Refractory epileptic state (RES) is defined by severe seizures that are resistant to antiepileptic drug treatment. Diagnostic errors such as pseudo-seizures and encephalopathies with triphasic waves must be distinguished at an early stage from cases of RES. The latter are symptomatic of a focal brain lesion or severe systemic disease, most frequently metabolic in origin. The treatment of such conditions is aimed at correction of the underlying cause. A nosographic issue that is still a subject of discussion and which requires further study, i.e., PLEDS, will also be discussed in this article.