This protocol applies to children (age <18 years) with NMDA-receptor antibody encephalitis of standard (non-severe) disease severity whose condition has not responded adequately to second-line therapy. It defines the next clinical step for this specific group.
Standard (non-severe) NMDA-receptor antibody encephalitis in a patient under 18. Severity is determined by the presence of markers affecting safety and function; none of those markers are present in this scenario. Any one or more of such markers would reclassify the case as severe.
The prior treatment step involved cyclophosphamide (if rituximab was used first, or vice versa) and/or escalation to IV tocilizumab in the most refractory patients.
Escalation to this protocol is indicated when adequate improvement has not been achieved, as assessed approximately 1–3 months after treatment with rituximab and/or cyclophosphamide.
When the above failure condition is met, the protocol calls for maintenance immune suppression extending beyond six months. Specific agents, criteria for their use, and monitoring requirements are detailed in the full protocol — not shown here.
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.
Maintenance (>6 mo) immune suppression can be considered in any patient who fails to improve adequately despite conventional second-line or escalation therapy.
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