Treatment of IGLON5 Antibody-Associated Encephalitis
Initial Treatment Protocol
- The American Academy of Neurology recommends initiating first-line immunotherapy with high-dose intravenous methylprednisolone (1g daily for 3-5 days) combined with IVIG immediately after excluding infection, as IGLON5 disease responds to aggressive early immunotherapy and has high mortality without treatment 1
- The European Academy of Neurology suggests screening for malignancy with CT chest/abdomen/pelvis, as paraneoplastic associations can occur 1
Second-Line Therapy for Inadequate Response
- The American Academy of Neurology recommends adding rituximab (375 mg/m² weekly for 4 weeks) as the preferred second-line agent for antibody-mediated autoimmunity 1, 2
- The European Academy of Neurology suggests considering plasma exchange (5-10 sessions every other day) as an alternative or additional therapy, particularly if there is severe presentation or contraindications to other treatments 1, 2
- Cyclophosphamide may be considered if there is evidence of cell-mediated inflammation, though rituximab is generally preferred 2