Anti-N-Methyl-D-Aspartate Receptor Encephalitis (Anti-NMDA Receptor Encephalitis)

Epidemiology

  • 80% of cases occur in females
  • Peak Age: 20’s

Associated Conditions

  • Ovarian Teratoma (see xxxx, [[xxxx]]): in females

Physiology

  • Autoantibody Against N-Methyl-D-Aspartate (NMDA) Receptor
    • NMDA Receptor Antagonists: ketamine, dextromethorphan, PCP, NO

Diagnosis

Head CT (see Head Computed Tomography, [[Head Computed Tomography]])

  • xxx

Brain MRI (see Brain Magnetic Resonance Imaging, [[Brain Magnetic Resonance Imaging]])

  • May Be Abnormal: although there is no specific site of involvement

Lumbar Puncture (LP) (see Lumbar Puncture, [[Lumbar Puncture]])

  • xxxx
  • CSF Anti-NMDA Antibody: diagnostic (only available from Mayo lab)

Electroencephalogram (EEG) (see Electroencephalogram, [[Electroencephalogram]])

  • xxxx

Serum Anti-NMDA Antibody

  • Diagnostic (If Present): only available from Mayo lab

Screening for Teratoma


Clinical Manifestations

Gastrointestinal Manifestations

  • Brief Gastrointestinal Illness: may precede the onset of symptoms

Neurologic Manifestations

  • Autonomic Symptoms
    • xxx
  • Prodromal Headache (see Headache, [[Headache]])
  • Delirium (see Delirium, [[Delirium]])
  • Opisthotonus (see Opisthotonus, [[Opisthotonus]])
  • Orofacial Dyskinesia
  • Psychiatric Symptoms
  • Seizures (see Seizures, [[Seizures]])
  • xxx

Treatment

Treatment of Seizures (see Seizures, [[Seizures]])

  • Standard Therapy

Immunosuppression

Corticosteroids (see Corticosteroids, [[Corticosteroids]])

  • First-Line Agent

Cyclophosphamide (Cytoxan) (see Cyclophosphamide, [[Cyclophosphamide]])

  • Second-Line Agent

Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin, [[Intravenous Immunoglobulin]])

  • First-Line Agent

Rituximab (Rituxan) (see Rituximab, [[Rituximab]])

  • Second-Line Agent

Plasma Exchange (see Plasmapheresis, [[Plasmapheresis]])

  • First-Line Therapy

Surgical Resection of Teratoma

  • If Present

Prognosis

  • Untreated: high mortality
  • Treated: xxx

References

  • Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008 Dec;7(12):1091–1098 [MEDLINE]
  • Challenges in providing critical care for patients with anti-N-methyl-D-aspartate receptor encephalitis. Chest. May 2014;145:1143–1147 [MEDLINE]