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Apr 19, 2017
Høgsbro-Rode F, Johannesen KMH, Stubbings V, Hjalgrim H
Ketogenic diet (KD) is used worldwide in the treatment of medically refractory epilepsy. Since the introduction of KD in the early 1900s, new approaches such as medium-chain triglyceride ketogenic diet, modified Atkins...
Apr 17, 2017
Socała K, Nieoczym D, Kowalczuk-Vasilev E, Wyska E, Wlaź P
Activation of Nrf2 with sulforaphane has recently gained attention as a new therapeutic approach in the treatment of many diseases, including epilepsy. As a plant-derived compound, sulforaphane is considered to be safe...
Apr 16, 2017
Kaya Ozcora GD, Gokay S, Canpolat M, Kardaş F, Kendirci M, Kumandaş S
BACKGROUND: Glutaric acidemia Type 1 (GA-1) is an autosomal recessively inherited metabolic disorder which is associated with GCDH gene mutations which alters the glutaryl-CoA dehydrogenase, an enzyme playing role in t...
Apr 14, 2017
Ramm-Pettersen A, Nakken KO, Haavardsholm KC, Selmer KK
INTRODUCTION: Glucose transporter type 1 deficiency syndrome (GLUT1-DS) is a rare metabolic encephalopathy with a wide variation of clinical phenotypes. Familial variants are often milder than de novo cases, and may th...
Apr 14, 2017
Myers KA, Scheffer IE
INTRODUCTION: Dishevelled, Egl-10 and Pleckstrin (DEP) domain-containing protein 5 (DEPDC5) is a protein subunit of the GTPase-activating proteins towards Rags 1 (GATOR1) complex. GATOR1 is a recently identified modula...
Apr 14, 2017
Albenzio M, Santillo A, Ciliberti MG, Figliola L, Caroprese M, Marino R, Polito AN
The present study was undertaken to evaluate the effect of protein fractions from bovine, caprine, and ovine milk on production of cytokines and reactive oxygen species (ROS) and reactive nitrogen species (RNS) by cult...
Apr 14, 2017
Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K
CLINICAL CHARACTERISTICS: Pyridoxine-dependent epilepsy is characterized by intractable seizures within the first weeks to months of life that are not controlled with antiepileptic drugs but respond both clinically and...
Apr 12, 2017
Simeone TA, Simeone KA, Rho JM
There is growing evidence that ketone bodies (KB)-derived from fatty acid oxidation and produced during fasting or consumption of high-fat diets-can exert broad neuroprotective effects. With respect to epilepsy, KB (su...
Apr 11, 2017
Hatori T, Sugiyama Y, Yamashita S, Hirakubo Y, Nonaka K, Ichihashi K
In a patient with cardiofaciocutaneous syndrome complicated by intractable infantile spasms (West syndrome), cardiac hypertrophy developed during adrenocorticotropic hormone treatment. Various types of antiepileptic dr...
Apr 11, 2017
Li J, O'Leary EI, Tanner GR
The high-fat, low-carbohydrate ketogenic diet (KD) is an effective clinical treatment for epilepsy in juveniles, especially for drug-resistant seizures. The KD results in elevated production of ketone bodies (KB's), su...

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