ºÚÁϳԹÏÍø

Home⇛The PCMC Journal⇛vol. 18 no. 2 (2022)

Factors affecting the clinical outcome of pediatric Anti- N-Methyl-Daspartate receptor encephalitis, a single center study.

Melady D Imperial-Gilbuena | Rose Daynielle A Cansanay | Madelyn P Pascual | Mel Michel Villaluz | Marilyn H Ortiz | Lillian V Lee

Discipline: medicine by specialism

 

Abstract:

Background: Anti-N-Methyl-D-Aspartate receptor (anti-NMDAR) Encephalitis is the most common type of autoimmune encephalitis that affects children, adolescents and young adults. Since its discovery in 2007, there is still a paucity of data on the disease and factors affecting its outcome. Objectives: To describe the clinical characteristics of children and adolescents with anti-NMDAR encephalitis and to analyze factors that may affect its outcome. Methods: Forty-three patient records of diagnosed anti-NMDAR Encephalitis were included. The outcome was evaluated using the modified Rankin Scale (mRS), and Clinical Assessment Scale for autoimmune Encephalitis (CASE). Results: Ages ranged from 2 years to 18 years old, majority in the 12-18 years age range. Sixty percent were female. First line treatment using immunotherapy was given to all patients: 37% as monotherapy and 84% combination therapy (MPT only 23%, IVIg only 4%, MPT + IVIg or TPE 21-26%, and MPT + IVIg + TPE 16%). Clinical outcomes on discharge and on follow-up were assessed using the mRS and CASE. On discharge the proportion of the patients who had mild impairment (mRS<2, CASE<9) was more than 50%. On median duration follow-up of 31 weeks (range 24-40 weeks), 96.8% had significant improvement (mRS<2, CASE<9). Among the possible factors that were assessed to affect outcome, only severity of the illness at the start of the treatment influenced clinical outcome. Conclusion: Early diagnosis and initiation of treatment before the progression of the disease will promote faster recovery and more optimal clinical outcome. CASE may be used as an additional tool in assessing response to treatment.



References:

  1. 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 Neurology. 2008;7(12):1091.Epub 2008 Oct 11, Available at; PMID:18851928; DOI: 10.1016/S1474-4422(08)70224-2
  2. Mary S Gable, Heather Sheriff, Josep Dalmau, Drake H Tilley, Carol A Glaser. 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. Clinical Infectious Disease. 2012 Apr;54(7):899-904. Epub 2012 Jan 26, Available at ; PMID: 22281844; doi:10.1093/cid/cir1038.
  3. Maarten J Titulaer, Lindsey McCracken, Iñigo Gabilondo, Thaís Armangué, Carol Glaser, Takahiro Iizuka, Lawrence S Honig, Susanne M Benseler, Izumi Kawachi, Eugenia Martinez-Hernandez, Esther Aguilar, Núria Gresa-Arribas, Nicole Ryan-Florance,  Abiguei Torrents, Albert Saiz, Myrna Rc Rosenfeld, Rita Balice-Gordon, Francesc Graus, Josep Dalmau. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurology. 2013 Feb;12(2):157-65. Available at: , DOI:0.1016/S1474-4422(12)70310-1
  4. Kayser MS, Titulaer MJ, Gresa- Arribas N, Dalmau J. Frequency and characteristics of isolated psychiatric episodes in anti–N-methyl-d-aspartate receptor encephalitis. JAMA Neurol 2013; 70:1133. Available at: ; PMDI: 32576635
  5. Dalmau J, Tuzun E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25-36. Available at: .PMID: 17262855, PMCID: PMC2430743, DOI: 10.1002/ana.21050
  6. 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. Clinical Infectious Disease. 2012 Apr;54(7):899-904. Epub 2012 Jan 26, Available at: PMCID: PMC3297648; DOI: 10.1093/cid/cir1038
  7. Florance NR, Davis RL, Lam C, et al. Anti-N-methyl-D-aspartate re- ceptor (NMDAR)  encephalitis in children and adolescents. Annals of Neurology. Florance NR, Davis RL, Lam C, et al. Anti-N-methyl-D-aspartate receptor (NMDAR)b encephalitis in children and adolescents. Ann Neurol.2009;66(1):11-18. Available at PMC2826225/, DOI:10.1002/ana.21756
  8. Wright S, Hacohen Y, Jacobson L, Agrawal S, Gupta R, Philip S, Smith M, Lim M, Wassamer E, Vincent A. NMDAR-Ab mediated neurological disease results of a U.K. based surveillance study in children. Archives of Disease in Childhood 30 Jan 2015;100:521–526; Available at: [PubMed] , DOI:10.1136/archdischild-2014-306795
  9. Treatment strategies for autoimmune encephalitis Yong-Won Shin, Soon-Tae Lee, Kyung-Il Park, Keun-Hwa Jung, Ki-Young Jung, Sang Kun Lee and Kon Chu. Therapeutic Advances in Neurologic Disorders, Shin YW, Lee ST, Park KI, et al. Treatment strategies for autoimmune encephalitis. Therapeutic Advances in Neurological Disorder 2017;11:1756285617722347.  Published 2017 Aug 16. [PMC] Available at: PMC5784571, DOI:10.1177/1756285617722347
  10. Josep Dalmau, Thais Armangué, Jesús Planagumà, Marija Radosevic, Francesco Mannara, Frank Leypoldt , Christian Geis, Eric Lancaster, Maarten J Titulaer, Myrna R Rosenfeld, Francesc Graus. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models Lancet Journal, 2019 Nov;18(11):1045-1057. [PubMed] Available at: . PMID: 31326280, DOI: 10.1016/S1474-4422(19)30244-3
  11. 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. [PMC] Available at: doi: 10.1002/ana.21756. PMID: 19670433; PMCID: PMC2826225.
  12. Sukhvir Wright, Yael Hacohen, Leslie Jacobson, Shakti Agrawal, Rajat Gupta, Sunny Philip, Martin Smith, Ming Lim, Evangeline Wassmer, Angela Vincent  -methyl-D-aspartate receptor antibody-mediated neurological disease: results of a UK-based surveillance study in children. Archives of Disease in Childhood 2015 Jun;100(6):521-6.[PubMed] Available at: .doi: 10.1136/archdischild-2014-306795
  13. Finke C, Kopp UA, Pruss H, et al. Cognitive deficits following anti-NMDA receptor  encephalitis. Journal Neurology, Neurosurgery and Psychiatry 2012;83:195–8. [PubMed] Available at:https://pubmed.ncbi.nlm.nih.gov/21933952/. DOI: 10.1136/jnnp-2011-300411; PMID: 21933952, PMCID: PMC3718487
  14. Anastasia Zekeridou , Evgenia Karantoni, Aurélien Viaccoz, François Ducray, Cyril Gitiaux, Frédéric Villega, Kumaran Deiva, Veronique Rogemond, Elodie Mathias, Géraldine Picard, Marc Tardieu, Jean-Christophe Antoine, Jean-Yves Delattre, Jerome Honnorat, Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis. 2015 Aug;262(8):1859-66. [PubMed] doi: 10.1007/s00415- 015-7781-9
  15. Rose Daynielle A. Cansanay, Md, Katrina Irene T. Manibog, Md, Madelyn P. Pascual Md, Lillian V. Lee, Md, Marilyn H. Ortiz Md, Clinical Profile And Outcome Of Filipino Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis Admitted In A Pediatric Tertiary Hospital From January 2011 To December 2018
  16. Jung-Ah Lim MD; Soon-Tae Lee MD,PhD; Jangsup Moon MD, PhD; Jin-Sun Jun MD ; Tae-Joon Kim MD; Yong-Won Shin MD, et. Al, Development of the clinical assessment scale in autoimmune encephalitis; 24 January 2019; Annals of Neurology 24 January 2019; 85 (3): 352-35 Available at: 
  17. Helena Ariño MD, PhD , Amaia Muñoz-Lopetegi MD , Eugenia Martinez-Hernandez MD, PhD , Thaís Armangue MD, PhD , Mireia Rosa- Justicia PhD , Domingo Escudero MD, et. al, Sleep disorders in Anti-NMDAR encephalitis, American Academy of Neurology, August 11,2020; 95 (6), Available at: . DOI:
  18. The MDS-sponsored Revision of the Unified Parkinson’s Disease Rating Scale, 2008 International Parkinson and Movement Disorder Society, www.movementdisorders.org,
  19. Zhang et al, Anti-N-methyl-D-aspartate receptor encephalitis with accompanying ovarian teratoma in female patients from East China: Clinical features, treatment, and prognostic outcomes. Seizure: European Journal of Epilepsy 2020 (75) 55-62 Available at: .PMID: 31874360; DOI: 10.1016/j.seizure.2019.12.016
  20. Adang, L. A., Lynch, D. R., & Panzer, J. A. (2014). Pediatric anti-NMDA receptor encephalitis is seasonal. Annals of clinical and translational neurology, 1(11), 921–925. Available at: .
  21. Nosadini, M., Mohammad, S. S., Corazza, F., Ruga, E. M., Kothur, K., & Perilongo, G. (2017). Herpes simplex virus-induced anti-N-methyl-D-aspartate receptor encephalitis: A systematic literature review with analysis of 43 cases. Developmental Medicine and Child Neurology, 59(8),796–805. Available at: 39890. PMID: 8439890. DOI: 10.1111/dmcn.13448
  22. Dalmau, J., Geis, C., & Graus, F. (2017). Autoantibodies to Synaptic Receptors and  euronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. Physiological reviews, 97(2), 839–887. [PubMed]
  23. Xu Liu, 1Bo Yan, Rui Wang, Chen Li, Chu Chen, Dong Zhou, and Zhen Hong ; Seizure outcomes in patients with anti-NMDAR encephalitis: A follow-up study. Epilepsia, 2017 Dec;58(12):2104-2111. [PubMed] Available at: doi: 10.1111/epi.13929
  24. Stefano Sartori, Margherita Nosadini, Elisabetta Cesaroni, Raffaele Falsaperla Giuseppe Capovilla, Francesca Beccaria, et al, Paediatric anti-N-methyl-D-aspartate receptor encephalitis: The first Italian multicenter case series. European Journal of pediatric Neurology 2015 Jul;19(4):453-63.[PubMed] doi:10.1016/j.ejpn.2015.02.006. Epub 2015 Mar 3. PMID: 25792293
  25. Clayton D. Hinkle, James N. Porter, et. al, Neuropsychological characterization of three adolescent females with anti-NMDA receptor encephalitis in the acute, post-acute, and chronic phases: an inter- institutional case series, The Clinical Neuropsychologist 2017 Jan;31(1):268-288. [PubMed] doi:10.1080/13854046.2016.1191676
  26. Rui Wang , Hong-Zhi Guan, Hai-Tao Ren, et. al, CSF findings in patients with anti-N-methyl-D-aspartate receptor-encephalitis, European Journal of Epilepsy, 2015-07-01, (29),137-142. [PubMed] PMID: 26076857 DOI: 10.1016/j.seizure.2015.04.005
  27. Jeannin-Mayer S, Andre-Obadia N, Rosenberg S, Boutet C, Honnorat J, Antoine JC, et al. EEG analysis in anti-NMDA receptor encephalitis: description of typical patterns. Clinical Neurophysiology 2019 Feb;130(2):289-296. [PubMed] doi:10.1016/j.clinph.2018.10.017
  28. Vlatko Sulentic, Zeljka Petelin Gadze et al, The effect of delayed anti-NMDAR encephalitis recognition on disease outcome, Journal of Neurovirology 2018 Oct;24(5):638-641. [PubMed] doi: 10.1007/s13365-018-0648-y
  29. McKeon GL, et al.; Cognitive Outcomes following Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Systematic Review, Journal of Clinical and Experimental Neuropsychology 2018 Apr;40(3):234-252. [PubMed] doi:10.1080/13803395.2017.1329408
  30. Lulu Yan, Shuang Zhang, et al, Clinical Study of Autonomic Dysfunction in Patients With Anti-NMDA Receptor Encephalitis; Frontiers of Neurology 2021 Feb 5 12 (609750) [PubMed] https://pubmed.ncbi.nlm.nih.gov/33613429/PMID: 33613429 PMCID: PMC7894204 DOI: 10.3389/fneur.2021.609750

Tools


Copyright © 2025   ºÚÁϳԹÏÍø