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May 27, 2015
Blaise JH, Ruskin DN, Koranda JL, Masino SA
Ketogenic diets are low-carbohydrate, sufficient protein, high-fat diets with anticonvulsant activity used primarily as a treatment for pediatric epilepsy. The anticonvulsant mechanism is thought to involve elevating i...
May 26, 2015
Ramm-Pettersen A, Nakken KO, Haavardsholm KC, Selmer KK
Glucose transporter 1 deficiency syndrome (GLUT1-DS) is a treatable metabolic encephalopathy caused by a mutation in the SLC2A1 gene. This mutation causes a compromised transport of glucose across the blood-brain barri...
May 26, 2015
Sivaraju A, Nussbaum I, Cardoza CS, Mattson RH
Ketogenic diet has been shown to be efficacious in some epileptic encephalopathies but rarely reported as being useful in children with Ohtahara syndrome. This could possibly be attributed to the rarity of the disease ...
May 24, 2015
Mescka CP, Guerreiro G, Donida B, Marchetti D, Wayhs CA, Ribas GS, Coitinho AS, Wajner M, Dutra-Filho CS, Vargas CR
Maple Syrup Urine Disease (MSUD) is a metabolic disorder caused by a severe deficiency of the branched-chain α-keto acid dehydrogenase complex activity which leads to the accumulation of branched-chain amino acids (BCA...
May 23, 2015
Finsterer J, Kothari S
May 23, 2015
Fung LW
May 23, 2015
Winesett SP, Bessone SK, Kossoff EH
Available pharmacologic treatments for seizures are limited in their efficacy. For a patient with seizures, pharmacologic treatment with available anticonvulsant medications leads to seizure control in
May 20, 2015
Reyes G, Flesler S, Armeno M, Fortini S, Ariela A, Cresta A, Mestre G, Caraballo RH
UNLABELLED: Epileptic encephalopathy with electrical status epilepticus during slow sleep (ESES) belongs to the group of epileptic encephalopathies that often prove refractory to AED treatment. The ketogenic diet (KD) ...
May 20, 2015
Chwiej J, Skoczen A, Matusiak K, Janeczko K, Patulska A, Sandt C, Simon R, Ciarach M, Setkowicz Z
A growing body of evidence demonstrates that dietary therapies, mainly the ketogenic diet, may be highly effective in the reduction of epileptic seizures. All of them share the common characteristic of restricting carb...
May 20, 2015
Schwarzkopf TM, Koch K, Klein J
Triheptanoin, an oily substance, consists of glycerol bound to three molecules of heptanoic acid, a C7 odd-chain fatty acid. A triheptanoin-rich diet has anaplerotic effects because heptanoate metabolism yields succina...

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