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Diabetes type 1 seems to be more prevalent in epilepsy, and low‐carbohydrate diets improve glycemic control in diabetes type 2, but data on the use of the classic ketogenic diet (KD) in epilepsy and diabetes are scarce. We present 15 months of follow‐up of a 3 years and 6 months old girl with diabetes type 1 (on the KD), right‐sided hemiparesis, and focal epilepsy due to a malformation of cortical...
Prolonged status epilepticus (SE) can be refractory to conventional interventions, with high rates of subsequent morbidity and mortality. A high fat, low protein, low carbohydrate ketogenic diet (KD) has been used successfully to treat intractable epilepsy. However, its possible role in prolonged SE has not been well described. We report successful use of the KD in two adult patients with prolonged...
Purpose: 2‐Deoxy‐d‐glucose (2‐DG), a glucose analog that accumulates in cells and interferes with carbohydrate metabolism by inhibiting glycolytic enzymes, has anticonvulsant actions. Recognizing that severe glucose deprivation can induce seizures, we sought to determine whether acute treatment with 2‐DG can promote seizure susceptibility by assessing its effects on seizure threshold. For comparison,...
Omega‐3 and omega‐6 polyunsaturated fatty acids (PUFAs) are dietary fatty acids that are involved in a myriad of physiologic processes in the brain. There is some evidence suggesting that PUFAs—and particularly omega‐3 PUFAs—may have anticonvulsant effects, both in humans and in animals. In the present review, we assess the evidence related to the antiseizure properties of the n‐3 PUFAs, discuss their...
Purpose: Calorie restriction can be anticonvulsant in animal models. The ketogenic diet was designed to mimic calorie restriction and has been assumed to work by the same mechanisms. We challenged this assumption by profiling the effects of these dietary regimens in mice subjected to a battery of acute seizure tests.
Methods: Juvenile male NIH Swiss mice received ketogenic diet or a normal diet...
Purpose: Although the number of antiepileptic drugs (AEDs) is increasing, none displays neuroprotective or antiepileptogenic properties that could prevent status epilepticus (SE)–induced drug‐resistant epilepsy. Ketogenic diet (KD) and calorie restriction (CR) are proposed as alternative treatments in epilepsy. Our goal was to assess the neuroprotective or antiepileptogenic effect of these diets...
Purpose: Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian refractory status epilepticus (SE) triggered by fever of unknown cause. SE may last more than 1 month, and this condition may evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed to report the effect of ketogenic...
We present an update of our experience with the ketogenic diet (KD) in patients with Dravet syndrome (DS) to evaluate the efficacy and tolerability and our short experience with vagus nerve stimulation (VNS) in the treatment of this syndrome. Between March 1, 1990 and May 31, 2007, 59 patients who met diagnostic criteria for DS were studied in our department. Twenty‐four of them were placed on the...
Ketogenic diet is a nonpharmacologic treatment for childhood epilepsy not amenable to drugs. At the present time, two works based on national research, one in Germany and one in the United States provide international guidelines to ensure a correct management of the ketogenic diet. Our Italian collaborative study group was set up in order to formulate a consensus statement regarding the clinical management...
Purpose: To compare the prognoses between short‐term (8 months) and conventional long‐term (>2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD).
Methods: Of 40 patients who achieved seizure‐free outcomes and showed improvement in hypsarrhythmic patterns within 6 months of the KD, with a 3:1 fat to nonfat ratio as an add‐on treatment,...
We aimed to test the efficacy of ketogenic diet (KD) in patients with Dravet syndrome (DS) not satisfactorily controlled by antiepileptic drugs (AEDs). We included prospectively 15 DS patients aged >3 years with partial response to AEDs including stiripentol. All patients had a seizure diary and clinical examination with Conners and Achenbach scales before KD, at 1 month following onset and every...
Purpose: The ketogenic diet (KD) has been used as an effective antiepileptic treatment for nearly a century. Inhibition of glycolysis and increased levels of ketone bodies are both known to contribute to the antiepileptic effects of the KD. Neuron‐restrictive silencer factor (NRSF), also known as RE‐1 silencing transcription factor (REST), is implicated in the antiepileptic effects of the glycolytic...
Ketogenic diet (KD) is known to be effective in intractable epilepsy. However, the role of KD in refractory status epilepticus (RSE) has not been well described. The aim of this study is to explore the role of KD in patients with RSE. We retrospectively reviewed the medical records of four children and one adult with RSE between October 2006 and August 2010. All presented with status epilepticus (SE)...
Purpose: We evaluated the ability of the ketogenic diet (KD) to improve thresholds to flurothyl‐induced seizures in two mouse lines with Scn1a mutations: one that models Dravet syndrome (DS) and another that models genetic (generalized) epilepsy with febrile seizures plus (GEFS+).
Methods: At postnatal day 21, mouse models of DS and GEFS+ were fasted for 12–14 h and then placed on either a 6:1 (fats...
We report the efficacy of the ketogenic diet in refractory epilepsies focusing on outcomes with regard to epilepsy syndromes and etiology in children and adults with refractory epilepsy. Sixty‐four consecutive children and four adults were prospectively enrolled from 2002 to 2009; seven were excluded from analysis. The classical ketogenic diet was initiated on an inpatient basis with dietary ratios...
Purpose: Glut 1 deficiency syndrome (DS) is defined by hypoglycorrhachia with normoglycemia, acquired microcephaly, episodic movements, and epilepsy refractory to standard antiepileptic drugs (AEDs). Gold standard treatment is the ketogenic diet (KD), which provides ketones to treat neuroglycopenia. Our purpose is (1) to describe epilepsy phenotypes in a large Glut 1 DS cohort, to facilitate diagnosis;...
Purpose: The low glycemic index treatment (LGIT) is a high fat, limited carbohydrate diet used in the treatment of epilepsy. The purpose of this study was to assess the efficacy and tolerability of the LGIT for the treatment of refractory seizures in pediatric patients with Angelman syndrome.
Methods: A pediatric Angelman syndrome cohort with refractory epilepsy was treated with the LGIT and followed...
For several decades, both in vitro and in vivo models of seizures and epilepsy have been employed to unravel the molecular and cellular mechanisms underlying the occurrence of spontaneous recurrent seizures (SRS)—the defining hallmark of the epileptic brain. However, despite great advances in our understanding of seizure genesis, investigators have yet to develop reliable biomarkers and surrogate...
Childhood is a vulnerable time—developmentally, socially, and cognitively. Any disruption may have long‐standing effects on the attainment of critical developmental milestones. The impact of ongoing seizures and epilepsy in children is significant and may result in cognitive delay, poorer educational outcomes, and lower employment rates in adulthood. The early recognition, intervention, and treatment...
Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection–related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear...
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