Keywords
Autoimmune
Anti-aquaporin-4
Child
Niger
How to Cite
Abstract
Introduction :
Pediatric autoimmune rhombencephalitis represents a major diagnostic and therapeutic challenge, characterized by progressively worsening manifestations that are often severe or require intensive care. MRI findings are of major importance, while a multidisciplinary approach and early treatment are key prognostic factors. The objective of this case report is to highlight the diagnostic and therapeutic challenges encountered in the management of pediatric autoimmune rhombencephalitis.
Case Report :
A 9-year-old boy presented with an emetic cough progressing to seizures and bulbar syndrome (ataxia, swallowing disorders). MRI revealed rhombencephalitis associated with positive anti-aquaporin-4 antibodies. Intensive immunotherapy (corticosteroids, immunoadsorption, rituximab) allowed for clinical improvement and extubation on day 35.
Conclusion :
This case report highlights the necessity of conducting an etiological work-up for an anti-aquaporin-4 autoimmune cause in any atypical pediatric rhombencephalitis. Early and aggressive immunotherapy can be determinant for functional recovery.
References
1. Jubelt B, Mihai C, Li T, Veerapaneni P. Rhombencephalitis / brainstem encephalitis. Curr Neurol Neurosci Rep. 2011;11(6):543–52.
2. Armstrong RW, Fung PC. Brainstem encephalitis (rhombencephalitis) due to Listeria monocytogenes: case report and review. Clin Infect Dis. 1993;16(5):689–702.
3. Miura S, Kurita T, Noda K, Nagasato K, Aizawa H. Symmetrical brainstem encephalitis caused by herpes simplex virus. J Clin Neurosci. 2009;16(4):589–90.
4. Koçer N, Islak C, Siva A, Saip S, Akman C, Kantarci O, et al. CNS involvement in neuroBehçet syndrome: an MR study. AJNR Am J Neuroradiol. 1999;20(6):1015–24.
5. Moragas M, Martínez-Yélamos S, Majós C, Fernández-Viladrich P, Rubio F, Arbizu T. Rhombencephalitis: a series of 97 patients. Medicine (Baltimore). 2011;90(4):256–61.
6. Cellucci T, Van Mater H, Graus F, Muscal E, Gallentine W, Klein-Gitelman MS, et al. Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. Neurol Neuroimmunol Neuroinflamm. 2020;7(2):e663.
7. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391–404.
8. Venkatesan A, Tunkel AR, Bloch KC, Lauring AS, Sejvar J, Bitnun A, et al. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis. 2013;57(8):1114–28.
9. Trindade RA, Faistauer A, Pérez JA, Vedolin LM, Duarte JA. Rhombencephalitis: pictorial essay. Radiol Bras. 2016;49(5):329–36.
10. Abboud H, Probasco JC, Irani S, Ances B, Benavides DR, Bradshaw M, et al. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry. 2021;92(7):757–68.
11. Dale RC, Brilot F, Duffy LV, Twilt M, Waldman AT, Narula S, et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology. 2014;83(2):142–50.
Metrics
Downloads
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2026 M Ali Bandiaré, J Donamou, F Hassane Djibo, S Boureima M, M Niandou, R Sabo