January 26, 2022

Keto Diet Resolves Non-Convulsive Seizures in 2 Girls With New Variant

Article appeared in Angelman Syndrome News

A ketogenic diet safely and rapidly controlled treatment-resistant prolonged non-convulsive seizure activity in two girls with Angelman syndrome caused by a new mutation in the UBE3A gene, a study shows.

These therapeutic benefits were observed before the detection of ketone bodies, the fat-derived molecules produced by the liver to serve as an energy source when glucose or sugar — the body’s go-to energy source — is not readily available.

These cases highlight the ketogenic diet as an effective approach to manage prolonged non-convulsive seizures related to Angelman and suggest that carbohydrate restriction may, by itself, have an effect on these seizures.

The study, “Novel UBE3A pathogenic variant in a large Georgian family produces non-convulsive status epilepticus responsive to ketogenic diet,” was published in the journal Seizure.