The ketogenic diet for paediatric patients with super-refractory status epilepticus in febrile infection-related epilepsy syndrome

Objective To investigate the effect and safety of ketogenic diet (KD) for the treatment of paediatric patients with super-refractory status epilepticus (SRSE) in febrile infection-related epilepsy syndrome (FIRES). Method From January 1, 2015 to October 31, 2017, ten critically ill paediatric patients with SRSE in FIRES were included in this study and treated with KD. The treatment effects of KD were evaluated by using continuous encephalography (CEEG) and amplitude-integrated electro-encephalography (aEEG). Results All 10 patients fulfilled the diagnostic criteria of SRSE in FIRES and achieved ketosis within 24–72 h following the administration of KD. CEEG and aEEG were monitored for several weeks to assess the efficacy of KD on status epilepticus (SE). SE was contained in 8 patients within 2 to 19 days after initiation of KD, and KD was discontinued in the other 2 patients. One of the 10 patients demonstrated severe adverse effects. Conclusion KD may be an alternative and safe treatment option in critical paediatric patients with SRSE in FIRES.