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Early infantile epileptic encephalopathy (EIEE), also known as Ohtahara syndrome, is characterized by intractable tonic seizures in the setting of a severe encephalopathy and a burst-suppression background pattern on EEG. Incidence has been estimated at 1/100 000 births in Japan and 1/50,000 births in the U.K. Approximately 100 cases total have been reported but this may be an underestimate. Ohtahara syndrome is a rare clinico-EEG syndrome. Gold standard investigation is genetic panel for epilepsy in case of nil structural abnormalities. It is refractory to any mode of treatment including ketogenic diet and surgery and carries highest mortality rate, die in infancy.

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