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DIETARY THERAPY FOR LENNOX-GASTAUT SYNDROME

 
KETOGENIC DIET
 
The Ketogenic Diet is used to treat children with refractory (resistant to treatment) epilepsy including Lennox-Gastaut Syndrome who have not responded well to anti-epileptic medicines.


What is the Ketogenic Diet?
The Ketogenic Diet is a high fat, low carbohydrate diet that forces the body to burn more fat than sugar. The diet is restrictive containing a 4:1 ratio by weight of fat to protein and carbohydrates, but has been shown to be an effective medical therapy for intractable childhood epilepsy. 
1


How Does it Work?
The Ketogenic Diet puts the body into a state of ketosis, which is said to have an anti-convulsant effect. When carbohydrates are restricted, the liver converts fat into fatty acids and ketone bodies. The ketones replace glucose as the energy source in the brain and sends the body into ketosis.

 

What is involved with the Ketogenic Diet?
The ketogenic diet starts with the strict supervision of a doctor and dietitian, followed by a 24 hour fasting period to raise the ketone level in the brain. The diet is then gradually introduced into the child's body.

How effective is the Ketogenic Diet?
Over one half of those children who are placed on the diet have a 50% reduction of seizures or more.
2 The diet requires strict supervision and even the slightest departure may cause the diet to lose its effect.

What about medications?
Medications are not discontinued once the diet is started. The doctor can determine when to decrease the medications while on the diet. Doctors usually recommend vitamins and mineral supplements to accompany the diet since a deficiency may otherwise by developed.

Side Effects:
Dehydration
Constipation
Kidney Stones
Bone Fractures
Vomiting
High cholesterol level
Slower growth rates in children
3

For more information on the ketogenic diet, please visit:
www.charliefoundation.org
www.matthewsfriends.org

 
MODIFIED ATKINS DIET
 
What is the Modified Atkins Diet?
The Modified Atkins diet is a modification of the traditional Ketogenic Diet that had been used for several years by families mostly who had been using the Ketogenic Diet for many years and eventually stopped weighing and measuring foods.
4 The diet is an effective and well-tolerated therapy for intractable pediatric epilepsy.5

How is it Different from the Ketogenic Diet?
With the Modified Atkins diet, there is no fluid or calorie restriction and there are no restrictions on proteins. In addition, foods are not weighed and measured, but carbohydrate counts are monitored by patients and parents.
6

Efficacy of the Diet:
According to studies, about 2/3 had a 50% reduction in seizures after 6 months. Many were able to reduce medications. 
6
 
LOW GLYCEMIC INDEX TREATMENT
 
Low Glycemic Index Treatment (LGIT) was first reported as an alternative Ketogenic Diet (KD) by Pfeifer et al. from Massachusetts General Hospital in 2005.7 LGIT is more liberal and less restrictive than the classical KD. This diet can be provided without restriction of calories, protein and fluid intake, and calculation of ketogenic ratio is not necessary.  LGIT restricts carbohydrate intake to food items with a glycemic index of less than 50 at 40-60 grams per day, in order to prevent large fluctuations in blood glucose concentrations, which are thought to exacerbate seizures.8 In the LGIT, a typical proportions of calories are as follows; 60-65% from fat, 20-30% from protein, and 10-15% from carbohydrates.
 
Approximately 16% of patients on the diet can achieve seizure freedom, while 33%-55% see a seizure reduction of more than 50%.9 LGIT is contraindicated in pyruvate decarboxylase deficiency, primary carnitine deficiency, fatty acid oxidation abnormalities, the porphyrias and some mitochondrial disorders.10
References
 

1. Kossoff EH. More fat and fewer seizures: dietary therapy for epilepsy. Lancet Neurol 2004;3: 415–420.

2. Charlie Foundation. http://www.charliefoundation.org/advisory-board/content/faq. Accessed 11/29/2011

3. Furth, Casey et al. Risk factors for urolithiasis in children on the ketogenic diet. Pediatr Nephrol 2000;15: 125–128.

4. Epilepsy.com. http://www.epilepsy.com/epilepsy/treatment_atkins_diet. Accessed 12/2/2011

5. Kossoff, et al.  A modified atkins diet is an effective treatment for intractable epilepsy. Epilepsia. Volume 47, Issue 2, pages 421–424, February 2006

6. Epilepsy.com. http://www.epilepsy.com/epilepsy/treatment_atkins_diet. Accessed 12/2/2011

7. Pfeifer HH, Thiele EA (2005) Low-glycemic-index treatment: a liberalized ketogenic diet for treatment of intractable epilepsy. Neurology 65: 1810-1812.

8. Greene AE, Todorova MT, Seyfried TN. Perspectives on the metabolic management of epilepsy through dietary reduction of glucose and       elevation of ketone bodies. J Neurochemistry. 2003;86(3):529–37.[PubMed]

9.  Lefevre F, Aronson N. Ketogenic diet for the treatment of refractory epilepsy in children: A systematic review of efficacy. Pediatrics. 2000;105(4):E46. [PubMed]

10. 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(2):304–17. [PubMed]