(J. Klepper, Aschaffenburg; updated 01.2007)
The only established therapy for GLUT1 deficiency syndrome is the ketogenic diet. It is a high-fat, low-carbohydrate diet mimicking the metabolic state of fasting
In starvation the body’s metabolism switches from carbohydrate to fat utilization for fuel. The liver generates ketones that cross the blood-brain barrier and serve as an alternative fuel to the brain. The high-fat ketogenic diet mimics the body’s response to starvation by supplying nutritional fat and very few carbohydrates, thus maintaining ketosis without weight loss.
The ketogenic diet should always be in the hands of an experienced team of physicians and dieticians. It is a very strict and limiting diet – vitamins, minerals and trace elements need to be supplemented and the fat to non-fat ratio needs to be strictly adhered to in every meal.
The response to the ketogenic diet in GLUT1 deficiency syndrome is striking. Almost all patients become seizure-free within weeks without comedication. Patients and parents also report a positive impact of the diet on mental alertness and endurance.
Several drugs (caffeine, phenobarbital, narcotics, chloral-hydrate) inhibit GLUT1 function. Others interact with the ketogenic mechanisms of the diet (valproate, topiramate, acetazolamide). These substances should be avoided in GLUT1 deficient patients.
*) GLUT1 patients on the ketogenic diet