Updated Clinical Guidelines on the Ketogenic Diet in Children with Epilepsy

June 20, 2018

Nearly a decade in the making, revised guidelines provide 8 key points for effective nonpharmacologic treatment of epilepsy in childhood.

The International Ketogenic Diet Study Group has released new clinical guidelines on the ketogenic diet in children with epilepsy.[1] The updated recommendations are the first change since the original guidelines on the subject were published almost 10 years ago.[2]

“Ketogenic dietary therapies (KDTs) are well-established, nonpharmacologic treatments used for children and adults with medication-refractory epilepsy… Given its efficacy, we strongly advocate that KDTs be considered earlier as an option for treatment of difficult-to-manage epilepsy,” wrote first author Eric Kossoff, MD, of Johns Hopkins University, and colleagues with the Practice Committee of the Child Neurology Society.

The classic ketogenic diet is most commonly used and consists of a high fat diet, with about 90% of calories from fat and a 4:1 fat to protein plus carbohydrate ratio. The medium chain triglyceride (MCT) diet is the second most commonly used diet, with 60% of energy from medium chain triglycerides. The modified Atkins diet (MAD) is a high-fat, low-carbohydrate diet similar to the classic ketogenic diet, but without a set ketogenic ratio. The low glycemic index treatment (LGIT) diet provides more liberal total daily carbohydrate intake, in favor of those with low glycemic indices.

1. Recommendations for nonfasting when initiating the classic ketogenic diet in children under age 2
2. Growth in evidence for the MAD and LGIT diets, especially in teens
3. Recommendation for 1-month follow-up visit after starting the diet
4. Clarification of ideal indications for ketogenic diet use

-Consider earlier initiation for several epilepsy syndromes (Angelman Syndrome, FIRES, infantile spasms, etc)

-KDTs are the treatment of choice in glucose transporter 1 deficiency syndrome (Glut1DS) and pyruvate dehydrogenase deficiency (PDHD)

Pre-diet evaluation should rule out contraindications to use, especially inborn errors of metabolism.

-Calorie and fluid restrictions no longer recommended

-Children under age 2 should start on the classic ketogenic diet

-MAD and LGIT can be recommended in teens

-Fasting and inpatient initiation are optional

-AEDs can be decreased after one month if the KDT is successful

-Use caution and a gradual taper when decreasing phenobarbital or benzodiazepines, due to increased risk of worsening seizure control

-Oral citrates may prevent kidney stones

-More frequent monitoring for infants and children at high risk for nutritional deficiencies

-GI problems and hyperlipidemia are most common

-Hyperlipidemia usually normalizes by 12 months

-Consider an EEG prior to discontinuation, for counseling about recurrence risk

-Gradually wean over 1-3 months, unless urgently indicated

References

1. Kossoff EH, Zupec-Kania BA, Auvin S, et al, for the Practice Committee of the Child Neurology Society. Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open. 2018;3:175-192.

2. Kossoff EH, Zupec-Kania BA, Amark PE, et al. Optimal clinical management of children receiving the ketogenic diet: recommendations of the international ketogenic diet study group. Epilepsia. 2009;50:304-317.