Sanaz Ahmadi Karvigh, MD. Assistant professor of neurology, Epileptologist, Tehran University of Medical Sciences,

When convulsive status epilepticus (SE) does not respond to the initial therapy benzodiazepines and second-line therapy (antiepileptic IV Load), IV anesthetics should be applied without hesitation within the first two hours. If SE dose not respond the first two IV medications, it is called refractory SE which occurs in up to 40% of cases of SE and after the first 24 hours it is called super refractory SE. Often refractory SE has a non-convulsive form, a term previously known as “subtle SE”. Although, occasional clonic jerks or nystagmus and rapid fluctuations in pupillary response can help to identify ongoing SE in the clinic, most of the time the only method to diagnose the NCSE is by electroencephalography. Simultaneous investigation of the underlying etiology is crucial while patient is kept under deep levels of anestheia. An acute symptomatic etiology is the most likely cause. Management of refractory status epilepticus involves treatment of the underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. If no clear explanation is found, autoimmune processes could be involved that justify immunomodulatory treatment. The new definition of NORSE (new onset refractory epilepticus) includes a few previously described conditions that still have an unclear underlying etiology. Anakinra, which is an anti IL-1 drug, has shown promise in controlling super-refractory SE in NORSE patients. Short-term and long-term outcomes of patients with refractory SE tend to be poor with high morbidity and mortality with only one-third of patients reaching baseline neurological status.