Epileptic spasms (ES) are a predominantly infantile seizure type observed frequently in certain genetic disorders. Ketogenic diet (high ratio of fat to carbohydrate/protein) is an established non-medication treatment for difficult to control seizures, including ES. Because ES are associated with worse developmental and cognitive outcomes if not detected or treated quickly and effectively, this trial aims to test the ketogenic diet to prevent ES in this high-risk population. This trial is a single-center pilot study of 10 infants with genetic seizure disorders to establish if the protocol of early ketogenic diet administration and ES evaluation is safe and feasible.
Plan for initiation of ketogenic diet by clinical team for treatment of epilepsy
The clinical team initiating the ketogenic diet agrees that the use of the KetoVie formula is appropriate for the subject, as all study subjects need to receive the same formula
Male or female, age 0 to less than 9 months (including neonates per investigator's judgment)
Epilepsy onset at less than 6 months of age
Epileptic spams prior to enrollment
Tuberous sclerosis complex, trisomy 21 (based on differential response to ES treatment)
Metabolic diagnosis with targeted treatment (including specific indication for ketogenic diet such as glucose transporter disorder, vitamin dependent epilepsies, and others) or exclusion for the ketogenic diet
Ongoing treatment with vigabatrin, ACTH, corticosteroids, topiramate or zonisamide. Other anti-seizure medications are permitted.
Heather Olson
Lead sponsor
Boston Children's Hospital
Sponsor institution
Boston Children's Hospital
Collaborator