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Frequency of diencephalic syndrome in NMOSD

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Abstract

Background

Diencephalic region of the brain harbors sites with a considerable amount of aquaporin-4 expression. Neuromyelitis optica spectrum disorder (NMOSD) primarily involves autoimmune processes against this molecule. However, little is known about the frequency of symptoms of diencephalic involvement in NMOSD patients.

Objective

To investigate the frequency of symptoms of diencephalic involvement in NMOSD patients and describe the associated characteristics in patients presenting such symptoms.

Materials and methods

This retrospective cohort included 145 NMOSD patients (39 males and 106 females) who visited Isfahan Multiple Sclerosis Center from January 2013 to February 2020 for approximately 61 months. Demographic and clinical information of patients and findings from radiological and serological investigations were retrieved.

Results

The frequency of diencephalic involvement in NMOSD patients was 3.4% (five cases). Diencephalic syndrome-associated symptoms observed in this cohort consisted of narcolepsy (n = 2; 40%), hypotension (n = 1; 20%), amenorrhea (n = 1; 20%), and syndrome of inappropriate antidiuretic hormone secretion (n = 1; 20%). These manifestations responded well to NMOSD-associated treatments, i.e., rituximab and azathioprine.

Conclusion

Although rarely manifested through symptoms suggestive of diencephalic involvement, NMOSD should be considered when encountering patients with the diencephalic syndrome to identify the primary cause of these manifestations.

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Availability of data and material

All data generated or analysed during this study are included in this published article.

Code availability

Not applicable.

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Funding

No funds, grants, or other support was received.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran

    Masoud Etemadifar

  2. Alzahra Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran

    Hosein Nouri, Reza Khorvash & Ali Aghababaee

  3. Department of Neurological Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran

    Mehri Salari

  4. Department of Biological Sciences and Technologies, Isfahan University, Isfahan, Iran

    Kimia Ghafari

Authors
  1. Masoud Etemadifar
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  2. Hosein Nouri
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  3. Reza Khorvash
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  4. Mehri Salari
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  5. Kimia Ghafari
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  6. Ali Aghababaee
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Contributions

Conceptualization: ME; Formal analysis and investigation: RK, KG, and AA; Writing—original draft preparation: RK; Writing—review and editing: ME, HN; Supervision: ME and MS.

Corresponding author

Correspondence to Reza Khorvash.

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Conflict of interest

The authors have no conflicts of interest to declare that are relevant to the content of this article.

Ethics approval

This study was approved by the ethical committee of Shahid Beheshti University of Medical Science.

Consent to participate

Written informed consent letters to participate were obtained from all participating individuals.

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Not applicable, since no data from the patients that could possibly endanger their anonymity were used in this article.

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Etemadifar, M., Nouri, H., Khorvash, R. et al. Frequency of diencephalic syndrome in NMOSD. Acta Neurol Belg 122, 961–967 (2022). https://doi.org/10.1007/s13760-021-01792-1

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  • DOI: https://doi.org/10.1007/s13760-021-01792-1

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