The New-Onset Refractory Status Epilepticus (NORSE) is a rare entity that is defined as a clinical presentation, in a patient without active epilepsy or other relevant neurological disorder, who presents a refractory status epilepticus (RSE) without an acute or active structural, toxic, or metabolic cause. NORSE constitutes a diagnostic challenge that implies high morbidity and mortality in the short and long term. Clinically, NORSE is characterized by a prodromal phase, an acute phase, where seizures with diverse semiology that evolve to an RSE occur, and a chronic phase, with neurological deterioration and drug-resistant epilepsy. After an extensive work-up, the cause of NORSE is identified in only half of the cases. The initial treatment is based on the use of conventional anti-seizure drugs, requiring most of patients the use of anesthetics and mechanical ventilation. Better results have been observed with immunotherapy, more recently with anti-cytokine drugs, such as tocilizumab and anakinra, consistent with the immunological/inflammatory mechanisms proposed for the pathophysiology of NORSE.
One of the most complex situations that neurologists face is brain death. It is a painful state for the patient’s family, but by the other hand, it constitutes the hope for patients waiting for organ donation. Therefore it is essential to have a clear concept to achieve a correct diagnosis and management of this delicate situation.