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Dec 9, 2016
Ashrafi MR, Hosseini SA, Zamani GR, Mohammadi M, Tavassoli A, Badv RS, Heidari M, Karimi P, Malamiri RA
To evaluate the efficacy, safety, and tolerability of a classic 4:1 ketogenic diet using a formula-based powder in infants and children with refractory seizures who are reluctant to eat homemade foods. We conducted an ...
Dec 9, 2016
Juozapaite S, Praninskiene R, Burnyte B, Ambrozaityte L, Skerliene B
Glucose transporter 1 deficiency syndrome (GLUT1-DS) is an inborn error of metabolism caused by impaired glucose transport through blood brain barrier due to mutation in SLC2A1 gene, encoding transporter protein. Clini...
Dec 6, 2016
van Baalen A, Vezzani A, Häusler M, Kluger G
Febrile infection-related epilepsy syndrome (FIRES, AERRPS, or DESC) is one of the most severe, mostly irreversible, and presumably immune-mediated epileptic encephalopathies affecting healthy children. Refractory stat...
Dec 2, 2016
Cioczek-Czuczwar A, Czuczwar P, Turski WA, Parada-Turska J
BACKGROUND: The mechanism of drug resistance in epilepsy remains unknown. Picolinic acid (PIC) is an endogenous metabolite of the kynurenine pathway and a chelating agent added to dietary supplements. Both inhibitory a...
Nov 25, 2016
Al Teneiji A, Bruun TU, Cordeiro D, Patel J, Inbar-Feigenberg M, Weiss S, Struys E, Mercimek-Mahmutoglu S
We report treatment outcome of eleven patients with pyridoxine-dependent epilepsy caused by pathogenic variants in ALDH7A1 (PDE-ALDH7A1). We developed a clinical severity score to compare phenotype with biochemical fea...
Nov 23, 2016
Soysal E, Gries H, Wray C
STUDY OBJECTIVE: To identify guidelines for anesthesia management and determine whether general anesthesia is safe for pediatric patients on ketogenic diet (KD). DESIGN: Retrospective medical record review. ...
Nov 23, 2016
Arslan N, Kose E, Guzel O
The aim of the present study was to evaluate serum selenium levels in children receiving olive oil-based ketogenic diet (KD) for intractable seizures for at least 1 year. Out of 320 patients who were initiated on KD, p...
Nov 22, 2016
Fogle KJ, Hertzler JI, Shon JH, Palladino MJ
Effective therapies are lacking for mitochondrial encephalomyopathies (MEs). MEs are devastating diseases that predominantly affect the energy-demanding tissues of the nervous system and muscle, causing symptoms such a...
Nov 22, 2016
Tefera TW, Tan KN, McDonald TS, Borges K
This review summarises the recent findings on metabolic treatments for epilepsy and Amyotrophic Lateral Sclerosis (ALS) in honour of Professor Ursula Sonnewald. The metabolic impairments in rodent models of these disor...
Nov 21, 2016
Arslan N, Guzel O, Kose E, Yılmaz U, Kuyum P, Aksoy B, Çalık T
PURPOSE: Long-term ketogenic diet (KD) treatment has been shown to induce liver steatosis and gallstone formation in some in vivo and clinical studies. The aim of this retrospective study was to evaluate the hepatic si...

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