Jane R. McGrogan

A modified Atkins diet is effective for the treatment of intractable pediatric epilepsy.

Summary:  Purpose: The Atkins diet may induce ketosis as does the ketogenic diet, without restrictions on calories, fluids, protein, or need for an inpatient fast and admission. Our objective was to evaluate the efficacy and tolerability of a modified Atkins diet for intractable childhood epilepsy.

Efficacy of the Atkins diet as therapy for intractable epilepsy

The ketogenic diet is effective for treating seizures in children with epilepsy. The Atkins diet can also induce a ketotic state, but has fewer protein and caloric restrictions, and has been used safely by millions of people worldwide for weight reduction. Six patients, aged 7 to 52 years, were started on the Atkins diet for the treatment of intractable focal and multifocal epilepsy. Five patients maintained moderate to large ketosis for periods of 6 weeks to 24 months; three patients had seizure reduction and were able to reduce antiepileptic medications. This provides preliminary evidence that the Atkins diet may have a role as therapy for patients with medically resistant epilepsy.

Growth of children on the ketogenic diet

This is a prospective cohort study of 237 children (130 males, 107 females) placed on the ketogenic diet for control of intractable epilepsy (mean age at starting diet 3 years 8 months; age range 2 months to 9 years 10 months); average length of follow-up was 308 days. There were 133 children on the diet at 1 year and 76 at 2 years. Height and weight measurements were converted into age- and sex-appropriate z scores. There was a rapid drop in weight z scores in the first 3 months. After this initial period, the weight z score remained constant in children who started the diet below the median weight for their age and sex, although z scores continued to decrease in children starting above the median. There was a small decrease in height z scores in the first 6 months (<0.5); however, there were larger changes by 2 years. There was no difference based on sex for either height or weight. The ketogenic diet generally provides sufficient nutrition to maintain growth within normal parameters over a defined period. Very young children grow poorly on the diet and should be followed-up carefully over long periods of use.

Worldwide use of the ketogenic diet

Summary:  Purpose: Over the past decade, the use of the ketogenic diet internationally has increased dramatically. The purpose of this survey was to evaluate the use of the diet worldwide.

Tuberous sclerosis complex and the ketogenic diet.

Summary:  Purpose: Tuberous sclerosis complex (TSC) is a condition that is frequently associated with intractable, early‐onset epilepsy, and often is first seen as infantile spasms. If medications fail and no clear epileptogenic tuber is identified, nonpharmacologic therapies are often attempted. The use of the ketogenic diet specifically for children with TSC and epilepsy has not been previously described.

Benefits of an All‐Liquid Ketogenic Diet

To the Editor: One of several barriers to the use of the ketogenic diet is its perceived unpalatability. We are still constantly surprised, however, by the lack of realization not only by pediatricians, but also by child neurologists and dietitians, that the diet can be easily provided as a liquid rather than heavy whipping cream, eggs, and tuna. Often physicians do not realize that the liquid form is perhaps the easiest method of providing the diet, most commonly for infants that are formula fed as well as for older children with gastrostomy tubes (1–3). Parents are often frustrated at their prior care after the diet is started conveniently and effectively as a liquid, after multiple medications had been tried and failed. Only a single abstract at the 2001 Child Neurology Society annual meeting described this so far, and this information apparently should be spread. We reviewed the 226 patients started on the ketogenic diet at our institution from October 1998 to February 2004. Sixty-one (27%) children were totally formula fed, with almost exactly half (31 patients) formula-fed infants (mean age, 1.1 years), and the remainder were gastrostomy tube–fed children (mean age, 3.8 years). Approximately half of the infants had recalcitrant infantile spasms, and half of the older gastrostomy tube–fed children had either Lennox–Gastaut syndrome or intractable myoclonic epilepsy. All patients were provided a combination of Ross Carbohydrate-Free, Mead Johnson Microlipid, and Ross Polycose formula, as calculated by our dietitian to a 3:1 or 4:1 ratio of fat to protein and carbohydrate. We found these 61 children had better seizure control than that described for the typical solid food–fed child, with 59% having >90% control at 12 months, compared with 27% in a larger cohort (4). Whereas 30% of children on the standard ketogenic diet discontinue because of restrictiveness, none of these 61 children did (4). Patients with excessive weight loss (or gain), low ketones, or hyperlipidemia had their diet quickly and easily titrated, as clinically necessary. Many benefits are found in an all-liquid ketogenic diet. The formula is very palatable, with a taste similar to that of most other infant formulas. Beyond the infant age group, however, solid foods tend to be more palatable. In addition, it is easy to calculate for the dietitian, with three components combined, as compared with a plethora of individually planned meals and snacks. Less room for error occurs, and education is involved for parents. The presence of a gastrostomy tube also allows medications to be provided without carbohydrate sweeteners or flavoring. Patients who are ill on the ketogenic diet can occasionally have acidosis and dehydration, and having a gastrostomy tube helps avoid this. However, we do not advocate gastrostomy tubes for our patients with epilepsy unless deemed medically necessary. Last, insurance companies often cover formula, as it is being used as a medical therapy rather than for solely nutritional purposes. In what other situations could a liquid ketogenic diet be beneficial? In any child in an intensive care setting for epilepsy, the diet can be easily started or continued via a temporary nasogastric tube. As the diet can be attempted for adults, perhaps a gastrostomy tube would be an ideal manner of providing such therapy (5). A liquid preparation also can be used as a supplement to solid foods.

Can you predict an immediate, complete, and sustained response to the ketogenic diet?

Summary:  Purpose: Although the ketogenic diet has been in use for >80 years, little agreement exists as to which patients are most likely to have dramatic, sudden, and complete seizure control.

Experience in the use of the ketogenic diet as early therapy.

The ketogenic diet has traditionally been considered an anticonvulsant therapy of last resort, despite excellent efficacy and limited side effects. We hypothesized that the ketogenic diet would have similar results in patients with new-onset epilepsy. A retrospective study was conducted of patients started on the ketogenic diet since 1994. Thirteen of 460 (2.8%) patients were started on the ketogenic diet as early (zero or one prior anticonvulsant) therapy for seizures. Of those remaining on the diet, 60% (6 of 10) had a > 90% seizure reduction at 6 months and 100% (6 of 6) had a > 90% reduction at 12 months. Patients with infantile spasms were as likely to achieve > 50% seizure reduction at 6 months as patients with other seizure types (75% vs 60%; P = .6). The ketogenic diet can be a valuable therapy before epilepsy becomes intractable. In the 13 patients reported, efficacy without side effects was achieved similarly to that with patients with intractable epilepsy. (J Child Neurol 2005;20:31—34).

Energy requirements of spasticity

Direct measurement of energy expended by spasticity in children with severe spastic quadriparesis is difficult. Insertion of an intrathecal baclofen pump in a 13-year-old boy with severe spasticity and profound mental retardation resulted in an estimated 30 to 40% decrease in his spasticity. As he had been on a carefully calculated ketogenic diet and fed by gastrostomy, his precise caloric intake was known. Decrease in spasticity, on the same caloric intake, led to marked weight gain. Reduction of 100 calories intake resulted in new weight stability. It was possible therefore, to estimate indirectly energy used by his spasticity. This 100 calories, representing 34% of calories above his resting energy requirement, corresponded to an independently estimated 30 to 40% of caloric expenditure of his spasticity. It was concluded that when calculation of calories is critical, energy utilization by spasticity must be taken into consideration.

The implementation and maintenance of the Ketogenic Diet in children.

The Ketogenic Diet is an effective treatment for epilepsy in children. At Johns Hopkins Hospital more than 400 children have been placed on the Ketogenic Diet. The implementation and maintenance of this treatment require significant collaboration between the family and Epilepsy Team. During initiation of the diet, in the hospital, parents attend classes on the history and mechanism of the diet, preparation of meals, psychological issues, complications and the management of childhood illnesses on the ketogenic diet. Many factors are considered in calculating a ketogenic formula. Age, weight, height and activity level are the obvious factors. However, secondary medical diagnoses, medications, neurological deficits, feeding issues and psychological issues are additional factors that affect the formulation of an optimal ketogenic prescription. Once this prescription has been formulated and implemented, many patients require fine-tuning of their ketogenic diets to get the best antiepileptic results while promoting growth. We believe that our success in sustaining this treatment is related to our fine-tuning and management practices.