Journal of General Internal Medicine

, Volume 26, Issue 7, pp 811–816 | Cite as

Anti-NMDA-Receptor Encephalitis: Case Report and Literature Review of an Under-Recognized Condition

  • Gregory S. Day
  • Sasha M. High
  • Bianca Cot
  • David F. Tang-Wai
Clinical Practice: Clinical Vignettes

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis is an immune-mediated syndrome that remains under-recognized despite a growing body of literature. This syndrome has been predominantly described in young females with a constellation of symptoms, including personality changes, autonomic dysfunction and neurologic decompensation. It is commonly associated with mature ovarian teratomas. We describe the classic presentation of anti-NMDA-R encephalitis in three dramatically different patients: Case A, a young woman with ovarian teratoma; Case B, the eldest case reported to date; and Case C, a young male with no identifiable tumor. We review the literature summarizing the differential diagnosis, investigative approach, treatment options and challenges inherent to this disorder. We advocate good supportive care, involvement of multiple health disciplines and use of immune-modulating therapies in patient management. These cases underscore the need for increased awareness and high diagnostic suspicion when approaching the patient with suspected viral encephalitis.

KEY WORDS

encephalitis NMDA-receptor teratoma autoimmune 

References

  1. 1.
    Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-98.PubMedCrossRefGoogle Scholar
  2. 2.
    Ishiura H, Matsuda S, Higashihara M, et al. Response of anti-NMDA receptor encephalitis without tumor to immunotherapy including Rituximab. Neurology 2008;71:1921-23.PubMedCrossRefGoogle Scholar
  3. 3.
    Gable MS, Gavali S, Radner A, et al. Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis. Eur J Clin Microbiol Infect Dis 2009;28:1421-29.PubMedCrossRefGoogle Scholar
  4. 4.
    Folstein MF, Folstein SE, McHugh PR. “Mini-mental state”: a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975:12:189-98.PubMedCrossRefGoogle Scholar
  5. 5.
    van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke 1988:19:604-07PubMedGoogle Scholar
  6. 6.
    Dalmau J, Tüzün E, Wu H, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007;61:25–36.PubMedCrossRefGoogle Scholar
  7. 7.
    Lynch DR, Anegawa NJ, Verdoorn T, Pritchett DB. N-methyl-D-aspartate receptors: different subunit requirements for binding of glutamate antagonists, glycine antagonists, and channel-blocking agents. Mol Pharmacol 1994;45:540–45.PubMedGoogle Scholar
  8. 8.
    Tüzün E, Zhou L, Baehring JM, Bannykh S, Rosenfeld MR, Dalmau J. Evidence for antibody-mediated pathogenesis in anti-NMDAR encephalitis associated with ovarian teratoma. Acta Neuropathol 2009;118:737-43PubMedCrossRefGoogle Scholar
  9. 9.
    Davies G, Irani SR, Coltart C, et al. Anti-N-methyl-D-aspartate receptor antibodies: A potentially treatable cause of encephalitis in the intensive care unit. Crit Care Med 2010;38:679-82.PubMedCrossRefGoogle Scholar
  10. 10.
    Prüss H, Dalmau J, Harms L, et al. Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin. Neurology:75:1735-39.Google Scholar
  11. 11.
    Wandinger KP, Saschenbrecker S, Stoecker W, Dalmau J. Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol 2010; Epub ahead of print: Oct 14Google Scholar
  12. 12.
    Iizuka T, Sakai F, Ide T, et al. Anti-NMDA receptor encephalitis in Japan: Long-term outcome without tumor removal. Neurology 2008;70(7):504-11.PubMedCrossRefGoogle Scholar

Copyright information

© Society of General Internal Medicine 2011

Authors and Affiliations

  • Gregory S. Day
    • 1
    • 3
  • Sasha M. High
    • 2
    • 3
  • Bianca Cot
    • 2
    • 3
  • David F. Tang-Wai
    • 1
    • 3
  1. 1.Department of Medicine, Division of NeurologyUniversity of TorontoToronto, OntarioCanada
  2. 2.Department of Medicine, Division of Internal MedicineUniversity of TorontoToronto, OntarioCanada
  3. 3.University Health Network - Mount Sinai HospitalToronto, OntarioCanada

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