Accuracy of Flash Glucose Monitoring in a Patient with Dravet Syndrome on a Ketogenic Diet.

We herein report the case of a 3-year-old girl with severe myoclonic epilepsy of infancy known as Dravet syndrome (DS) on a ketogenic diet (KD) whose glucose concentrations were controlled by using a flash glucose monitoring system. Two-hundred ninety-three events of moderate hypoglycemia with a minimum of 45 mg/dL, not related to day or night, were recorded during the observational period. Hypoglycemia rate declined from 24.5% of all measurements to 11.8% over time; one hypoglycemia-associated seizure and one seizure due to ketone concentrations below the therapeutic range were observed. In summary, this case report broadens our understanding of hypoglycemia risk in patients with DS on a KD. Especially in childhood, the painless and easy detection of low glucose concentrations might lead to improved cognitive performance, and the reduction of hypoglycemia-induced seizures.

Potentiating α2 subunit containing perisomatic GABAA receptors protects against seizures in a mouse model of Dravet syndrome.

KEY POINTS: Dravet syndrome mice (Scn1a+/- ) demonstrate a marked strain dependence for the severity of seizures which is correlated with GABAA receptor α2 subunit expression. The α2 /α3 subunit selective positive allosteric modulator (PAM) AZD7325 potentiates inhibitory postsynaptic currents (IPSCs) specifically in perisomatic synapses. AZD7325 demonstrates stronger effects on IPSCs in the seizure resistant mouse strain, consistent with higher α2 subunit expression. AZD7325 demonstrates seizure protective effects in Scn1a+/- mice without apparent sedative effects in vivo. ABSTRACT: GABAA receptor potentiators are commonly used for the treatment of epilepsy, but it is not clear whether targeting distinct GABAA receptor subtypes will have disproportionate benefits over adverse effects. Here we demonstrate that the α2 /α3 selective positive allosteric modulator (PAM) AZD7325 preferentially potentiates hippocampal inhibitory responses at synapses proximal to the soma of CA1 neurons. The effect of AZD7325 on synaptic responses was more prominent in mice on the 129S6/SvEvTac background strain, which have been demonstrated to be seizure resistant in the model of Dravet syndrome (Scn1a+/- ), and in which the α2 GABAA receptor subunits are expressed at higher levels relative to in the seizure prone C57BL/6J background strain. Consistent with this, treatment of Scn1a+/- mice with AZD7325 elevated the temperature threshold for hyperthermia-induced seizures without apparent sedative effects. Our results in a model system indicate that selectively targeting α2 is a potential therapeutic option for Dravet syndrome.

Treatment of myoclonic-atonic epilepsy caused by SLC2A1 de novo mutation with ketogenic diet: A case report.

RATIONALE: The SLC2A1 gene encodes glucose transporter 1 on blood-brain barrier, which plays an important role in the energy supply for neurons. Mutations in SLC2A1 gene can cause many clinical syndromes, including glucose transporter type 1 deficiency syndrome and many types of epilepsy syndromes such as childhood absence epilepsy and myoclonic-atonic epilepsy, etc. Ketogenic diet has been proved to be very effective on those cases. Clinically, SLC2A1 gene mutations are quite rare. PATIENT CONCERNS: Repeated unconsciousness and bilateral limb weakness lasted for 3 years. DIAGNOSES: Myoclonic-atonic epilepsy. LESSONS: After taking whole exome sequencing, we found out that there is a de novo insertion mutation in the patient's SLC2A1 gene, leading to frameshift. As a result, ketogenic diet (2:1, 4 times a day) was used as the treatment. As for the patient, total calories intake per day was controlled at 1190 kcal. The calories per kg per day were 66.11 kcal/kg. The amount of ketone bodies was controlled at 2 to 3 mmol/L and the concentration of plasma glucose was controlled at 4 to 5 mmol/L. OUTCOMES: After the launch of ketogenic diet, the patient has been seizure free for nearly a year and stopped all his antiepileptic drugs. CONCLUSION: Our case suggests that gene examination is very important part of the diagnosis of epilepsy etiology and epilepsy syndromes. Ketogenic diet should be considered as the first line therapy with SLC2A1 gene mutations.

Efficacy of the ketogenic diet in Chinese children with Dravet syndrome: A focus on neuropsychological development.

OBJECTIVES: In this retrospective study, we evaluated the efficacy of the ketogenic diet (KD) treatment in Chinese children with Dravet syndrome (DS) as well as its effect on neuropsychological development. METHODS: Twenty-six children (14 male) living with DS and being treated with KD at our department between July 2014 and December 2017 were enrolled in the study. The efficacy of KD was measured by seizure frequency before and after the diet. Additionally, children's neuropsychological development, as evaluated by the Gesell developmental schedule, was compared between the KD and a non-KD group. RESULTS: After 3, 6, 12, 18, 24, and 30 months, 92.3%, 84.6%, 46.2%, 30.8%, 19.2%, and 19.2% remained on the KD, while 38.4%, 34.6%, 38.4%, 23.0%, 15.4%, and 15.4% showed >50% reduction in seizure. The development age (DA) subscores of 12 children, as measured by the Gesell developmental schedule, increased after commencement of KD. However, children's development quotient (DQ) subscores (age-adjusted) decreased after KD. In the non-KD group (40 participants), an increase of DA subscores and decrease of DQ subscores were also observed. Results found no difference in changes of DQ subscores over time between the two groups. The DQ subscores after the diet in the KD group (20 participants) did not differ significantly when compared to the DQ subscores at same age in the non-KD group (20 patients) (t-test). CONCLUSIONS: The DA subscores of 12 children in KD group increased after KD; when compared with that of the non-KD group, no significant difference was observed in respect to the changes of DQ subscore over time. Effects on cognitive and other neuropsychological development outcomes of KD for children living with DS require further study.

The glucose transporter type 1 (Glut1) syndromes.

The glucose transporter type 1 (Glut1) is the most important energy carrier of the brain across the blood-brain barrier. In the early nineties, the first genetic defect of Glut1 was described and known as the Glut1 deficiency syndrome (Glut1-DS). It is characterized by early infantile seizures, developmental delay, microcephaly, and ataxia. Recently, milder variants have also been described. The clinical picture of Glut1 defects and the understanding of the pathophysiology of this disease have significantly grown. A special form of transient movement disorders, the paroxysmal exertion-induced dyskinesia (PED), absence epilepsies particularly with an early onset absence epilepsy (EOAE) and childhood absence epilepsy (CAE), myoclonic astatic epilepsy (MAE), episodic choreoathetosis and spasticity (CSE), and focal epilepsy can be based on a Glut1 defect. Despite the rarity of these diseases, the Glut1 syndromes are of high clinical interest since a very effective therapy, the ketogenic diet, can improve or reverse symptoms especially if it is started as early as possible. The present article summarizes the clinical features of Glut1 syndromes and discusses the underlying genetic mutations, including the available data on functional tests as well as the genotype-phenotype correlations. This article is part of the Special Issue "Individualized Epilepsy Management: Medicines, Surgery and Beyond".

Prospective study of the efficacy of a ketogenic diet in 20 patients with Dravet syndrome.

PURPOSE: We evaluated the efficacy and tolerability of the ketogenic diet (KD) on generalised convulsions and status epilepticus (SE) in patients with Dravet syndrome (DS). METHODS: Patients with DS having ≥2 generalised convulsions/month despite drug treatment were included in this study and placed on a KD for 6 months. From 3 months before (baseline) to 6 months after KD initiation, caregivers recorded patients' seizure activity, antiepileptic drug use, and adverse events. The KD efficacy was determined by examining the frequency and duration of seizures at 3 and 6 months vs. baseline. Responders were defined as individuals whose generalised convulsions decreased in frequency by ≥50% vs. baseline. Seizures lasting ≥5 min and SE were specifically evaluated. Patients' cognition was also assessed at 3 and 6 months via questionnaire. RESULTS: Twenty patients continued the KD for at least 3 months. Of the 17 responders identified at month 3, seizures decreased by 50-89% and 90-99% in nine and two patients, respectively; six patients were seizure free. The KD was ineffective in three patients, who discontinued the diet. By month 6, seizures decreased by 50-89% and 90-99% in six and one patient(s), respectively; 10 patients were seizure free. The frequency of other seizure types also improved. During all 6 months, neither generalised convulsions lasting ≥5 min nor SE was detected in the 17 responders. The KD also improved patients' cognition. CONCLUSION: The KD is a good treatment option for medically intractable epilepsy.

The ketogenic diet in patients with myoclonic status in non-progressive encephalopathy.

Myoclonic status in non-progressive encephalopathy (MSNPE) is characterized by the recurrence of long-lasting atypical status epilepticus associated with attention impairment and continuous polymorphous jerks, mixed with other complex abnormal movements, in infants suffering from a non-progressive encephalopathy. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies. PURPOSE: In this study we assess the efficacy and tolerability of the KD in patients with MSNPE. METHODS: Between March 1, 1980 and August 31, 2013, 99 patients who met the diagnostic criteria of MSNPE were seen (58 patients in Verona and 41 patients in Buenos Aires). Six of these 99 patients were placed on the KD using the Hopkins protocol and followed for a minimum period of 24 months. RESULTS: Twelve months after initiating the diet, three patients had a 75%-99% decrease in seizures, two had a 50%-74% decrease in seizures, and the remaining child had a less than 50% seizure reduction. In five patients with a seizure reduction of more than 50%, the myoclonic status epilepticus disappeared within 6 months after starting the diet. All patients had very good tolerability and no adverse events were identified. In most of the patients AEDs were reduced. CONCLUSION: The KD is a promising therapy for MSNPE, with most of our patients showing a more than 50% seizure reduction. In patients that responded well to the diet cognitive performance and quality of life also improved.

Efficacy of a ketogenic diet in resistant myoclono-astatic epilepsy: A French multicenter retrospective study.

OBJECTIVE: Recent studies have suggested that the early introduction of a ketogenic diet (KD) could improve seizure control in myoclono-astatic epilepsy (MAE). This multicenter study sought to identify the benefits of KD use on seizure control and epilepsy and on developmental outcomes in children with resistant MAE. METHODS: Fifty children who were diagnosed with severe MAE in the French network of Reference Centers for Rare Epilepsies and who were treated with KD between 2000 and 2013 were included in this study. The seizure frequency and EEG recordings were assessed two weeks before KD introduction, 2 and 6 months after, and during the last follow-up, which also included an assessment of developmental outcome. RESULTS: Patients had a median follow up of 52 months (range 13-136) and received 4.3 antiepileptic drugs [2-9] before KD introduction. Fifty-four percent (54%) of our patients were seizure-free after 6 months of KD or more, and 86% experienced more than a 70% seizure reduction after 2 months of KD. Forty-four percent (44%) of them had a clear benefit of early KD treatment (after four AEDs failed). Early KD treatment did not result in a greater seizure reduction (p=0.055), but significantly resulted in remission (p<0.028). Fifty percent of patients with resistant MAE had normal development outcomes. Earlier KD treatment, after three AEDs failed, was correlated with a better cognitive outcome (p<0.01). SIGNIFICANCE: Early introduction of KD treatment in resistant MAE has a strong, persistent anticonvulsant effect with long-term remission and better cognitive outcomes.

Modified Atkins diet is an effective treatment for children with Doose syndrome.

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. METHODS: Four participating centers retrospectively identified all patients with MAE in whom a MAD had been started before June 2015. Seven children were recruited from a cohort included in an open prospective controlled trial. A retrospective review of all available charts was performed in the other patients. RESULTS: Thirty patients (24 boys) were included. Mean age at epilepsy onset was 3.1 years (range 1.5-5.6). MAD was started at a mean age of 4.5 years (range 2.2-9.1) after the children had received an average of six different AEDs (range 2-15). Mean MAD observation time was 18.7 months (range 1.5-61.5). Twenty of 30 patients were still on MAD at the end of study (duration range 1.5-61.5, mean 18.5 months). MAD was stopped without relapse in three patients after sustained seizure freedom for >2 years. For the other seven cases, ineffectiveness (three patients), loss of efficacy (two), or noncompliance (two) led to termination. No severe adverse effects were noted. By the end of the observation period, 25 (83%) of 30 patients experienced a seizure reduction by ≥50% and 14 (47%) of 30 were seizure-free. None of the evaluated factors differed significantly between the groups of seizure-free and non-seizure-free children. SIGNIFICANCE: MAD is an effective treatment for MAE. It should be considered as an alternative to AEDs or the more restrictive classic ketogenic diet.

SLC6A1 Mutation and Ketogenic Diet in Epilepsy With Myoclonic-Atonic Seizures.

BACKGROUND: Epilepsy with myoclonic-atonic seizures, also known as myoclonic-astatic epilepsy or Doose syndrome, has been recently linked to variants in the SLC6A1 gene. Epilepsy with myoclonic-atonic seizures is often refractory to antiepileptic drugs, and the ketogenic diet is known for treating medically intractable seizures, although the mechanism of action is largely unknown. We report a novel SLC6A1 variant in a patient with epilepsy with myoclonic-atonic seizures, analyze its effects, and suggest a mechanism of action for the ketogenic diet. METHODS: We describe a ten-year-old girl with epilepsy with myoclonic-atonic seizures and a de novo SLC6A1 mutation who responded well to the ketogenic diet. She carried a c.491G>A mutation predicted to cause p.Cys164Tyr amino acid change, which was identified using whole exome sequencing and confirmed by Sanger sequencing. High-resolution structural modeling was used to analyze the likely effects of the mutation. RESULTS: The SLC6A1 gene encodes a transporter that removes gamma-aminobutyric acid from the synaptic cleft. Mutations in SLC6A1 are known to disrupt the gamma-aminobutyric acid transporter protein 1, affecting gamma-aminobutyric acid levels and causing seizures. The p.Cys164Tyr variant found in our study has not been previously reported, expanding on the variants linked to epilepsy with myoclonic-atonic seizures. CONCLUSION: A 10-year-old girl with a novel SLC6A1 mutation and epilepsy with myoclonic-atonic seizures had an excellent clinical response to the ketogenic diet. An effect of the diet on gamma-aminobutyric acid reuptake mediated by gamma-aminobutyric acid transporter protein 1 is suggested. A personalized approach to epilepsy with myoclonic-atonic seizures patients carrying SLC6A1 mutation and a relationship between epilepsy with myoclonic-atonic seizures due to SLC6A1 mutations, GABAergic drugs, and the ketogenic diet warrants further exploration.

The Impact of 3:1 Ketogenic Diet on Cardiac Repolarization Changes in Children with Refractory Seizures: A Prospective Follow-Up Study.

Background The association between ketogenic diet (KD) and prolonged QT interval, life-threatening ventricular arrhythmias, and sudden death is controversial. Aim We aimed to prospectively evaluate the effect of KD on electrocardiography (ECG) measures in children with refractory epilepsy. Method A total of 70 children with drug-resistant epilepsy who received a KD for at least 12 months were included in the study. The standard 12-lead electrocardiography was performed in all patients before the beginning and in the 12th month of KD. Heart rate, P-wave duration and dispersion, corrected QT interval and QT dispersion, and Tp-e interval were measured. Results All ECG-derived parameters, but P-wave dispersion increased after 12 months of KD compared with the baseline values. However, these changes were not statistically significant. Conclusion A 12-month long 3:1 KD treatment exerts no deleterious effect on cardiac repolarization measures.

Altered Glycolysis and Mitochondrial Respiration in a Zebrafish Model of Dravet Syndrome.

Altered metabolism is an important feature of many epileptic syndromes but has not been reported in Dravet syndrome (DS), a catastrophic childhood epilepsy associated with mutations in a voltage-activated sodium channel, Nav1.1 (SCN1A). To address this, we developed novel methodology to assess real-time changes in bioenergetics in zebrafish larvae between 4 and 6 d postfertilization (dpf). Baseline and 4-aminopyridine (4-AP) stimulated glycolytic flux and mitochondrial respiration were simultaneously assessed using a Seahorse Biosciences extracellular flux analyzer. Scn1Lab mutant zebrafish showed a decrease in baseline glycolytic rate and oxygen consumption rate (OCR) compared to controls. A ketogenic diet formulation rescued mutant zebrafish metabolism to control levels. Increasing neuronal excitability with 4-AP resulted in an immediate increase in glycolytic rates in wild-type zebrafish, whereas mitochondrial OCR increased slightly and quickly recovered to baseline values. In contrast, scn1Lab mutant zebrafish showed a significantly slower and exaggerated increase of both glycolytic rates and OCR after 4-AP. The underlying mechanism of decreased baseline OCR in scn1Lab mutants was not because of altered mitochondrial DNA content or dysfunction of enzymes in the electron transport chain or tricarboxylic acid cycle. Examination of glucose metabolism using a PCR array identified five glycolytic genes that were downregulated in scn1Lab mutant zebrafish. Our findings in scn1Lab mutant zebrafish suggest that glucose and mitochondrial hypometabolism contribute to the pathophysiology of DS.

The ketogenic diet in two paediatric patients with refractory myoclonic status epilepticus.

AIM: We describe two patients with refractory myoclonic status epilepticus treated with the ketogenic diet. METHODS: Between May 1, 2014 and January 1, 2015, two patients who met the diagnostic criteria for refractory myoclonic status epilepticus, seen at our department, were placed on the ketogenic diet and followed for a minimum of six months. RESULTS: One patient with myoclonic epilepsy of unknown aetiology had a 75-90% seizure reduction, and the other with progressive encephalopathy associated with myoclonic epilepsy had a 50% seizure reduction. Both patients retained good tolerability for the diet. At the last control, one patient had isolated myoclonias and EEG showed occasional generalized spike-and-polyspike waves; the patient is now successfully attending kindergarten. The quality of life of the second patient improved significantly. In both cases, the number of antiepileptic drugs was reduced. CONCLUSION: The ketogenic diet is an effective and well-tolerated treatment option for patients with refractory myoclonic status epilepticus and should be considered earlier in the course of treatment.

Glucose Transporter 1 Deficiency: A Treatable Cause of Opsoclonus and Epileptic Myoclonus.

BACKGROUND: Epileptic myoclonus in infancy is associated with various pathological conditions. In the absence of an identifiable central nervous system lesion, an underlying metabolic genetic cause is often suspected. PATIENTS: We describe two infants with glucose transporter 1 deficiency syndrome who presented with epileptic myoclonus. One infant presented with an electroclinical phenotype mimicking benign myoclonic epilepsy of infancy; the other infant had a novel mutation and presented with opsoclonus and epileptic myoclonus with a robust response to high-dose steroids. Both infants began the ketogenic diet after the diagnosis of glucose transporter 1 deficiency syndrome, with good yet variable treatment responses. CONCLUSIONS: These infants demonstrate that an evaluation for glucose transporter 1 deficiency syndrome is warranted in patients presenting with an electroclinical picture compatible with benign myoclonic epilepsy of infancy as well as in patients with intractable epilepsy who demonstrate a significant response to steroid therapy.

High-fat diets and seizure control in myoclonic-astatic epilepsy: a single center's experience.

PURPOSE: To determine the efficacy of the Modified Atkins Diet (MAD) and Ketogenic Diet (KD) in seizure control within a population of myoclonic-astatic epilepsy (MAE) patients. METHODS: This was a retrospective, single center study evaluating the seizure control by high fat diets. Seizure diaries kept by the parents performed seizure counts. All patients met the clinical criteria for MAE. RESULTS: Nine patients met the clinical criteria. We found that both the MAD and KD were efficacious in complete seizure control and allowed other medications to be stopped in seven patients. Two patients had greater than 90% seizure control without medications, one on the KD and the other on the MAD. Seizure freedom has ranged from 13 to 36 months, and during this time four patients have been fully weaned off of diet management. One patient was found to have a mutation in SLC2A1. CONCLUSION: Our results suggest that strictly defined MAE patients respond to the MAD with prolonged seizure control. Some patients may require the KD for seizure freedom, suggesting a common pathway of increased requirement for fats. Once controlled, those fully responsive to the Diet(s) could be weaned off traditional seizure medications and in many, subsequently off the MAD or KD.

The ketogenic diet for the treatment of myoclonic astatic epilepsy in a child with type 1 diabetes mellitus.

Initiation of the ketogenic diet in a child with epilepsy and type 1 diabetes mellitus presents a challenge because the distinction between diet-induced ketosis and diabetic ketoacidosis is difficult to discern. We report the successful use of the ketogenic diet in a child with myoclonic astatic epilepsy and type 1 diabetes.

The ketogenic diet in Dravet syndrome.

Dravet syndrome is an infantile epilepsy syndrome with intractable pleomorphic seizures, cognitive impairment, and a number of comorbidities including ataxia/gait abnormalities and behavioral issues. Antiseizure medications are only partially effective in controlling seizures. Secondary to the intractable epilepsy, patients are often on multiple antiseizure medications with significant accumulative neurotoxic side effects. Specifically for Dravet syndrome, the medical literature includes both laboratory and clinical research that supports the use of the ketogenic diet. In addition, a review of the children with Dravet syndrome who were treated with the ketogenic diet at our center was undertaken. Thirteen of the 20 children (65%) with Dravet syndrome treated with the ketogenic diet experienced a greater than 50% reduction in seizure frequency. The ketogenic diet is a good alternative to medication for seizure management in children with Dravet syndrome.

Myoclonic astatic epilepsy and the use of the ketogenic diet.

Myoclonic astatic epilepsy (MAE) is a rare childhood generalized epilepsy syndrome of unknown incidence and etiology. Onset may be explosive with a myriad of different seizure types and children may become severely affected with an epileptic encephalopathy. This disorder may be particularly sensitive to the ketogenic diet (KD). This article will briefly review the background, diagnostic criteria's and our current information regarding the use of dietary therapies in MAE.

Experiencia con la dieta cetogénica como tratamiento en la epilepsia refractaria / Experience with ketogenic diet as treatment for refractory epilepsy

Introducción. La epilepsia es una enfermedad neurológica que se controla con fármacos antiepilépticos en la mayoría de casos. Sin embargo, aproximadamente un 25% de epilepsias son refractarias al tratamiento farmacológico. La dieta cetogénica es una de las opciones terapéuticas para este tipo de epilepsia. A pesar del aumento en los últimos años de la popularidad de ésta como tratamiento anticonvulsivo, no se ha establecido un consenso internacional para sus indicaciones y manejo. Objetivo. Evaluar la respuesta, tolerancia y efectos secundarios en los pacientes con epilepsias refractarias que han recibido dieta cetogénica en nuestra institución en un período de 20 años. Pacientes y métodos. Se revisaron las historias clínicas de 30 pacientes que utilizaron dieta cetogénica como coadyuvante al tratamiento de epilepsia refractaria seguidos en nuestro centro. Se obtuvo información completa para el estudio en 27 casos. Resultados. Diez pacientes (35,7%) presentaron una respuesta positiva en la reducción del número de crisis por más de seis meses; cinco de ellos con una disminución del 50-75% de las crisis y cinco de más del 75%. Los efectos adversos más frecuentes a corto plazo fueron diarrea, vómitos e hipoglucemias, y a largo plazo, estreñimiento y aumento de peso. Conclusiones. Existe una respuesta positiva con la dieta cetogénica en una tercera parte de nuestros pacientes con epilepsia refractaria. La tolerancia es aceptable y los efectos adversos existentes pueden prevenirse o corregirse. Puede considerarse una opción terapéutica frente a epilepsias refractarias antes de iniciar otras medidas más agresivas o cuando no es factible la opción quirúrgica (AU)

Introduction. Epilepsy is a disease where most patients have a good control with pharmacological antiepileptic treatment. Nevertheless, 25% of the patients have a refractory epilepsy to usual antiepileptic drugs. Ketogenic diet is one of the treatment options for this type of epilepsy. In spite of the increased popularity of it as an antiepileptic treatment, it does not exist an international consensus of its indications and management. Aim. To evaluate the response, tolerance and adverse effects of the patients with refractory epilepsy at our hospital during the last 20 years. Patients and methods. We reviewed the data of 30 patients with ketogenic diet and the follow-up at the Neurology and Nutrition Services in our Hospital. Results. Ten patients (35.7%) had a positive response with reduction of their seizures for more than six months; five of them had a 50-75% decrease in seizures and five of them had more than 75% of seizure reduction. The most common short term adverse effects were diarrhea, vomiting and hypoglicemia whereas long term adverse effects were constipation and weight gain. Conclusions. We recommend to use ketogenic diet as treatment in refractory epilepsy since there is a positive response in seizure control in some cases. The adverse effects seen could be prevented or treated without complications. It is a preferabletreatment option before using other aggressive therapeutical measures or when surgery is not feasible (AU)

Myoclonic astatic epilepsy and the role of the ketogenic diet.

The ketogenic diet remains one of the most effective treatments for medically refractory childhood epilepsy. In spite of the long history of its use, relatively little is known about the mechanism of action. The diet's efficacy in a wide range of epilepsy syndromes suggests that it may have multiple mechanisms of action, each of which may be more relevant to a specific disease state. Further research is necessary to define the mechanism of action, which may, in turn, lead to easier means to provide the therapeutic benefit.