Anti-NMDA receptor encephalitis

Background

Anti-NMDA receptor encephalitis is an under-recognized neurologic described disorder described in 2007 due to antibodies to the NMDA receptor and is often associated with GYN tumors (most commonly ovarian teratoma)[1]

Clinical Features

History

  • Female predominance (up to 80-90%)
  • Predominantly in children and young adults, however can be found at any age[2][3]
  • Viral like prodrome (headache, low-grade fever, malaise)
  • Psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, etc) and/or decreased level of consciousness [2]
  • Dyskinesia, movement disorders and increased rigidity
  • Autonomic instability: hyperthermia, tachycardia/bradycardia, BP fluctuations, hypoventilation
  • Lethargy, seizures

Physical

  • Abnormality in vitals as above, rarely may find abdominal mass

Differential Diagnosis

Movement Disorders and Other Abnormal Contractions

Altered mental status

Diffuse brain dysfunction

Primary CNS disease or trauma

Psychiatric

Evaluation

  • Diagnosis confirmed by detection of antibodies to NR1 subunit of NMDAR in CSF or serum (typically send-out lab)
  • LP: CSF lymphocytic pleocytosis or oligoclonal bands (can be normal initially)
  • EEG: to rule out seizure with movement disorders
  • MRI brain: normal or transient FLAIR or contrast enhancing abnormalities in cortical or subcortical regions
  • Pelvic ultrasound or CT or MRI to evaluate for associated ovarian teratoma

Management

Disposition

  • Admission with neurology consult

See Also

References

  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; Dec;7(12); 191-8. PMID: 18851928
  2. Dalmau J, Lancaster E, Martinez-Hernandez E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011 Jan;10(1):63-47. PMID: 21163445
  3. Armangue T, Petit-Pedrol M, Dalmau J Autoimmune Encephalitis in Children. J Child Neurol. 2012 Nov;27(11):1460-9. PMID: 2293555
  4. Wandinger K, Saschenbrecker S, Stoecker W, Dalmau J Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol. 2011 Feb;231(1-2):86-91. PMID: 20951441
  5. Punja M, Pomerleau JJ, Devlin MW, et al. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: an etiology worth considering in the differential diagnosis of delirium. Clin Toxicol 2013 Sep-Oct;51:794-7. PMID: 23962100
  6. Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013 Feb;12(2):157-65. PMID: 23290630
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