How effective is the ketogenic diet for electrical status epilepticus of sleep?

Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation. Clinical reports of the KD for ESES have been heterogeneous, but to date 38 children have been described in six publications. Overall, 53% had EEG improvement, 41% had >50% seizure reduction, 45% had cognitive improvement, but only 9% had EEG normalization. This review will assess the efficacy of the KD in the treatment of ESES based on known data as well as possible mechanisms of action and the need for future study.