This protocol applies to children under 18 years of age with standard (non-severe) NMDA receptor antibody encephalitis whose initial immunotherapy has failed to deliver meaningful functional gains within the expected window.
The patient is a child (age <18 years) with NMDA receptor antibody encephalitis of standard disease severity. Severe disease is defined by issues affecting safety and function; this protocol requires confirmation that none of the severe safety and function markers are present.
First-line immunotherapy was started: corticosteroids alongside therapeutic plasma exchange (TPE), IV immunoglobulin (IVIg), or both. Escalation to this protocol is triggered when significant gains in function are not achieved approximately 2 weeks after initiation of two or more first-line therapies.
A targeted second-line immunotherapy agent is the next step — with a defined alternative if the preferred agent is contraindicated or unavailable. Full dosing, sequencing, and monitoring details are in the complete protocol.
Adequate improvement, assessed 1–3 months following initiation of the first second-line immunotherapy.
DOI: 10.1212/NXI.0000000000001052
Severe is defined by issues affecting SAFETY and FUNCTION. Any (≥1) of the severity markers present below (items a–i) scoring “severe” rather than “standard” would classify as severe disease.
In patients who are failing to improve approximately 2 wk after initiation of 2 or more first-line therapies, second-line therapy is favored over further first-line therapies.
Rituximab (RTX) is generally the second-line therapy of choice. Cyclophosphamide (CYC) may be considered if RTX is contraindicated or not available.
Another second-line therapy (i.e., CYC, if RTX was used first, or vice versa) can be used in any patient with severe disease who fails to improve adequately 1–3 mo following initiation of the first second-line immunotherapy.
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