Anti-NMDA Receptor Encephalitis — Immunotherapy — Clinical Pathway: Inpatient

Anti-NMDA Receptor Encephalitis Clinical Pathway — Inpatient

Immunotherapy

The decision regarding all treatment should be made in collaboration with the neuroimmunology attending.

Early diagnosis and aggressive treatment with immunotherapies are essential to optimizing outcomes. Treatment prior to laboratory confirmation may be considered in clinically consistent cases.

Appropriate therapies include high-dose IV steroids as 1st line and intravenous immunoglobulin (IVIG) or plasma exchange as second line. Currently, there are no clinical trials that evaluate these therapies in anti-NMDARE; thus, there is limited data to support a specific order of treatments. Secondary treatments may include rituximab and cyclophosphamide.

Medications

  • First Line
    • IV Methylprednisolone
      • 30 mg/kg IV for 3-5 days
      • Maximum: 1,000 mg
      • If first dose given in the evening, consider adjustment of subsequent administration times to improve sleep/wake cycle
  • Second Line
  • Third Line
    • Rituximab
      • 750 mg/m2 IV every 2 wks for 2 doses
      • Maximum: 1,000 mg
      • Review vaccination and TB status for all patients
      • Consider pre-rituximab screening labs if undergoing plasmapheresis. Be sure to administer a second dose of rituximab after completing plasmapheresis.
      • Premedicate with acetaminophen, diphenhydramine, +/- methylprednisolone
      • Genentech Patient Assistance Program is available for outpatients with an insurance barrier
  • Fourth Line
    • Cyclophosphamide
      • 750 mg/m2 IV once — Further doses to be considered on a case-by-case basis
      • Maximum: 1,500 mg
      • Consider Rheumatology consult
      • Administer fluids, ondansetron, mesna, and cyclophosphamide per formulary guidance
      • Obtain CBC with differential on days 7, 10, and prior to next infusion if given
      • Ensure information regarding potential effect on fertility and preservation options is discussed with child and/or family