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Modified ketogenic diet

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https://www.readbyqxmd.com/read/28437535/clinical-aspects-of-glucose-transporter-type-1-deficiency-information-from-a-global-registry
#1
Jian Hao, Dorothy I Kelly, Jianzhong Su, Juan M Pascual
Importance: Case reports regularly document unique or unusual aspects of glucose transporter type 1 deficiency (G1D). In contrast, population studies from which to draw global inferences are lacking. Twenty-five years after the earliest case reports, this deficiency still particularly affects treatment and prognostic counseling. Objective: To examine the most common features of G1D. Design, Setting, and Participants: In this study, data were collected electronically from 181 patients with G1D through a web-based, worldwide patient registry from December 1, 2013, through December 1, 2016...
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28427869/ischemic-preconditioning-with-ketogenic-diet-improves-brain-ischemic-tolerance-through-increased-extracellular-adenosine-levels-and-hypoxia-inducible-factors
#2
Qi Yang, Min Guo, Xun Wang, Yanxin Zhao, Hongyan Ding, Mei Cui, Qiang Dong
Achieving a prolonged neuroprotective state reduces brain damage and neurological dysfunction characteristic of brain ischemia. The ketogenic diet (KD) has disease-modifying effects in several neurodegenerative disorders. In this study, we fed mice with KD for three weeks and performed reversible middle cerebral artery occlusion (MCAO) in the animals. KD-fed mice had significantly reduced infarct volume, increased regional cerebral blood flow (rCBF) and extracellular adenosine levels in both ischemic and reperfusion stages...
April 18, 2017: Brain Research
https://www.readbyqxmd.com/read/28416069/-dietary-treatment-of-medically-refractory-epilepsy-in-children-and-adolescents
#3
Frederikke Høgsbro-Rode, Katrine M Harris Johannesen, Vibeke Stubbings, Helle Hjalgrim
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 diet and low glycaemic index treatment have been suggested as alternative treatments. Several studies have documented significant seizure reduction from all four diets. The aim of this article is to give an overview of the effect of dietary treatment and to discuss advantages in initiating dietary treatment as an early treatment instead of as a last option...
April 3, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28294308/neuronal-inhibition-and-seizure-suppression-by-acetoacetate-and-its-analog-2-phenylbutyrate
#4
Atsushi Kadowaki, Nagisa Sada, Narinobu Juge, Ayaka Wakasa, Yoshinori Moriyama, Tsuyoshi Inoue
OBJECTIVE: The ketogenic diet is clinically used to treat drug-resistant epilepsy. The diet treatment markedly increases ketone bodies (acetoacetate and β-hydroxybutyrate), which work as energy metabolites in the brain. Here, we investigated effects of acetoacetate on voltage-dependent Ca(2+) channels (VDCCs) in pyramidal cells of the hippocampus. We further explored an acetoacetate analog that inhibited VDCCs in pyramidal cells, reduced excitatory postsynaptic currents (EPSCs), and suppressed seizures in vivo...
March 11, 2017: Epilepsia
https://www.readbyqxmd.com/read/28288483/efficacy-of-treatments-for-infantile-spasms-a-systematic-review
#5
Ji Min Song, Jongsung Hahn, Se Hee Kim, Min Jung Chang
OBJECTIVES: West syndrome (also known as infantile spasm because of its main seizure type) is a rare form of epilepsy that begins during early infancy. Recent guidelines and reviews on West syndrome recommend the use of adrenocorticotropic hormone steroids, or vigabatrin, as the first-line treatment. However, West syndrome remains to be one of the most challenging epilepsies to treat. Here, we systematically reviewed the current literature obtained during the previous decade. This article provides an overview of the current treatment of infantile spasms...
March 2017: Clinical Neuropharmacology
https://www.readbyqxmd.com/read/28264719/the-efficacy-of-the-modified-atkins-diet-in-north-sea-progressive-myoclonus-epilepsy-an-observational-prospective-open-label-study
#6
Martje E van Egmond, Amerins Weijenberg, Margreet E van Rijn, Jan Willem J Elting, Jeannette M Gelauff, Rodi Zutt, Deborah A Sival, Roald A Lambrechts, Marina A J Tijssen, Oebele F Brouwer, Tom J de Koning
BACKGROUND: North Sea Progressive Myoclonus Epilepsy is a rare and severe disorder caused by mutations in the GOSR2 gene. It is clinically characterized by progressive myoclonus, seizures, early-onset ataxia and areflexia. As in other progressive myoclonus epilepsies, the efficacy of antiepileptic drugs is disappointingly limited in North Sea Progressive Myoclonus Epilepsy. The ketogenic diet and the less restrictive modified Atkins diet have been proven to be effective in other drug-resistant epilepsy syndromes, including those with myoclonic seizures...
March 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28262406/a-novel-inherited-scn1a-mutation-associated-with-gefs-in-benign-and-encephalopathic-epilepsy
#7
Angela C Gauthier, Louis N Manganas, Richard H Mattson
Generalized epilepsy with febrile seizures plus (GEFS+) is an autosomal dominant condition often caused by mutations in SCN1A that usually first manifests as childhood simple febrile seizures but may progress to a variety of afebrile generalized seizure types. Here, we describe the case of an 8-year-old boy with a novel SCN1A mutation who developed febrile seizures at 10months of age which eventually advanced to frequent afebrile tonic-clonic seizures. His condition was unresponsive to several antiepileptic drugs and the ketogenic diet, and he experienced gradual cognitive decline...
March 2, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28229464/modified-atkins-diet-is-an-effective-treatment-for-children-with-doose-syndrome
#8
Adelheid Wiemer-Kruel, Edda Haberlandt, Hans Hartmann, Gabriele Wohlrab, Thomas Bast
OBJECTIVE: Children with myoclonic astatic epilepsy (MAE; Doose syndrome) whose seizures do not respond immediately to standard antiepileptic drugs (AEDs) are at high risk of developing an epileptic encephalopathy with cognitive decline. A classic ketogenic diet (KD) is a highly effective alternative to AEDs. To date, there are only limited data on the effectiveness of the modified Atkins diet (MAD), which is less restrictive and more compatible with daily life. We report findings from a retrospective study on 30 MAE patients treated with MAD...
April 2017: Epilepsia
https://www.readbyqxmd.com/read/28141738/new-insights-into-the-mechanisms-of-the-ketogenic-diet
#9
Detlev Boison
PURPOSE OF REVIEW: High-fat, low-carbohydrate ketogenic diets have been used for almost a century for the treatment of epilepsy. Used traditionally for the treatment of refractory pediatric epilepsies, in recent years the use of ketogenic diets has experienced a revival to include the treatment of adulthood epilepsies as well as conditions ranging from autism to chronic pain and cancer. Despite the ability of ketogenic diet therapy to suppress seizures refractory to antiepileptic drugs and reports of lasting seizure freedom, the underlying mechanisms are poorly understood...
April 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28110175/ketogenic-diet-therapy-for-epilepsy-during-pregnancy-a-case-series
#10
Elles J T M van der Louw, Tanya J Williams, Bobbie J Henry-Barron, Joanne F Olieman, Johannes J Duvekot, Marijn J Vermeulen, Natalja Bannink, Monique Williams, Rinze F Neuteboom, Eric H Kossoff, Coriene E Catsman-Berrevoets, Mackenzie C Cervenka
PURPOSE: Evaluation of ketogenic diet (KD) therapies for seizure control during pregnancy when safety and appropriate management become considerations. Until now, no information has been available on seizure reduction and human pregnancy related outcomes in women treated with KD therapies. METHOD: We describe two cases of pregnant women with epilepsy treated with KD therapy either as monotherapy (Case 1) or as adjunctive therapy (Case 2). RESULTS: Case 1: A 27 year old woman, gravida1, started the classic KD with medium chain triglyceride (MCT) emulsion and 75g carbohydrate-restriction, later reduced to 47g...
February 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28053859/is-there-a-role-for-diet-monotherapy-in-adult-epilepsy
#11
Mackenzie C Cervenka, Bobbie J Henry-Barron, Eric H Kossoff
Ten adults were treated with ketogenic diet monotherapy for epilepsy. Four patients were naïve to antiseizure drugs (ASDs), and six previously tried and stopped ASDs. Of four treatment-naïve participants, two (50%) were free from disabling seizures on Modified Atkins Diet (MAD) monotherapy for > 1 year. Two (50%) stopped. Four of six patients (67%) who had previously tried ASDs became seizure-free on diet monotherapy, and two experienced > 50% seizure reduction. Side effects included amenorrhea, weight loss, osteoporosis, and hyperlipidemia...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28008324/vitamin-d3-for-the-treatment-of-epilepsy-basic-mechanisms-animal-models-and-clinical-trials
#12
REVIEW
Kevin Pendo, Christopher M DeGiorgio
There is increasing evidence supporting dietary and alternative therapies for epilepsy, including the ketogenic diet, modified Atkins diet, and omega-3 fatty acids. Vitamin D3 is actively under investigation as a potential intervention for epilepsy. Vitamin D3 is fat-soluble steroid, which shows promise in animal models of epilepsy. Basic research has shed light on the possible mechanisms by which Vitamin D3 may reduce seizures, and animal data support the efficacy of Vitamin D3 in rat and mouse models of epilepsy...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/27759811/ketogenic-diet-for-epilepsy-treatment
#13
Letícia Pereira de Brito Sampaio
The ketogenic diet (KD), a high-fat, low-carbohydrate, and adequate-protein diet is an established, effective nonpharmacologic treatment option for intractable childhood epilepsy. The KD was developed in 1921 and even though it has been increasingly used worldwide in the past decade, many neurologists are not familiar with this therapeutic approach. In the past few years, alternative and more flexible KD variants have been developed to make the treatment easier and more palatable while reducing side effects and making it available to larger group of refractory epilepsy patients...
October 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27647878/modified-atkins-diet-induces-subacute-selective-ragged-red-fiber-lysis-in-mitochondrial-myopathy%C3%A2-patients
#14
Sofia Ahola, Mari Auranen, Pirjo Isohanni, Satu Niemisalo, Niina Urho, Jana Buzkova, Vidya Velagapudi, Nina Lundbom, Antti Hakkarainen, Tiina Muurinen, Päivi Piirilä, Kirsi H Pietiläinen, Anu Suomalainen
Mitochondrial myopathy (MM) with progressive external ophthalmoplegia (PEO) is a common manifestation of mitochondrial disease in adulthood, for which there is no curative therapy. In mice with MM, ketogenic diet significantly delayed progression of the disease. We asked in this pilot study what effects high-fat, low-carbohydrate "modified Atkins" diet (mAD) had for PEO/MM patients and control subjects and followed up the effects by clinical, morphological, transcriptomic, and metabolomic analyses. All of our five patients, irrespective of genotype, showed a subacute response after 1...
2016: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/27594980/ketogenic-diet-modifies-the-gut-microbiota-in-a-murine-model-of-autism-spectrum-disorder
#15
Christopher Newell, Marc R Bomhof, Raylene A Reimer, Dustin S Hittel, Jong M Rho, Jane Shearer
BACKGROUND: Gastrointestinal dysfunction and gut microbial composition disturbances have been widely reported in autism spectrum disorder (ASD). This study examines whether gut microbiome disturbances are present in the BTBR(T + tf/j) (BTBR) mouse model of ASD and if the ketogenic diet, a diet previously shown to elicit therapeutic benefit in this mouse model, is capable of altering the profile. FINDINGS: Juvenile male C57BL/6 (B6) and BTBR mice were fed a standard chow (CH, 13 % kcal fat) or ketogenic diet (KD, 75 % kcal fat) for 10-14 days...
2016: Molecular Autism
https://www.readbyqxmd.com/read/27525031/modified-atkins-diet-in-advanced-malignancies-final-results-of-a-safety-and-feasibility-trial-within-the-veterans-affairs-pittsburgh-healthcare-system
#16
Jocelyn L Tan-Shalaby, Jennifer Carrick, Krystal Edinger, Dana Genovese, Andrew D Liman, Vida A Passero, Rashmikant B Shah
BACKGROUND: Dysfunctional mitochondrial processes limit malignant cells ability to use energy from fatty acids and ketones. Animal studies using ketogenic diets for cancer show encouraging results. We tested the diet's safety and feasibility in cancer patients across a broad variety of solid tumors. METHODS: We recruited 17 advanced cancer patients who were not on chemotherapy. They consumed 20 to 40 g of carbohydrates daily with evaluations performed weekly until week 4, then every 4 weeks until 16 weeks...
2016: Nutrition & Metabolism
https://www.readbyqxmd.com/read/27381979/ketogenic-diet-prevents-neuronal-firing-increase-within-the-substantia-nigra-during-pentylenetetrazole-induced-seizure-in-rats
#17
Andrea Viggiano, Madison Stoddard, Simone Pisano, Francesca Felicia Operto, Valentina Iovane, Marcellino Monda, Giangennaro Coppola
The mechanism responsible for the anti-seizure effect of ketogenic diets is poorly understood. Because the substantia nigra pars reticulata (SNr) is a "gate" center for seizures, the aim of the present experiment was to evaluate if a ketogenic diet modifies the neuronal response of this nucleus when a seizure-inducing drug is administered in rats. Two groups of rats were given a standard diet (group 1) or a ketogenic diet (group 2) for four weeks, then the threshold for seizure induction and the firing rate of putative GABAergic neurons within the SNr were evaluated with progressive infusion of pentylenetetrazole under general anesthesia...
July 2016: Brain Research Bulletin
https://www.readbyqxmd.com/read/27273526/use-of-modified-atkins-diet-in-glucose-transporter-type-1-deficiency-syndrome
#18
Sofiane Amalou, Domitille Gras, Adina Ilea, Marie-Odile Greneche, Laurent Francois, Vanina Bellavoine, Catherine Delanoe, Stéphane Auvin
AIM: Glucose transporter type 1 deficiency syndrome (GLUT1-DS) results from impaired glucose transport into the brain, and is treated with a ketogenic diet. A few reports have suggested effectiveness of treatment using the modified Atkins diet (MAD). We aimed to assess the efficacy of MAD as a treatment for GLUT1-DS. METHOD: We evaluated the efficacy of MAD in 10 patients (four males, six females; mean age at diagnosis [SD] 6.2y [1.7], min-max: 4mo-12y) with GLUT1-DS...
November 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27168769/non-invasive-treatment-options-for-focal-cortical-dysplasia
#19
Ting-Ting Wang, Dong Zhou
Focal cortical dysplasia (FCD) presents a strong clinical challenge especially for the treatment of the associated epilepsy. Epilepsy in FCD is often treatment-resistant and constitutes 50% of treatment-resistant cases. Antiepileptic drugs (AEDs) have been widely used in the treatment of FCD. However, evidence to suggest their specific effect on the treatment of FCD remains to be established. In view of this resistance, several alternative treatments have been suggested. Although treatment currently involves surgical management, non-invasive treatments have been identified...
May 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27064917/-national-consensus-on-the-modified-atkins-diet
#20
María Vaccarezza, Ariela Agustinho, M Julia Alberti, Laura Argumedo, Marisa Armeno, Virginia Blanco, Cecilia Bouquet, Analía Cabrera, Roberto Caraballo, Luciana Caramuta, Araceli Cresta, Elizabeth S de Grandis, Martha G DeMartini, Cecilia Diez, Mariana Diz, Corina Dlugoszewski, Nidia Escobal, Hilario Ferrero, Santiago Galicchio, Victoria Gambarini, Beatriz Gamboni, Lara Gonzalez, Silvina Guisande, Amal Hassan, Pablo Matarrese, Graciela Mestre, Laura Pesce, Viviana Rios, Marcos Semprino, Patricia Sosa, Marisol Toma, Rocío Viollaz, Luis Panico
INTRODUCTION: Epilepsy is a chronic disease that affects 0.5-1% of the population. One third of the patients become refractory to antiepileptic drugs. Among the non-pharmacological treatments available, the modified Atkins diet is an effective treatment used since 2003 as another alternative for children and adults with refractory epilepsy. DEVELOPMENT: The Ketogenic Diet National Committee, which depends on the Argentine Society of Pediatric Neurology, elaborated this consensus on the modified Atkins diet, basing itself on a review of the literature and on their clinical experience...
April 16, 2016: Revista de Neurologia
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