Neurocritical Care

, Volume 18, Issue 1, pp 89–92 | Cite as

Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Favorable Outcome Despite Prolonged Status Epilepticus

  • Xavier Finné Lenoir
  • Christian Sindic
  • Vincent van Pesch
  • Souraya El Sankari
  • Marianne de Tourtchaninoff
  • Roger Denays
  • Philippe HantsonEmail author
Practical Pearl



To describe a case of auto-immune encephalitis in an adolescent with favorable outcome despite prolonged status epilepticus.


A 17 year old Asian man without previous medical history developed alteration of consciousness and partial seizures. The diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was confirmed by the detection of specific antibodies in both cerebrospinal fluid and serum.


The clinical course was complicated by prolonged status epilepticus which was refractory to a large number of antiepileptic drugs, including barbiturate coma. Immunomodulatory therapy included steroids, plasma exchanges, and intravenous immunoglobulins. After 86 days of intensive therapy, the patient regained consciousness progressively. Brain magnetic resonance imaging never demonstrated any lesion. Extensive search for a tumor was negative. At 12 month follow-up, the patient had made an excellent recovery.


Auto-immune encephalitis is likely underdiagnosed in adolescents. In their most severe presentation, seizures may be resistant to a large number of anti-epileptic drugs, and the clinical improvement seems to be mainly because of the immunomodulatory therapy. Relapse is possible, as well as the delayed development of a teratoma or other tumor.


Encephalitis Anti-NMDA receptor antibodies Status epilepticus Seizure management Ketamine Immunosuppressive therapy 

Supplementary material

Recording of orofacial dyskinesias and stereotypic movements of the limbs present between day 34 and 40. (MP4 10182 kb)


  1. 1.
    Florance NR, Davis RL, Lam C, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009;66:11–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091–8.PubMedCrossRefGoogle Scholar
  3. 3.
    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.PubMedCrossRefGoogle Scholar
  4. 4.
    Niehusmann P, Dalmau J, Rudlowski C, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol. 2009;66:458–64.PubMedCrossRefGoogle Scholar
  5. 5.
    Dalmau J, Tüzün E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25–36.PubMedCrossRefGoogle Scholar
  6. 6.
    Poloni C, Korff CM, Ricotti V, et al. Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-D-aspartate receptor encephalitis. Dev Med Child Neurol. 2009;52:e78–82.PubMedCrossRefGoogle Scholar
  7. 7.
    De Nayer AR, Mylant N, Sindic CJM. A subacute behavioral disorder in a female adolescent: autoimmune anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Biol Psychiatry. 2009;66:e13–4.PubMedCrossRefGoogle Scholar
  8. 8.
    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
  9. 9.
    Smith JH, Dhamija R, Moseley BD, et al. N-methyl-D-aspartate receptor autoimmune encephalitis presenting with opsoclonus-myoclonus: treatment response to plasmapheresis. Arch Neurol. 2011;68:1069–72.PubMedCrossRefGoogle Scholar
  10. 10.
    Prüss H, Holtkamp M. Ketamine successfully terminates malignant status epilepticus. Epilepsy Res. 2008;82:219–22.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Xavier Finné Lenoir
    • 1
  • Christian Sindic
    • 2
    • 3
  • Vincent van Pesch
    • 2
    • 3
  • Souraya El Sankari
    • 2
  • Marianne de Tourtchaninoff
    • 2
  • Roger Denays
    • 4
  • Philippe Hantson
    • 1
    Email author
  1. 1.Department of Intensive Care, Cliniques St-LucUniversité catholique de LouvainBrusselsBelgium
  2. 2.Department of Neurology, Cliniques St-LucUniversité catholique de LouvainBrusselsBelgium
  3. 3.Laboratory of Neurochemistry, Cliniques St-LucUniversité catholique de LouvainBrusselsBelgium
  4. 4.Department of NeurologyCentre Hospitalier Régional de NamurNamurBelgium

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