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Status epilepticus that persists despite at least 2 standard anticonvulsant medications is termed refractory status epilepticus (RSE). New-onset refractory status epilepticus (NORSE) is defined as a condition, not a specific diagnosis, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause in a patient without active epilepsy. Most of the common causes of RSE can be identified within 24-72 hours of presentation. In up to half of the cases of NORSE, a possible or probable cause is ultimately found, most often autoimmune or paraneoplastic encephalitis, with infectious causes less common. Identifying patients with an underlying autoimmune origin is critical because these patientschr('39') condition may remain refractory to conventional antiseizure medications but may respond to immunotherapy.
 
The most common causes of NORSE and FIRES are autoimmune/paraneoplastic disorders, such as encephalitis associated with anti-neuronal antibodies (anti-NMDA receptor, anti-voltage-gated potassium channel complex, etc.), followed by viral encephalitis. When an underlying cause is identified it should be appropriately treated.it is common to use approaches that modulate the immune system. These options include IV steroids, IV immunoglobulins, plasma exchange therapy (plasmapheresis) and some monoclonal antibodies against inflammatory cells (e.g., rituximab).
Conclusion: Status Epilepticus is an epilepsy emergency and neurologists try to treat it emergency to reduce the morbidity and mortality of this grave disease recently one of the refractory causes of status epilepticus is autoimmune encephalitis and Response to Immunotherapy has been reported satisfactory in some cases. But more investigation must be performed.

Keywords: epilepsy, antiseizure, Immunotherapy

     

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