title: Substantial and sustained seizure reduction with ketogenic diet in a patient with Ohtahara syndrome.
abstract: Ketogenic diet has been shown to be efficacious in some epileptic encephalopathies but rarely reported as being useful in children with Ohtahara syndrome. This could possibly be attributed to the rarity of the disease and associated short survival period. We report on a 5-year-old child with Ohtahara syndrome, whose seizures failed to improve with all known medications, continued to show persistent suppression-burst pattern on the electroencephalography (EEG) and had substantial reduction in seizure frequency for one year post-initiation of ketogenic diet. He has not had a single visit to the emergency room because of seizures in the last one year, and more importantly, there has been a clear improvement noted in his level of interaction and temperament. Patients with Ohtahara syndrome invariably have medically intractable seizures and catastrophic neurodevelopmental outcome. Ketogenic diet is a treatment modality that might be worth considering even in this group of patients.
authors.affiliation: Comprehensive Epilepsy Center, Department of Neurology, 15 York Street, LLCI 716, Yale University School of Medicine, Yale-New Haven Hospital, CT 06520, USA.
authors.forename: Adithya
authors.initials: A
authors.lastname: Sivaraju
authors.affiliation: Comprehensive Epilepsy Center, Department of Neurology, 15 York Street, LLCI 716, Yale University School of Medicine, Yale-New Haven Hospital, CT 06520, USA.
authors.forename: Ilisa
authors.initials: I
authors.lastname: Nussbaum
authors.affiliation: Comprehensive Epilepsy Center, Department of Neurology, 15 York Street, LLCI 716, Yale University School of Medicine, Yale-New Haven Hospital, CT 06520, USA.
authors.forename: Candace S
authors.initials: CS
authors.lastname: Cardoza
authors.affiliation: Comprehensive Epilepsy Center, Department of Neurology, 15 York Street, LLCI 716, Yale University School of Medicine, Yale-New Haven Hospital, CT 06520, USA.
authors.forename: Richard H
authors.initials: RH
authors.lastname: Mattson
keywords: EEG, Electroencephalography/electroencephalogram; ER, Emergency room; Epileptic encephalopathies; G tube, Gastric tube; Ketogenic diet; Ohtahara syndrome; Persistence of suppression-burst; Q-EEG, Quantitative electroencephalogram