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Modified ketogenic diet

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
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...
September 19, 2016: EMBO Molecular Medicine
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
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
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
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...
June 8, 2016: Developmental Medicine and Child Neurology
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
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
Mackenzie C Cervenka, Bobbie J Henry, Elizabeth A Felton, Katlyn Patton, Eric H Kossoff
OBJECTIVES: Over 250 medical centers worldwide offer ketogenic diets to children with epilepsy; however, access to these therapies has been extremely limited for adults until recent years. We examine our 5-year experience creating and implementing a dedicated Adult Epilepsy Diet Center designed to provide adults with epilepsy access to ketogenic diets. MATERIAL AND METHODS: Outpatients seen at the Johns Hopkins Adult Epilepsy Diet Center from August 2010 thru September 2015 age 18years and older were enrolled in a prospective open-label observational study...
May 2016: Epilepsy & Behavior: E&B
Jane Y Chen, Conny Tran, Lin Hwang, Gang Deng, Michael E Jung, Kym F Faull, Michael S Levine, Carlos Cepeda
BACKGROUND: Huntington's disease (HD) is a fatal, inherited neurodegenerative disorder characterized by uncontrollable dance-like movements, as well as cognitive deficits and mood changes. A feature of HD is a metabolic disturbance that precedes neurological symptoms. In addition, brain cholesterol synthesis is significantly reduced, which could hamper synaptic transmission. OBJECTIVE: Alterations in lipid metabolism as a potential target for therapeutic intervention in the R6/2 mouse model of HD were examined...
2016: Journal of Huntington's Disease
S Auvin
The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet that has been employed as a non-pharmacologic therapy for refractory epilepsy. Several multicenter and two randomized studies have demonstrated the efficacy of the ketogenic diet and the modified Atkins diet for children and adolescent with pharmacoresitant epilepsy. In order to facilitate patient tolerability and palatability, the diet protocols are gradually modified including changes in ratios of the fat versus non-fat components and the initiation of the diet with or without fasting...
March 2016: Revue Neurologique
Eric H Kossoff, Sarah C Doerrer, Steven P Winesett, Zahava Turner, Bobbie J Henry, Stacey Bessone, Anthony Stanfield, Mackenzie C Cervenka
The outcome for patients attempting dietary therapy for epilepsy a second time is unknown. Twenty-six subjects treated with the ketogenic diet as children who then began either the ketogenic diet or a Modified Atkins Diet (MAD) at least 6 months later were evaluated. The mean age at the first diet trial was 5.6 years and at the second diet trial was 11.5 years. Most restarted dietary therapy because of persistent seizures (65%) or recurrence after seizure freedom (19%). Overall, 77% had a ≥50% seizure reduction with the first diet, and 50% with the second diet, P = ...
July 2016: Journal of Child Neurology
Tatsuya Fujii, Yasushi Ito, Satoru Takahashi, Kuriko Shimono, Jun Natsume, Keiko Yanagihara, Hirokazu Oguni
OBJECTIVES: To evaluate the outcome of ketogenic diets (KDs) in patients with glucose transport type 1 deficiency syndrome (GLUT1DS) in Japan. METHODS: A nationwide survey for GLUT1DS was conducted by sending questionnaires to board-certified pediatric neurologists nationwide to obtain clinical and laboratory data. RESULTS: Among 39 patients whose diagnosis was confirmed molecularly or by the 3-O-methylglucose uptake assay, 31 were treated with KDs for longer than 1month...
August 2016: Brain & Development
Kirsty Martin, Cerian F Jackson, Robert G Levy, Paul N Cooper
BACKGROUND: The ketogenic diet (KD), being high in fat and low in carbohydrates, has been suggested to reduce seizure frequency. It is currently used mainly for children who continue to have seizures despite treatment with antiepileptic drugs. Recently, there has been interest in less restrictive KDs including the modified Atkins diet (MAD) and the use of these diets has extended into adult practice. OBJECTIVES: To review the evidence for efficacy and tolerability from randomised controlled trials regarding the effects of KD and similar diets...
February 9, 2016: Cochrane Database of Systematic Reviews
Eunjoo Lee, Hoon-Chul Kang, Heung Dong Kim
A ketogenic diet (KD) is a dietary approach to treat intractable epilepsy. The KD begins with hospitalization and the child and their parents can adapt to the KD for 1-2 weeks. Recently, various type of dietary intervention such as the modified Atkins diet (MAD) and the low glycemic index treatment (LGIT) have been performed. Since 2010, we carried out the KD, MAD, and LGIT for total of 802 patients; 489 patients (61%) for the KD, 147 patients (18.3%) with the MAD, and 166 patients (20.7%) for the LGIT. In this report, application of these dietary practices in Severance Hospital is shared...
January 2016: Clinical Nutrition Research
Hannah R Kass, S Parrish Winesett, Stacey K Bessone, Zahava Turner, Eric H Kossoff
PURPOSE: GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. METHODS: A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously, and information analyzed in a database. RESULTS: Surveys were received from 92 families, of which 90 (98%) had been treated with dietary therapies...
February 2016: Seizure: the Journal of the British Epilepsy Association
Sviatlana V Hrynevich, Tatyana V Waseem, Audrey Hébert, Luc Pellerin, Sergei V Fedorovich
The ketogenic diet is used as a prophylactic treatment for different types of brain diseases, such as epilepsy or Alzheimer's disease. In such a diet, carbohydrates are replaced by fats in everyday food, resulting in an elevation of blood-borne ketone bodies levels. Despite clinical applications of this treatment, the molecular mechanisms by which the ketogenic diet exerts its beneficial effects are still uncertain. In this study, we investigated the effect of replacing glucose by the ketone body β-hydroxybutyrate as the main energy substrate on synaptic vesicle recycling in rat brain synaptosomes...
February 2016: Neurochemistry International
Jeong A Kim, Jung-Rim Yoon, Eun Joo Lee, Joon Soo Lee, Jeong Tae Kim, Heung Dong Kim, Hoon-Chul Kang
OBJECTIVE: We aimed to compare the efficacy, safety, and tolerability of a modified Atkins diet (MAD) with the classic ketogenic diet (KD) for the treatment of intractable childhood epilepsy. METHODS: From March 2011 to March 2014, 104 patients aged 1-18 years who had refractory epilepsy were randomly assigned to each diet group (, number NCT2100501). A seizure diary record was used to compare seizure frequencies with the baseline prediet seizure frequency at the third and sixth months after diet therapy initiation...
January 2016: Epilepsia
Rocío Valdebenito, Iván Ruminot, Pamela Garrido-Gerter, Ignacio Fernández-Moncada, Linda Forero-Quintero, Karin Alegría, Holger M Becker, Joachim W Deitmer, L Felipe Barros
The effectiveness of ketogenic diets and intermittent fasting against neurological disorders has brought interest to the effects of ketone bodies on brain cells. These compounds are known to modify the metabolism of neurons, but little is known about their effect on astrocytes, cells that control the supply of glucose to neurons and also modulate neuronal excitability through the glycolytic production of lactate. Here we have used genetically-encoded Förster Resonance Energy Transfer nanosensors for glucose, pyruvate and ATP to characterize astrocytic energy metabolism at cellular resolution...
October 29, 2015: Journal of Cerebral Blood Flow and Metabolism
Roy E Strowd, Mackenzie C Cervenka, Bobbie J Henry, Eric H Kossoff, Adam L Hartman, Jaishri O Blakeley
Dietary glycemic modulation through high-fat, low-carbohydrate diets, which induce a state of systemic ketosis and alter systemic metabolic signaling, have been incorporated into the clinical management of patients with neurological disease for more than a century. Mounting preclinical evidence supports the antitumor, proapoptotic, and antiangiogenic effects of disrupting glycolytic metabolism through dietary intervention. In recent years, interest in incorporating such novel therapeutic strategies in neuro-oncology has increased...
September 2015: Neuro-oncology Practice
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