Introduction: Early use of the ketogenic diet (KD) is described as having a particular interest for super-refractory status epilepticus and febrile infection-related epilepsy syndrome. The authors conducted a systematic review of the available data on the KD for refractory and super-refractory status epilepticus.
Areas covered: Following a systematic bibliographic search, the authors found 15 published papers: 2 prospective and 13 retrospective studies. Most often, the primary aim of the retrospective studies was the efficacy evaluation of the KD for refractory or super-refractory status epilepticus. Four studies focused on the use of KD for NORSE/FIRES. These initial studies suggested that KD was effective in these conditions, and that it showed mild and manageable side effects.
Expert opinion: The published studies provided enough preliminary data to validate the feasibility and safety of the use of KD for refractory and super-refractory status epilepticus. Further studies demonstrating the efficacy of the KD in these indications are needed. Possible design and endpoints are discussed.
Keywords: Ketogenic diet; NORSE; refractory status epilepticus; super-refractory status epilepticus.