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Adam L Hartman - One of the best experts on this subject based on the ideXlab platform.
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The Ketogenic Diet: Uses in epilepsy and other neurologic illnesses
Current Treatment Options in Neurology, 2008Co-Authors: Kristin W. Barañano, Adam L HartmanAbstract:The Ketogenic Diet is well established as therapy for intractable epilepsy. It should be considered first-line therapy in glucose transporter type 1 and pyruvate dehydrogenase deficiency. It should be considered early in the treatment of Dravet syndrome and myoclonic-astatic epilepsy (Doose syndrome). Initial studies indicate that the Ketogenic Diet appears effective in other metabolic conditions, including phosphofructokinase deficiency and glycogenosis type V (McArdle disease). It appears to function in these disorders by providing an alternative fuel source. A growing body of literature suggests the Ketogenic Diet may be beneficial in certain neurodegenerative diseases, including Alzheimer disease, Parkinson's disease, and amyotrophic lateral sclerosis. In these disorders, the Ketogenic Diet appears to be neuroprotective, promoting enhanced mitochondrial function and rescuing adenosine triphosphate production. Dietary therapy is a promising intervention for cancer, given that it may target the relative inefficiency of tumors in using ketone bodies as an alternative fuel source. The Ketogenic Diet also may have a role in improving outcomes in trauma and hypoxic injuries.
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the neuropharmacology of the Ketogenic Diet
Pediatric Neurology, 2007Co-Authors: Adam L Hartman, Eileen P G Vining, Maciej Gasior, Michael A. RogawskiAbstract:The Ketogenic Diet is a valuable therapeutic approach for epilepsy, one in which most clinical experience has been with children. Although the mechanism by which the Diet protects against seizures is unknown, there is evidence that it causes effects on intermediary metabolism that influence the dynamics of the major inhibitory and excitatory neurotransmitter systems in brain. The pattern of protection of the Ketogenic Diet in animal models of seizures is distinct from that of other anticonvulsants, suggesting that it has a unique mechanism of action. During consumption of the Ketogenic Diet, marked alterations in brain energy metabolism occur, with ketone bodies partly replacing glucose as fuel. Whether these metabolic changes contribute to acute seizure protection is unclear; however, the ketone body acetone has anticonvulsant activity and could play a role in the seizure protection afforded by the Diet. In addition to acute seizure protection, the Ketogenic Diet provides protection against the development of spontaneous recurrent seizures in models of chronic epilepsy, and it has neuroprotective properties in diverse models of neurodegenerative disease.
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the Ketogenic Diet one decade later
Pediatrics, 2007Co-Authors: J M Freeman, Eric H Kossoff, Adam L HartmanAbstract:The Ketogenic Diet, a high fat, adequate protein, low carbohydrate Diet, has, during the past decade, had a resurgence of interest for the treatment of difficult-tocontrol seizures in children. This review traces its history, reviews its uses and side effects, and discusses possible alternatives and the Diet’s possible mechanisms of action. Finally, this review looks toward possible future uses of the Ketogenic Diet for conditions other than epilepsy.
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The Neuropharmacology of the Ketogenic Diet
Pediatric Neurology, 2007Co-Authors: Adam L Hartman, Eileen P G Vining, Maciej Gasior, Michael A. RogawskiAbstract:The Ketogenic Diet is a valuable therapeutic approach for epilepsy, one in which most clinical experience has been with children. Although the mechanism by which the Diet protects against seizures is unknown, there is evidence that it causes effects on intermediary metabolism that influence the dynamics of the major inhibitory and excitatory neurotransmitter systems in brain. The pattern of protection of the Ketogenic Diet in animal models of seizures is distinct from that of other anticonvulsants, suggesting that it has a unique mechanism of action. During consumption of the Ketogenic Diet, marked alterations in brain energy metabolism occur, with ketone bodies partly replacing glucose as fuel. Whether these metabolic changes contribute to acute seizure protection is unclear; however, the ketone body acetone has anticonvulsant activity and could play a role in the seizure protection afforded by the Diet. In addition to acute seizure protection, the Ketogenic Diet provides protection against the development of spontaneous recurrent seizures in models of chronic epilepsy, and it has neuroprotective properties in diverse models of neurodegenerative disease. © 2007 Elsevier Inc. All rights reserved.
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clinical aspects of the Ketogenic Diet
Epilepsia, 2007Co-Authors: Adam L Hartman, Eileen P G ViningAbstract:The Ketogenic Diet remains a valuable therapeutic option for patients with intractable epilepsy. Clinical aspects of the Diet's success may provide insights into epileptogenesis and anticonvulsant action. The Diet's efficacy has been established primarily through large case series. The Diet has been used successfully in patients with many different epilepsy syndromes in countries around the world. Potential adverse effects can be avoided with careful attention during the Diet's initiation and maintenance phases. In the last decade, variations to the classical Ketogenic Diet have been utilized. Ketogenic Diets now are being used for diseases other than epilepsy. This critical analysis of the Diet should provide the impetus for further clinical and basic research into the Diet's application and mechanisms of action.
Darryl C De Vivo - One of the best experts on this subject based on the ideXlab platform.
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experience with the Ketogenic Diet in infants
Pediatrics, 2001Co-Authors: Douglas R Nordli, Maxine M Kuroda, Joanne Carroll, Dorcas Koenigsberger, Lawrence J Hirsch, Harlan J Bruner, William T Seidel, Darryl C De VivoAbstract:Objective. To evaluate the effectiveness, tolerability, and adverse effects of the Ketogenic Diet in infants with refractory epilepsy. Methods. A retrospective review of 32 infants who had been treated with the Ketogenic Diet at a large metropolitan institution. Results. Most infants (71%) were able to maintain strong ketosis. The overall effectiveness of the Diet in infants was similar to that reported in the literature for older children; 19.4% became seizure-free, and an additional 35.5% had >50% reduction in seizure frequency. The Diet was particularly effective for patients with infantile spasms/myoclonic seizures. There were concomitant reductions in antiepileptic medications. The majority of parents reported improvements in seizure frequency and in their child9s behavior and function, particularly with respect to attention/alertness, activity level, and socialization. The Diet generally was well-tolerated, and 96.4% maintained appropriate growth parameters. Adverse events, all reversible and occurring in one patient each, included renal stone, gastritis, ulcerative colitis, alteration of mentation, and hyperlipidemia. Conclusion. The Ketogenic Diet should be considered safe and effective treatment for infants with intractable seizures.
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the Ketogenic Diet revisited back to the future
Epilepsia, 1997Co-Authors: Douglas R Nordli, Darryl C De VivoAbstract:There has been a recent extraordinary resurgence of interest in the Ketogenic Diet. Some commentaries in the professional and lay literature have been instructive and informative, others have been less so, and some have been frankly contentious. Books, an informative videotape, a made-for-television movie starring Meryl Streep, and many websites are just some of the current venues that are providing information regarding the Diet (1). An unfortunate byproduct of this popularization has been a fruitless dialectic: “Does the Ketogenic Diet deserve a prominent role in the management of children with refractory epilepsy?” The answer to this question has always been yes. In our view, this is a distracting issue and one that should be put aside, because the Diet is a time-tested, effective, and safe method for treatment of intractable childhood epilepsy. Only bromides, discovered in 1857, and phenobarbital, discovered in 1910, can match the 76 years of experience with the Ketogenic Diet. Numerous centers have documented its efficacy, and clinical reports indicate that at least one-third to two-thirds of patients benefit substantially from the Diet (2-5). Results of treatment with few antiepileptic drugs (AEDs) can match these statistics. Like AEDs the Ketogenic Diet has also demonstrated efficacy in experimental models of seizures (6-8). At the Neurological Institute of New York, clinicians have used the conventional long-chain Diet as a mainstay for the treatment of drug-resistant childhood epilepsy for >30 years, with good success (9). In the last several years, our Dietitians and nutritionists have used a novel and effective approach. The Diet is organized into food groups so that substitutions among different foods can be made easily. This makes the Diet simpler to use and provides parents with greater flexibility. The broad picture regarding the mechanics and the effectiveness of the Ketogenic Diet has been clear since 1921. Therefore, we regard
Eileen P G Vining - One of the best experts on this subject based on the ideXlab platform.
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Experience in the Use of the Ketogenic Diet as Early Therapy
Journal of Child Neurology, 2016Co-Authors: James E. Rubenstein, Eileen P G Vining, Eric H Kossoff, P L Pyzik, Jane R. Mcgrogan, J M FreemanAbstract:The Ketogenic Diet has traditionally been considered an anticonvulsant therapy of last resort, despite excellent efficacy and limited side effects. We hypothesized that the Ketogenic Diet would have similar results in patients with new-onset epilepsy. A retrospective study was conducted of patients started on the Ketogenic Diet since 1994. Thirteen of 460 (2.8%) patients were started on the Ketogenic Diet as early (zero or one prior anticonvulsant) therapy for seizures. Of those remaining on the Diet, 60% (6 of 10) had a > 90% seizure reduction at 6 months and 100% (6 of 6) had a > 90% reduction at 12 months. Patients with infantile spasms were as likely to achieve > 50% seizure reduction at 6 months as patients with other seizure types (75% vs 60%; P = .6). The Ketogenic Diet can be a valuable therapy before epilepsy becomes intractable. In the 13 patients reported, efficacy without side effects was achieved similarly to that with patients with intractable epilepsy. (J Child Neurol 2005;20:31—34).
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the neuropharmacology of the Ketogenic Diet
Pediatric Neurology, 2007Co-Authors: Adam L Hartman, Eileen P G Vining, Maciej Gasior, Michael A. RogawskiAbstract:The Ketogenic Diet is a valuable therapeutic approach for epilepsy, one in which most clinical experience has been with children. Although the mechanism by which the Diet protects against seizures is unknown, there is evidence that it causes effects on intermediary metabolism that influence the dynamics of the major inhibitory and excitatory neurotransmitter systems in brain. The pattern of protection of the Ketogenic Diet in animal models of seizures is distinct from that of other anticonvulsants, suggesting that it has a unique mechanism of action. During consumption of the Ketogenic Diet, marked alterations in brain energy metabolism occur, with ketone bodies partly replacing glucose as fuel. Whether these metabolic changes contribute to acute seizure protection is unclear; however, the ketone body acetone has anticonvulsant activity and could play a role in the seizure protection afforded by the Diet. In addition to acute seizure protection, the Ketogenic Diet provides protection against the development of spontaneous recurrent seizures in models of chronic epilepsy, and it has neuroprotective properties in diverse models of neurodegenerative disease.
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The Neuropharmacology of the Ketogenic Diet
Pediatric Neurology, 2007Co-Authors: Adam L Hartman, Eileen P G Vining, Maciej Gasior, Michael A. RogawskiAbstract:The Ketogenic Diet is a valuable therapeutic approach for epilepsy, one in which most clinical experience has been with children. Although the mechanism by which the Diet protects against seizures is unknown, there is evidence that it causes effects on intermediary metabolism that influence the dynamics of the major inhibitory and excitatory neurotransmitter systems in brain. The pattern of protection of the Ketogenic Diet in animal models of seizures is distinct from that of other anticonvulsants, suggesting that it has a unique mechanism of action. During consumption of the Ketogenic Diet, marked alterations in brain energy metabolism occur, with ketone bodies partly replacing glucose as fuel. Whether these metabolic changes contribute to acute seizure protection is unclear; however, the ketone body acetone has anticonvulsant activity and could play a role in the seizure protection afforded by the Diet. In addition to acute seizure protection, the Ketogenic Diet provides protection against the development of spontaneous recurrent seizures in models of chronic epilepsy, and it has neuroprotective properties in diverse models of neurodegenerative disease. © 2007 Elsevier Inc. All rights reserved.
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clinical aspects of the Ketogenic Diet
Epilepsia, 2007Co-Authors: Adam L Hartman, Eileen P G ViningAbstract:The Ketogenic Diet remains a valuable therapeutic option for patients with intractable epilepsy. Clinical aspects of the Diet's success may provide insights into epileptogenesis and anticonvulsant action. The Diet's efficacy has been established primarily through large case series. The Diet has been used successfully in patients with many different epilepsy syndromes in countries around the world. Potential adverse effects can be avoided with careful attention during the Diet's initiation and maintenance phases. In the last decade, variations to the classical Ketogenic Diet have been utilized. Ketogenic Diets now are being used for diseases other than epilepsy. This critical analysis of the Diet should provide the impetus for further clinical and basic research into the Diet's application and mechanisms of action.
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kidney stones carbonic anhydrase inhibitors and the Ketogenic Diet
Epilepsia, 2002Co-Authors: Eric H Kossoff, J M Freeman, Susan L Furth, P L Pyzik, Heather Hladky, Eileen P G ViningAbstract:PURPOSE: Because carbonic anhydrase inhibitors and the Ketogenic Diet are each known risk factors for kidney stones, simultaneous use of these therapies has been discouraged. The objective of this study was to establish the prevalence of nephrolithiasis in children in this combination-therapy population. METHODS: Since 1996, 301 children have been started on the Ketogenic Diet at our institution. A retrospective cohort study of renal calculi in Ketogenic Diet patients was performed to evaluate the increased risk with combined use of a carbonic anhydrase inhibitor. RESULTS: In 15 (6.7%) of 221 children on the Ketogenic Diet without the use of carbonic anhydrase inhibitors, stones developed. In five (6.3%) of the 80 children on the Diet in combination with topiramate or zonisamide, stones developed. There was no difference between these two groups (p = 0.82). No child was treated with either acetazolamide or more than one carbonic anhydrase inhibitor simultaneously. Prior Ketogenic Diet duration was shorter (10.4 vs. 22.4 months; p = 0.03), and more children had either a family history of renal stones or significant urologic abnormalities (80 vs. 27%; p = 0.04) in the combination-therapy group. CONCLUSIONS: The combined use of carbonic anhydrase inhibitors and the Ketogenic Diet does not increase the risk of kidney stones. We recommend that all patients treated with combination therapy should be treated with increased hydration. Urine alkalinization should be considered for children with previous renal abnormalities, family histories of kidney stones, hematuria, or elevated urine calcium-to-creatinine ratios. If renal stones are found, we advocate discontinuation of the carbonic anhydrase inhibitor.
Eric H Kossoff - One of the best experts on this subject based on the ideXlab platform.
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Experience in the Use of the Ketogenic Diet as Early Therapy
Journal of Child Neurology, 2016Co-Authors: James E. Rubenstein, Eileen P G Vining, Eric H Kossoff, P L Pyzik, Jane R. Mcgrogan, J M FreemanAbstract:The Ketogenic Diet has traditionally been considered an anticonvulsant therapy of last resort, despite excellent efficacy and limited side effects. We hypothesized that the Ketogenic Diet would have similar results in patients with new-onset epilepsy. A retrospective study was conducted of patients started on the Ketogenic Diet since 1994. Thirteen of 460 (2.8%) patients were started on the Ketogenic Diet as early (zero or one prior anticonvulsant) therapy for seizures. Of those remaining on the Diet, 60% (6 of 10) had a > 90% seizure reduction at 6 months and 100% (6 of 6) had a > 90% reduction at 12 months. Patients with infantile spasms were as likely to achieve > 50% seizure reduction at 6 months as patients with other seizure types (75% vs 60%; P = .6). The Ketogenic Diet can be a valuable therapy before epilepsy becomes intractable. In the 13 patients reported, efficacy without side effects was achieved similarly to that with patients with intractable epilepsy. (J Child Neurol 2005;20:31—34).
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Hepatic Dysfunction as a Complication of Combined Valproate and Ketogenic Diet
Pediatric Neurology, 2015Co-Authors: Clare E. Stevens, Zahava Turner, Eric H KossoffAbstract:Abstract Background The Ketogenic Diet has long been shown to be an effective therapy for children with medication-refractory seizures. Most complications of the Ketogenic Diet include short-lived gastrointestinal disturbances, acidosis, and dyslipidemia. Hepatic dysfunction and pancreatitis are among the less common but more serious complications of the Ketogenic Diet. Many patients on the Ketogenic Diet receive adjunct treatment with an anticonvulsant drug, and valproate is frequently used. Methods and Results We describe a child who developed hepatic dysfunction in association with the combined use of valproate and the Ketogenic Diet. After stopping the valproate and then restarting the Ketogenic Diet, her liver enzymes normalized, and she was able to achieve markedly improved seizure control and quality of life. Conclusions Although caution should be advised when using both treatments simultaneously, the development of hepatic dysfunction should not preclude continuation of the Ketogenic Diet, as the hepatotoxic effects may be completely reversed once the valproate is stopped.
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Is there an ideal way to discontinue the Ketogenic Diet
Epilepsy Research, 2011Co-Authors: Lila T. Worden, P L Pyzik, Zahava Turner, James E. Rubenstein, Eric H KossoffAbstract:Summary It is unclear what the ideal weaning speed of the Ketogenic Diet should be and the resultant risk of seizure worsening. A retrospective chart review was performed of children who discontinued the Ketogenic Diet at Johns Hopkins Hospital from January 2000 to June 2010. Speed of discontinuation was categorized into immediate ( 6 weeks) rates. One hundred and eighty-three children were identified. Children with both a longer Diet duration ( p =0.004) and lower seizure frequency ( p p p =0.01). In summary, there does not appear to be an increased risk of seizure exacerbation with rapid Ketogenic Diet discontinuations. Those who improved 50–99% and were receiving more anticonvulsants were at the highest risk overall. Discontinuing the Ketogenic Diet over weeks rather than months appears safe.
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long term outcomes of children treated with the Ketogenic Diet in the past
Epilepsia, 2010Co-Authors: Amisha Patel, P L Pyzik, Zahava Turner, James E. Rubenstein, Eric H KossoffAbstract:Summary Purpose: The Ketogenic Diet has well-established short- and long-term outcomes for children with intractable epilepsy, but only for those actively receiving it. However, no information exists about its long-term effects years after it has been discontinued. Methods: Living subjects were identified who were treated at the Johns Hopkins Hospital with the Ketogenic Diet from November 1993 to December 2008 for ≥1 month, and had discontinued it ≥6 months prior to this study. Of 530 patients who were eligible, 254 were successfully contacted by phone or e-mail with a survey and request for laboratory studies. Results: Questionnaires were completed by 101 patients, with a median current age of 13 years (range 2–26 years). Median time since discontinuing the Ketogenic Diet was 6 years (range 0.8–14 years). Few (8%) still preferred to eat high fat foods. In comparison to the 52% responder rate (>50% seizure reduction) at Ketogenic Diet discontinuation, 79% were now similarly improved (p = 0.0001). Ninety-six percent would recommend the Ketogenic Diet to others, yet only 54% would have started it before trying anticonvulsants. Lipids were normal (mean total cholesterol 158 mg/dl), despite most being abnormal while on the Ketogenic Diet. The mean Z scores for those younger than age 18 years were −1.28 for height and −0.79 for weight. In those 18 years of age or older, the mean body mass index (BMI) was 22.2. Discussion: This is the first study to report on the long-term effects of the Ketogenic Diet after discontinuation. The majority of subjects are currently doing well with regard to health and seizure control.
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the Ketogenic Diet one decade later
Pediatrics, 2007Co-Authors: J M Freeman, Eric H Kossoff, Adam L HartmanAbstract:The Ketogenic Diet, a high fat, adequate protein, low carbohydrate Diet, has, during the past decade, had a resurgence of interest for the treatment of difficult-tocontrol seizures in children. This review traces its history, reviews its uses and side effects, and discusses possible alternatives and the Diet’s possible mechanisms of action. Finally, this review looks toward possible future uses of the Ketogenic Diet for conditions other than epilepsy.
Jung Mi Rho - One of the best experts on this subject based on the ideXlab platform.
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The Ketogenic Diet and epilepsy
Current Opinion in Clinical Nutrition and Metabolic Care, 2008Co-Authors: Do-young Kim, Jung Mi RhoAbstract:bodies may attenuate spontaneous firing of ATP-sensitive potassium channels in central neurons, and pharmacological inhibition of glycolysis has been shown to retard epileptogenesis in a rat kindling model. Summary While the mechanisms underlying the broad clinical efficacy of the Ketogenic Diet remain unclear, there is growing evidence that the Ketogenic Diet alters the fundamental biochemistry of neurons in a manner that not only inhibits neuronal hyperexcitability but also induces a protective effect. Thus, the Ketogenic Diet may ultimately be useful in the treatment of a variety of neurological disorders
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The Ketogenic Diet and epilepsy: Nutritional aspects
Epilepsy Currents, 2008Co-Authors: Do-young Kim, Jung Mi RhoAbstract:Rationale: The Ketogenic Diet is a high fat, adequate protein, low carbohydrate Diet that mimics the physiological state of starvation. It has been observed that this state has anti-epileptic effects, yet the underlying mechanism is still unknown. This therapeutic form of treatment is often prescribed to patients with medically refractory epilepsy, specifically in the pediatric population. Given that the best responders to the Diet are children, it is imperative to understand how a metabolic imbalance during critical growth periods can present clinically in the future. Patients that experience a reduction in seizures often remain on the Ketogenic Diet for an extended period of time, for as long as two or more years. There is minimal primary research investigating the long-term nutritional and neurological outcomes of adopting the Ketogenic Diet. We examined 18 clinical trials that investigate the influence of the Ketogenic Diet on seizure reduction and 12 clinical trials investigating the nutritional implications of prolonged exposure to the Ketogenic Diet. Methods: We have performed literature searches in order to locate primary sources of research that measure the nutritional adequacy and neurological responses to the Ketogenic Diet. Each article has been evaluated based on the methods, time span, sample size, efficacy, and results. Results: The Ketogenic Diet has been shown to be an effective treatment alternative especially for young children with intractable seizures. The nutritional risks that present with the Diet include decreased vitamin A, selenium, magnesium, phosphorus, folate, and calcium levels. These vitamins and minerals are crucial to proper growth and development. Despite their supplementation, weight gain and height growth is very minimal on the Diet. Conclusions: : It is questionable whether or not the benefits outweigh the risks when putting a child on the Ketogenic Diet. Nutritional deficiencies at a young age can have major consequences in the future. Since pediatric patients have better compliance and success rates with the Diet, there is a need for more prospective longterm studies that follow pediatric patients over time in order to track seizure activity and brain development into adulthood.
