When status epilepticus (SE) remains refractory to appropriate therapy, it is associated with high mortality and with substantial morbidity in survivors. Many outcome predictors such as age, seizure type, level of consciousness before treatment, and mostly, etiology, are well-established. A longer duration of SE is often associated with worse outcome, but duration may lose its prognostic value after several hours. Several terms and definitions have been used to describe prolonged, refractory SE, including “malignant SE,” “prolonged” SE, and more recently, “super refractory” SE, defined as “SE that has continued or recurred despite 24 hours of general anesthesia (or coma-inducing anticonvulsants).” There are few data available regarding the outcome of prolonged refractory SE, and even fewer for SE remaining refractory to anesthetic drugs. This article reviews reports of outcome after prolonged, refractory, and “super refractory” SE. Most information detailing the clinical outcome of patients surviving these severe illnesses, in which seizures can persist for days or weeks (and especially those concerning “super-refractory” SE) come from case reports and retrospective cohort studies. In many series, prolonged, refractory SE has a mortality of 30% to 50%, and several studies indicate that most survivors have a substantial decline in functional status. Nevertheless, several reports demonstrate that good functional outcome is possible even after several days of SE and coma induction. Treatment of refractory SE should not be withdrawn from younger patients without structural brain damage at presentation solely because of the duration of SE.
*Department of Neurology, Hôpital du Valais, Sion, Switzerland;
†Department of Clinical Neurosciences, CHUV and University of Lausanne, Lausanne, Switzerland;
‡Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, U.S.A.; and
§Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, U.S.A.
Address correspondence and reprint requests to Frank W. Drislane, MD, Department of Neurology, KS 457, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02115, U.S.A.; e-mail: firstname.lastname@example.org.
V.A. is funded by the Swiss National Science Foundation, grant: P2GEP3_148510 and the Gottfried und Julia Bangerter-Rhyner Foundation.