Feasibility of ketogenic diet therapy variants for refractory epilepsy in neonates to infants under 2 years old

Hsieh, Tzu-Yun; Su, Ting-Yu; Hung, Kuo‐Chi; Hsu, Mei-Chin; Lin, Ying-Jui; Kuo, Hsuan-Chang; Hung, Po-Cheng

doi:10.1016/j.yebeh.2023.109315

Cited by 4 publications

(2 citation statements)

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“… 49 and encourages the implementation of KD in patients with KCNT1‐related epilepsy. Its feasibility, safety, and efficacy for pharmacoresistant epilepsy in infants under 2 years old, especially for those with genetic etiology, has already been shown, 51 and this also applies to KCNT1 ‐related epilepsies.…”

Section: Discussionmentioning

confidence: 95%

Efficacy of anti‐seizure medications and alternative therapies (ketogenic diet, CBD, and quinidine) in KCNT1‐related epilepsy: A systematic review

Gras,

Bearden,

West

et al. 2024

Epilepsia Open

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ObjectiveKCNT1‐related epilepsies encompass three main phenotypes: (i) epilepsy of infancy with migrating focal seizures (EIMFS), (ii) autosomal dominant or sporadic sleep‐related hypermotor epilepsy [(AD)SHE], and (iii) different types of developmental and epileptic encephalopathies (DEE). Many patients present with drug‐resistant seizures and global developmental delays. In addition to conventional anti‐seizure medications (ASM), multiple alternative therapies have been tested including the ketogenic diet (KD), cannabidiol (CBD—including Epidyolex © and other CBD derivatives) and quinidine (QUIN). We aimed to clarify the current state of the art concerning the benefits of those therapies administered to the three groups of patients.MethodsWe performed a literature review on PubMed and EMBase with the keyword “KCNT1” and selected articles reporting qualitative and/or quantitative information on responses to these treatments. A treatment was considered beneficial if it improved seizure frequency and/or intensity and/or quality of life. Patients were grouped by phenotype.ResultsA total of 43 studies including 197 patients were reviewed. For EIMFS patients (32 studies, 135 patients), KD resulted in benefit in 62.5% (25/40), all types of CBD resulted in benefit in 50% (6/12), and QUIN resulted in benefit in 44.6% (25/56). For (AD)SHE patients (10 studies, 32 patients), we found only one report of treatment with KD, with no benefit noted. QUIN was trialed in 8 patients with no reported benefit. For DEE patients (10 studies, 30 patients), KD resulted in benefit for 4/7, CBD for 1/2, and QUIN for 6/9. In all groups, conventional ASM are rarely reported as beneficial (in 5%–25% of patients).SignificanceKetogenic diet, CBD, and QUIN treatments appear to be beneficial in a subset of patient with drug‐resistant epilepsy. The KD and CBD are reasonable to trial in patients with KCNT1‐related epilepsy. Further studies are needed to identify optimal treatment strategies and to establish predictive response factors.Plain Language SummaryWe performed an extensive review of scientific articles providing information about the therapeutic management of epilepsy in patients with epilepsy linked to a mutation in the KCNT1 gene. Conventional anti‐seizure treatments were rarely reported to be beneficial. The ketogenic diet (a medical diet with very high fat, adequate protein and very low carbohydrate intake) and cannabidiol appeared to be useful, but larger studies are needed to reach a conclusion.

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Section: Discussionmentioning

confidence: 95%

Efficacy of anti‐seizure medications and alternative therapies (ketogenic diet, CBD, and quinidine) in KCNT1‐related epilepsy: A systematic review

Gras,

Bearden,

West

et al. 2024

Epilepsia Open

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“… 87 , 88 , 89 While concerns exist regarding potential growth retardation in children following the ketogenic diet due to reduced ghrelin and des-acyl ghrelin levels, studies on growth retardation in relation to the ketogenic diet have yielded conflicting results. 90 , 91 , 92 , 93 …”

Section: Complications Of Ketogenic Dietmentioning

confidence: 99%

Drug resistant epilepsy and ketogenic diet: A narrative review of mechanisms of action

Mishra,

Singh,

Ramadass

2024

World Neurosurgery: X

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Antiepileptic-drugs

2024

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Feasibility of ketogenic diet therapy variants for refractory epilepsy in neonates to infants under 2 years old

Cited by 4 publications

References 40 publications

Efficacy of anti‐seizure medications and alternative therapies (ketogenic diet, CBD, and quinidine) in KCNT1‐related epilepsy: A systematic review

Efficacy of anti‐seizure medications and alternative therapies (ketogenic diet, CBD, and quinidine) in KCNT1‐related epilepsy: A systematic review

Drug resistant epilepsy and ketogenic diet: A narrative review of mechanisms of action

Antiepileptic-drugs

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