Treatment of Paraneoplastic Cerebellar Degeneration When Corticosteroids Have Not Controlled Brain Inflammation
This protocol applies to patients with rapidly progressive cerebellar syndrome in whom antineuronal antibodies against cell-surface antigens have been detected, and in whom first-line therapy has not achieved the required treatment goals.
Clinical scenario
Rapidly progressive cerebellar syndrome with detected antineuronal antibodies directed against cell-surface antigens. The antibody subtype — here, antibodies directed against cell-surface antigens rather than intracellular antigens — determines the applicable escalation strategy.
First-line failure condition
This protocol is indicated when first-line treatment — early management of the underlying tumor alongside corticosteroids (intravenous methylprednisolone, with or without a subsequent oral prednisolone taper) — has not achieved adequate reduction of brain inflammation and circulating antibody levels.
Next-line approach (partial overview)
Escalation targets the reduction of circulating autoantibodies through an intravenous approach; the complete options, criteria, and sequencing are contained in the full protocol.
References
DOI: 10.3390/brainsci11111414
- If a specific antibody has been detected, one can distinguish between antibodies directed against intracellular antigens and antibodies directed against cell-surface antigens.
- Concepts targeting B-cells alone are primarily used if antibodies against cell-surface antigens have been detected.
- Reduction of circulating autoantibodies can also be addressed by intravenous immunoglobulins (IVIG) or plasma exchange (PLEX).
- In case of missing effect of monotherapy with corticosteroids, IVIG or PLEX should be initiated.