European Journal of Clinical and Biomedical Sciences

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Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma

Received: 16 March 2023    Accepted: 1 April 2023    Published: 25 September 2023
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Abstract

Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a rare condition, often associated with neoplasms and characterized by neuropsychiatric symptoms and dysautonomia. Affecting approximately 0.01% of cancer patients, it is the second most frequent autoimmune encephalitis after acute disseminated encephalomyelitis, its clinical presentation is usually acute and severe, so prompt identification and treatment improve prognosis. We present the case of a 23-year-old female patient from a rural area of Caquetá, Colombia, previously healthy, who presented persistent psychotic symptoms of two weeks of evolution and was initially treated in a psychiatric unit where she evolved with systemic compromise. Finally, a diagnosis of paraneoplastic anti-NDMAR encephalitis was made. The patient received IV immunoglobulin G plus adjuvant systemic management with chemotherapy. She had complete resolution of her neuropsychiatric disorder and was asymptomatic when discharged. In conclusion, anti-NMDAR encephalitis is a condition with an underlying autoimmune mechanism, with production of autoantibodies mainly against the NR1 and NR2 subunits of NMDA receptors in the brain. These autoantibodies are produced in response to a breakdown of immune tolerance usually secondary to a neoplastic process, mostly ovarian teratoma, or infectious as in the case of herpes viral encephalitis. Despite its severe and potentially fatal nature, AE typically exhibits a positive response to treatment if a prompt diagnosis is established and management is initiated. However, it is acknowledged in some texts that there is still a gap in recognizing this entity, which encourages future valuable research.

DOI 10.11648/j.ejcbs.20230904.12
Published in European Journal of Clinical and Biomedical Sciences (Volume 9, Issue 4, August 2023)
Page(s) 51-54
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Encephalitis, Psychosis, Autoimmune Diseases, Paraneoplastic Encephalitis

References
[1] Fu P, He L, Tang N, Nie Q, Li Z. A single center retrospective study of paraneoplastic neurological syndromes with positive onconeural antibodies. Journal of Clinical Neuroscience. 2021; 89: 336–42.
[2] Patel, A., Koul, P., & Lado, F. (2021). The Autoimmune Encephalitis: Historical Overview (2587). Neurology, 96 (15 Supplement), 2587.
[3] Vogrig A, Gigli GL, Segatti S, Corazza E, Marini A, Bernardini A, et al. Epidemiology of paraneoplastic neurological syndromes: a population-based study. Journal of Neurology. 2020; 267 (1): 26–35.
[4] Midgley, L., Kelleher, E., & Zandi, M. S. Autoimmune encephalitis. British Journal of Hospital Medicine. 2018; 79 (9), C134–C137.
[5] Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, Baehring JM, Shimazaki H, Koide R, King D, Mason W, Sansing LH, Dichter MA, Rosenfeld MR, Lynch DR. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007; 61 (1): 25-36.
[6] Vaux, A., Robinson, K., Saglam, B., Cheuk, N., Kilpatrick, T., Evans, A., & Monif, M. Autoimmune Encephalitis in Long-Standing Schizophrenia: A Case Report. Frontiers in Neurology. 2022. 12, 1–7.
[7] Prüss H, Dalmau J, Harms L, Höltje M, Ahnert-Hilger G, Borowski K, et al. Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin. Neurology. 2010; 75 (19): 1735–9.
[8] 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. Vol. 92, Journal of Neurology, Neurosurgery and Psychiatry. BMJ; 2021. p. 757–68.
[9] 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.
[10] Gresa-Arribas N, Titulaer MJ, Torrents A, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol 2014; 13: 167.
[11] Vedeler CA, Antoine JC, Giometto B, et al. Management of paraneoplastic neurological syndromes: report of an EFNS Task Force. Eur J Neurol 2006; 13: 682.
[12] Lejuste F, Thomas L, Picard G, et al. Neuroleptic intolerance in patients with anti-NMDAR encephalitis. Neurol Neuroimmunol Neuroinflamm 2016; 3: e280.
[13] Granerod J, Dphil A, Clewley JP, Walsh AL, Morgan D, Brown Frcpath WG, et al. Causes of encephalitis and diff erences in their clinical presentations in England: a multicentre, population- based prospective study. Lancet Infect Dis. 2010 Dec; 10 (12): 835-44.
[14] Lawn ND, Westmoreland BF, Kiely MJ, et al. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc 2003; 78: 1363.
[15] Dalmau J, Graus F. Autoimmune Encephalitis-Misdiagnosis, Misconceptions, and How to Avoid Them. JAMA Neurol. 2023 Jan 1; 80 (1): 12-14.
[16] Britton, J. W., & Dalmau, J. Recognizing autoimmune encephalitis as a cause of seizures: Treating cause and not effect. Neurology. 2019; 92 (19), 877–878.
Cite This Article
  • APA Style

    Gonzalez Manrique Guillermo, Zabaleta Orozco Mario Alberto, Claros Ortiz Katherine Viviana, Cano Arias Luis Guillermo, Velasco Hoyos Maria Fernanda, et al. (2023). Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma. European Journal of Clinical and Biomedical Sciences, 9(4), 51-54. https://doi.org/10.11648/j.ejcbs.20230904.12

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    ACS Style

    Gonzalez Manrique Guillermo; Zabaleta Orozco Mario Alberto; Claros Ortiz Katherine Viviana; Cano Arias Luis Guillermo; Velasco Hoyos Maria Fernanda, et al. Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma. Eur. J. Clin. Biomed. Sci. 2023, 9(4), 51-54. doi: 10.11648/j.ejcbs.20230904.12

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    AMA Style

    Gonzalez Manrique Guillermo, Zabaleta Orozco Mario Alberto, Claros Ortiz Katherine Viviana, Cano Arias Luis Guillermo, Velasco Hoyos Maria Fernanda, et al. Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma. Eur J Clin Biomed Sci. 2023;9(4):51-54. doi: 10.11648/j.ejcbs.20230904.12

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  • @article{10.11648/j.ejcbs.20230904.12,
      author = {Gonzalez Manrique Guillermo and Zabaleta Orozco Mario Alberto and Claros Ortiz Katherine Viviana and Cano Arias Luis Guillermo and Velasco Hoyos Maria Fernanda and Solano Vega Irina Isabel and Lizcano Aros Jose Alfredo and Luna Tavera Rina Liliana},
      title = {Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma},
      journal = {European Journal of Clinical and Biomedical Sciences},
      volume = {9},
      number = {4},
      pages = {51-54},
      doi = {10.11648/j.ejcbs.20230904.12},
      url = {https://doi.org/10.11648/j.ejcbs.20230904.12},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ejcbs.20230904.12},
      abstract = {Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a rare condition, often associated with neoplasms and characterized by neuropsychiatric symptoms and dysautonomia. Affecting approximately 0.01% of cancer patients, it is the second most frequent autoimmune encephalitis after acute disseminated encephalomyelitis, its clinical presentation is usually acute and severe, so prompt identification and treatment improve prognosis. We present the case of a 23-year-old female patient from a rural area of Caquetá, Colombia, previously healthy, who presented persistent psychotic symptoms of two weeks of evolution and was initially treated in a psychiatric unit where she evolved with systemic compromise. Finally, a diagnosis of paraneoplastic anti-NDMAR encephalitis was made. The patient received IV immunoglobulin G plus adjuvant systemic management with chemotherapy. She had complete resolution of her neuropsychiatric disorder and was asymptomatic when discharged. In conclusion, anti-NMDAR encephalitis is a condition with an underlying autoimmune mechanism, with production of autoantibodies mainly against the NR1 and NR2 subunits of NMDA receptors in the brain. These autoantibodies are produced in response to a breakdown of immune tolerance usually secondary to a neoplastic process, mostly ovarian teratoma, or infectious as in the case of herpes viral encephalitis. Despite its severe and potentially fatal nature, AE typically exhibits a positive response to treatment if a prompt diagnosis is established and management is initiated. However, it is acknowledged in some texts that there is still a gap in recognizing this entity, which encourages future valuable research.},
     year = {2023}
    }
    

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  • TY  - JOUR
    T1  - Paraneoplastic Encephalitis Due to Anti-NMDA Receptor Antibodies in a Young Woman with Ovarian Teratoma
    AU  - Gonzalez Manrique Guillermo
    AU  - Zabaleta Orozco Mario Alberto
    AU  - Claros Ortiz Katherine Viviana
    AU  - Cano Arias Luis Guillermo
    AU  - Velasco Hoyos Maria Fernanda
    AU  - Solano Vega Irina Isabel
    AU  - Lizcano Aros Jose Alfredo
    AU  - Luna Tavera Rina Liliana
    Y1  - 2023/09/25
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    N1  - https://doi.org/10.11648/j.ejcbs.20230904.12
    DO  - 10.11648/j.ejcbs.20230904.12
    T2  - European Journal of Clinical and Biomedical Sciences
    JF  - European Journal of Clinical and Biomedical Sciences
    JO  - European Journal of Clinical and Biomedical Sciences
    SP  - 51
    EP  - 54
    PB  - Science Publishing Group
    SN  - 2575-5005
    UR  - https://doi.org/10.11648/j.ejcbs.20230904.12
    AB  - Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a rare condition, often associated with neoplasms and characterized by neuropsychiatric symptoms and dysautonomia. Affecting approximately 0.01% of cancer patients, it is the second most frequent autoimmune encephalitis after acute disseminated encephalomyelitis, its clinical presentation is usually acute and severe, so prompt identification and treatment improve prognosis. We present the case of a 23-year-old female patient from a rural area of Caquetá, Colombia, previously healthy, who presented persistent psychotic symptoms of two weeks of evolution and was initially treated in a psychiatric unit where she evolved with systemic compromise. Finally, a diagnosis of paraneoplastic anti-NDMAR encephalitis was made. The patient received IV immunoglobulin G plus adjuvant systemic management with chemotherapy. She had complete resolution of her neuropsychiatric disorder and was asymptomatic when discharged. In conclusion, anti-NMDAR encephalitis is a condition with an underlying autoimmune mechanism, with production of autoantibodies mainly against the NR1 and NR2 subunits of NMDA receptors in the brain. These autoantibodies are produced in response to a breakdown of immune tolerance usually secondary to a neoplastic process, mostly ovarian teratoma, or infectious as in the case of herpes viral encephalitis. Despite its severe and potentially fatal nature, AE typically exhibits a positive response to treatment if a prompt diagnosis is established and management is initiated. However, it is acknowledged in some texts that there is still a gap in recognizing this entity, which encourages future valuable research.
    VL  - 9
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Author Information
  • Neurophysiology Department, Hospital Universitario Hernando Moncaleano Perdomo, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Neurophysiology Department, Hospital Universitario Hernando Moncaleano Perdomo, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Neurophysiology Department, Hospital Universitario Hernando Moncaleano Perdomo, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Gynecologic Oncology Department, Hospital Universitario Hernando Moncaleano Perdomo, Neiva, Colombia

  • Internal Medicine Department, Universidad Surcolombiana, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Internal Medicine Department, Universidad Surcolombiana, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Internal Medicine Department, Universidad Surcolombiana, Investigation Group MI DNeuropsy, Neiva, Colombia

  • Department of Pathology, Hospital Universitario Hernando Moncaleano Perdomo, Neiva, Colombia

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