Neuro-Cranio-Vertebral Syndrome Associated with Ehlers–Danlos Syndrome: Diagnosis and Treatment

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

The article by Miguel B. Royo-Salvador and colleagues details the diagnosis and treatment for Neuro-Cranio-vertebral syndrome associated with Ehlers-Danlos syndrome. In this article, the authors suggest a new treatment for filum terminale collagen dysfunction NCVS. The study included complete imaging and clinical characteristics of ten patients with EDS and NCVS. It compared the 373 patients with NCVS in a previous Filum disease (FD) and NCVS survey. The authors further suggest postsurgical findings in four EDS-NCVS patients after sectioning of the filum terminale. Overall, this is a well-written article that suggests spinal fusion surgery as a last resort in cases where sectioning of the filum terminale does not have a satisfactory effect and severe vertebral instability is demonstrated by dynamic imaging tests of the spine. The overall quality of the article can be improved, and the following changes are needed.

 

The article needs overall improvements for the Figures. The authors should make the following changes to the Figures. 

1. The individual figure should be labeled e.g., A, B, C, D, etc. This will further help to understand which figure the authors are referring to.
2. The figure legends would benefit from additional detail for the Figures. More comprehensive explanations of each figure's content, mechanistic approach, and an arrow showing the specific parts they are referring to from the imaging system will benefit the reader's interest. 
3. Figures 11 to 17 need additional changes: The font sizes on the X and Y axes should be increased.
4. Figure 17B should be rechecked for corrections. This doesn't add to 100%.

Author Response

Comment 1 :The individual figure should be labeled e.g., A, B, C, D, etc. This will further help to understand which figure the authors are referring to.

Response 1: Thank you for pointing this out. We agree with this comment and we have therefore labeled the figures as suggested.

Comment 2: The figure legends would benefit from additional detail for the Figures. More comprehensive explanations of each figure's content, mechanistic approach, and an arrow showing the specific parts they are referring to from the imaging system will benefit the reader's interest.

Response 2: Thank you for the suggestion. We have expanded the majority figure legends to further explain the shown content (except charts). Please see the modified figure legends marked in red in the manuscript.

Comment 3: Figures 11 to 17 need additional changes: The font sizes on the X and Y axes should be increased.

Response 3: Thank you for pointing this out. We have doubled font size and hope that readability will know be enhanced. If some of the chart labelling remains too small, we hope the editorial office can increase the size of the figure.

Comment 4: Figure 17B should be rechecked for corrections. This doesn't add to 100%.

 Response 4: Thank you for the observation. This has been doublechecked and amended.

Reviewer 2 Report

Comments and Suggestions for Authors

Dear Editor,

Thank you for your kind invitation to review the article titled "Neuro-Cranio-Vertebral Syndrome Associated with Ehlers-Danlos Syndrome: Diagnosis and Treatment." The authors aim to present both the outcomes of their patients and a discussion of these results in the context of the existing literature.

I find the authors' work to be quite comprehensive and believe it will contribute significantly to the literature. It represents an important addition for both clinicians and patients. In this regard, I congratulate the authors on their efforts.

The article is quite detailed and extensive. Some of my suggestions are outlined below:

• The keywords of the article can be selected from MeSH terms.
• The authors mentioned in the Methods section that they used a Jamar dynamometer for assessment; however, they have not provided any results related to this. If they do not intend to present such results, they may consider removing this statement.
• In Table 1, are the values associated with idiopathic scoliosis classified specifically for these patients? Normally, curves above 45 degrees are classified as severe. I recommend that the authors review this information and use a current reference.
• Table 5 is not referenced within the text; if possible, Tables 1 and 5 could be combined.
• Some information presented in the text is also included in the tables, leading to repetition. Information that is already clearly expressed in the tables may not need to be presented in narrative form.
• The tables could be slightly condensed, though this decision is up to the authors and the editor.
•  

Author Response

Comment 1: The keywords of the article can be selected from MeSH terms.

Response 1: Thank you for the suggestion. Key words have been modified to correspond to MeSH terms (except Filum Terminale) 

Comment 2: The authors mentioned in the Methods section that they used a Jamar dynamometer for assessment; however, they have not provided any results related to this. If they do not intend to present such results, they may consider removing this statement.

Response 2: Thank you very much for pointing this out. We have eliminated this from the Methods section as in fact we have not included the corresponding data in the manuscript.

Comment 3: In Table 1, are the values associated with idiopathic scoliosis classified specifically for these patients? Normally, curves above 45 degrees are classified as severe. I recommend that the authors review this information and use a current reference.

Response 3: Thank you for the observation This was indeed an error, the values we make reference to classify severe scoliosis from 40º. We have corrected this in the manuscript. Table one has been eliminated from the methods section and only the reference to the employed scales has been left in.

Comment 4: Table 5 is not referenced within the text; if possible, Tables 1 and 5 could be combined.

Response 4: Thank you, we have indeed proceeded to indicated the used scale within table 5 (now table 4) and added the mention in the text. (Please see marked in red in the manuscript).

Comment 5:  Some information presented in the text is also included in the tables, leading to repetition. Information that is already clearly expressed in the tables may not need to be presented in narrative form.

Response 5: Thank you for pointing this out. We have edited some of the text to avoid repeating the data presented in the tables. See marked in red in the manuscript.

Comment 6: The tables could be slightly condensed, though this decision is up to the authors and the editor.

Response 6: Thank you for the suggestion, we have slightly condensed the most extensive table, (the new table 1). See marked red in the resubmitted manuscript.