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Immunomodulatory therapy for autoimmune encephalitis

Immunomodulatory therapy for autoimmune encephalitis
Autoimmune encephalitis is a heterogeneous entity that includes paraneoplastic, idiopathic, drug-induced, and postviral causes. In addition to immunomodulatory therapy, potential triggers of autoimmunity should be treated promptly. This includes treatment of the associated tumor in paraneoplastic cases.

NMDAR: N-methyl-D-aspartate receptor; IVMP: intravenous methylprednisolone; IVIG: intravenous immune globulin; TPE: therapeutic plasma exchange; LGI1: leucine-rich glioma inactivated protein 1; CASPR2: contactin-associated protein-like 2.

* Examples of antibody-mediated encephalitides include those associated with antibodies against neuronal cell-surface antigens, such as anti-NMDAR, anti-LGI1, and anti-CASPR2. Examples of T-cell mediated encephalitides include paraneoplastic cases associated with high-risk onconeuronal antibodies.

¶ IVMP dose is for adults. For children, use 20 to 30 mg/kg once daily (maximum 1000 mg per day) for 3 to 5 days.

Δ The choice between IVIG and TPE is individualized and may vary by center. IVIG is often favored because it is more readily available and does not require a central line. TPE is an alternative to IVIG when there are relative contraindications to IVIG, such as high thromboembolic risk or severe hyponatremia.

◊ Before each escalation step, it is important to review the diagnosis and reconsider alternative diagnoses.

§ In severe cases, cyclophosphamide may be started at the same time as rituximab.
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