Autoimmune encephalitis mediated by antibodies (AMAE) targeting cell-surface antigens

Includes but not limited to: 

  • Encephalitis associated with antibodies to: NMDA receptor, VGKC, LGI1, CASPR2, DPPX, AMPA receptor, glycine receptor, or GABA (A or B) receptor.
  • Highly suspected autoimmune encephalitis
  • Paraneoplastic encephalitis
  • Seronegative autoimmune encephalitis
  • Seronegative limbic encephalitis
  • Suspected autoimmune limbic encephalitis

(See separate entry for Rasmussen syndrome)

RecommendationsIVIG may be used as an option with expert consultation.
Dose/Frequency of AdministrationInduction: 2 g/kg adjusted body weight divided over 2 to 5 days.

Maintenance: 0.5 to 2 g/kg adjusted body weight monthly, if necessary.