Evaluation of Safety and Tolerability of a Nutritional Formulation in AS

Lowering carbohydrate content of the diet and increasing the fat content (through either a ketogenic diet, MCT based diet, modified Atkins diet, or the low glycemic index diet [LGIT]) has been shown to effectively decrease seizure frequency in patients with epilepsy and AS. Specialized medical food formations have been developed to transition patients to low carb, high fat formations, all of which are very high in fat. The medical food under assessment in this study is a specialized high fate formulation containing a ketone known as beta-hydroxybutyrate (BHB). BHB is a natural fat metabolite (ketone) that shifts metabolism away from carbs toward fats. This BHB will be delivered to those affected with AS to those that are on a standard American diet, the LGIT diet or the ketogenic diet. The metabolic sensitivity of AS forms the baseline for patients, but metabolic pathways used are different depending on diet. The hypothesis of the study is that BHB will enhance the effectiveness of dietary intervention by providing fuel substrates that push metabolism away from carbohydrates and toward fat utilization. The specific aims of the study are the following:

1) Assess tolerability of this supplement in patients with AS

2) Assess ketosis in AS patients

3) Assess safety in AS patients