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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Treatment and Outcome

Autoimmune encephalitis is often associated with a favorable outcome after tumor removal and antineoplastic treatment (if applicable), as well as immunotherapy. In general, steroids, intravenous immunoglobulin, and plasmapheresis are the first line of immunotherapy especially when a tumor is detected and treated [9, 39]. Rituximab and cyclophosphamide comprise the second-line immunotherapy when the first-line treatment fails. Although seizures must be addressed aggressively during the acute phase of the disease, patients often do not require long-term antiepileptic medication.

In a large multiinstitutional observational study, over 500 patients with anti- NMDA receptor encephalitis were treated and monitored up to 2 years. Out of 501 patients, 94% received first-line immunotherapy (steroids, intravenous immunoglobulin, plasmapheresis) or tumor removal, resulting in improvement within

4 weeks in 53% of patients. More than half of patients who failed first-line therapy received second-line immunotherapy (rituximab, cyclophosphamide), resulting in better outcome than those who did not. During the first 24 months, almost 80% of patients reached a good outcome, where relapses occurred in approximately 12% of the patients. About 6% of patients died [39].

Predictors of poor outcome in anti-NMDA receptor encephalitis are a delay in diagnosis and treatment, the need for intensive care, high titer of antibody in CSF and serum, and the presence of teratoma [39, 74]. The overall prognosis for patients with autoimmune encephalitis is variable. Some patients have a complete recovery, while others die or develop a permanent neurologic disability.

 
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