Background Generalized convulsive status epilepticus (GCSE) is a medical emergency associated with high morbidity and mortality that requires prompt medical intervention. Topiramate (TPM) is an antiepileptic drug effective against a broad spectrum of seizure types, and has been proposed as a possible therapeutic option for super-refractory status epilepticus (SRSE), the most severe form of GCSE. Aim This review aimed to evaluate the role of TPM in GCSE, including SRSE. Methods MEDLINE, CENTRAL, ClinicalTrials.gov, LILACS, Google Scholar, and Opengrey.eu were systematically searched. We compared: (1) patients who did and who did not receive TPM as their last drug; (2) patients receiving TPM as the last drug and achieving SE control and patients receiving TPM as the last drug but without termination of SE. Results The literature search yielded 1164 results, with individual data available for 35 patients (six with SRSE) from four studies. SE was controlled in 68.6% of patients receiving TPM either as the last drug (20) or not (15), and in 14 of the 20 patients receiving TPM as the last drug (70%). Only six patients received TPM for SRSE; in five of them, TPM was administered as the last drug with resolution of SE in four. When comparing patients who did and did not receive TPM as the last drug, no statistically significant difference was found for any of the variables considered; similarly, no difference was found comparing patients receiving TPM as the last drug and achieving SE control with those receiving TPM as the last drug but without termination of SE. Conclusions The lack of a statistically significant difference is likely to be due to the small sample size. In only a few patients was TPM used for SRSE. There is an unmet need for high-quality studies to evaluate the role of TPM in GCSE. Electronic supplementary material The online version of this article (doi:10.1007/s40265-016-0672-2) contains supplementary material, which is available to authorized users.
