Abstract
ObjectiveTo 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).MethodFrom 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).ResultsAll 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.ConclusionKD may be an alternative and safe treatment option in critical paediatric patients with SRSE in FIRES.
Highlights
Status epilepticus (SE) is one of the most life-threatening medical emergencies in children
febrile infection-related epilepsy syndrome (FIRES) is a sub-type of new-onset refractory status epilepticus (NORES) that occurs after a prior febrile infection, with
EEG changes EEG recordings performed before the ketogenic diet (KD) showed multifocal interictal epileptic discharge, which was consistent with SE that originated from focal areas and migrated over large areas of unilateral or bilateral hemispheres
Summary
Status epilepticus (SE) is one of the most life-threatening medical emergencies in children. Super-refractory status epilepticus (SRSE) is defined as an epileptic status that continues or recurs 24 h or more after the initiation of anaesthetic therapy, including reduction or withdrawal of anaesthetic agents [1]. The mortality of SRSE ranges from 23–85% [2,3,4], and current therapies demonstrate various drawbacks in treatment outcomes, adverse effects, and intolerance. There is an urgent need to develop alternative or novel therapies to facilitate clinical management. Ketogenic diet (KD) is a high fat, adequate protein, low carbohydrate diet, and it is currently recommended as a
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