This protocol addresses children aged under 18 years diagnosed with NMDA receptor antibody encephalitis who present with severe disease — a classification defined by features that directly compromise patient safety or functional status.
Severe disease is defined by issues affecting safety and function. At least one of the following severity markers must be present:
Management involves first-line immunotherapy built around IV corticosteroid therapy combined with additional immunotherapy components selected on the basis of disease severity — the specific combination, sequencing, and decision criteria are defined in the full structured protocol.
The primary measure of early response is significant functional improvement, assessed approximately one week after initiating corticosteroid therapy. Failure to achieve meaningful functional gains defines treatment failure and signals the need for escalation.
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.
IV corticosteroids should be the first immunotherapy used (i.e., IV methylprednisolone, IVMP). If IV corticosteroids are not available or are contraindicated, oral corticosteroids (prednisone, OP or dexamethasone, DEX) should be used.
During the clinical course, a “failure to improve” (required to determine need for escalation of therapy) is defined as failure to achieve significant gains in function.
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