This protocol addresses children (age <18 years) with confirmed NMDA receptor antibody encephalitis of standard (non-severe) severity whose functional recovery fell short of the expected threshold approximately one week after corticosteroid initiation.
Children under 18 years of age with NMDA receptor antibody encephalitis, standard (non-severe) disease severity — none of the severe safety and function markers are present. Severity is classified by the presence or absence of markers affecting safety and function.
The prior treatment line centred on first-line immunotherapy with IV corticosteroids (with optional IV immunoglobulin add-on). The trigger for escalating to this protocol is failure to achieve significant gains in function (clinical improvement) at approximately 1 week after corticosteroid initiation.
This protocol calls for adding a further first-line immunotherapy alongside ongoing corticosteroids. One approach in this step involves plasma exchange therapy. The full protocol specifies agent selection, any sequencing requirements, and the criteria for choosing among options — none of those details are presented 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.
In a child who has only received one first-line immunotherapy and who has severe disease or is failing to improve after 1 wk postinitiation of corticosteroids, another first-line immunotherapy (i.e., corticosteroids + TPE or corticosteroids + IVIg) should be considered.
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.
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