Anti-NMDA-receptor encephalitis
ICD-10 G04.8 · ICD-11 8E4A.0.3
Pediatric — Age <18 years · Relapse

Treatment of Anti-NMDA-receptor Encephalitis Relapse in Children Under 18 Years

This protocol addresses management at relapse of NMDA receptor antibody encephalitis in patients younger than 18 years — a clinical situation that calls for a distinct treatment approach beyond the initial line of therapy.

Clinical Scenario

Relapse is defined as the return of previously resolved symptoms and signs, or the appearance of new symptoms or signs associated with change in function — lasting more than 1 week (or a shorter period if associated with a change in safety) — that cannot be explained by adverse reactions to current medications or intercurrent illness, following a period of stability or improvement of at least 1 month.

Treatment Direction

At relapse in pediatric patients, escalation to second-line immunotherapy and/or maintenance immunotherapy becomes a central consideration, typically initiated within weeks of first-line treatment — the specific agents, criteria, and sequencing are set out in the full protocol.

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References

DOI: 10.1212/NXI.0000000000001052

Return of previous resolved symptoms and signs, or appearance of new symptoms or signs, associated with change in function, lasting more than 1 wk (or shorter if associated with change in safety), that cannot be explained by adverse reactions to current medications or intercurrent illness, after a period of stability or improvement of at least 1 mo.

In patients who relapse, second-line immunotherapy and/or maintenance (>6 mo) immunotherapy (mycophenolate mofetil or rituximab [RTX] redosing) should be considered, started within median 2 wk after initiation of first-line treatment (range 1–3 wk).

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