The Efficacy of the Ketogenic Diet—1998: A Prospective Evaluation of Intervention in 150 Children

Freeman, John M.; Vining, Eileen P.G.; Pillas, Diana J.; Pyzik, Paula L.; Casey, Jane; Kelly, Louise A.

doi:10.1542/peds.102.6.1358

Cited by 561 publications

(417 citation statements)

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“…The KGD has a greater efficacy in younger children, with widespread onset seizures, either multifocal or generalized, rather than with a well-defined focal onset (12). However, with starvation and ketosis, patients are predisposed to metabolic acidosis during treatment with the KGD, especially during induction (9).…”

Section: Discussionmentioning

confidence: 99%

Concomitant Treatment with Topiramate and Ketogenic Diet in Pediatric Epilepsy

et al. 2002

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Summary:Purpose: Topiramate (TPM) is widely used as add-on therapy for epilepsy. TPM inhibits carbonic anhydrase, which may result in metabolic acidosis from decreased serum bicarbonate. The ketogenic diet (KGD) predisposes patients to metabolic acidosis, especially during induction. In children with refractory epilepsy, cotreatment with TPM and KGD may be considered, but special attention should be paid to the combined risks for metabolic acidosis and nephrolithiasis. We report our experience in 14 children cotreated with TPM and the KGD.Methods: Medical records of 14 children cotreated with the KGD and TPM for medically refractory epilepsy were reviewed retrospectively. Bicarbonate levels were analyzed and correlated with clinical profiles, including duration of cotreatment, TPM dose, KGD ratio, and seizure control.Results: Nine children had a <20% decrease in bicarbonate levels, from 5.3 to 12.3 mEq/L (mean, 7.6 mEq/L). Cotreatment was continued in all patients for duration of 33 to 544 days (seven had remained on cotreatment at the end of the study period), although two children required bicarbonate supplements to continue the KGD. No patient had nephrolithiasis.Conclusions: Although a large decrease in bicarbonate level occurred in the majority of children, the decrease appeared mostly at the time of KGD induction when added to prior TPM therapy. Bicarbonate levels should be monitored carefully with TPM and KGD cotreatment, and bicarbonate supplements given when symptomatic.

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Section: Discussionmentioning

confidence: 99%

Concomitant Treatment with Topiramate and Ketogenic Diet in Pediatric Epilepsy

et al. 2002

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“…The ketogenic diet (KD) is an established nonpharmacologic treatment for patients with intractable epilepsy (1,2) and was designed to mimic the biochemical changes seen upon fasting, specifically the formation of ketone bodies: ␤-hydroxybutyrate (BHB), acetoacetate (ACA), and to a lesser extent, acetone. Despite decades of clinical experience with the KD, the mechanisms underlying its anticonvulsant activity remain poorly understood.…”

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confidence: 99%

Acetoacetate, Acetone, and Dibenzylamine (a Contaminant in l‐(+)‐β‐Hydroxybutyrate) Exhibit Direct Anticonvulsant Actions in Vivo

et al. 2002

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Summary:Purpose: To investigate whether ketone bodies are directly anticonvulsant.Methods: We tested the effects of acetoacetate (ACA), acetone, and both stereoisomers, D-(−)-and L-(+), of ␤-hydroxybutyrate (BHB) on sensory-evoked seizures in Frings audiogenic seizure-susceptible mice.Results: We found that these ketone bodies, with the exception of the D-(−)-isomer of BHB, were anticonvulsant in this model. Furthermore, with gas chromatography-mass spectrometry, we confirmed that the activity of L-(+)-BHB was due to dibenzylamine, a chemical contaminant.Conclusions: Our data indicate that the anticonvulsant efficacy of the ketogenic diet may be due in part to the direct actions of ACA and acetone.

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“…Patients who initiated the classic ketogenic diet at another institution but were then treated at the Johns Hopkins Hospital were included. 2 Records were reviewed for patient demographics, efficacy (parental seizure calendars), side effects, and most recent laboratory values (including lipid profiles, liver enzymes, urine calcium:creatinine ratios, and ketosis level). After 2 years on the ketogenic diet, follow-up visits and laboratory studies were typically obtained every 6 to 12 months.…”

Section: Methodsmentioning

confidence: 99%

“…1 Previous studies have demonstrated that the efficacy of the diet in providing seizure control for children with difficult-to-manage seizures is at least equal to many of the newer anticonvulsant medications and often allows a decrease in medication. 2,3 Most epilepsy centers advocate use of the diet for 2 to 3 years if it controls seizures, followed by a gradual discontinuation in a manner similar to the withdrawal of anticonvulsants. Effects of the diet have been reported in patients who were on it for as long as 3 to 6 years' duration.…”

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confidence: 99%

Long‐term use of the ketogenic diet in the treatment of epilepsy

Groesbeck

Bluml

Kossoff

2006

Develop Med Child Neuro

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Long-term outcomes of the ketogenic diet in the treatment of epilepsy have not previously been reported. A retrospective chart review of children treated with the ketogenic diet for more than 6 years at the Johns Hopkins Hospital was performed. The response was documented at clinic visits and by telephone contacts; laboratory studies were obtained approximately every 6 to 12 months. Satisfaction and tolerability were assessed by means of a brief parental telephone questionnaire. In all, 28 patients (15 males, 13 females), currently aged 7 to 23 years, were identified. The median baseline seizure frequency per week at diet onset was 630 (range 1-1400). Diet duration ranged from 6 to 12 years; 19 remain on the diet currently. After 6 years or more, 24 children experienced a more than 90% decrease in seizures, and 22 parents reported satisfaction with the diet's efficacy. Ten children were at less than the 10th centile for height at diet initiation; this number increased to 23 at the most recent follow-up (p=0.001). Kidney stones occurred in seven children and skeletal fractures in six. After 6 years or more the mean cholesterol level was 201mg/dl, high-density lipoprotein was 54mg/dl, low-density lipoprotein was 129mg/dl, and triglycerides were 97mg/dl. Efficacy and overall tolerability for children are maintained after prolonged use of the ketogenic diet. However, side effects, such as slowed growth, kidney stones, and fractures, should be monitored closely.The ketogenic diet is a high-fat, low-carbohydrate, adequateprotein diet that was developed more than 80 years ago as a method of managing intractable epilepsy in children. 1 Previous studies have demonstrated that the efficacy of the diet in providing seizure control for children with difficult-to-manage seizures is at least equal to many of the newer anticonvulsant medications and often allows a decrease in medication. 2,3 Most epilepsy centers advocate use of the diet for 2 to 3 years if it controls seizures, followed by a gradual discontinuation in a manner similar to the withdrawal of anticonvulsants. Effects of the diet have been reported in patients who were on it for as long as 3 to 6 years' duration. 4 However, the ketogenic diet has been the only successful therapy for many children with epilepsy, and often these families continue the diet for longer periods.There are many questions regarding the long-term use of the ketogenic diet. Is it feasible for patients to remain on the diet for prolonged periods of time? Does the efficacy of the diet fluctuate over time? Does the diet become more tolerable over time, or do families eventually tire of the strict regimen of calculating ratios and weighing out foods? Established side effects of the ketogenic diet include kidney stones, dyslipidemia, changes in bone density, and slowed growth. [5][6][7][8][9][10][11][12][13] Most studies reporting on side effects have a follow-up period of less than 2 years, whereas a recent review examined diet complications after up to 4 years of treatment. 13 Are children on t...

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The Efficacy of the Ketogenic Diet—1998: A Prospective Evaluation of Intervention in 150 Children

Abstract: The ketogenic diet should be considered as alternative therapy for children with difficult-to-control seizures. It is more effective than many of the new anticonvulsant medications and is well tolerated by children and families when it is effective.

Cited by 561 publications

References 17 publications

Concomitant Treatment with Topiramate and Ketogenic Diet in Pediatric Epilepsy

Concomitant Treatment with Topiramate and Ketogenic Diet in Pediatric Epilepsy

Acetoacetate, Acetone, and Dibenzylamine (a Contaminant in l‐(+)‐β‐Hydroxybutyrate) Exhibit Direct Anticonvulsant Actions in Vivo

Long‐term use of the ketogenic diet in the treatment of epilepsy

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