Cognitive and psychiatric evolution in autoimmune encephalitis in a school-aged child: Clinical case

Main Article Content

Dra. Myriam Guadalupe Márquez Zapata https://orcid.org/0009-0002-6276-8739
Dr. Gustavo Joaquín Cruz Ruelas https://orcid.org/0009-0000-4841-856X
Dra. Alma Patricia González https://orcid.org/0000-0002-3401-7519
Dr. Carlos Paque Bautista https://orcid.org/0000-0002-2658-0491
Dra. Cecilia Elizabeth González Delgado https://orcid.org/0009-0002-2919-100X
Gloria Patricia Sosa-Bustamante https://orcid.org/0000-0002-8460-4965

Keywords

Autoimmune Diseases of the Nervous System, Neuropsychiatry, Mental Status and Dementia Tests, Sleep Disorders, Circadian Rhythm, Neuropsychology

Abstract

Background: Autoimmune encephalitis (AE) is a rare entity in children and can manifest psychiatric symptoms without evident neurological signs.


Clinic case: A 10-year-old female with a subacute case of 3 weeks of evolution, denotating disinhibition, behavioral errors, mood swings with irritability, auditory stimuli intolerance, restlessness in social situations; sleep cycle disturbances, right arm weakness upon physical exertion that progressed into right hemiparesis, adynamia, verbal apraxia, and agraphia. The patient was hospitalized, and infectious processes were excluded. Brain imaging with nuclear magnetic resonance showed lesions compatible with basal leptomeningitis. The electroencephalogram did not show cortical irritability. Suspecting an autoimmune etiology, the patient received therapy with immunomodulators, showing a gradual response without reaching to previous functionality state. Sertraline and second-generation antipsychotics were added, with the patient being discharged due to overall improvement. At 3 months of follow-up, the patient persisted with moderate cognitive impairment. Fifteen months after disease debut, the patient's cognitive status score was normal, with mild irritability, and continued avoidance of intense auditory stimuli and crowded places.


Conclusions: AE diagnosis is challenging for pediatricians, as a high suspicion level is required when facing nonspecific symptoms; the prognosis depends on early identification and initiation of immunotherapy.

Abstract 18 | PDF (Spanish) Downloads 16

References

1. García RV, Savransky A, Reyes Valenzuela G, et al. Tratamiento y evolución de la encefalitis inmunomediada en edad pediátrica, serie de casos. Medicina Infantil, 2024;XXXI(1):16-25. Disponible en: https://revistamedicinainfantil.org.ar/2024/04/23/numero-1-6/

2. Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9.

3. Santoro JD, Demakakos P, He S, et al. A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making. Front Neurol. 2024;15:1408606. doi: 10.3389/fneur.2024.1408606.

4. Dubey D, Pittock SJ, Kelly CR, et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177. doi: 10.1002/ana.25131.

5. Kang Q, Liao H, Yang L, et al. Clinical Characteristics and Short-Term Prognosis of Children With Antibody-Mediated Autoimmune Encephalitis: A Single-Center Cohort Study. Frontiers in Pediatrics. 2022;10. doi: 10.3389/fped.2022.880693.

6. Menculini G, Chipi E, Paolini Paoletti F, et al. Insights into the Pathophysiology of Psychiatric Symptoms in Central Nervous System Disorders: Implications for Early and Differential Diagnosis. Int J Mol Sci. 2021;22(9):4440. doi: 10.3390/ijms22094440.

7. Nguyen L, Yang JH, Goyal S, et al. A systematic review and quantitative synthesis of the long-term psychiatric sequelae of pediatric autoimmune encephalitis. J Affect Disord. 2022;308:449-457. doi: 10.1016/j.jad.2022.04.027.

8. Sachs N, Zohar-Dayan E, Ben Zeev B, et al. Autoimmune encephalitis in Israeli children - A retrospective nationwide study. Eur J Paediatr Neurol. 2024;50:1-5. doi: 10.1016/j.ejpn.2024.03.001.

9. Hauptman AJ, Ferrafiat V. Neuroinflammatory syndromes in children. Curr Opin Psychiatry. 2023;36(2):87-95. doi: 10.1097/YCO.0000000000000846.

10. Kendall, F.P., McCreary, E.K. and Provance, P.G. Muscle Testing and Function. 4th Edition, Lippincott, Williams and Wilkins. Philadelphia: Scientific Research Publishing; 2015. Disponible en: https://www.scirp.org/reference/referencespapers?referenceid=1601849

11. Nasreddine ZS, Phillips NA, Bédirian V, et al. The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005;53(4):695-9. doi: 10.1111/j.1532-5415.2005.53221.x. Erratum in: J Am Geriatr Soc. 2019;67(9):1991. doi: 10.1111/jgs.15925.

12. Graus F, Vogrig A, Muñiz-Castrillo S, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021;8(4):e1014. doi: 10.1212/NXI.0000000000001014.

13. Lim JA, Lee ST, Moon J, et al. Development of the clinical assessment scale in autoimmune encephalitis. Ann Neurol. 2019;85(3):352-358. doi: 10.1002/ana.25421.

14. Li Q, Fu N, Han Y, et al. Pediatric Autoimmune Encephalitis and Its Relationship With Infection. Pediatr Neurol. 2021;120:27-32. doi: 10.1016/j.pediatrneurol.2021.04.001.

15. Cellucci T, Van Mater H, Graus F, et al. Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. Neurol Neuroimmunol Neuroinflamm. 2020;7(2):e663. doi: 10.1212/NXI.0000000000000663. Erratum in: Neurol Neuroimmunol Neuroinflamm. 2020;7(4):e730. doi: 10.1212/NXI.0000000000000730.

16. Hacohen Y, Wright S, Waters P, et al. Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens. J Neurol Neurosurg Psychiatry. 2013;84(7):748-55. doi: 10.1136/jnnp-2012-303807.

17. Nosadini M, Mohammad SS, Ramanathan S, et al. Immune therapy in autoimmune encephalitis: a systematic review. Expert Rev Neurother. 2015;15(12):1391-419. doi: 10.1586/14737175.2015.1115720.

18. Madani J, Yea C, Mahjoub A, et al. Clinical features and outcomes in children with seronegative autoimmune encephalitis. Dev Med Child Neurol. 2024;66(10):1310-1318. doi: 10.1111/dmcn.15896.

19. Panda PK, Sharawat IK, Ramachandran A, et al. Validity and prognostic utility of clinical assessment scale for autoimmune encephalitis (CASE) score in children with autoimmune encephalitis. Brain Dev. 2023;45(1):8-15. doi: 10.1016/j.braindev.2022.09.009.

20. Zhou H, Deng Q, Yang Z, et al. Performance of the clinical assessment scale for autoimmune encephalitis in a pediatric autoimmune encephalitis cohort. Front Immunol. 2022;13:915352. doi: 10.3389/fimmu.2022.915352.

21. Nosadini M, Mohammad SS, Ramanathan S, et al. Immune therapy in autoimmune encephalitis: a systematic review. Expert Rev Neurother. 2015;15(12):1391-419. doi: 10.1586/14737175.2015.1115720.

22. Dalmau J, Armangué T, Planagumà J, et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol. 2019;18(11):1045-1057. doi: 10.1016/S1474-4422(19)30244-3.

23. Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12(2):157-65. doi: 10.1016/S1474-4422(12)70310-1.

24. Lennon VA, Kryzer TJ, Pittock SJ, et al. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202(4):473-7. doi: 10.1084/jem.20050304.

25. Finke C, Kopp UA, Prüss H, et al. Cognitive deficits following anti-NMDA receptor encephalitis. J Neurol Neurosurg Psychiatry. 2012;83(2):195-8. doi: 10.1136/jnnp-2011-300411.

26. Adana Díaz L, Arango A, Parra C, et al. Impact of Educational Level on Versions (Basic and Complete) of the Montreal Cognitive Assessment. Dement Geriatr Cogn Disord. 2021;50(4):341-348. doi: 10.1159/000518747.

27. Scheuermeier M, Chaves KQ, Marín-Sanabria D, et al. First Pediatric Case of Autoimmune Encephalitis Associated With COVID-19 in Costa Rica. Cureus. 2022;14(10):e30616. doi: 10.7759/cureus.30616.

28. Zhang M, Zhu X, Yu L, et al. Pediatric anti-CaVα2δ autoimmune encephalitis: A case report and literature review. J Neuroimmunol. 2025;401:578550. doi: 10.1016/j.jneuroim.2025.578550.

29. Abderrahman Y, Chegondi M. A Case of Autoimmune Encephalitis in Disguise in an Adolescent Female. Pediatrics. 2022;149(1 Meeting Abstracts February 2022):389.

30. Sabanathan S, Abdel-Mannan O, Mankad K, et al. Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study. Ann Clin Transl Neurol. 2022;9(1):67-78. doi: 10.1002/acn3.51494.

31. Ramírez-Campos J, Núñez-Velázquez M, Cárdenas-Rojo N, et al. Confiabilidad intra e interobservador de la lectura del EEG en pacientes pediátricos con alteraciones neurológicas [Intra and inter-observer reliability of electroencephalogram interpretation in pediatric patients with neurological alterations]. Rev Med Inst Mex Seguro Soc. 2014;52(4):376-81.