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. Author manuscript; available in PMC: 2013 Jul 9.
Published in final edited form as: Curr Treat Options Neurol. 2011 Jun;13(3):324–332. doi: 10.1007/s11940-011-0116-y

Table 1.

General treatment approach to patients with synaptic autoimmunities

  1. Search for and remove a tumor as appropriate for syndrome (e.g., teratoma in anti–NMDA-receptor encephalitis, systemic cancer in anti–AMPA-receptor encephalitis).a

  2. After tumor removal or if no tumor is found, proceed with 5-day course of concurrent IVIg and methylprednisolone.

  3. If clear improvement is seen within 10 days, continue with supportive care.

  4. If there is no response or limited response after 1 cycle of immunosuppression, initiate cyclophosphamide (monthly) and rituximab (weekly for 4 weeks starting with the first dose of cyclophosphamide).

  5. For patients with limited or no response to the approaches above, consider other forms of immunosuppression.b

  6. For patients without tumors, continue immunosuppression with mycophenolate mofetil or azathioprine for at least 1 year after initial treatments are discontinued (to reduce the high rate of relapses after recovery).

a

Although some surgeons are hesitant to take these patients to surgery (especially those requiring intensive care, including ventilator support), tumor removal is critical in decreasing time to recovery and increasing the chance of more complete recovery

b

Single cases of responses to methotrexate have been reported [23]

(Based on series of patients with anti–NMDA-receptor encephalitis, especially Florance et al. [8••, Class III])