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

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. METHODS: Twenty children were treated prospectively in a hospital-based ambulatory clinic from September 2003 to May 2005. Children aged 3-18 years, with at least three seizures per week, who had been treated with at least two anticonvulsants, were enrolled and received the diet over a 6-month period. Carbohydrates were initially limited to 10 g/day, and fats were encouraged. Parents measured urinary ketones semiweekly and recorded seizures daily. All children received vitamin and calcium supplementation. RESULTS: In all children, at least moderate urinary ketosis developed within 4 days (mean, 1.9). Sixteen (80%) completed the 6-month study; 14 chose to remain on the diet afterward. At 6 months, 13 (65%) had >50% improvement, and seven (35%) had >90% improvement (four were seizure free). Mean seizure frequency after 6 months was 40 per week (p = 0.005). Over a 6-month period, mean serum blood urea nitrogen increased from 12 to 17 mg/dl (p = 0.01); creatinine was unchanged. Cholesterol increased from 192 to 221 mg/dl, (p = 0.06). Weight did not change significantly (34.0-33.7 kg); only six children lost weight. A stable body mass index over time correlated with >90% improvement (p = 0.004). CONCLUSIONS: A modified Atkins diet is an effective and well-tolerated therapy for intractable pediatric epilepsy.

Tuberous sclerosis complex and the ketogenic diet.

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. METHODS: A chart review was performed of patients with TSC treated with the ketogenic diet over a 5-year period at Johns Hopkins Hospital and Massachusetts General Hospital. RESULTS: Twelve children, ages 8 months to 18 years, were identified. Eleven (92%) children had a >50% reduction in their seizures at 6 months on the diet, and 8 (67%) had a >90% response. Five children had at least a 5-month seizure-free response. Diet duration ranged from 2 months to 5 years (mean, 2 years). CONCLUSIONS: In this limited-duration case series of 12 patients, the ketogenic diet was a generally effective therapeutic modality for the intractable epilepsy occasionally seen in children with TSC.

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

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. METHODS: A case-control study was performed of children with intractable epilepsy started on the ketogenic diet at our institution since June 2001. Patients with a dramatic response were defined as those becoming seizure free within 2 weeks of diet onset. These children were compared with all other patients treated with the diet over the same time period in terms of patient demographics, epilepsy characteristics, and diet parameters. RESULTS: Eighteen early, dramatic responders over a 3-year period were identified and compared with 89 patients who were not similarly improved. The absence of complex partial seizures as the predominant seizure type (0 vs. 23%; p = 0.02) correlated with this dramatic success. The presence of infantile spasms (39% vs. 20%; p = 0.09) approached significance, but all other variables did not. CONCLUSIONS: An early, dramatic response to the ketogenic diet is more likely in patients with predominant seizure types other than complex partial. It may also be more likely to occur in children who have infantile spasms. In all other patient demographics and diet parameters, an equal likelihood of similar success was found.

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.

Worldwide use of the ketogenic diet.

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. METHODS: With the use of the Internet, e-mail requests for information about international ketogenic diet centers (outside the United States) were made over a 9-month period. Assistance also was obtained from the Child Neurology Society and International League Against Epilepsy. Questions included patient enrollment (total and annually), year the diet was first offered, unique cultural and religious issues in the country, community opinion, and research interests. RESULTS: Successful communication was made with 73 academic centers in 41 countries outside the United States. Sixteen (39%) countries provided information from multiple centers. The median duration offering the diet was 8 years (range, 1-45 years). The average number of patients enrolled to date was 71.6 per country, with 5.4 new patients annually. Common difficulties included avoiding rice intake, tolerating higher fat-to-protein and carbohydrate ratios (e.g., 4:1), finding specific nutritional labels on foods, and handling the growing interest from large populations with limited resources. Nevertheless, cultural and religious issues were generally not limiting; physician and patient acceptance of the diet as an option is high; and most meals were similar among countries. Centers often had great pride in their programs, and international collaborative groups are forming rapidly. A website is now available with updated center information at http://www.neuro.jhmi.edu/Epilepsy/Peds/ketoworldwide.htm CONCLUSIONS: Despite occasional difficulties, the ketogenic diet is being used worldwide.

The impact of early versus late anticonvulsant reduction after ketogenic diet initiation.

The ideal timing of anticonvulsant reduction for a child started on the ketogenic diet is unclear. The records of 81 children started on the ketogenic diet consecutively over a 2-year period were reviewed for a 6-month period after diet initiation. During their first 6 months on the ketogenic diet, medications were tapered in 53 of 81 (65%) patients, with 30 of 53 (57%) considered "early" (tapered during diet initiation or within the first month afterward). No differences were seen between the early and late groups with respect to percentage with > 90% seizure reduction at 3 months (47% vs 48%), diet duration (1.1 vs 0.9 years), percentage remaining on the ketogenic diet to date (73% vs 65%), or improved alertness (63% vs 57%). Nine of fifty-three (17%) had transient seizure increases during medication tapering, with no correlation to the timing in which this occurred; however, five were taking benzodiazepines or barbiturates. All had > 50% seizure reduction at 3 months despite the transient worsening. Early reduction of anticonvulsants in children initiated on the ketogenic diet appears to be safe and well tolerated. However, it offers no specific advantage compared with a later taper.

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.

Efficacy of the ketogenic diet for infantile spasms.

OBJECTIVE: The objective of this study was to determine whether the ketogenic diet is safe, well-tolerated, and efficacious in the treatment of infantile spasms. METHODS: During a 4-year period, 23 children with infantile spasms, aged 5 months to 2 years, were started on the ketogenic diet; 9 (39%) had symptomatic infantile spasms, and 16 (70%) had hypsarrhythmia. Children had an average prediet exposure to 3.3 anticonvulsants. Two children were enrolled before any medication had been tried. Seizure reduction was analyzed retrospectively, using parent reports and electroencephalograms (EEGs) when available. RESULTS: At 3, 6, 9, and 12 months, 38%, 39%, 53%, and 46%, respectively, of all patients currently on the diet were >90% improved (3 were seizure-free at 12 months); 67%, 72%, 93%, and 100% were >50% improved. Fifty-six percent remained on the diet at 12 months, 46% of whom were >90% improved and 100% were >50%. Fifty percent of those with hypsarrhythmia and follow-up EEGs had EEG improvement. Fifty-seven percent had their medications reduced or discontinued by 12 months. Fifty-seven percent had improvement in development, which was correlated with seizure control. Independent factors that predicted improvement included age younger than 1 year and previous exposure to 3 or fewer anticonvulsants. No child has died, and 7 children had diet-related adverse reactions (nephrolithiasis, gastroesophageal reflux). DISCUSSION: The ketogenic diet is a safe, well-tolerated, and possibly effective potential alternative to other therapies for infantile spasms.