The ketogenic diet is a high-fat, adequate-protein, low-carbohydrate mainstream dietary therapy that in medicine is used mainly to treat hard-to-control (refractory) epilepsy in children. The diet forces the body to burn fats rather than carbohydrates.
Normally carbohydrates in food are converted into glucose, which is then transported around the body and is important in fueling brain function. However, if only a little carbohydrate remains in the diet, the liver converts fat into fatty acids and ketone bodies, the latter passing into the brain and replacing glucose as an energy source. An elevated level of ketone bodies in the blood (a state called ketosis) eventually lowers the frequency of epileptic seizures. Around half of children and young people with epilepsy who have tried some form of this diet saw the number of seizures drop by at least half, and the effect persists after discontinuing the diet. Some evidence shows that adults with epilepsy may benefit from the diet and that a less strict regimen, such as a modified Atkins diet, is similarly effective. Side effects may include constipation, high cholesterol, growth slowing, acidosis, and kidney stones.
In 1994, Hollywood producer Jim Abrahams, whose son's severe epilepsy was effectively controlled by the diet, created the Charlie Foundation for Ketogenic Therapies to further promote diet therapy. Publicity included an appearance on NBC's Dateline program and …First Do No Harm (1997), a made-for-television film starring Meryl Streep. The foundation sponsored a research study, the results of which—announced in 1996—marked the beginning of renewed scientific interest in the diet.
Possible therapeutic uses for the ketogenic diet have been studied for many additional neurological disorders, some of which include: Alzheimer's disease, amyotrophic lateral sclerosis, headache, neurotrauma, pain, Parkinson's disease, and sleep disorders.
The ketogenic diet is calculated by a dietitian for each child. Age, weight, activity levels, culture, and food preferences all affect the meal plan. First, the energy requirements are set at 80–90% of the recommended daily amounts (RDA) for the child's age (the high-fat diet requires less energy to process than a typical high-carbohydrate diet). Highly active children or those with muscle spasticity require more food energy than this; immobile children require less. The ketogenic ratio of the diet compares the weight of fat to the combined weight of carbohydrate and protein. This is typically 4:1, but children who are younger than 18 months, older than 12 years, or who are obese may be started on a 3:1 ratio. Fat is energy-rich, with 9 kcal/g (38 kJ/g) compared to 4 kcal/g (17 kJ/g) for carbohydrate or protein, so portions on the ketogenic diet are smaller than normal. The quantity of fat in the diet can be calculated from the overall energy requirements and the chosen ketogenic ratio. Next, the protein levels are set to allow for growth and body maintenance, and are around 1 g protein for each kg of body weight. Lastly, the amount of carbohydrate is set according to what allowance is left while maintaining the chosen ratio. Any carbohydrate in medications or supplements must be subtracted from this allowance. The total daily amount of fat, protein, and carbohydrate is then evenly divided across the meals.
The classic ketogenic diet is not a balanced diet and only contains tiny portions of fresh fruit and vegetables, fortified cereals, and calcium-rich foods. In particular, the B vitamins, calcium, and vitamin D must be artificially supplemented. This is achieved by taking two sugar-free supplements designed for the patient's age: a multivitamin with minerals and calcium with vitamin D. A typical day of food for a child on a 4:1 ratio, 1,500 kcal (6,300 kJ) ketogenic diet comprises three small meals and three small snacks:
|Breakfast||Egg with bacon||
|Morning snack||Peanut butter ball
(serving size: 0.5 ounce)
|Afternoon snack||Keto yogurt
(serving size: 1.3 ounces)
|Dinner||Cheeseburger (no bun)||
|Evening snack||Keto custard
(serving size: 1.2 ounces)
Normal dietary fat contains mostly long-chain triglycerides (LCTs). Medium-chain triglycerides (MCTs) are more ketogenic than LCTs because they generate more ketones per unit of energy when metabolised. Their use allows for a diet with a lower proportion of fat and a greater proportion of protein and carbohydrate, leading to more food choices and larger portion sizes. The original MCT diet developed by Peter Huttenlocher in the 1970s derived 60% of its calories from MCT oil. Consuming that quantity of MCT oil caused abdominal cramps, diarrhea, and vomiting in some children. A figure of 45% is regarded as a balance between achieving good ketosis and minimising gastrointestinal complaints. The classical and modified MCT ketogenic diets are equally effective and differences in tolerability are not statistically significant. The MCT diet is less popular in the United States; MCT oil is more expensive than other dietary fats and is not covered by insurance companies.
The modified Atkins diet reduces seizure frequency by more than 50% in 43% of patients who try it and by more than 90% in 27% of patients. Few adverse effects have been reported, though cholesterol is increased and the diet has not been studied long term. Although based on a smaller data set (126 adults and children from 11 studies over five centres), these results from 2009 compare favorably with the traditional ketogenic diet.
Low glycemic index treatment
The low glycemic index treatment (LGIT) is an attempt to achieve the stable blood glucose levels seen in children on the classic ketogenic diet while using a much less restrictive regimen. The hypothesis is that stable blood glucose may be one of the mechanisms of action involved in the ketogenic diet, which occurs because the absorption of the limited carbohydrates is slowed by the high fat content. Although it is also a high-fat diet (with approximately 60% calories from fat), the LGIT allows more carbohydrate than either the classic ketogenic diet or the modified Atkins diet, approximately 40–60 g per day. However, the types of carbohydrates consumed are restricted to those that have a glycaemic index lower than 50. Like the modified Atkins diet, the LGIT is initiated and maintained at outpatient clinics and does not require precise weighing of food or intensive dietitian support. Both are offered at most centres that run ketogenic diet programmes, and in some centres they are often the primary dietary therapy for adolescents.
Infants and patients fed via a gastrostomy tube can also be given a ketogenic diet. Parents make up a prescribed powdered formula, such as KetoCal, into a liquid feed. Gastrostomy feeding avoids any issues with palatability, and bottle-fed infants readily accept the ketogenic formula. Some studies have found this liquid feed to be more efficacious and associated with lower total cholesterol than a solid ketogenic diet. KetoCal is a nutritionally complete food containing milk protein and is supplemented with amino acids, fat, carbohydrate, vitamins, minerals and trace elements. It is used to administer the 4:1 ratio classic ketogenic diet in children over one year. The formula is available in both 3:1 and 4:1 ratios, either unflavoured or in an artificially sweetened vanilla flavour and is suitable for tube or oral feeding. Other formula products include KetoVolve and Ketonia. Alternatively, a liquid ketogenic diet may be produced by combining Ross Carbohydrate Free soy formula with Microlipid and Polycose.
In theory, there are no restrictions on where the ketogenic diet might be used, and it can cost less than modern anticonvulsants. However, fasting and dietary changes are affected by religious and cultural issues. A culture where food is often prepared by grandparents or hired help means more people must be educated about the diet. When families dine together, sharing the same meal, it can be difficult to separate the child's meal. In many countries, food labelling is not mandatory, so calculating macronutrients such as fat, protein and carbohydrates can be difficult. In some countries, it may be hard to find sugar-free forms of medicines and supplements, to purchase an accurate electronic scale, or to afford MCT oils.
The ketogenic diet is a mainstream dietary therapy that was developed to reproduce the success and remove the limitations of the non-mainstream use of fasting to treat epilepsy. Although popular in the 1920s and '30s, it was largely abandoned in favour of new anticonvulsant drugs. Most individuals with epilepsy can successfully control their seizures with medication. However, 25–30% fail to achieve such control despite trying a number of different drugs. For this group, and for children in particular, the diet has once again found a role in epilepsy management.