Medicine Section - Ketogenic Diet for Seizure Control

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Description
The ketogenic diet is a high-fat, low-carbohydrate, low-protein diet that has been used to treat some cases of refractory epilepsy. The composition of the diet induces ketosis, a physiologic state in which circulating ketone bodies are used as the primary fuel source in the absence of simple sugars. Ketosis may inhibit seizures through an unknown mechanism. The diet was developed in the 1920s, but used infrequently subsequent to the introduction of anti-epileptic drugs. Over the last several decades, the ketogenic diet has gained attention as a treatment option in patients with epilepsy that is refractory to medications.

The ketogenic diet is quite restrictive, requiring the cooperation of the patient, family, and an appropriately trained dietitian. The ratio of fat to carbohydrates must be strictly maintained, meaning that the precise contents of each food item must be known, and exactly measured. Given the restrictions, compliance with the diet can be problematic, especially in children over 10 years of age who have well-established dietary habits and preferences.

As currently practiced, the diet is initiated in the hospital setting. Children are admitted to the hospital and fasted for 1 to 2 days. The diet is then instituted gradually over a number of days. A full ketogenic diet is attained by approximately day 5 in most children, at which time the patient is discharged home and followed as an outpatient. The main reason for hospitalization is the period of fasting. Fasting potentially exposes children to dehydration and metabolic derangements that could become life threatening if not properly monitored and treated.

Policy/Criteria
Active policy - no longer scheduled for routine literature review.

A ketogenic diet may be considered medically necessary in children with refractory epilepsy. Refractory epilepsy is defined by inadequate control of seizures despite attempts at seizure control using multiple conventional anticonvulsants.

Scientific Background
This policy is based on a 1998 BlueCross BlueShield Association Technology Evaluation Center (TEC) assessment that offered the following conclusions:

• While the published data regarding ketogenic diets consists of uncontrolled case series, the data are consistent in showing that some children benefit from the ketogenic diet, as demonstrated by a significant reduction in seizure frequency; e.g., complete cessation of seizures in 16% of children, a greater than 90% reduction in 32%, and a greater than 50% reduction in 56%.
• These results exceed any expected placebo effect or spontaneous remission of seizures.

As currently practiced, the ketogenic diet is typically initiated in an inpatient setting, principally to monitor the patient during the initial fasting period, but also presumably to provide the intense education required to maintain a ketogenic diet once discharged. There are currently no data that focus on initiating the diet in the outpatient environment. However, the published studies do not explicitly delineate the adverse effects that occurred during the inpatient stay, and whether their management required hospitalization. Another possibility is the gradual initiation of the diet such that fasting (and hospitalization) would not be required. This approach should, in principle, achieve the same endpoint of ketosis, although over a longer time period. However, it is also possible that the fast itself is responsible for some degree of response seen in the published studies.

As part of the TEC assessment, 14 programs offering ketogenic diets were surveyed. Thirteen of the 14 programs reported that they always or virtually always instituted the diet in the inpatient setting. Four programs reported that they would rarely institute the diet in the outpatient setting under special circumstances, such as when it was being restarted after a period off the diet. One program reported that they routinely initiate the diet in the outpatient setting and that they feel that their results were comparable to other centers that followed the inpatient protocol. This program reported that they have not published any data on their outcomes nor formally presented outcome data in any scientific forums. While these data do not represent a comprehensive catalogue of practice patterns, it is clear that the most common approach is initiation of the diet in the inpatient setting, but that there are instances in which the diet has been successfully initiated in the outpatient setting.

An updated MEDLINE search found no new studies that conflict with the above analysis. Lefevre and Aronson published a review article in 2000 that systematically reviewed and synthesized the available evidence on the efficacy of the ketogenic diet in reducing seizure activity in children with refractory epilepsy. (2) The evidence consisted entirely of uncontrolled studies. Of the 11 studies identified for the review, 9 were retrospective series from a single institution, two were prospective studies, one of which was a multicenter trial. The results of these studies were consistent in showing that some children benefit from the ketogenic diet, demonstrated by a significant reduction in seizure frequency. Estimates of the rates of improvement by combined analysis were as follows: complete cessation of all seizures in 16% of children; >90% reduction in seizures in 32%; and >50% reduction in seizures in 56%. The authors doubted that this degree of benefit could be attributed to a placebo effect and concluded that the evidence was sufficient to determine that the ketogenic diet is efficacious in reducing seizure frequency in children with refractory epilepsy.

References

1. TEC Assessment, 1998; Tab 20
2. Lefevre F; Aronson N. Ketogenic diet for the treatment of refractory epilepsy in children: a systematic review of efficacy. Pediatrics 2000;105(4):E46

Cross References
None

Codes	Number	Description
CPT	No code
HCPCS	No code

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