Most children with epilepsy achieve seizure control with antiepileptic medications or outgrow their epilepsy. However, despite the introduction of new antiepileptic medications, 30% to 40% of patients have poorly controlled epilepsy that is resistant to medication. Ongoing seizures can be debilitating for these children. Uncontrolled seizures are associated with a range of unfavorable effects, including cognitive, behavioral, and psychosocial comorbidities. Some of these children may benefit from intracranial surgical interventions. For those who do not have a surgical option, it is essential to consider palliative surgical options and dietary therapy that may improve or even achieve seizure control. For children whose seizure control improves, the dose of antiepileptic medications may be reduced, thereby improving quality of life by reducing side effects.
DIETARY TREATMENTS FOR EPILEPSY
Dietary therapy has been used to treat epilepsy for more than 9 decades beginning with the ketogenic diet in 1921 followed by the medium-chain triglyceride diet in 1971. In the past decade, the modified Atkins diet and low glycemic index treatment have emerged as alternatives. The common characteristic of all dietary therapies to treat epilepsy is the restriction in carbohydrate intake to shift the predominant caloric source to fat. Restriction of carbohydrates forces body tissues to catabolize fats as their primary source of energy, which results in the production of ketone bodies and free fatty acids that the body uses for energy production. Contraindications are the same for all ketogenic therapies, and all require close monitoring and fine-tuning to achieve an optimal outcome. All providers involved in the care of these patients must understand the importance of strict adherence to the dietary regimen.
The mechanism by which the ketogenic diet yields its antiseizure effect is not known. Hypotheses for which there is some experimental support includes carbohydrate reduction, activation of adenosine triphosphate (ATP)–sensitive potassium channels by mitochondrial metabolism, inhibition of the mammalian target of rapamycin activity, and inhibition of glutamatergic excitatory synaptic transmission. The relationship between ketones and seizure control is unclear, but all of these mechanisms depend on ketones.
Evaluation and Counseling
Evaluation and counseling for dietary treatments for epilepsy are similar. All share the property of changing the primary energy source for the body and brain, and all require medical supervision. Henceforth, diets will be referred to collectively as ketogenic diets unless otherwise specified. The complex needs of these patients are best met by an interdisciplinary team consisting of a neurologist, nurse, dietitian, pharmacist, and social worker. At minimum, a physician familiar with ketogenic diets and a dietitian are mandatory for a ketogenic diet team with limited resources.
Before initiating the ketogenic diet, disorders of fatty acid mitochondrial transport, β-oxidation, and other mitochondrial cytopathies should be excluded. The ketogenic diet is contraindicated in these patients because it can precipitate a potentially fatal metabolic crisis in an affected individual due to its reliance on mitochondrial fatty ...