(J. Klepper, Aschaffenburg; updated 01.2007)
The ketogenic diet can be used effectively in:
- intractable childhood epilepsy
- disorders of brain energy metabolism
- GLUT1 deficiency syndrome
- Pyruvate dehydrogenase deficiency
ad 1. intractable childhood epilepsy
Approximately 70% of children with epilepsy will respond effectively to an anticonvulsant drug. 30% of children will need a second anticonvulsant, which will control seizures in yet again 70% of children. Epilepsy in the remaining 30% will usually respond poorly to further anticonvulsant treatment. In these patients the ketogenic diet is a therapeutic option that has regained the interest of pediatricians.There is ample evidence that in these children the ketogenic diet is effective. A metaanalysis of 11 studies on the ketogenic diet (Lefevre, Pediatrics 2000) showed - independent of age and seizure frequency – complete seizure control in 16%, a major seizure reduction in 32% and a moderate seizure reduction in 33% of patients.
ad 2. disorders of brain energy metabolism
In the resting state the human brain requires about 20% of total body energy consumption. In infants this can increase up to 80%.
In GLUT1 deficiency syndrome the supply of glucose to the brain is impaired due to a transport defect of the glucose transporter GLUT1 in the blood-brain barrier. This “energy crisis” in brain results in early-onset epilepsy, developmental delay and a complex movement disorder. The ketogenic diet provides ketones as an alternative fuel to the brain restoring energy metabolism in the developing brain. Seizures are controlled effectively within weeks, the impact on developmental delay and the movement disorder respond to a lesser extent.
Pyruvate dehydrogenase deficiency is a severe disorder of brain energy metabolism resulting in hypotonia, epilepsy, episodic apneas, and death in infancy. Glucose can not be metabolized into acetyl-CoA fueling the citrate cycle and the respiratory chain for energy production. The ketogenic diet provides ketones potentially restoring acetyl-CoA to fuel energy production.