Dravet syndrome is a severe form of epilepsy marked by frequent, prolonged, and uncontrollable seizures starting in infancy. Available medications do not improve symptoms in approximately one-third of Dravet syndrome patients.

The ketogenic diet is a high-fat, low-carbohydrate, and limited protein diet developed by physicians in the 1920s to treat epilepsy. With the introduction of modern medicines, the ketogenic diet was vastly abandoned, but in the past few decades, it has begun to gain ground again as an effective therapy for epileptic disorders.

How the ketogenic diet works

The body uses carbohydrates, proteins, and fats found in foods to produce energy. Carbohydrates found in bread, rice, and potatoes are the body’s primary source of energy as they are rapidly turned into glucose (sugar) and immediately broken down to produce the energy needed for the activity of different organs.

In the absence of glucose, liver enzymes turn fats into ketone bodies in a process called ketogenesis. Being the only source of energy for the brain other than glucose, ketone bodies can fulfill two-thirds of the energy requirement of the brain.

The exact mechanism by which the ketogenic diet helps reduce seizures in epilepsy and Dravet syndrome is not fully understood. However, scientists have several theories about the protective effects of ketone bodies in the brain. For example, they think ketone bodies may increase the levels of chemicals that transmit signals through the nerve cells, as well as the number of mitochondria, the main energy-producing units of cells.

Ketogenic diet research

A clinical study showed that, of 13 children with Dravet syndrome who stayed on the ketogenic diet for longer than a year, two remained seizure-free, and 11 had a more than 50% decrease in the frequency of their seizures. Six of those patients stopped the ketogenic diet later, and only one remained seizure-free, while two patients had infrequent seizures, and three lost the diet’s positive effects. Another study found that more than 60 percent of Dravet syndrome patients on the ketogenic diet improved within the first year.

Another study included 20 children with Dravet syndrome who had at least two seizures per month despite medication. In 17 patients, seizure frequency and duration decreased by at least 50% after three months on the ketogenic diet compared with baseline, or the beginning of the study. Six patients were seizure-free. Three patients did not respond to the ketogenic diet and discontinued the therapy. After six months, in the 17 remaining patients, seizure frequency and duration remained below 50% compared with baseline, and 10 patients were seizure-free.

The ketogenic diet can also be used in combination with anti-epileptic medications to improve seizure control when these medicines are only partially successful. A clinical study found that the ketogenic diet, in addition to standard anti-epileptic medications, improves not only seizure frequency, but also the behavioral abnormalities associated with the condition such as hyperactivity, attention deficit, and aggressiveness.

The ketogenic diet is generally prescribed to children with epilepsy, from ages 1 to 8, and it long has been thought not to benefit adults with the disease. However, more recent studies have shown that adults may in fact benefit from the diet.

How the ketogenic diet is practiced

A team of physicians and dietitians initiate the diet at a medical center, where the patient’s family can be trained to prepare meals according to the specific requirements of the ketogenic diet. In some centers, the diet may start with a fasting period of about a day, resulting in a quicker switch to ketones as an energy source.

The aim of the ketogenic diet is to establish a one-in-four carbohydrate and protein to fat ratio in meals. It is a very restrictive and demanding lifestyle for the whole family. It requires weighing every ingredient and reading all the labels for the exact calculations, which can be overwhelming. For this reason, a dietitian closely monitors the entire course of the diet.

The food amounts in a ketogenic diet are customized to each patient based on his or her needs. Typical high-fat foods included in a ketogenic diet are oils, nuts, eggs, heavy cream, butter, cheese, and mayonnaise. Vegetables are also an integral part of a well-formulated ketogenic diet. Leafy greens such as spinach and cabbage are recommended; however, starchy vegetables such as legumes, squash, and sweet corn should be avoided. Foods such as bread, rice, pasta, potatoes, and sugar-containing sweets are strictly prohibited. Due to the very limited variety of ingredients, vitamin and mineral supplements are included in the diet.

Side effects of the ketogenic diet

Constipation, acidosis, and carnitine deficiency are among the most frequent side effects of the ketogenic diet. Less commonly, patients may have high cholesterol, slow growth, kidney stones, low blood sugar, and nausea. Pre-existing conditions may increase the risk of some of these side effects.

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