Article

Ketogenic Diet in Patients with Dravet Syndrome

Paediatric Hospital Dr. Juan P. Garrahan, Buenos Aires, Buenos Aires F.D., Argentina
Epilepsia (Impact Factor: 4.58). 10/2005; 46(9):1539-44. DOI: 10.1111/j.1528-1167.2005.05705.x
Source: PubMed

ABSTRACT The ketogenic diet (KD) has been used as a therapeutic alternative to antiepileptic drugs (AEDs) for refractory epilepsy. Severe myoclonic epilepsy in infants or Dravet syndrome (DS) is one of the most malignant epileptic syndromes. In this retrospective study, we evaluated the efficacy and tolerability of the KD in patients with diagnostic criteria of DS.
Between March 1, 1990, and August 31, 2004, 52 patients who met diagnostic criteria for DS were enrolled in a study at our department. Twenty of them were placed on the KD with the Hopkins protocol and followed up for a minimum of 1 year.
Three of the 20 original children stayed on the diet for 12 months, four children for 2 years, four children for 3 years, and two children for 4 years. One year after initiating the diet, 13 (65%) of the initial patients remained on the diet. Two (15%) patients were seizure free, eight (61.7%) children had a 75-99% decrease in seizures, and the remaining three (23%) children had a 50-74% decrease in seizures. Thus 1 year after starting the diet, 10 (77%) children had achieved a >75% decrease in their seizures. Four patients have been off the diet for >2 years; one of them is seizure free, two have sporadic seizures, and one, who abandoned the diet after 2 years of adhering to it, relapsed. No differences in seizure control when compared with age, sex, or seizure type were found.
Considering the severity and intractability of seizures in patients with DS, the fact that 10 of the 13 children who remained on the diet had a significant reduction in number of seizures shows that the KD is at present an interesting therapeutic alternative. Even in patients in whom seizure reduction was not dramatic, quality of life improved, and in all of them, the number of AEDs was reduced to one or two. We consider that children with DS should be offered the KD immediately after three adequate trials of AEDs have failed.

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    • "On the other hand, the KD seems to be mainly effective for of seizure worsening and functional deterioration [7], which is the case in status epilepticus and also epileptic encephalopathy [15]. The KD was indeed reported as largely useful in epileptic encephalopathies through retrospective studies, namely Dravet syndrome [16] [17], myoclonic– astatic epilepsy [4] [18], infantile spasms [19], Lennox–Gastaut syndrome [20], FIRES [5], and refractory absences [21]. In all these conditions, the diet was added to the antiepileptic medication. "
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    ABSTRACT: Hormonal therapy or ketogenic diet often permits overcoming the challenging periods of many epileptic encephalopathies (West and Lennox-Gastaut syndromes and encephalopathy with continuous spike-waves in slow sleep), but relapse affects over 20% of patients. We report here a monocenter pilot series of 42 consecutive patients in whom we combined oral steroids with the ketogenic diet for corticosteroid-resistant or -dependent epileptic encephalopathy. We retrospectively evaluated the effect on seizure frequency, interictal spike activity, neuropsychological course, and steroid treatment course. Twenty-three patients had West syndrome (WS), 13 had encephalopathy with continuous spike-waves in slow sleep (CSWS), and six others had miscellaneous epileptic encephalopathies. All patients succeeded to reach 0.8 to 1.6g/l ketone bodies in the urine following the usual KD regimen. For at least 6months, 14/42 responded to the addition of the ketogenic diet: 4/23 with WS, 8/13 with CSWS, and 2/6 with miscellaneous epileptic encephalopathies. The addition of the KD allowed withdrawing steroids in all responders. Among them, 10/15 had been patients with steroid-dependent epileptic encephalopathy and 4/27 patients with steroid-resistant epileptic encephalopathy. Therefore, the ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies. Patients presenting with steroid-dependent CSWS seem to be the best candidates. Copyright © 2015 Elsevier Inc. All rights reserved.
    Epilepsy & Behavior 06/2015; 48:61-65. DOI:10.1016/j.yebeh.2015.03.003 · 2.06 Impact Factor
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    • "Twenty patients were given the diet and thirteen continued on the diet for at least one year. There was more than 75% reduction in seizures in ten children with two who became seizure-free40). Other studies have also reported similar results41,42). "
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    ABSTRACT: The ketogenic diet has been widely used and proved to be effective for intractable epilepsy. Although the mechanisms underlying its anti-epileptic effects remain to be proven, there are increasing experimental evidences for its neuroprotective effects along with many researches about expanding use of the diet in other neurologic disorders. The first success was reported in glucose transporter type 1 deficiency syndrome, in which the diet served as an alternative metabolic source. Many neurologic disorders share some of the common pathologic mechanisms such as mitochondrial dysfunction, altered neurotransmitter function and synaptic transmission, or abnormal regulation of reactive oxygen species, and the role of the ketogenic diet has been postulated in these mechanisms. In this article, we introduce an overview about the expanding use and emerging trials of the ketogenic diet in various neurologic disorders excluding intractable epilepsy and provide explanations of the mechanisms in that usage.
    Korean Journal of Pediatrics 09/2012; 55(9):316-21. DOI:10.3345/kjp.2012.55.9.316
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    • "One notable example is West syndrome (infantile spasms), where the ketogenic diet is successfully used as a first line agent.72 The same diet is also thought to be especially effective in unspecified symptomatic generalized epilepsy, multifocal epilepsy, juvenile absence epilepsy, continuous spike and slow wave of sleep, myoclonic-astatic epilepsy (MAE or Doose syndrome), severe myoclonic epilepsy of infancy (Dravet syndrome), and seizures in patients with Tuberous Sclerosis Complex and Landau-Kleffner syndrome.68,73,74 Ketogenic diets are also recommended for patients suffering from glucose transporter defects and pyruvate dehydrogenase deficiency, who are unable to utilize glucose for brain metabolism.75,76 "
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    ABSTRACT: It is currently estimated that about 20%-30% of adults and 10%-40% of children diagnosed with epilepsy suffer from uncontrolled or poorly controlled seizures, despite optimal medical management. In addition to its huge economic costs, treatment-refractory epilepsy has a widespread impact on patients' health-related quality of life. The present paper focuses on the concepts of refractory and difficult-to-treat seizures and their pharmacological management. Evidence on efficacy and tolerability of rational pharmacotherapy with antiepileptic drug combinations and of non-pharmacological treatment options such as epilepsy surgery, neurostimulation, metabolic treatment and herbal remedies is reviewed. The importance of early identification of the underlying etiology of the specific epilepsy syndrome is emphasized, to inform early prognosis and therapeutic strategies.
    06/2012; 4:105-15. DOI:10.4137/JCNSD.S8315
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