Anti-NMDA-receptor encephalitis
ICD-10 G04.8 · ICD-11 8E4A.0.3

Treatment of NMDA Receptor Encephalitis Relapse in Children When Rituximab or Cyclophosphamide Has Not Worked

Paediatric <18 years · Relapse · Refractory

This protocol applies to children under 18 years of age who experience a relapse of anti-NMDA-receptor antibody encephalitis and who have not achieved adequate improvement following treatment with second-line immunotherapy. Relapse is defined as the return of previously resolved symptoms and signs, or the appearance of new symptoms or signs associated with a change in function.

When does this protocol apply?

The previous step in management involved a second-line immunosuppressive regimen — either rituximab or cyclophosphamide (or both, used sequentially). After that treatment, adequate clinical improvement was expected within approximately 1–3 months.

Escalation is indicated when adequate improvement has not been achieved approximately 1–3 months after treatment with rituximab and/or cyclophosphamide. This protocol describes the next step for those most refractory cases.

Next-line approach

For children with NMDA-receptor antibody encephalitis relapse who remain refractory after the preceding second-line regimen, escalation to IV tocilizumab is considered. This is reserved for the most refractory patients and involves a sustained course of treatment.

The full eligibility criteria, weight-based dosing schedule, treatment duration, and monitoring guidance are available in the complete structured regimen below.

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 with NMDARE relapse, escalation to IV tocilizumab should be considered only in the most refractory patients who fail to improve adequately after about 1–3 mo of treatment with RTX and/or CYC.

12 mg/kg/dose (<30 kg), 8 mg/kg/dose (≥30 kg) (max 800 mg) given monthly over 6 mo or more (duration of required immunosuppression)

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