Assistive Communication Devices in Rett Syndrome: A Case Report and Narrative Review

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

Assistive communication devices are increasingly used in Rett syndrome (RTT).  Several recent reviews have covered the topic as well as other related communication issues in RTT.  Therefore, it is expected that the current review will provide a new perspective.  Adding a “case report” is didactic but not sufficient for differentiating this manuscript.  The main shortcoming in the field of interventions in neurodevelopmental disorders, such as RTT, continues to be limited evidence of efficacy or effectiveness.  This affects particularly non-pharmacological interventions.  The present review lacks critical appraisals of assistive communication devices.  Before advocating for access to them, it is important to know to what extent are they effective and in which way effectiveness could be increased.

 

In addition to the aforementioned shortcomings, the manuscript includes outdated references in particular those referring to the medical aspects of RTT.  It even uses an inaccurate and controversial label to define RTT.  The disorder is developmental and progressive but not degenerative; the evidence supporting this notion is quite extensive.  Also, the staging scheme proposed by Hagberg several decades ago is no longer accepted by most clinical investigators and, from the management perspective, it has been replaced by more conventional age periods (Fu et al. 2020).  Finally, the manuscript will benefit from careful proofreading and appropriate review of the list of references (e.g. several without volumes or pages).

Comments on the Quality of English Language

Minor editing will be beneficial.

Author Response

Please find attached revision letter. Thank you

Author Response File: Author Response.pdf

Reviewer 2 Report

Comments and Suggestions for Authors

The manuscript by Lee et al. describes assistive devices for communication in the context of Rett Syndrome, and an analysis of different types of technology that are used in this context. The subject of the manuscript is an important one, both for improving care of patients with Rett Syndrome, and also for the larger context of healthcare disparities for individuals with disability. The authors advocate for further study of these technologies, including longer-term studies, which is clearly needed in this arena. The manuscript itself is a little unfocused and would benefit from added detail, added literature citations, and improved focus.

 

- the introduction would benefit from a little more treatment of Rett Syndrome, and particularly about the types of communication deficits these individuals have. As it stands, the introduction really just states that they loss of communication abilities. The authors could, for example, discuss how Rett Syndrome affects communication processes such as expressive vs receptive aphasia, dysarthria, speech apraxia, etc.

- Introduction, line 55: The mention of NJC would benefit from at least one citation of their work/statements.

- It is not clear why assistive technologies in the mobility aids and daily living aids categories are mentioned. Although these are adaptive technologies that can be used in individuals with Rett Syndrome, they are not part of augmentative communication, and thus are tangential at best to the stated goals of the manuscript. Also, it is not correct to class mobility aids, educational tools, and daily living aids as types of AAC technologies as is done in lines 96-97.

- The section on Educational tools (section 5) might be better couched as a discussion of how communication technology could be used to improve education.

- Section 6 contains an analysis of different types of devices. This section would benefit from more explanation of how the effectiveness (as outlined in Table 1) is scored. What constitutes a high score vs medium or low? The authors could consider breaking down the analysis for each technology by each of the criteria shown in Table 1.

- It is not clear to me how the categories of device shown in Table 2 were determined. As shown, there is significant overlap between the technology shown, and a conflation of AAC per se (speech generating device) with the technology used to control the SGD (eye-gaze, switch).

- The authors cite limited generalizability of some types of technology. However, it is not really clear what is meant by this. The manuscript would benefit from a better treatment of what the authors mean by generalizability, and more detail about how technologies (e.g. speech generating devices) display low generalizability. Examples would help make the case more clearly.

- Section 6 does not cite any literature except what is in Table 1 and 2.

- In the context of the overall manuscript, the case report would benefit from analysis using the criteria outlined in Section 6, applied to her AAC device.

- the authors cite a significant increase in happiness in the patient in the case report (line 263). How was this assessed?

- In section 8, lines 316-319, the idea of using communication technology for better insight into cognitive processes is mentioned. I agree this is a significant advantage of using AAC in individuals with Rett Syndrome. I think the paper would be strengthened with more discussion of this concept.

- the manuscript at several points states that these findings “extend the literature” or “contribute new insights.” The authors may wish to expressly state what some of these new findings are, as it is not very clear from how the manuscript is currently written.

- Ethical approval: while a full IRB review is not needed for a case review, the authors should at least mention whether they had permission from the patient/family to publish the case report section of this paper. 

Author Response

Please find attached revision letter. Thank you

Author Response File: Author Response.pdf

Round 2

Reviewer 2 Report

Comments and Suggestions for Authors

The authors have revised their submission, and significantly improved the focus and quality of the manuscript. They have addressed my concerns. My only optional comment regards line 267 in the revised manuscript, where they have removed the mention of increasing the patient’s overall happiness. It is fine to leave this out, but if the authors wish to retain this point, they need only mention that this was assessed subjectively by the patient’s caregivers.