Who Is Ketogenic Therapy Suitable For?
Ketogenic therapy should be considered as a treatment for epilepsy after two appropriate anticonvulsant medications have failed to be effective or produced unacceptable side effects. This treatment has been shown to be successful in treating a wide range of seizure types and syndromes (references 1, 2, 3) although may be particularly beneficial in myoclonic epilepsies, infantile spasms and tuberous sclerosis complex (reference 4). Guidelines from the UK National Institute for Health and Care Excellence (NICE), updated in 2012, recommend ‘Refer children and young people with epilepsy whose seizures have not responded to appropriate anti-epileptic drugs to a tertiary paediatric epilepsy specialist for consideration of the use of a ketogenic diet.’ (http://www.nice.org.uk).
The ketogenic diet is a first-line treatment for the neurometabolic diseases glucose transporter type 1 (GLUT1) deficiency syndrome and pyruvate dehydrogenase (PDH) deficiency. Although clinical evidence is more limited, ketogenic therapy is also increasingly being explored as a treatment option for other disorders such as neurological cancers.
The traditional or modified ketogenic diets should not be used in individuals who have fatty acid oxidation defects, pyruvate carboxylase deficiency, primary carnitine deficiencies, organic acidurias, defects in ketone metabolism, disorders that require high dietary carbohydrates as part of their treatment, severe liver disease or hypoglycaemia under investigation (4). They should be used with caution in those with a history of renal stones, hyperlipidemia, severe gastro-oesophageal reflux, diabetes mellitus and parent or care-giver non-compliance. Concomitant steroid use will limit ketosis. Although possible, they will be more difficult to implement if there are pre-existing dietary restrictions and food allergy, especially if associated with behavioural feeding issues. A ketogenic diet can be administered to enterally-fed individuals via naso-gastric tube or gastrostomy.
Both traditional and modified diets have been shown to be successful in the treatment of epilepsy. Choice will depend on the individual’s age, dietary preference, and lifestyle, taking into account palatability and ease of use for both the child/adult and the family. The classical ketogenic diet is usually preferred in infants and classical ketogenic formulae are used for tube feeding. Modified regimes have benefits for the older child and adult who prefer a less restricted diet.
1. Is the ketogenic diet effective in specific epilepsy syndromes?
1. Efficacy of the ketogenic diet: which epilepsies respond?
2. Do patients with absence epilepsy respond to ketogenic diets?
3. Optimal clinical management of children receiving the ketogenic diet: Recommendations
of the International Ketogenic Diet Study Group.
Kossoff EH, Zupec-Kania BA, Amark PE, et al. Epilepsia. Feb 2009; 50(2):304-17.