Your epilepsy treatment team
Northeast Regional Epilepsy Group
 
User   Password
Forgot your password? | New patients

ImageKetogenic Diet for the Treatment of Epilepsy - Feature article

For this edition of the newsletter, Sloka Iyengar sat down with Angela Samuels – the staff nutritionist at Northeast Regional Epilepsy Group (NEREG) to learn more about the ketogenic diet for the treatment of epilepsy. Our ketogenic specialist explained how the ketogenic diet was discovered, how it works, if there are certain epilepsy patients that can benefit more from it, the success rate as well as risks to keep in mind.

Angela Samuels studied Nutritional Sciences at Rutgers University, and has more than 8 years of experience with the ketogenic diet.  Before joining the Northeast Regional Epilepsy Group, she was the dietitian for the ‘Keto’ program at New York Presbyterian Hospital. 

Sloka Iyengar, PhD is an epilepsy researcher at the Nathan Kline Institute, New York and has a strong interest in epilepsy advocacy and education; and disseminating scientific findings to non-scientists.  

How was the ketogenic diet discovered? 
The ketogenic diet is a very high-fat, very low-carbohydrate diet that is used to treat patients with intractable epilepsy.   

The observation that certain kinds of refractory epilepsies can be treated by fasting had been made by the ancient Greek physicians. Indeed, until the early 1900’s (when the only drugs available for seizures were the bromides), a handful of physicians had tried this approach with reasonable success. The 1920’s and 1930’s saw the release of drugs like phenytoin and sodium valproate making the ketogenic diet a bit obscure. However, recently, this diet therapy has seen a revival – some credit goes to Hollywood producer Jim Abrahams whose son Charlie Abrahams had difficult-to-treat epilepsy, and became seizure-free, thanks to this diet. John Abrahams also founded the Charlie Foundation (http://www.charliefoundation.org/) to educate people about this diet and its effectiveness. 

What is the ketogenic diet? Is it similar to the Atkins diet?
The ketogenic diet is prescribed by ratio of grams of fat: combined grams of carbohydrate and protein, which determines the degree of ketosis (typically for ketogenic diet 3:1- 4:1). Conceptually, the high-fat and low- carbohydrate ketogenic diet is similar to an Atkins diet. There are some differences, however. The ketogenic type of diet is extremely calorie-controlled, and all of the food must be precisely weighed and measured according to the prescription developed by a registered dietitian. Another type of diet that can be used in some people with epilepsy is the modified Atkin’s diet (MAD), where the breakdown of foods is less strict than in the ketogenic diet.  Also, with MAD, the non-carbohydrate foods may be eaten liberally instead of weighed and measured.

How does the ketogenic diet work? 
It is uncertain how the diet works to help seizures. The body and the brain normally use glucose for energy. When someone is on the ketogenic diet, the main source of energy changes from glucose to fat. The liver can break down fats to form ketone bodies; these ketones circulate through the blood in the body and are ultimately excreted into the urine. The elevated state of ketones in the blood called ‘ketosis’ is supposed to reduce the frequency of seizures. The three ketone bodies (β-hydroxybutyrate, acetoacetate and acetone) are proposed to be anticonvulsant by themselves, as seen in experiments done in rodent models of epilepsy. 

Is there a population group that this diet works better for as compared to others? 
The ketogenic diet is more often used in children, whereas the MAD can be used in children and adults. Some of the special patient groups that have experienced a decrease in seizure frequency with this diet are -  

- Individuals with West syndrome – an uncommon to rare disorder that is characterized by infantile spasms, hypsarrhythmia and developmental regression

- Landau-Kleffner syndrome –a rare childhood neurological disorder characterized by aphasia and convulsions

- Rett syndrome – a neurodevelopmental disorder that primarily affects girls. 

- Some mitochondrial disorders 

- Dravet syndrome – also called severe myoclonic epilepsy of infancy

- Tuberous sclerosis complex – a genetic disorder where non-malignant tumors are seen in the brain and other vital organs. Seizures are common (along with neurodevelopmental abnormalities, and those of the kidneys, lungs and skin).  

- Lennox-Gastaut syndrome – which is characterized by daily seizures (of multiple types) as well as developmental delay, behavioral and neuropsychological problems. 

Surprisingly, there are disorders other than epilepsy where people have been found to benefit from a high-fat, low-carbohydrate diet. Some of them are Alzheimer’s disease, autism, brain tumors (glioblastomas), migraine, Parkinson’s disease and amyotrophic lateral sclerosis

What is the success rate of the diet? 
The rate of success of the diet is measured by seizure freedom or a decrease in seizure frequency. The success rate is similar for the ketogenic and the MAD. Prospective and retrospective studies have shown that approximately half of the patients on one of these diets will have at least 50% or better decrease in seizure frequency, 30% of patients will have 90% reduction in seizure frequency, and 10-15% of patients can be completely seizure-free. 

What are some of the adverse reactions? Is it necessary to take vitamins and mineral supplements if one is on the diet?
Short-term side effects during diet initiation include dehydration (as ketosis inhibits thirst), hypoglycemia, vomiting and acidosis.  Long -term side effects can include constipation, stunted growth, vitamin and mineral deficiencies, hyperlipidemia and kidney stones. All patients on the diet must take a daily multivitamin and calcium supplement.

What are some resources where epilepsy and their caregivers can find recipes and practical tips for staying on the ketogenic diet?
The internet offers a number of valuable resources for individuals on this diet. The Charlie Foundation website (www.charliefoundation.org) and the ketocalculator (http://keto-calculator.ankerl.com/) are online resources for people on this diet. Books like the Keto cookbook and Keto kid are also useful resources. 

Are there contraindications for the diet?
There are definitely some conditions where it is not advisable to implement the ketogenic diet. Some of them are:

- Initiation of diet during stress/sepsis/infection

- Inborn errors of metabolism such as pyruvate carboxylase deficiency, organic acidurias

- Disorders which involve defects in fatty acid transport 

- Family/caregiver noncompliance 

- Hyperlipidemia 

- When the patient is on medications that interfere with carbohydrate metabolism e.g. corticosteroids. 

How is the diet implemented? What are the practical considerations that have to be kept in mind when someone is on the ketogenic diet?
The diet is started by an outpatient family meeting where the dietitian and epileptologist sit with the patient (and the family) and discuss the diet. This is followed by an inpatient admission coordinated with the dietitian and the Epilepsy team. Patients are admitted to the Epilepsy Monitoring Unit, where they stay for 3-5 days. Prior to admission, all meds are converted to sugar free form (since the ketogenic diet is very low in carbohydrate), and formulas for food are worked out prior to admission. 

There is also blood-work that is done before the patient is started on the diet. Once the diet starts, there is follow-up at 3, 6, 9, 12 months and then every 6 months after that if patient is doing well.

Meal plans are developed taking personal preferences and cultural considerations into account.

It seems that the ketogenic diet is a concerted effort that is made possible by a number of medical professionals. Could you tell us something about each professional’s part in ensuring success and compliance on the diet?
Role of the physician:

- To identify appropriate patients for this therapy

- Making sure that the patient does not have a medical condition that could worsen in the setting of diet

- Monitoring cholesterol, bone density, electrolyte abnormalities.

Role of the social worker: 

- To identify family needs during the diet

- To identify concerns regarding compliance

- To provide support to families

Role of the nursing professional 

- To act as the point person for the patient

- To Help direct patient to appropriate party

Role of the dietitian 

- To address nutritional content and supplementation of food

- To help make adjustments to the food –plan to optimize seizure control or alleviate side effects

- To help design meal plans that work with the child’s tastes

- To help the family check the carbohydrate content of foods and medicines usin online tools like the ketocalculator

For subjects that are helped by this diet, does it mean that they can be off anti-epileptic drugs (AEDs) for the rest of their lives?
Some patients can have a reduction in seizure meds, some can come off seizure meds completely and some are under good control with a combination of meds and diet.

Are there support groups for parents of children who are on this diet?
Yahoo keto group for parents and caregivers is a way for parents, family members and patients to learn more and connect with other people also on the ketogenic diet. https://groups.yahoo.com/neo/groups/ketogenic/info 

Thank you, Angela, for your time and for enlightening us about the ketogenic diet! 

Twitter Facebook