Data Availability
The data that support the findings of this study are available from the corresponding authors upon request. All data are published anonymously, and the identity of the patient described cannot be retrieved from the data provided in the present case report.
References
Zhang SY. Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity. Hum Genet. 2020;139(6):911–8. https://doi.org/10.1007/s00439-020-02127-5.
Consonni F, Ciullini Mannurita S, Gambineri E. Atypical presentations of IPEX: expect the unexpected. Front Pediatr. 2021;9. https://doi.org/10.3389/FPED.2021.643094/BIBTEX
Rosenberg JM, Maccari ME, Barzaghi F, et al. Neutralizing anti-cytokine autoantibodies against interferon-α in immunodysregulation polyendocrinopathy enteropathy X-linked. Front Immunol. 2018;9(MAR). https://doi.org/10.3389/FIMMU.2018.00544
Gervais A, Rovida F, Avanzini MA, et al. Autoantibodies neutralizing type I IFNs underlie West Nile virus encephalitis in ∼40% of patients. J Exp Med. 2023;220(9): e20230661. https://doi.org/10.1084/jem.20230661.
Bastard P, Rosen LB, Zhang Q, et al. Autoantibodies against type I IFNs in patients with life-threatening COVID-19. Science. 2020;370(6515):eabd4585. https://doi.org/10.1126/science.abd4585
Kobayashi I, Shiari R, Yamada M, et al. Novel mutations of FOXP3 in two Japanese patients with immune dysregulation, polyendocrinopathy, enteropathy, X linked syndrome (IPEX) [8]. J Med Genet. 2001;38(12):874–6. https://doi.org/10.1136/jmg.38.12.874.
Lapides DA, McDonald MM. Inflammatory manifestations of systemic diseases in the central nervous system. Curr Treat Options Neurol. 2020;22(9):1–15. https://doi.org/10.1007/S11940-020-00636-2/TABLES/1.
Armangue T, Olivé-Cirera G, Martínez-Hernandez E, et al. Neurologic complications in herpes simplex encephalitis: clinical, immunological and genetic studies. Brain. 2023. https://doi.org/10.1093/BRAIN/AWAD238.
Acknowledgements
We thank the patient and his family. We thank Associazione di Promozione Sociale “Genitori in fuga” (https://www.genitoriinfuga.org/) for their support to our scientific work.
Funding
This paper did not receive any grant from funding agencies in the public, commercial or not-for- profit sectors.
Author information
Authors and Affiliations
Contributions
Data collection: CMB, LR. Conception and design of the study: LR, CMB, AM. Generation, collection, assembly, analysis, and/or interpretation of data: LR, CMB, SB, ET, AM. Drafting paper: LR, CMB, AM. Editing and approval of the final version of the manuscript: LR, CMB, SB, ET, AM.
Corresponding author
Ethics declarations
Ethics Approval
Not applicable.
Consent to Participate
Not applicable.
Consent for Publication
All data are published anonymously and the identity of the patient described cannot be retrieved from the data provided in the present paper. Written informed consent for publication was obtained from the patient’s parents.
Conflicts of Interest
The authors confirm that there are no known conflicts of interest associated with this publication and there has been no significant financial support for this work that could have influenced its outcome.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Rossini, L., Bonardi, C.M., Bresolin, S. et al. Severe Herpes Simplex Encephalitis: an Unusual Presentation of IPEX. J Clin Immunol 44, 100 (2024). https://doi.org/10.1007/s10875-024-01702-z
Received:
Accepted:
Published:
DOI: https://doi.org/10.1007/s10875-024-01702-z