Short-term trial of a liquid ketogenic milk to infants with West syndrome

Abstract

After the resurgence of the ketogenic diet (KD) since the mid 1990s, the diet had been worldwidely used for the treatment of intractable childhood epilepsies, including Asian countries [1] and [2]. However, we are reluctant to introduce it to infants because of the complications and intolerance of high fat diet derived from low lipase activity in neonates and young infants [3]. We developed a liquid ketogenic milk diet that consists mainly of medium-chain triglycerides (60% of total lipid content). In addition, because of potential problems associated with long-term feeding of unbalanced diets [4], we conducted a short-term trial over a period of 8 months, which included a tapering period of 2 months. The subjects were two infants with cryptogenic West syndrome; to the male patient (Patient 1), a liquid ketogenic milk was executively tried since six months old and to the female patient (Patient 2) since the age of 5 months old. Before receiving the diet, both infants exhibited frequent daily spasms despite treatment with maximum doses of vigabatrin and topiramate. There was a complete remission of seizures after the diet had been fed for 1 and 2 weeks to Patient 1 and 2, respectively. Previous electroencephalographic findings which had showed hypsarrythmic patterns were also normalized and showed appropriate for age. Both patients displayed catch-up developmental progress without any neurological disabilities for 35 months (Patient 1) and 11 months (Patient 2) after a successful discontinuation of the diet and antiepileptic drugs. During the trial, the weight gain was appropriate for age and no other complications were observed. On the basis of these positive preliminary data, we are intended to conduct a comparative trial in which eligible infants will be randomly assigned to either a classic long-term KD protocol or a short-term a liquid ketogenic milk protocol.