From the Guidelines
The treatment for autoimmune encephalitis typically involves immunotherapy to suppress the abnormal immune response attacking the brain, with first-line treatments including high-dose corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchange, as recommended by the most recent guidelines 1. The choice of first-line therapy may depend on the severity of the presentation and the specific antibody involved, with corticosteroids alone or combined with other agents being the most popular choice among clinicians 2.
- High-dose corticosteroids, such as methylprednisolone 1000mg IV daily for 3-5 days, are often used as the initial treatment.
- IVIG at 2g/kg divided over 2-5 days and plasma exchange (5-7 exchanges over 10-14 days) may be used alone or in combination with corticosteroids, depending on the severity of the disease. If patients don't respond adequately within 2-4 weeks, second-line therapies are considered, including:
- Rituximab (375mg/m² weekly for 4 weeks or 1000mg given twice, two weeks apart) for known or highly suspected antibody-mediated autoimmunity, such as NMDAR-antibody encephalitis 1.
- Cyclophosphamide (750mg/m² monthly for 3-6 months) for known or highly suspected cell-mediated autoimmunity, such as classical paraneoplastic syndrome 1. Supportive care is essential, including:
- Seizure management with antiepileptic drugs.
- ICU care if needed.
- Treating underlying tumors if the encephalitis is paraneoplastic. Recovery can take months to years, and some patients require long-term immunosuppression with agents like mycophenolate mofetil or azathioprine to prevent relapse, as suggested by recent studies 1, 3. Early treatment is crucial for better outcomes, as it helps limit immune-mediated neuronal damage and preserves brain function, highlighting the importance of prompt diagnosis and initiation of therapy 4, 5.
From the Research
Treatment Overview
The treatment for autoimmune encephalitis typically involves immunotherapy, with the goal of reducing inflammation and modulating the immune system.
- Conventional treatments include corticosteroids, intravenous immunoglobulin (IVIg), and/or therapeutic plasma exchange (TPE) 6.
- Earlier administration of immunotherapies in tumor-negative patients may facilitate faster recoveries 6.
- The choice of treatment may depend on the specific subtype of autoimmune encephalitis, as well as the individual patient's response to treatment.
Treatment Options
Some common treatment options for autoimmune encephalitis include:
- Corticosteroids, such as oral prednisone 7
- Intravenous immunoglobulin (IVIg) 6, 7
- Therapeutic plasma exchange (TPE) 6, 7
- Rituximab, a monoclonal antibody that targets B cells 7, 8
- Other immunosuppressive medications, such as azathioprine or mycophenolate mofetil 8
Emerging Therapies
There are several emerging therapies for autoimmune encephalitis, including:
- Targeted therapies that specifically target the underlying autoantibodies or immune cells involved in the disease 8
- New immunomodulatory therapies, such as interleukin-2 (IL-2) or interleukin-6 (IL-6) inhibitors 8
- Ongoing clinical trials are investigating the safety and efficacy of these new therapies 8
Management Recommendations
Proposed recommendations for symptomatic and long-term management of autoimmune encephalitis include:
- Bridging therapy with oral prednisone taper or other immunosuppressive medications 7
- Maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies or seronegative autoimmune encephalitis 7
- Cancer screening for 4 years in patients with neuronal surface antibodies or limbic encephalitis 7