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, tachy/brady,BP fluctuations, hypoventilation
  • Lethargy, seizures

Physical

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

Differential Diagnosis

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

External Links

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. 2.0 2.1 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. 4.0 4.1 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. 6.0 6.1 6.2 6.3 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