The Ketogenic Diet Including Breast Milk for Treatment of Infants with Severe Childhood Epilepsy: Feasibility, Safety, and Effectiveness

Dressler, Anastasia; Häfele, Chiara; Giordano, Vito; Benninger, Franz; Trimmel-Schwahofer, Petra; Gröppel, Gudrun; Samueli, Sharon; Feucht, Martha; Male, Christoph; Repa, Andreas

doi:10.1089/bfm.2019.0190

Cited by 14 publications

(17 citation statements)

References 35 publications

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“…In patients who were established on KMT and those who were new to KMT, the WBME formula was well tolerated. Our data are consistent with data from other studies of formula-fed patients on KMT, which also showed low dropout rates (0-20%) and high diet response rates (67-90%) [4,11,[13][14][15]. These results are likely related to improved compliance and fewer errors in food preparation because the patients received the majority of their total calories from formula rather than solid foods, providing a more consistent and rigid adherence to KMT.…”

Section: Discussionsupporting

confidence: 90%

“…Among patients with cerebral palsy, constipation was reported in 40% of patients who were fed exclusively with formula versus 23% of patients who were orally fed (P = 0.03) [25]. Constipation rates were reported to be between 25% and 67% in 3 similar studies that involved patients receiving ketogenic formula; these rates were similar to our results of 69% [95% CI, 48-86%] [11,13,15]. Our study categorized constipation as infrequent or hard stools that required laxative use for management.…”

Section: Discussionsupporting

confidence: 87%

“…Of note, 2 of 10 patients dropped out of the study, leaving 8 patients in the formula group, all of whom were diet responsive. A recently published study of 79 patients receiving either 100% ketogenic formula or at least 90% ketogenic formula plus up to 10% total calories from breastmilk had a combined diet response rate of 68% [15]. Our combined diet response rate of 96% supports that patients who receive a majority of calories from ketogenic formula may have improved seizure control related to greater compliance, ease of administration, or fewer errors in meal preparation.…”

Section: Discussionsupporting

confidence: 64%

“…Benefits of a liquid ready-to-feed ketogenic formula include palatability, ease of diet titration, reduced risk of calculation or preparation error, and improved compliance, all of which can potentially improve efficacy [3,4]. In contrast to studies reporting efficacy rates of 31.5-70% for patients consuming a solid-food ketogenic diet [5][6][7][8][9][10][11][12], diet response rates among those who use formulas to provide a majority of total energy intake appear higher, between 67% and 90% [4,11,[13][14][15].…”

Section: Introductionmentioning

confidence: 96%

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Efficacy and tolerability of a whey-based, medium-chain triglyceride–enhanced ketogenic formula in children with refractory epilepsy: A retrospective study

Wheeler

Temkit

Wilfong

et al. 2021

Seizure

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Ketogenic metabolic therapy (KMT) has demonstrated effectiveness in seizure reduction. However, patient compliance and adverse effects limit its use. Ready-to-feed (RTF) ketogenic formulas improve compliance and include components that mitigate adverse effects. This study is the first to evaluate the efficacy and tolerability of an RTF, whey-based, medium-chain triglyceride-enhanced (WBME) ketogenic formula. Methods: Retrospective data from patients who received KMT between January 1, 2015, and February 28, 2018, were analyzed. Patients who received ≥75% of their total calories from the WBME formula and who were monitored for 3 months were included. Outcome measures were gastrointestinal issues, acidosis, serum blood glucose and beta-hydroxybutyrate levels, unintentional weight changes, diet response (≥50% reduction in seizures), seizure freedom, and change in formula or discontinuation of therapy. Patients with incomplete outcome data or who received <75% of total calories from the formula were excluded. Results: Twenty-six patients (13 males; mean [SD] age, 6.1 [5.8] years) met the inclusion criteria. Thirteen patients were established patients who received a standard ketogenic formula before changing to the WBME formula; 13 were patients new to KMT whose therapy was initiated using the WBME formula. This formula was well tolerated; no patient in either group discontinued therapy or required a change in formula. The combined diet response rate (95% CI) for established and new patients was 96% (80-100%). Seizure-freedom (95% CI) for both groups at 3 months posttreatment was 20% (7-41%). The most prevalent adverse effect was constipation (69% [95% CI, 48-86%]). Conclusion:The WBME ketogenic formula appears to be effective and well tolerated by pediatric patients with refractory epilepsy.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 87%

Section: Discussionsupporting

confidence: 64%

Section: Introductionmentioning

confidence: 96%

See 2 more Smart Citations

Efficacy and tolerability of a whey-based, medium-chain triglyceride–enhanced ketogenic formula in children with refractory epilepsy: A retrospective study

Wheeler

Temkit

Wilfong

et al. 2021

Seizure

View full text Add to dashboard Cite

show abstract

“…Recent data, however, suggest that breastfeeding may be continued along with ketogenic diet without loss of ketosis. 21…”

Section: Discussionmentioning

confidence: 99%

Evaluation of the Modified Atkins Diet for the Treatment of Epileptic Spasms Refractory to Hormonal Therapy: A Randomized Controlled Trial

et al. 2021

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Objectives: We aimed to evaluate the efficacy of the modified Atkins diet in children with epileptic spasms who had failed hormonal therapy. Methods: Children aged 9 months to 3 years having daily epileptic spasms despite a trial of ACTH or oral prednisolone and 1 additional anticonvulsant medication were enrolled. Children were randomly assigned to receive the modified Atkins diet either immediately or after a delay of 4 weeks. The ongoing anticonvulsant medications were continued unchanged. The primary outcome variable was the proportion of children who achieved spasm freedom as per parental reports at 4 weeks. Secondary outcomes included time to spasm cessation, proportion of children with electroclinical remission, the proportion of children with >50% reduction of spasms at 4 weeks, and adverse effects of the diet. ( ClinicalTrials.gov Identifier: NCT03807141). Results: A total of 91 children were enrolled in the study; 46 in the diet group and 45 in the control group. At the end of 4 weeks, 11 children in the diet group were spasm free compared with none in the control group ( P ≤ .001). The median time to achieve spasm cessation was 10 days (interquartile range 9-20). Nine of these had resolution of hypsarrhythmia on electroencephalography (EEG). Thirty (65.2%) in the diet group had >50% reduction in spasms, compared with none in the control group ( P < .001). The most common side effect was constipation, noted in 34.8% of the children. Conclusions: The modified Atkins diet was found to be effective and well tolerated in children with epileptic spasms refractory to hormonal therapy.

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Use of ketogenic dietary therapy for drug‐resistant epilepsy in early infancy

Armeno,

Calligaris,

Gagiulo

et al. 2023

Epilepsia Open

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ObjectiveThere is growing evidence that ketogenic dietary therapy (KDT) can be safely and efficiently used in young children, but little evidence exists on its use in newborns. Developmental and epileptic encephalopathies starting in the neonatal period or early infancy usually present a poor prognosis. The aim of this study was to evaluate effectiveness, safety, and survival of infants younger than 3 months of age with drug‐resistant epilepsy in whom KDT was used.MethodsA retrospective study was conducted to evaluate neonates and infants younger than 3 months who started KDT for drug‐resistant developmental and epileptic encephalopathies at three referral centers. Data were collected on demographic features, time of epilepsy onset, epilepsy syndrome, seizure type, seizure frequency at diet onset, etiology, details regarding diet initiation, type of ketogenic formula, breastfeeding, route of administration, blood‐ketones, growth, length of NICU stay, and survival.ResultsNineteen infants younger than 12 weeks of life who received KDT with a minimum follow‐up of one month were included; 13 had early‐infantile developmental and epileptic encephalopathy, four epilepsy of infancy with migrating focal seizures, and two focal epilepsy. A >50% response was observed in 73.7 % at 1 month on the diet; 37% achieved a >75% seizure reduction, and 10.5% became seizure free. At 3 months, a >50% decrease in seizure frequency was observed in 72.2%; 15.8% had a >75% reduction and 21% became seizure free. Overall survival was 76% at 1 year on diet. Incidence of acute and late adverse effects was low and most adverse effects were asymptomatic and manageable.SignificanceOur experience suggests that KDT is safe and effective in newborns and very young infants; however, further studies on the management of the diet in this vulnerable age group are necessary.

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The Ketogenic Diet Including Breast Milk for Treatment of Infants with Severe Childhood Epilepsy: Feasibility, Safety, and Effectiveness

Cited by 14 publications

References 35 publications

Efficacy and tolerability of a whey-based, medium-chain triglyceride–enhanced ketogenic formula in children with refractory epilepsy: A retrospective study

Efficacy and tolerability of a whey-based, medium-chain triglyceride–enhanced ketogenic formula in children with refractory epilepsy: A retrospective study

Evaluation of the Modified Atkins Diet for the Treatment of Epileptic Spasms Refractory to Hormonal Therapy: A Randomized Controlled Trial

Use of ketogenic dietary therapy for drug‐resistant epilepsy in early infancy

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