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A Case Report and Literature Review on Suspected Anti N Methyl D Aspartate Receptor Encephalitis in a Child with Type 1 Diabetes at Al Sabah Children Hospital, South Sudan

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DOI: 10.21522/TIJMD.2013.09.04.Art011

Authors : Kenneth Sube, Jenty Parata, Joseph Lako

Abstract:

Anti‑N‑Methyl‑D‑Aspartate (NMDA) receptor encephalitis is a rare form of limbic encephalitis but represents the most common autoimmune encephalitis in children. Its clinical manifestations and disease course differ from adults in terms of tumor association, neurological presentation, and symptom frequency. While associations with autoimmune diseases such as Graves’ disease, autoimmune polyglandular syndrome, and type 1 diabetes have been reported in adults, a link with type 1 diabetes has not previously been described in children. We conducted a case review of a 16‑year‑old adolescent with known type 1 diabetes admitted to Al Sabah Children’s Hospital, Juba, South Sudan. Clinical features, treatment, and outcomes were documented. Literature was reviewed to contextualize findings and highlight diagnostic challenges in resource‑limited settings. The patient presented with altered consciousness, seizures, psychotic symptoms, and movement disorder. Following initiation of high‑dose corticosteroid therapy, the patient achieved complete recovery within four weeks. This case suggests a possible association between type 1 diabetes and anti‑NMDA receptor encephalitis in pediatric patients, an observation not previously documented. Anti‑NMDA receptor encephalitis should be considered in pediatric patients with type 1 diabetes presenting with neuropsychiatric symptoms. This case emphasizes the need for improved diagnostic capacity, awareness, and access to advanced neuroimaging and serological markers in South Sudan and other resource‑limited settings.

References:

[1].   Wu PY, Chi CS, Tsai CR, Yang YL, Lee HF. Long-Term Outcome of Pediatric Patients with Anti-NMDA Receptor Encephalitis in a Single Center. Children (Basel). 2023 Jan 18;10(2):182. doi: 10.3390/children10020182. PMID: 36832312; PMCID: PMC9954979.

[2].   Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, Campen CJ, Moss H, Peter N, Gleichman AJ, Glaser CA, Lynch DR, Rosenfeld MR, Dalmau J. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009 Jul;66(1):11-8. doi: 10.1002/ana.21756. PMID: 19670433; PMCID: PMC2826225.

[3].   Zhang M, Li W, Zhou S, Zhou Y, Yang H, Yu L, Wang J, Wang Y, Zhang L. Clinical Features, Treatment, and Outcomes Among Chinese Children with Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis. Front Neurol. 2019 Jun 6;10:596. doi: 10.3389/fneur.2019.00596. PMID: 31244759; PMCID: PMC6562280.

[4].   Moen A. Diagnosing and Treating Anti N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Pediatric Perspective 2014, VOLUME 23, NUMBER 4

[5].   Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis. 2012 Apr;54(7):899-904. doi: 10.1093/cid/cir1038. Epub 2012 Jan 26. PMID: 22281844; PMCID: PMC3297648.

[6].   Dubey D, Pittock SJ, Kelly CR, McKeon A, Lopez-Chiriboga AS, Lennon VA, Gadoth A, Smith CY, Bryant SC, Klein CJ, Aksamit AJ, Toledano M, Boeve BF, Tillema JM, Flanagan EP. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018 Jan;83(1):166-177. doi: 10.1002/ana.25131. PMID: 29293273; PMCID: PMC6011827.

[7].   Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, Dessain SK, Rosenfeld MR, Balice-Gordon R, Lynch DR. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008 Dec;7(12):1091-8. doi: 10.1016/S1474-4422(08)70224-2. Epub 2008 Oct 11. PMID: 18851928; PMCID: PMC2607118.

[8].   Mann AP, Grebenciucova E, Lukas RV. Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges. Ther Clin Risk Manag. 2014 Jul 1;10:517-25. doi: 10.2147/TCRM.S61967. PMID: 25061311; PMCID: PMC4085332.

[9].   Agarwal R, Gupta V. Anti-NMDAR Encephalitis in Children. 2023 Jul 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan–. PMID: 32965889.

[10].  Sandweiss AJ, Erickson TA, Jiang Y, Kannan V, Yarimi JM, Levine JM, Fisher K, Muscal E, Demmler-Harrison G, Murray KO, Ronca SE. Infectious profiles in pediatric anti-N-methyl-d-aspartate receptor encephalitis. J Neuroimmunol. 2023 Aug 15;381:578139. doi: 10.1016/j.jneuroim.2023.578139. Epub 2023 Jun 20. PMID: 37364517.

[11].  Manto M, Jissendi P. Brain imaging in cerebellar ataxia associated with autoimmune polyglandular syndrome type 2. J Neuroimaging. 2012 Jul;22(3):308-11. doi: 10.1111/j.1552-6569.2010.00542.x. Epub 2010 Nov 17. PMID: 21091821.

[12].  Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013 Feb;12(2):157-65. doi: 10.1016/S1474-4422(12)70310-1. Epub 2013 Jan 3. PMID: 23290630; PMCID: PMC3563251.

[13].  Ramanathan S, Mohammad SS, Brilot F, Dale RC. Autoimmune encephalitis: recent updates and emerging challenges. J Clin Neurosci. 2014 May;21(5):722-30. doi: 10.1016/j.jocn.2013.07.017. Epub 2013 Sep 15. PMID: 24246947.

[14].  Liba Z, Sebronova V, Komarek V, Sediva A, Sedlacek P. Prevalence and treatment of anti-NMDA receptor encephalitis. Lancet Neurol. 2013 May;12(5):424-5. doi: 10.1016/S1474-4422(13)70070-X. PMID: 23602156.

[15].  Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004; 75:1135–40.

[16].  Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000 Jul;123 ( Pt 7):1481-94. doi: 10.1093/brain/123.7.1481. PMID: 10869059.

[17].  Favier M, Joubert B, Picard G, Rogemond V, Thomas L, Rheims S, Bailhache M, Villega F, Pédespan JM, Berzero G, Psimaras D, Antoine JC, Desestret V, Honnorat J. Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis. Eur J Paediatr Neurol. 2018 May;22(3):404-11. doi: 10.1016/j.ejpn.2017.12.014. Epub 2017 Dec 28. PMID: 29310866.