Autoimmune encephalitis due to antibodies against N-methyl-D-aspartate (NMDA) receptor N-methyl-D-aspartate (NMDA) receptor: Case series in children
Keywords:
Autoimmune encephalitis, anti-NMDA antibodies, children, pediatric
Abstract
We describe a case series of pediatric patients with autoimmune encephalitis (AE) due to antibodies against the N-methyl-D-aspartate (NMDA) receptor, treated in tertiary hospitals in Mexico and Paraguay, and their evolution with immunotherapy. The predominant symptoms were fever, sleep disorders, hallucinations, dyskinesias, choreoathetotic movements, spasticity, seizures, and dysautonomia. Patients were treated with IVIG and IV methylprednisolone as first line therapy. In some cases, use of plasmapheresis and anti-CD20 monoclonal antibodies was required. Timely treatment initiation was related to a shorter hospital stay and less development of complications. Timely diagnosis should be emphasized, implementing its identification based on the clinical picture and supporting the diagnosis with adequate serological tests and CSF findings, thus favoring the start of early treatment to reduce complications and days of hospitalization.
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References
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(3) 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 Jul;92(7):757-768. doi: 10.1136/jnnp-2020-325300.
(4) 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 Apr;15(4):391-404. doi: 10.1016/S1474-4422(15) 00401-9.
(5) Erazo Torriceli R. Encefalitis autoinmunes. Receptor Anti-NMDA y nuevos inmunofenotipos. MEDICINA (Buenos Aires) 2019; Vol.79(Supl. III):54-59.
(6) Gastaldi M, Nosadini M, Spatola M, Sartori S, Franciotta D. N-methyl-D-aspartate receptor encephalitis: laboratory diagnostics and comparative clinical features in adults and children. Expert Rev Mol Diagn. 2018 Feb;18(2):181-193. doi: 10.1080/ 14737159.2018.1431124.
(7) Irani SR, Bera K, Waters P, Zuliani L, Maxwell S, Zandi MS, et al. N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non- paraneoplastic disorder of both sexes. Brain. 2010 Jun;133 (Pt 6):1655-67. doi: 10.1093/brain/awq113.
(8) Erazo R, González J, Quintanilla C, Devaud C, Gayoso C, Toledo X, et al. Encefalitis subaguda por anticuerpos anti receptor de N-metil-D-aspartato: Serie de 13 casos pediátricos. Rev. chil. pediatr. [Internet]. 2016 Dic [citado 2022 Mar 23];87(6):487-493.
(9) Hunt DL, Castillo PE. Synaptic plasticity of NMDA receptors: mechanisms and functional implications. Curr Opin Neurobiol. 2012 Jun;22(3):496-508. doi: 10.1016/j.conb.2012.01.007.
(10) Waxman EA, Lynch DR. N-methyl-D-aspartate receptor subtypes: multiple roles in excitotoxicity and neurological disease. Neuro- scientist. 2005 Feb;11(1):37-49. doi: 10.1177/1073858404269012.
(11) van Coevorden-Hameete MH, de Graaff E, Titulaer MJ, Hoogenraad CC, Sillevis Smitt PA. Molecular and cellular mechanisms under- lying anti-neuronal antibody mediated disorders of the central nervous system. Autoimmun Rev. 2014 Mar;13(3):299-312. doi: 10.1016/j.autrev.2013.10.016.
(12) Newman MP, Blum S, Wong RC, Scott JG, Prain K, Wilson RJ, Gillis D. Autoimmune encephalitis. Intern Med J. 2016 Feb;46(2):148-57. doi: 10.1111/imj.12974.
(13) Zuliani L, Nosadini M, Gastaldi M, Spatola M, Iorio R, Zoccarato M, et al. Management of antibody-mediated autoimmune ence- phalitis in adults and children: literature review and consensus- based practical recommendations. Neurol Sci. 2019 Oct;40(10):2017-2030. doi: 10.1007/s10072-019-03930-3.
(14) 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 Neuroin flamm. 2020 Jan 17;7(2):e663. doi: 10.1212/NXI.0000000000000663.
(15) Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, et al. 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.
(16) Lim JA, Lee ST, Moon J, Jun JS, Kim TJ, Shin YW, et al. Develop- ment of the clinical assessment scale in autoimmune encephalitis. Ann Neurol. 2019 Mar;85(3):352-358. doi: 10.1002/ana.25421.
(17) Scheer S, John RM. Anti-N-Methyl-D-Aspartate Receptor Ence- phalitis in Children and Adolescents. J Pediatr Health Care. 2016 Jul-Aug;30(4):347-58. doi: 10.1016/j.pedhc.2015.09.004.
(18) Steriade C, Moosa ANV, Hantus S, Prayson RA, Alexopoulos A, Rae-Grant A. Electroclinical features of seizures associated with autoimmune encephalitis. Seizure. 2018 Aug;60:198-204. doi: 10.1016/j.seizure.2018.06.021.
(19) Dale RC, Gorman MP, Lim M. Autoimmune encephalitis in children: clinical phenomenology, therapeutics, and emerging challenges. Curr Opin Neurol. 2017 Jun;30(3):334-344.
Published
2022-06-04
How to Cite
1.
Suárez Martínez JM, Soto Zurita C, Aparicio Vera LA, Florentín C, Medina Barrios J, Vázquez J, Morel Z. Autoimmune encephalitis due to antibodies against N-methyl-D-aspartate (NMDA) receptor N-methyl-D-aspartate (NMDA) receptor: Case series in children. Rev. parag. reumatol. [Internet]. 2022Jun.4 [cited 2025May11];8(1):21-6. Available from: https://revista.spr.org.py/index.php/spr/article/view/155
Section
ORIGINAL ARTICLES
Copyright (c) 2022 Paraguayan Journal of Rheumatology
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