The ketogenic diet for infants: How long can you go?

Dressler, Anastasia; Trimmel-Schwahofer, Petra

doi:10.1016/j.eplepsyres.2020.106339

Cited by 19 publications

(19 citation statements)

References 48 publications

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“…Side effects were frequent in our cohort, but only four infants withdrew due to adverse effects or poor tolerability. Our data are similar to those published by Dressler in a recent review [30]. Most frequent side effects were hypoglycemia and gastrointestinal disturbances in a short-term, and dyslipidemia and hypercalciuria in a long-term.…”

Section: Discussionsupporting

confidence: 91%

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Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age

Ruiz-Herrero

Villarroya

Perez-Sebastian

et al. 2021

Seizure

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Ketogenic dietary therapies (KDT) are high-fat and low-carbohydrate diets that may achieve seizure control and improve cognitive state. We describe our KDT experience in infants (children less than two years of age). Research methods & procedures:We conducted a retrospective, descriptive and observational study of 42 infants treated with KDT between 2000-2018.Results: The types of KDT started were: classic ketogenic diet ratio 3:1 (40), ratio 4:1 (1) and modified ketogenic diet with medium-chain triglycerides (1). Four patients switched to a modified Atkins diet. During follow-up, 79%, 57%, 38% and 17% of infants remained on KDT at 3, 6, 12 and 24 months, respectively. Seizure reduction ≥50% compared to baseline was achieved in 50%, 45%, 38% and 17% at 3, 6, 12 and 24 months, respectively. Seizure control was excellent (reduction >90%) in 33%, 31%, 26% and 12%, and seizure-free infants were 9, 9, 10 and 4, at different follow-up intervals, respectively. Sixty-three percent of infants with West syndrome were responders to KDT. Mean length of KDT was 390 days (16 days-4.9 years). Ineffectiveness was the reason for withdrawal in 50% of patients. Early adverse effects (during first month) occurred in 40% of infants. The most frequent early side effects were asymptomatic hypoglycemia and gastrointestinal disturbances. Lateonset side effects occurred in 55-14% of infants during therapy, and most frequent were hypercalciuria and dyslipidaemia. Conclusion: KDT are useful and effective treatments in infancy. Side effects are frequent but mild and easy to manage.

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Section: Discussionsupporting

confidence: 91%

“…Maybe an earlier use of the diet would have improved the results of our patients. The most frequent used and recommended type of KDT in infancy is CKD ratio 3:1 [11,30] because it meets protein requirements for a proper growth. The ratio can be adjusted based on acceptance, adverse effects, ketosis, or intercurrent situations.…”

Section: Discussionmentioning

confidence: 99%

Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age

Ruiz-Herrero

Villarroya

Perez-Sebastian

et al. 2021

Seizure

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“…It is also of interest that the KD/ MCT diet, with its long-established application in the treatment of retractable epilepsies, may also inhibit mTOR pathway signalling, suggesting that the KD/MCT diet could be a useful early disease-modifying treatment. There is now overwhelming evidence regarding the feasibility and effectiveness of the KD in infants as young as 3 weeks old 116 , 144 , 145 ; a recent meta-analysis in infants less than 2 years of age with drug-resistant epilepsy reported ⩾50% responder rates of 59%, while 33% of infants attained seizure freedom. 146 A limitation of the KD, however, especially in older children and adults, is that it is difficult to maintain; the recent evidence that decanoic acid reduces mTORC1 activity in model systems, including astrocytes derived from TSC patients, suggests that a more sustainable diet rich in decanoic acid may be able to produce similar results to the KD, with better compliance.…”

Section: Discussionmentioning

confidence: 99%

“… 115 With this in mind, the KD has been reported to be effective and well tolerated in very young infants with refractory epilepsy, including in infants maintaining a breast milk diet. 116 …”

Section: Non-pharmacologic Agentsmentioning

confidence: 99%

Review of the treatment options for epilepsy in tuberous sclerosis complex: towards precision medicine

Schubert‐Bast

Strzelczyk

2021

Ther Adv Neurol Disord

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Tuberous sclerosis complex (TSC) is a rare genetic disorder caused by mutations in the TSC1 or TSC2 genes, which encode proteins that antagonise the mammalian isoform of the target of rapamycin complex 1 (mTORC1) – a key mediator of cell growth and metabolism. TSC is characterised by the development of benign tumours in multiple organs, together with neurological manifestations including epilepsy and TSC-associated neuropsychiatric disorders (TAND). Epilepsy occurs frequently and is associated with significant morbidity and mortality; however, the management is challenging due to the intractable nature of the seizures. Preventative epilepsy treatment is a key aim, especially as patients with epilepsy may be at a higher risk of developing severe cognitive and behavioural impairment. Vigabatrin given preventatively reduces the risk and severity of epilepsy although the benefits for TAND are inconclusive. These promising results could pave the way for evaluating other treatments in a preventative capacity, especially those that may address the underlying pathophysiology of TSC, including everolimus, cannabidiol and the ketogenic diet (KD). Everolimus is an mTOR inhibitor approved for the adjunctive treatment of refractory TSC-associated seizures that has demonstrated significant reductions in seizure frequency compared with placebo, improvements that were sustained after 2 years of treatment. Highly purified cannabidiol, recently approved in the US as Epidiolex® for TSC-associated seizures in patients ⩾1 years of age, and the KD, may also participate in the regulation of the mTOR pathway. This review focusses on the pivotal clinical evidence surrounding these potential targeted therapies that may form the foundation of precision medicine for TSC-associated epilepsy, as well as other current treatments including anti-seizure drugs, vagus nerve stimulation and surgery. New future therapies are also discussed, together with the potential for preventative treatment with targeted therapies. Due to advances in understanding the molecular genetics and pathophysiology, TSC represents a prototypic clinical syndrome for studying epileptogenesis and the impact of precision medicine.

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“…Certain ketogenic dietary protocols use medium-chain triglycerides (MCT) to help boost ketone production as they are more rapidly broken down into ketones and energy [ 8 ]. This form of dietary modifications was documented since 1925 to treat drug-resistant epilepsy [ 9 , 10 ]. However, a prolonged ketogenic diet is not endured well as there are several drawbacks such as retardation of growth in children and gastrointestinal side effects [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

A Concise Review of Ketogenic Dietary Interventions in the Management of Rare Diseases

Hettiarachchi

Lakmal

Dissanayake

2021

Journal of Nutrition and Metabolism

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Dietary interventions are now being used as an adjunct therapy in the treatment of rare diseases. One such method is the high-fat, moderate-protein, and very low-carbohydrate diet which produces ketosis and therefore called the ketogenic diet. Some of the more common conditions that are treated with this method are pharmacoresistant epilepsy, infantile spasms, glycogen storage diseases, and other forms of rare metabolic disturbances. With this review, we look at different uses of the ketogenic diet in treating rare diseases and the recommendations based on current evidence.

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The ketogenic diet for infants: How long can you go?

Cited by 19 publications

References 48 publications

Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age

Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age

Review of the treatment options for epilepsy in tuberous sclerosis complex: towards precision medicine

A Concise Review of Ketogenic Dietary Interventions in the Management of Rare Diseases

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