Ketogenesis is the conversion of acetyl-CoA to the ketone bodies acetoacetate and beta-hydroxybutyrate (BHBA). In hepatic ketogenesis, which occurs during fasting in both nonruminant and ruminant animals, the source of acetyl-CoA is the mitochondrial oxidation of predominantly long-chain fatty acids. In the mature, fed ruminant animal, the ruminal epithelium is also capable of producing ketone ...

PURPOSE GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. METHODS A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously...

Researchers from the Children's National Health System in Washington, D.C. studied the feasibility, rate of complications, and effect on seizures of initiating the Ketogenic Diet (KD) in pediatric patients with Super-Refractory Status Epilepticus (SRSE).

The ketogenic diet with extremely high fat and very low carbohydrate levels is popular in society today. Although it has beneficial effects on epilepsy neurodegenerative diseases, how diets impact host glucose lipid metabolism gut microbiota still needs further investigation.

Children with epilepsy, especially those facing intractable seizures, experience a great impact on the quality of their lives. Effective treatment is essential, and although new anti-epileptic drugs have shown an improved profile of action, still a substantial number of children look for more efficacious ways of treatment that are far away from potential toxicity and ineffectiveness. The ketoge...

The medium chain triglyceride (MCT) ketogenic diet is a major treatment of drug-resistant epilepsy but is problematic, particularly in adults, because of poor tolerability. Branched derivatives of octanoic acid (OA), a medium chain fat provided in the diet have been suggested as potential new treatments for drug-resistant epilepsy, but the structural basis of this functionality has not been det...

A variety of observations suggest that decreasing glycolysis and increasing levels of reduced glutathione, generated by metabolism of glucose through the pentose phosphate pathway, would have an anticonvulsant effect. Because fructose-1,6-bisphosphate (F1,6BP) shifts the metabolism of glucose from glycolysis to the pentose phosphate pathway, it was hypothesized to have anticonvulsant activity. ...

Normally, the brain's fuel is glucose, but during fasting it increasingly relies on ketones (beta-hydroxybutyrate, acetoacetate, and acetone) produced in liver mitochondria from fatty acid beta-oxidation. Although moderately raised blood ketones produced on a very high fat ketogenic diet have important clinical effects on the brain, including reducing seizures, ketone metabolism by the brain is...

PURPOSE To examine the process for transitioning adolescents on dietary management for intractable epilepsy to adult neurologic care. METHODS Ten patients, ages 6-16 years when the ketogenic or modified Atkins diet was initiated, were identified; age at transition was 18-43 years. All patients were seen at Johns Hopkins Hospital, Baltimore, Maryland. RESULTS Seven remain on diets (4 Ketogen...