Epilepsy of infancy with migrating focal seizures: three patients treated with the ketogenic diet

Caraballo, Roberto; Noli, Daniel; Cachia, Pedro

doi:10.1684/epd.2015.0741

Cited by 52 publications

(16 citation statements)

References 14 publications

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“…One of two patients with MFSI became seizure free at 3 months, whereas the other patients started to show seizure reduction after 3 months but eventually discontinued the diet due to an adverse event (gastric disturbance). These results are similar to those of Caraballo et al [30]. However, the KD response was low in EME, unclassified generalized epilepsy, and unclassified focal epilepsy.…”

Section: Discussionsupporting

confidence: 91%

Short- and long-term seizure-free outcomes of dietary treatment in infants according to etiology

Riantarini

Kim

et al. 2019

Seizure

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It is important to determine whether specific etiology is more effective to dietary treatment so that the diet can be started earlier for infants. We evaluated etiology-specific, seizure-free outcomes of dietary treatment in infants < 1 year of age. Methods: We conducted a 10-year, retrospective, longitudinal observational study of 115 infants treated with ketogenic diet (KD) or modified Atkins diet (MAD). Results: Most patients (70%) received classical KD; 30% received MAD. During follow-up, 90%, 73%, and 61% of the patients remained on the diet at 3, 6, and 12 months, respectively. Seizure-free outcomes were reported in 50%, 44%, and 50% of the patients at 3, 6, and 12 months, respectively. Long-term seizure-free outcomes over 12 months were reported in 43 (74%) of 58 infants who were seizure-free at 3 months. Etiologies were mostly symptomatic (structural brain abnormalities, genetic, or metabolic) in 83 (72%) of 115 patients. According to underlying etiology, long-term seizure-free outcomes were observed in 14 (33%) of 42 patients with structural brain abnormalities, 7 (33%) of 21 with genetic etiologies, 7 (35%) of 20 with metabolic etiologies, and 15 (47%) of 32 with unknown etiologies. There were no etiology-based differences with respect to long-term seizure-free outcomes (P = 0.63). Conclusion:The high rate of long-term seizure-free outcomes can be predicted based on the seizure freedom at 3 months regardless of etiology. Early dietary treatment is beneficial, even in infants < 1 year of age with specific symptomatic etiologies such as genetic, structural brain abnormalities, and metabolic etiology.

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Section: Discussionsupporting

confidence: 91%

Short- and long-term seizure-free outcomes of dietary treatment in infants according to etiology

Riantarini

Kim

et al. 2019

Seizure

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“…Preliminary experience also showing some beneficial effects of the KD has been reported in epileptic encephalopathies such as Lafora body disease, 58 Rett syndrome, 59,60 Landau-Kleffner syndrome, 61 and subacute sclerosing panencephalitis ( Table 2). 62 Single reports describe the use of the diet in metabolic disorders such as phosphofructokinase deficiency, adenylosuccinate lyase deficiency, and glycogenosis type V. [63][64][65] Other conditions with limited data include juvenile myoclonic epilepsy, 66 CDKL5 encephalopathy, 67 epilepsy of infancy with migrating focal seizures, 68 childhood absence epilepsy, 69 and epileptic encephalopathy with continuous spike-and-wave during sleep. 70 All of the above strong and moderate indications have sufficient data in our guideline group's opinion to justify the use of KDT.…”

Section: Indications and Contraindicationsmentioning

confidence: 99%

“…Adenylosuccinate lyase deficiency 64 CDKL5 encephalopathy 67 Childhood absence epilepsy 69 Cortical malformations 73,74 Epilepsy of infancy with migrating focal seizures 68 Epileptic encephalopathy with continuous spike-and-wave during sleep 70 Glycogenosis type V 65 Juvenile myoclonic epilepsy 66 Lafora body disease 58 Landau-Kleffner syndrome 61 Lennox-Gastaut syndrome 26 Phosphofructokinase deficiency 63 Rett syndrome 59,60 Subacute sclerosing panencephalitis (SSPE) 62 Long-chain fatty acids are transported across the mitochondrial membrane by carnitine, facilitated by carnitine palmitoyltransferase (CPT) I and II and carnitine translocase. 71 Once in the mitochondrion, fatty acids are b-oxidized to 2-carbon units of acetyl-CoA that can then enter the tricarboxylic acid cycle and be used for energy.…”

Section: Indications and Contraindicationsmentioning

confidence: 99%

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Kossoff¹,

Zupec‐Kania²,

et al. 2018

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SummaryKetogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre‐KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow‐up, side events, and KDT discontinuation. It has been helpful in outlining a state‐of‐the‐art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organizers and authors of this guideline present a revised version with additional authors, in order to include recent research, especially regarding other dietary treatments, clarifying indications for use, side effects during initiation and ongoing use, value of supplements, and methods of KDT discontinuation. In addition, authors completed a survey of their institution's practices, which was compared to responses from the original consensus survey, to show trends in management over the last 10 years.

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“…in a small group of infants (13.8 ± 5.7 months) showed that a ketogenic diet was effective and safe [ 85 ]. Other reports have reported the use of a ketogenic diet in young patients (less than 2 years) [ [86] , [87] , [88] , [89] , [90] , [91] , [92] , [93] , [94] , [95] ]. Most of the patients had infantile spasms and received ketogenic diet therapy during the chronic stage.…”

Section: Use Of a Ketogenic Diet In Nicusmentioning

confidence: 99%

Application of ketogenic diets for pediatric neurocritical care

Lin

Wang

2020

Biomedical Journal

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In this review, we summarize the general mechanisms of the ketogenic diet, and the application of a ketogenic diet in pediatric intensive care units for the neurological disorders of children and young infants. A ketogenic diet is a high-fat, low-carbohydrate, adequate-protein diet. It can alter the primary cerebral energy metabolism from glucose to ketone bodies, which involves multiple mechanisms of antiepileptic action, antiepileptogenic properties, neuro-protection, antioxidant and anti-inflammatory effects, and it is potentially a disease-modifying intervention. Although a ketogenic diet is typically used for the chronic stage of pharmacoresistant of epilepsy, recent studies have shown its efficacy in patients with the acute stage of refractory/super-refractory status epilepticus. The application of a ketogenic diet in pediatric intensive care units is a challenge because of the critical status of the patients, who are often in a coma or have a nothing by mouth order. Moreover, a ketogenic diet needs to be started early and sometimes through parenteral administration in patients with critical conditions such as refractory status epilepticus or febrile infection-related epilepsy syndrome. Animal models and some case reports have shown that the neuro-protective effects of a ketogenic diet can be extended to other emergent neurological diseases, such as traumatic brain injury and ischemic stroke.

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Epilepsy of infancy with migrating focal seizures: three patients treated with the ketogenic diet

Cited by 52 publications

References 14 publications

Short- and long-term seizure-free outcomes of dietary treatment in infants according to etiology

Short- and long-term seizure-free outcomes of dietary treatment in infants according to etiology

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Application of ketogenic diets for pediatric neurocritical care

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