An autoimmune etiology for seizures, epilepsy, and status epilepticus is becoming increasingly recognized. The role of autoimmunity in epilepsy has been highlighted in the literature and the International League Against Epilepsy now recognizes autoimmune epilepsy as a distinct entity. An appropriate and thorough work-up of all new-onset seizures and status epilepticus is paramount in determining the likely efficacy of immunotherapeutic agents in treating seizures and status epilepticus. Criteria for the clinical diagnosis of autoimmune mediated epilepsy and encephalitis have been published by expert consensus and validated models to predict response to immunotherapy exist. These guidelines should guide clinicians about when to promptly start immunotherapy. Immunotherapy has been shown to improve outcomes and may reduce relapse rates in autoimmune encephalitis. Treatment algorithms with immunotherapeutic agents have been established by expert opinion and multiple observational retrospective trials in the past 10 years. However, future prospective randomized controlled trials are still needed to better understand the optimal regimen, dosing schedule, and duration of treatment with immunotherapeutic agents.
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