How Can the Ketogenic Diet Help My Child With Angelman Syndrome?

A ketogenic diet can be effective in controlling the frequency of seizures in Angelman syndrome, particularly in children who don’t respond to medication. If your child has Angelman syndrome, here are some considerations before starting your child on the diet.

What is Angelman syndrome?

Angelman syndrome is a complex neurological disorder characterized by delayed mental and physical development, speech impediments, frequent bursts of unexplainable laughter, and seizures that usually start in children ages 18 months to 2 years.

Up to 90% of Angelman syndrome patients experience difficult-to-treat and potentially life-threatening seizures.

What is the ketogenic diet?

The ketogenic diet takes its name from the ketones formed when the body uses fat as an energy source in a process called nutritional ketosis. Usually, the body uses carbohydrates for fuel. In the “keto diet,” fats including butter, heavy whipping cream, mayonnaise, and oils become the primary fuel. Specifically, the diet provides three to four grams of fat for every gram of carbohydrate and protein.

The plan is stricter than the modified Atkins diet, requiring specific amounts of calories, fluids, and proteins. All food must be weighed and measured. If your child strays from the diet for even one meal, the benefits of ketosis could be lost.

Children who are on the diet continue to take seizure medications, although some may be able to take smaller doses or fewer medications.

How can I start my child on this diet?

If you’re interested in starting your child on the keto diet, consult your child’s physician who can explain and prescribe a regimen. Speak with a registered dietitian and your treatment team to establish the ideal plan for your child. The dietitian will also carefully monitor the diet, and teach you and your child what foods are allowed. Your child will undergo blood and urine tests to make sure there are no medical problems. Because the diet does not provide all the vitamins and minerals of a balanced diet, the dietitian will likely recommend supplements.

Your child will usually start the diet in the hospital. This is to monitor seizures, ensure all medications are carbohydrate-free, and educate the family. For infants, doctors can start them on what is usually a formula-only dietary therapy.

Does the ketogenic diet work?

A recent study found that a high-fat, low-carbohydrate diet was successful in reducing seizures in six children with Angelman syndrome.

In a case study involving a five-year-old Angelman syndrome patient, a ketogenic diet was effective in reducing uncontrollable daily seizures.

Overall, several studies have shown that the ketogenic diet can reduce or prevent seizures that could not be controlled by medications. More than half of the children who go on the diet experienced at least a 50% reduction in the number of seizures, and between 10%–15% became seizure-free.

A randomized, double-blind, placebo-controlled clinical trial (NCT03644693) is currently recruiting Angelman syndrome patients, ages 4 to 11, at Vanderbilt University Medical Center in Tennessee, U.S., to test the safety and tolerability of a fat-based nutritional formula containing exogenous ketones.

What are the side effects?

A person starting the ketogenic diet may feel sluggish for a few days. Other potential long-term side effects are kidney stones, high blood cholesterol levels, constipation, slowed growth, and bone fractures.

Can my child ever stop the ketogenic diet?

If a child on the ketogenic diet experiences reduced seizures for some time, usually two years, doctors might suggest the child discontinue the diet. However, the child may remain on the ketogenic diet if it’s led to significant but not total seizure control.

Last updated: June 22, 2020

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Angelman Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.