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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

Abstract

Background

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

Methods

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.

Results

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.

Conclusion

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.

Keywords

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)

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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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