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.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.

OBJECTIVE Kidney stones are an adverse event with the ketogenic diet (KD), occurring in approximately 6% of children who are started on this therapy for intractable epilepsy. Potassium citrate (Polycitra K®) is a daily oral supplement that alkalinizes the urine and solubilizes urine calcium, theoretically reducing the risk of kidney stones. METHODS Children at Johns Hopkins Hospital starting the KD from 2000–2008, with at least 1 month of follow-up, were evaluated (n=313). From 2000–2005, children were treated with daily Polycitra K® at 2 meq/kg/day only in the setting of identified hypercalciuria; whereas since 2006 it has been started in all children empirically at KD onset. RESULTS Polycitra K® was administered to 198 children preventatively overall, of whom 4 (2.0%) developed kidney stones, compared to 11 of 105 (10.5%) who did not receive Polycitra K®, P= .003. Two children since 2006 refused Polycitra K®, one of whom developed a kidney stone. Successful empiric administration of Polycitra K® at KD onset resulted in a kidney stone incidence of 0.9% (1 of 106) compared to administration only due to hypercalciuria, 6.7% (13 of 195), P =.02. Polycitra K® resulted in less acidic urine (mean pH 6.8 vs. 6.2, P = .002), but not reduced serum acidosis. No side effects of oral citrates were reported. CONCLUSIONS Oral potassium citrate is an effective preventative supplement against kidney stones in children receiving the KD, achieving its goal of urine alkalinization. Universal supplementation is warranted.

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LGS Lennox-Gastaut syndrome AIM To determine the efficacy of the ketogenic diet for children with Lennox-Gastaut syndrome (LGS) at our institution and in the literature. METHODThe records of children with LGS initiated on the ketogenic diet at our institution from 1994 to 2010 were reviewed. Inclusion criteria included the presence of £2.5Hz spike-and-wave complexes on electroencephalogram, multiple seizure types including tonic, atonic, or atypical absence, developmental delay, and age under 1 year. We additionally reviewed the literature for cases of LGS treated with the ketogenic diet and their outcomes.RESULTS Seventy-one children (41 males, 30 females, median age 3y 6mo, range 18mo-18y), withLGS were initiated on the ketogenic diet. Using an intent-to-treat analysis, after 6 months, 36 (51%) achieved more than 50% seizure reduction, 16 (23%) experienced more than 90% seizure reduction, and 1 (1%) achieved seizure freedom. Results were similar after 12 months. Age, sex, side effects, valproate use, and history of infantile spasms were not predictive of more than 90% seizure reduction. In the literature, 88 of 189 (47%) children with LGS had more than 50% seizure reduction after 3 to 36 months of ketogenic diet treatment. INTERPRETATIONThe ketogenic diet is efficacious in the treatment of LGS, with approximately one-half of children responding at 12 months.The ketogenic diet is a high fat, low carbohydrate dietary treatment for intractable epilepsy, predominantly used in children. Since its development, the ketogenic diet has become widely used in a variety of epilepsy syndromes including myoclonicastatic epilepsy, Dravet syndrome, and infantile spasms. 1,2 Since its introduction 90 years ago, one of the most common epilepsy syndromes for which the ketogenic diet is used in clinical practice is Lennox-Gastaut syndrome (LGS).LGS was first reported in 1950, though the electroclinical features were first described by the Marseille School in France in 1966. 3,4 The criteria for the syndrome have been modified on several occasions; however, the International League Against Epilepsy Classification Commission adopted a formal definition in 1989. 5 Most clinicians agree that patients with LGS have an age at onset of less than 8 years (peak 3-5y), multiple seizure types that include tonic seizures (with often atypical absence, atonic, and other seizure types), intellectual disability, and the presence of diffuse, slow spike-wave complexes on electroencephalogram (EEG). [5][6][7] While many large ketogenic diet trials have included children with LGS, outcome data were primarily reported by seizure type rather than any specific epilepsy syndrome. 8 As a result, when the International Ketogenic Diet Consensus Guideline was published in 2009, LGS was not included as either a clear or possible 'indication' for the ketogenic diet primarily owing to insufficient data. 1 This study sought to determine the efficacy of the ketogenic diet for children with LGS at our institution and in the literature. We hypothesized that the ...

SUMMARYPurpose: The ketogenic diet has well-established shortand long-term outcomes for children with intractable epilepsy, but only for those actively receiving it. However, no information exists about its long-term effects years after it has been discontinued. Methods: Living subjects were identified who were treated at the Johns Hopkins Hospital with the ketogenic diet from November 1993 to December 2008 for ‡1 month, and had discontinued it ‡6 months prior to this study. Of 530 patients who were eligible, 254 were successfully contacted by phone or e-mail with a survey and request for laboratory studies. Results: Questionnaires were completed by 101 patients, with a median current age of 13 years (range 2-26 years). Median time since discontinuing the ketogenic diet was 6 years (range 0.8-14 years). Few (8%) still preferred to eat high fat foods. In comparison to the 52% responder rate (>50% seizure reduction) at ketogenic diet discontinuation, 79% were now similarly improved (p = 0.0001). Ninety-six percent would recommend the ketogenic diet to others, yet only 54% would have started it before trying anticonvulsants. Lipids were normal (mean total cholesterol 158 mg/dl), despite most being abnormal while on the ketogenic diet. The mean Z scores for those younger than age 18 years were )1.28 for height and )0.79 for weight. In those 18 years of age or older, the mean body mass index (BMI) was 22.2. Discussion: This is the first study to report on the longterm effects of the ketogenic diet after discontinuation. The majority of subjects are currently doing well with regard to health and seizure control.