the use of microswitch technology to enable self-determined responding in children with profound and multiple disabilities. Identified studies that met pre-determined inclusion criteria were summarized in terms of (a) participants, (b) experimental design, (c) microswitches and procedures used, and (d) main results. The 18 studies formed three groups based on whether the microswitch technology was primarily intended to enable the child to (a) access preferred stimuli (7 studies), (b) choose between stimuli (6 studies), or (c) recruit attention/initiate social interaction (5 studies). The results of these studies were consistently positive and support the use of microswitch technology in educational programs for children with profound and multiple disabilities as a means to impact their environment and interact with others. Implications for delivery of augmentative and alternative communication intervention to children with profound and multiple disabilities are discussed.

EXTENDING THE ASSESSMENT OF TECHNOLOGY-AIDED PROGRAMS TO SUPPORT LEISURE AND COMMUNICATION IN PEOPLE WITH ACQUIRED BRAIN INJURY AND EXTENSIVE MULTIPLE DISABILITIES.

Intervention programs for people with acquired brain injury and extensive motor and communication impairment need to be diversified according to their characteristics and environment. These two studies assessed two technology-aided programs for supporting leisure (i.e., access to songs and videos) and communication (i.e., expressing needs and feelings and making requests) in six of those people. The three people participating in Study 1 did not possess speech but were able to understand spoken and written sentences. Their program presented leisure and communication options through written phrases appearing on the computer screen. The three people participating in Study 2 did not possess any speech and were unable to understand spoken or written language. Their program presented leisure and communication options through pictorial images. All participants relied on a simple microswitch response to enter the options and activate songs, videos, and communication messages. The data showed that the participants of both studies learned to use the program available to them and to engage in leisure and communication independently. The importance of using programs adapted to the participants and their environment was discussed.

En route to disentangle the impact and neurobiological substrates of early vocalizations: learning from Rett syndrome.

Research on acoustic communication and its underlying neurobiological substrates has led to new insights about the functioning of central pattern generators (CPGs). CPG-related atypicalities may point to brainstem irregularities rather than cortical malfunctions for early vocalizations/babbling. The "vocal pattern generator," together with other CPGs, seems to have great potential in disentangling neurodevelopmental disorders and potentially predict neurological development.

Tangible Symbols as an AAC Option for Individuals with Developmental Disabilities: A Systematic Review of Intervention Studies.

We reviewed nine studies evaluating the use of tangible symbols in AAC interventions for 129 individuals with developmental disabilities. Studies were summarized in terms of participants, tangible symbols used, communication functions/skills targeted for intervention, intervention procedures, evaluation designs, and main findings. Tangible symbols mainly consisted of three-dimensional whole objects or partial objects. Symbols were taught as requests for preferred objects/activities in five studies with additional communication functions (e.g., naming, choice making, protesting) also taught in three studies. One study focused on naming activities. With intervention, 54% (n = 70) of the participants, who ranged from 3 to 20 years of age, learned to use tangible symbols to communicate. However, these findings must be interpreted with caution due to pre-experimental or quasi-experimental designs in five of the nine studies. Overall, tangible symbols appear promising, but additional studies are needed to establish their relative merits as a communication mode for people with developmental disabilities.

Two men with advanced amyotrophic lateral sclerosis operate a computer-aided television system through mouth or throat microswitches.

This study assessed a simple technology to enable two men affected by amyotrophic lateral sclerosis, in an advanced stage (i.e., with pervasive motor disabilities and lack of speech), to operate a computer-aided television system. The technology included microswitches, a portable computer, an interface connecting the microswitches to the computer, a commercial software package to allow the possibility of watching television via computer, and specific software to allow microswitch activations to be recorded as forward commands for channel change. The participants (a) gained control over the television programs to watch or not to watch (i.e., through channel changes) and (b) showed increased attention to the programs (i.e., an increase in their watching time). The practical relevance of the findings, the integration of the technology used in this study within the participants' wider recreation and communication program, and ways of extending the research were discussed.

Case studies of technology for adults with multiple disabilities to make telephone calls independently.

Recent literature has shown the possibility of enabling individuals with multiple disabilities to make telephone calls independently via computer-aided telephone technology. These two case studies assessed a modified version of such technology and a commercial alternative to it for a woman and a man with multiple disabilities, respectively. The modified version used in Study 1 (a) presented the names of the persons available for a call and (b) reminded the participant of the response she needed to perform (i.e., pressing a microswitch) if she wanted to call any of those names/persons. The commercial device used in Study 2 was a Galaxy S3 (Samsung) equipped with the S-voice module, which allowed the participant to activate phone calls by uttering the word "Call" followed by the name of the persons he wanted to call. The results of the studies showed that the participants learned to make phone calls independently using the technology/device available. Implications of the results are discussed.

Enabling People With Intellectual and Sensory Disabilities to Trigger a Tablet's Delivery of Task Instructions by Walking to the Tablet: Proof-of-Concept Study.

BACKGROUND: People with intellectual and sensory or sensory-motor disabilities tend to have problems performing multistep tasks. To alleviate their problems, technological solutions have been developed that provide task-step instructions. Instructions are generally delivered at people's request (eg, as they touch an area of a computer or tablet screen) or automatically, at preset intervals. OBJECTIVE: This study carried out a preliminary assessment of a new tablet-based technology system that presented task-step instructions when participants with intellectual and sensory disabilities walked close to the tablet (ie, did not require participants to perform fine motor responses on the tablet screen). METHODS: The system entailed a tablet and a wireless camera and was programmed to present instructions when participants approached the tablet, that is, when the camera positioned in front of the tablet detected them. Two instructions were available for each task step. One instruction concerned the object(s) that the participants were to collect, and the other instruction concerned the "where" and "how" the object(s) collected would need to be used. For 3 of the six participants, the two instructions were presented in succession, with the second instruction presented once the required object(s) had been collected. For the other 3 participants, the two instructions were presented simultaneously. Instructions consisted of pictorial representations combined with brief verbal phrases. The impact of the system was assessed for each of the 2 groups of participants using a nonconcurrent multiple baseline design across individuals. RESULTS: All participants were successful in using the system. Their mean frequency of correct task steps was close to or above 11.5 for tasks including 12 steps. Their level of correct performance tended to be much lower during the baseline phase when they were to receive the task-step instructions from a regular tablet through scrolling responses. CONCLUSIONS: The findings, which need to be interpreted with caution given the preliminary nature of the study, suggest that the new tablet-based technology system might be useful for helping people with intellectual and sensory disabilities perform multistep tasks.

Teaching multi-step requesting and social communication to two children with autism spectrum disorders with three AAC options.

The present study involved comparing the acquisition of multi-step requesting and social communication across three AAC options: manual signing (MS), picture exchange (PE), and speech-generating devices (SGDs). Preference for each option was also assessed. The participants were two children with autism spectrum disorders (ASD) who had previously been taught to use each option to request preferred items. Intervention was implemented in an alternating-treatments design. During baseline, participants demonstrated low levels of correct communicative responding. With intervention, both participants learned the target responses (two- and three-step requesting responses, greetings, answering questions, and social etiquette responses) to varying levels of proficiency with each communication option. One participant demonstrated a preference for using the SGD and the other preferred PE. The importance of examining preferences for using one AAC option over others is discussed.

Barriers and facilitators for obtaining support services among underserved families with an autistic child: A systematic qualitative review.

LAY ABSTRACT: Families from underrepresented ethnic or racial groups and those with limited financial resources could experience more difficulty in accessing support services for their autistic child due to certain types of barriers. We searched academic journals, websites, and other sources for studies which looked at what barriers might be present for such families and what might help families access support services for their autistic child. The search found 18 studies. Results from each study were examined and coded into themes. Parents reported that accessibility, diversity of support services, and stigma influenced their experiences with support services. We discuss what these findings might mean for future research and for service delivery.

Parent reported barriers and facilitators to support services for autistic children in Aotearoa New Zealand.

LAY ABSTRACT: Parents might have problems in getting support services for their autistic child due to certain barriers. However, there might also be things that can ease or facilitate parents' access to support services. In this study, New Zealand parents were asked about their experiences in getting support services for their autistic child. We also looked at differences in reported barriers and facilitators based on several demographic factors with a focus on family level of financial resourcing. A total of 173 parents completed a survey. The results suggested that parents experienced several barriers, particularly related to service pathways. Facilitators were also experienced, predominantly related to providers. Financial resourcing predicted the number of parent-reported barriers. Both lower level of family financial resourcing and having a non-binary child predicted parents' rating of the extent of barriers. Child age and level of speech were predictors for reports of experiencing a higher number of facilitators, with parents of younger children or of non-speaking autistic children reporting a greater number of facilitators. We discuss how these results may be useful to support service delivery and identify areas for future research.

Use of technology to sustain mobility in older people with cognitive impairment and dementia: a scoping review.

PURPOSE: This paper provides a review of studies that evaluated technology devices to sustain various forms of mobility in older people with cognitive impairment or dementia and mobility problems (e.g., inability to ambulate or difficulties to orient with consequent failures to reach relevant destinations). METHODS: A literature search was performed for English language articles indexed in PubMed, PsycINFO, and Web of Science. This search was then supplemented with an ancestral and forward search. The search covered the period ranging between January 2010 and October 2020. RESULTS: Twenty-seven articles were included in the review. They were divided into four groups according to whether they aimed to promote (a) supported ambulation, (b) use of safe power wheelchair, (c) ambulation guided by orientation cues, and (d) ambulation with GPS-based assistance. The studies were coded in terms of the objective pursued, technology used, participants involved, strategies applied to assess the technology, and outcome reported. CONCLUSION: A variety of technology devices are available to address the mobility problems of older people with cognitive impairment or dementia. The devices' accessibility and usability differ widely. Data on their impact are still largely preliminary and new systematic research is needed.IMPLICATIONS FOR REHABILITATIONA variety of mobility problems may afflict older people with cognitive impairment or dementia and curtail their opportunities to freely move indoor or outdoor.Technology devices have been developed to (a) promote forms of supported ambulation, (b) facilitate safe use of power wheelchairs, (c) deliver orientation/navigation cues, and (d) ensure tracking and possible assistance.The technology devices differ in terms of accessibility and affordability, with some of them readily available and others at a developmental stage.Data on the effectiveness of the various technology devices in reducing the impact of the mobility problems are still largely preliminary and new systematic research is needed.

Learning about neurodiversity from parents - Auditory gestalt perception of prelinguistic vocalisations.

BACKGROUND: Infants with Rett syndrome (RTT) may have subtle anomalies in their prelinguistic vocalisations but the detection of these is difficult, since their conspicuous vocalisations are often interspersed with inconspicuous ones. AIMS AND METHODS: Extending a previous study with predominantly non-parents, the present study sampled parents of children with RTT and aimed to examine their gestalt perception of prelinguistic vocalisations. METHODS AND PROCEDURE: Parents (n = 76) of female children with RTT listened to vocalisation recordings from RTT and typically developing (TD) infants, including an inconspicuous vocalisation from a RTT girl. For each recording, parents indicated if the vocalisation was produced by a RTT or a TD child. RESULTS: Overall correct to incorrect identification rate was 2:1, which was comparable to that of the previous study. Intriguingly, parents of RTT children seemed to be sensitive to features characterising the vocalisations of RTT infants, which has especially influenced their perception of the inconspicuous vocalisation from a RTT girl. CONCLUSIONS AND IMPLICATIONS: These results invite further research on the potential characterising differences between vocalisations from TD infants and infants with divergent neurodevelopment.