The ketogenic diet in children with epilepsy

Papandreou, Dimitrios; Pavlou, Evangelos; Kalimeri, Eleni; Mavromichalis, I.

doi:10.1079/bjn20051591

Cited by 35 publications

(33 citation statements)

References 67 publications

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“…This was evidenced by significantly higher daily weight gain in the KD fed mutants. Clinically, the KD is associated with significantly attenuated growth compared to humans consuming a non-KD (Vining et al, 2002;Liu et al, 2003;Papandreou et al, 2006). This has also been shown in rats fed a KD (Zhao et al, 2004;Nylen et al, 2005;Nylen et al, 2006).…”

Section: Ataxia and Weightsmentioning

confidence: 59%

A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype

Nylen

Velázquez

Likhodii

et al. 2008

Experimental Neurology

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Succinic semialdehyde dehydrogenase (SSADH) deficiency is an heritable disorder of GABA degradation characterized by ataxia, psychomotor retardation and seizures. To date, there is no effective treatment for SSADH deficiency. We tested the hypothesis that a ketogenic diet (KD) would improve outcome in an animal model of SSADH deficiency, the SSADH knockout mouse (Aldh5a1 −/− ). Using a 4:1 ratio of fat to combined carbohydrate and protein KD we set out to compare the general phenotype, in vivo and in vitro electrophysiology and [ 35 S]TBPS binding in both Aldh5a1 −/− mice and control (Aldh5a1 +/+ ) mice. We found that the KD prolonged the lifespan of mutant mice by >300% with normalization of ataxia, weight gain and EEG compared to mutants fed a control diet. Aldh5a1 −/− mice showed significantly reduced mIPSC frequency in CA1 hippocampal neurons as well as significantly decreased [ 35 S]TBPS binding in all brain areas examined. In KD fed mutants, mIPSC activity normalized and [ 35 S]TBPS binding was restored in the cortex and hippocampus. The KD appears to reverse toward normal the perturbations seen in Aldh5a1 −/− mice. Our data suggest that the KD may work in this model by restoring GABAergic inhibition. These data demonstrate a successful experimental treatment for murine SSADH deficiency using a KD, giving promise to the idea that the KD may be successful in the clinical treatment of SSADH deficiency.

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Section: Ataxia and Weightsmentioning

confidence: 59%

A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype

Nylen

Velázquez

Likhodii

et al. 2008

Experimental Neurology

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“…pyruvate dehydrogenase deficiency) (Keene, 2006;Klepper and Voit, 2002;Wexler et al, 1997;Vining, 1999). A number of adverse effects related to such diets have been reported including constipation, nutritional deficiencies and deaths (Wheless, 2001;Papandreou et al, 2006). Severe acidosis is another reported adverse effects of diets very low in carbohydrates consumed by adults who wanted to loose weight (IoM, 2005;Shah and Isley, 2006;Chen et al, 2006a).…”

Section: Dietary Requirementsmentioning

confidence: 99%

Scientific Opinion on Dietary Reference Values for carbohydrates and dietary fibre

Products¹

2010

EFS2

415

162

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This Opinion of the EFSA Panel on Dietetic Products, Nutrition, and Allergies (NDA) deals with the establishment of Dietary Reference Values for carbohydrates and dietary fibre. Nutritionally, two broad categories of carbohydrates can be differentiated: "glycaemic carbohydrates", i.e. carbohydrates digested and absorbed in the human small intestine, and "dietary fibre", non-digestible carbohydrates passing to the large intestine. In this Opinion, dietary fibre is defined as non-digestible carbohydrates plus lignin. The absolute dietary requirement for glycaemic carbohydrates is not precisely known but will depend on the amount of fat and protein ingested. The Panel proposes 45 to 60 E% as the reference Intake range for carbohydrates applicable to both adults and children older than one year of age. Although high frequency of intake of sugar-containing foods can increase the risk of dental caries, there are insufficient data to set an upper limit for (added) sugar intake. Based on the available evidence on bowel function, the Panel considers dietary fibre intakes of 25 g/day to be adequate for normal laxation in adults. A fibre intake of 2 g/MJ is considered adequate for normal laxation in children from the age of one year. Although there is some experimental evidence that a reduction of the dietary glycaemic index and glycaemic load may have favourable effects on some metabolic risk factors such as serum lipids, the evidence for a role in weight maintenance and prevention of diet-related diseases is inconclusive. KEY WORDSCarbohydrates, dietary fibre, sugars, added sugars, glycaemic carbohydrates, oligosaccharides, starch, lignin, glycaemic index, glycaemic load, dietary requirements, blood lipids, lipid profile, glucose tolerance, insulin sensitivity, body weight, type 2 diabetes, blood pressure, cardiovascular disease, coronary heart disease, dental caries, gastrointestinal function, colorectal cancer, mineral absorption SUMMARYFollowing a request from the European Commission, the EFSA Panel on Dietetic Products, Nutrition and Allergies (NDA) was asked to deliver a scientific opinion on Population Reference Intakes for the European population, including carbohydrates and dietary fibre.Nutritionally, two broad categories of carbohydrates can be differentiated: "glycaemic carbohydrates", i.e. carbohydrates digested and absorbed in the human small intestine, and "dietary fibre", non-digestible carbohydrates passing to the large intestine.The main glycaemic carbohydrates are monosaccharides, disaccharides, malto-oligosaccharides, and starch. In this Opinion the term "sugars" is used to cover monosaccharides and disaccharides. The term "added sugars" refers to sucrose, fructose, glucose, starch hydrolysates (glucose syrup, highfructose syrup) and other isolated sugar preparations used as such or added during food preparation and manufacturing. Sugar alcohols (polyols) such as sorbitol, xylitol, mannitol, and lactitol, are usually not included in the term "sugars". However, they are partly metabolised and i...

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“…The classical KD contains insufficient micronutrients [23,24] The potential presence of absorbable / resorbable carbohydrate in vitamin/mineral supplements however should not be overlooked. It is known that various anticonvulsants have interactions with some minerals and vitamins, for example, influencing the absorption of folic acid and the metabolism of calcium and vitamin D [22].…”

Section: Vitamins and Mineralsmentioning

confidence: 99%