Pediatric Narcolepsy Type 1: A State-of-the-Art Review

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

Thank you for letting me review this well written manuscript. I find  ambitious and well written.

 

I have a few comments

1. There are many abbreviations in this article. Sometimes I do not find if you  use the abbreviation later on.  it is also hard to follow.

 HCTR  is used but also hcrt-1.   I do not understand the difference  between these . QRFP is not explained , ASO is not explained  ROHHAD is explained but not used later on . I do not find ODD later on either .  Please try to diminish the number of abbreviations and check them all.

2. Epidemiology:

There are more authors giving proof on the effect of the Pandemrix vaccination  especially from Finland  and Sweden. Please  add more references here.  I do not agree that the role of Pandemrix is inconclusive. please change this sentence. 

Clinical picture

3.What is the meaning of oneiric ? Is it correct ? 

4.Semeiology is misspelled 

5.Cataplectic  facies may sometimes persist in adults. please  add this  or explain 

 6.I do not understand the connection between RBD and status dissociates. please clarify.

7.Endocrinological  comorbidities    Do you mean   sodium oxide or oxybate ? 

 

 

Author Response

1. There are many abbreviations in this article. Sometimes I do not find if you  use the abbreviation later on.  it is also hard to follow.  HCTR  is used but also hcrt-1.   I do not understand the difference  between these . QRFP is not explained , ASO is not explained  ROHHAD is explained but not used later on . I do not find ODD later on either.  Please try to diminish the number of abbreviations and check them all.

             Reply: We thank the reviewer for the comment. We modified the paper accordingly.

2. Epidemiology: There are more authors giving proof on the effect of the Pandemrix vaccination  especially from Finland  and Sweden. Please  add more references here.  I do not agree that the role of Pandemrix is inconclusive. please change this sentence. 

Reply: Thank for the suggestion, we modified accordingly. Please see page 3: “This association has underscored the importance of rigorous post-marketing surveillance and further research to ensure the safety of vaccines.” We also add references for this part (Gauffin et al. 2024; Hovi et al. 2022).

3. Clinical picture What is the meaning of oneiric ? Is it correct ? 

Reply: Oneiric means “propers of dreams”.  

4. Semeiology is misspelled

Reply: Thanks for the comment, we modified it.

5. Cataplectic  facies may sometimes persist in adults. please  add this  or explain 

             Reply: We thank the reviewer, please see page 4 “As the child grows, this phenotype of             cataplexy evolves into the classical picture, however, cataplectic facies may persist during adulthood”

      6.I do not understand the connection between RBD and status dissociates. please clarify.

         Reply: We modified the text to explain how RBD in NT1 can represent a form of status dissociatus and added a definition. “RBD in pediatric NT1 can also be severe and pervasive, also occurring during daytime SOREMPs and resembling automatic behaviors, possibly representing a form of “status dissociatus” (i.e the co-occurrence of features proper of different behavioral states, that prevents the unambiguous recognition of the state itself)”

      7.Endocrinological  comorbidities    Do you mean   sodium oxide or oxybate ? 

         Reply: We mean Sodium Oxybate, which we modified in the paper.

Author Response File: Author Response.docx

Reviewer 2 Report

Comments and Suggestions for Authors

Overall, the scientific review provides a comprehensive overview of narcolepsy type 1 (NT1) in pediatric populations, covering various aspects including comorbidities, cognitive aspects, diagnosis, and treatment.

 

While the review covers a wide range of topics, the organization could be improved for better coherence. Breaking down the review into subsections with clear headings would enhance readability and help readers navigate through the content more easily.

 

The review contains a vast amount of information, which at times can be overwhelming. Streamlining the text by focusing on key points and avoiding repetition would improve clarity and make the review more concise.

 

While the review presents findings from various studies, there is limited synthesis of the evidence presented. Providing more interpretation of the findings and discussing their implications for clinical practice and future research would enhance the value of the review.

 

 

The review lacks critical analysis of the strengths and limitations of the studies discussed. Providing insights into the methodological rigor of the studies, potential biases, and areas for further investigation would add depth to the review.

 

 

Some sections of the review could benefit from better integration of findings from different studies. For example, discussing how findings from neuroimaging studies align with cognitive deficits observed in children with NT1 would strengthen the coherence of the narrative.

 

 

While the review briefly mentions the need for future research, more emphasis could be placed on identifying specific gaps in the literature and outlining potential directions for future studies.

 

Comments on the Quality of English Language

The quality of English language in the scientific review is generally good. The sentences are well-structured, and the vocabulary is appropriate for a scientific audience. However, there are some areas where language could be improved for clarity and precision. For example, there are instances of long and complex sentences that could be broken down for better readability. Additionally, ensuring consistency in terminology usage and avoiding jargon where possible would enhance clarity for readers who may not be familiar with specialized scientific language.

Author Response

1.Overall, the scientific review provides a comprehensive overview of narcolepsy type 1 (NT1) in pediatric populations, covering various aspects including comorbidities, cognitive aspects, diagnosis, and treatment.

 While the review covers a wide range of topics, the organization could be improved for    better coherence. Breaking down the review into subsections with clear headings would enhance readability and help readers navigate through the content more easily. The review contains a vast amount of information, which at times can be overwhelming. Streamlining the text by focusing on key points and avoiding repetition would improve clarity and make the review more concise.

Reply: We thank the reviewer for the suggestion, we added new subsections as “H1N1 Influenza and Pandemrix”, “Excessive Daytime Sleepiness”, “Cataplexy”, “Sleep paralysis and sleep-related hallucinations”.

 

 

 

 

 

 

2.While the review presents findings from various studies, there is limited synthesis of the evidence presented. Providing more interpretation of the findings and discussing their implications for clinical practice and future research would enhance the value of the review.

The review lacks critical analysis of the strengths and limitations of the studies discussed. Providing insights into the methodological rigor of the studies, potential biases, and areas for further investigation would add depth to the review.

Some sections of the review could benefit from better integration of findings from different studies. For example, discussing how findings from neuroimaging studies align with cognitive deficits observed in children with NT1 would strengthen the coherence of the narrative.

Reply: Thanks for the suggestion. Please see on page 15: “Neuroimaging data on NT1 children consistently align with the cognitive deficits identified in other studies of this population. Imaging studies reveal structural and functional brain abnormalities, particularly in areas associated with memory, attention, and executive function, which corroborate the cognitive impairments observed clinically. These findings provide a robust neurobiological basis for the cognitive challenges experienced by children with NT1, highlighting the importance of addressing these deficits in both diagnostic and therapeutic strategies. This convergence of neuroimaging evidence with clinical observations underscores the critical need for comprehensive cognitive assessments and targeted interventions to support the cognitive development of affected children.”

 

3.While the review briefly mentions the need for future research, more emphasis could be placed on identifying specific gaps in the literature and outlining potential directions for future studies.

Reply: We thank the reviewer for the comment, please see at page 20 in the conclusion:

“Longitudinal studies are essential to comprehend the long-term progression and psychosocial impact of the disease on children and adolescents. Additionally, there is a need for more comprehensive studies on the efficacy and safety of emerging treatment options, including immunotherapy and novel pharmacological agents. Investigating the role of environmental factors and their interplay with genetic susceptibility could also provide insights into prevention strategies. Furthermore, enhancing diagnostic criteria and developing more accessible, non-invasive diagnostic tools will be crucial in facilitating early diagnosis and intervention, ultimately improving the quality of life for pediatric patients.”

 

 

4.Comments on the Quality of English Language

The quality of English language in the scientific review is generally good. The sentences are well-structured, and the vocabulary is appropriate for a scientific audience. However, there are some areas where language could be improved for clarity and precision. For example, there are instances of long and complex sentences that could be broken down for better readability. Additionally, ensuring consistency in terminology usage and avoiding jargon where possible would enhance clarity for readers who may not be familiar with specialized scientific language.

Reply: We thank the reviewer, and we modified the manuscript accordingly.

Author Response File: Author Response.docx