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Part of the book series: Neuropsychiatric Symptoms of Neurological Disease ((NSND))

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Abstract

Autoimmune encephalitis associated with antibodies targeting neural cell surface antigens have emerged in the past 10 years as a major cause of encephalitis. Those associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) is the most frequent. This newly recognized disease is characterized by a stereotyped clinical phenotype. The clinical course usually begins in the majority of cases by psychiatric symptoms, sometimes preceded by prodromal symptoms. The psychiatric stage is usually followed by severe fluctuations in consciousness with neurologic involvement with cognitive impairment, speech impairment, movement disorders, seizures, and behavioral problems. Typically the disease affects young women, and an ovarian teratoma is frequently associated, but cases have been reported in children, in men, and in patients without tumors. The treatment consists of early immunotherapy and, if necessary, tumor removal. The outcome is good in many cases if the treatment is started early, but severe sequelae or death is possible. The management of psychiatric symptoms can be difficult.

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References

  1. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1135–40.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  2. Honnorat J, Cartalat-Carel S, Ricard D, Camdessanche JP, Carpentier AF, Rogemond V, et al. Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry. 2009;80:412–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Graus F, Saiz A, Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol. 2010;257:509–17.

    Article  CAS  PubMed  Google Scholar 

  4. Zuliani L, Graus F, Giometto B, Bien C, Vincent A. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry. 2012;83:638–45.

    Article  PubMed Central  PubMed  Google Scholar 

  5. Coutinho E, Harrison P, Vincent A. Do neuronal autoantibodies cause psychosis? A neuroimmunological perspective. Biol Psychiatry. 2014;75:269–75.

    Article  CAS  PubMed  Google Scholar 

  6. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000;123:1481–94.

    Article  PubMed  Google Scholar 

  7. Dalmau J, Tüzün 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;61:25–36.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. 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;10:63–74.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Kayser MS, Dalmau J. Anti-NMDA receptor encephalitis in psychiatry. Curr Psychiatry Rev. 2011;7:189–93.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  10. Kayser MS, Dalmau J. The emerging link between autoimmune disorders and neuropsychiatric disease. J Neuropsychiatry Clin Neurosci. 2011;23:90–7.

    Article  PubMed Central  PubMed  Google Scholar 

  11. Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain. 2010;133:2734–48.

    Article  PubMed Central  PubMed  Google Scholar 

  12. 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. Clin Infect Dis. 2012;54:899–904.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  13. Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, Jackson M, et al. Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol. 2001;50:73–8.

    Article  CAS  PubMed  Google Scholar 

  14. Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9:776–85.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  15. Takahashi Y, Mori H, Mishina M, Watanabe M, Fujiwara T, Shimomura J, et al. Autoantibodies to NMDA receptor in patients with chronic forms of epilepsia partialis continua. Neurology. 2003;61:891–6.

    Article  CAS  PubMed  Google Scholar 

  16. Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol. 2005;58:594–604.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Ances BM, Vitaliani R, Taylor RA, Liebeskind DS, Voloschin A, Houghton DJ, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain. 2005;128:1764–77.

    Article  PubMed Central  PubMed  Google Scholar 

  18. Dalmau J, Tüzün 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;61:25–36.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  19. 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;10:63–74.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  20. Miya K, Takahashi Y, Mori H. Anti-NMDAR autoimmune encephalitis. Brain Dev. 2014;36:645–52.

    Article  PubMed  Google Scholar 

  21. Rosenthal-Simons A, Durrant AR, Heresco-Levy U. Autoimmune-induced glutamatergic receptor dysfunctions: conceptual and psychiatric practice implications. Eur Neuropsychopharmacol. 2013;23:1659–71.

    Article  CAS  PubMed  Google Scholar 

  22. Farber NB. The NMDA receptor hypofunction model of psychosis. Ann N Y Acad Sci. 2003;1003:119–30.

    Article  CAS  PubMed  Google Scholar 

  23. Gruber O, Chadha Santuccione A, Aach H. Magnetic resonance imaging in studying schizophrenia, negative symptoms, and the glutamate system. Front Psychiatry. 2014;5:32.

    Article  PubMed Central  PubMed  Google Scholar 

  24. 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.

    Article  PubMed  Google Scholar 

  25. Granerod J, Ambrose HE, Davies NW, Clewley JP, Walsh AL, Morgan D, et al. Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis. 2010;10:835–44.

    Article  PubMed  Google Scholar 

  26. 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. Clin Infect Dis. 2012;54:899–904.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  27. Sansing LH, Tüzün E, Ko MW, Baccon J, Lynch DR, Dalmau J. A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol. 2007;3:291–6.

    Article  PubMed Central  PubMed  Google Scholar 

  28. 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;70:504–11.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. 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;7:1091–8.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  30. Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009;66:11–8.

    Article  PubMed Central  PubMed  Google Scholar 

  31. Niehusmann P, Dalmau J, Rudlowski C, Vincent A, Elger CE, Rossi JE, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol. 2009;66:458–64.

    Article  PubMed  Google Scholar 

  32. Rubio-Agustí I, Dalmau J, Sevilla T, Burgal M, Beltrán E, Bataller L. Isolated hemidystonia associated with NMDA receptor antibodies. Mov Disord. 2011;26:351–2.

    Article  PubMed  Google Scholar 

  33. Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol. 2014;13:167–77.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

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Correspondence to Bruno Brochet MD .

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Brochet, B. (2015). Psychiatric Presentation of Brain Inflammation. In: Brochet, B. (eds) Neuropsychiatric Symptoms of Inflammatory Demyelinating Diseases. Neuropsychiatric Symptoms of Neurological Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-18464-7_7

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  • DOI: https://doi.org/10.1007/978-3-319-18464-7_7

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-18463-0

  • Online ISBN: 978-3-319-18464-7

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