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Abstract

Background: Autoimmune encephalitis (AIE) are distinct group of encephalitis where production of autoimmune antibody causes neuroinflammation. The core clinical features are encephalopathy, psychiatric disorder, movement disorder and seizure. The investigation and treatment modalities are different from that of infectious encephalitis. There are limited studies in pediatric population in particularly in developing country like Bangladesh. Thus this study has been done to describe patients with AIE from a tertiary care hospital.


Method: This is a retrospective study done in children of 1-16 year from January 2018 to December 2019. AIE was diagnosed on the basis of clinical, electrographic and neuroimaging features and was confirmed with detection of autoantibody in CSF. Treatment was given according to the published literature with immunotherapy mainly.


Results: Total 15 children were studied, 14 patients were antiNMDAR encephalitis and 1 was antiMOG antibody syndrome. Mean age was 5.98 and 4.5 year respectively. Seizure was the most common clinical feature, mostly focal in nature. Other manifestations were movement disorder, psychiatric disorder, loss of consciousness etc. Most of the patients had abnormal EE, focal epileptic discharge being the commonest. Eight out of 15 had abnormal MRI of brain. Cortical  hyperintensity was important feature located mostly in temporal region. In the case of antiMOG antibody syndrome there was demyelinating lesion in multiple areas. Cornerstone of the treatment was mostly combination immunotherapy with IV methylprednisolone and IV immunoglobulin followed by oral steroid. Majority of the patients showed improvement however 3 patients had complete recovery. Complications observed were epilepsy, speech disorder, cognitive disorder, behavioural disorder, ataxia and visual impairment.


Conclusion:  Timely diagnosis and prompt treatment of AIE is very important as proper treatment can cause significant improvement.  

Keywords

Autoimmune encephalitis, antiNMDAR encephalitis, antiMOG antibody syndrome

Article Details

How to Cite
Fatema, K., & Rahman, M. M. . (2021). Pediatric Autoimmune encephalitis: Experience from a Tertiary Care Hospital in Bangladesh. Journal of the International Child Neurology Association, 1(1). https://doi.org/10.17724/jicna.2021.200

References

    1. Blaabjerg M, Seifert-Held T, Sellner J. Emerging Challenges in the Diagnosis and Treatment of Autoimmune Encephalitis. Front. Neurol. 2019. 10:146. doi: 10.3389/fneur.2019.00146

    2. Dalmau J, Tuzun E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36.

    3. Saraya A, Mahavihakanont A, Shuangshoti S, Sittidetboripat N, Deesudchit T, Callahan M, et al. Autoimmune causes of encephalitis syndrome in Thailand: prospective study of 103 patients. BMC Neurol. 2013;13:150.

    4. Kamble N, Netravathi M, Saini J, Mahadevan A, Yadav R, Nalini A, et al. Clinical and imaging characteristics of 16 patients with autoimmune neuronal synaptic encephalitis. Neurol India. 2015;63(5):687–96.

    5. 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. doi:10.1016/S1474-4422(15)00401-9.

    6. Khadilkar S, Soni G, Patil S, Huchche A, Faldu H. Autoimmune Encephalitis: An update. J Assoc Physicians India 2017 ;65(2):62-69.

    7. Sai Y, Zhang X, Feng M, Tang J, Liao H, Tan L. Clinical diagnosis and treatment of pediatric anti-N-methyl-D-aspartate receptor encephalitis: A single center retrospective study. Exp Ther Med. 2018; 16(2):1442–1448. doi:10.3892/etm.2018.6329

    8. Saraya AW , Worachotsueptrakun W , Vutipongsatorn K, Sonpee C , Hemachudha T. Differences and diversity of autoimmune encephalitis in 77 cases from a single tertiary care center. BMC Neurology 2019; 19:273. https://doi.org/10.1186/s12883-019-1501-5

    9. Dutra LA, Abrantes F, Toso FFT, Pedroso JL, Barsottini OGP, Hoftberger R. Autoimmune encephalitis: a review of diagnosis and treatment . Arq Neuropsiquiatr 2018; 76(1):41-49.

    10. Zhang J, Ji T, Chen Q, Jiang, Y., Cheng, H., Zheng, P et al. Pediatric Autoimmune Encephalitis: Case Series From Two Chinese Tertiary Pediatric Neurology Centers. Front Neurol. 2019; 10:906. Published 2019 Aug 22. doi:10.3389/fneur.2019.00906

    11. Zekeridou A, Karantoni E, Viaccoz A, Ducray F, Gitiaux C, Villega F, et al. Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis. J Neurol. (2015) 262:1859– 66. doi: 10.1007/s00415-015-7781-9

    12. Viaccoz A, Desestret V, Ducray F, Picard G, Cavillon G, Rogemond V, et al. Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis. Neurology. (2014) 82:556–63. doi: 10.1212/WNL.0000000000000126

    13. Gable MS, Gavali S, Radner A, Tilley DH, Lee B, Dyner L. Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis. Eur J Clin Microbiol Infect Dis. 2009 Dec;28(12):1421-9. doi: 10.1007/s10096-009-0799-0.
    14. Dalmau J, Gleichman AJ, Hughes EG , Rossi JE, Peng X, Lai M et al . Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; 7:1091–1098.
    15. Lancaster E, Leypoldt F, Titulaer MJ, Honnorat J, Waters PJ, Reindl M, et al. Immunoglobulin G antibodies to the N-Methyl-D-aspartate receptor are distinct from immunoglobulin A and immunoglobulin M responses. Ann Neurol 2015; 77:183.

    16. Masdeu JC, González-Pinto A, Matute C, Ruiz-de-Azúa S, Palomino A, De Leon J, et al. Serum IgG antibodies against the NR1 subunit of the NMDA receptor not detected in schizophrenia. Am J Psychiatry 2012;169:1120-1121.

    17. T. Zhang, Y. Duan, J. Ye, W. Xu, N. Shu, C. Wang, K. Li and Y. Brain MRI Characteristics of Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Their Associations with 2-Year Clinical Outcome. American Journal of Neuroradiology March 2018, DOI: https://doi.org/10.3174/ajnr.A5593
    18. 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; 12(2):157-65. https://doi.org/10.1016/S1474-4422 (12)70310-1

    19. Shin YW, Lee ST, Park KI, Jung KH, Jung KY, Lee SK et al. Treatment strategies for autoimmune encephalitis. Ther Adv Neurol Disord. 2017;11 1756285617722347. doi: 10.1177/1756285617722347.

    20. Nosadini M, Mohammad SS, Ramanathan S, Brilot F, Dale RC et al. Immune therapy in autoimmune encephalitis: a systematic review.Expert Rev Neurother. 2015; 15(12):1391-419.

    21. Wiseman AC . Immunosuppressive Medications . Clin J Am Soc Nephrol. 2016 ; 11(2):332-43.

    22. Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011 Jan; 10(1):63-74.

    23. Lee WJ, Lee ST, Byun JI, Sunwoo JS, Kim TJ, Lim JA, et al. Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort. Neurology. 2016 May 3; 86(18):1683-91.

    24. Lana-Peixoto MA, Talim N . Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes. Biomedicines. 2019 Jun; 7(2): 42. doi: 10.3390/biomedicines7020042.

    25. Jarius S, Metz I, Konig FB, Ruprecht K, Reindl M, Paul F et al. Screening for MOG IgG and 27 other anti-glial and anti-neuronal autoantibodies in ‘Pattern II multiple sclerosis’ and brain biopsy findings in a MOG IgG-positive case. Mult. Scler. 2016;22:1541–1549. doi: 10.1177/1352458515622986.
    26. Spadaro M, Gerdes LA, Mayer MC, Ertl-Wagner B, Laurent S, Krumbholz M, et al. Histopathology and clinical course of mog-antibody-associated encephalomyelitis. Ann. Clin. Transl. Neurol. 2015;2:295–301. doi: 10.1002/acn3.164.
    27. Reindl M., Rostasy K. MOG antibody-associated diseases. Neurol. Neuroimmunol. Neuroinflamm. 2015;2:e60. doi: 10.1212/NXI.0000000000000060.

    28. Di Pauli F, Hoftberger R, Reindl M, Beer R, Rhomberg P, Schanda K, et al. Fulminant demyelinating encephalomyelitis: Insights from antibody studies and neuropathology. Neurol. Neuroimmunol. Neuroinflamm. 2015;2:e175. doi: 10.1212/NXI.0000000000000175.

    29. Jarius S, Ruprecht K, Kleiter I, Borisow N, Asgari N, Pitarokoili K et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association withAQP4-IgG, and origin. J Neuroinflammation 2016; 13: 279.

    30. Weber MS, Derfuss T, Metz I, Brück W. Defining distinct features of anti-MOG antibody associated central nervous system demyelination. Ther Adv Neurol Disord. 2018;11:1756286418762083. Published 2018 Mar 29. doi:10.1177/1756286418762083

    31. Hacohen Y, Banwell B. Treatment Approaches for MOG-Ab-Associated Demyelination in Children. Curr Treat Options Neurol. 2019;21(1):2.

    32.Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology. 2008; 70(7):504-11.

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