Ketogenic Diet as Preferred Treatment of FIRES

Jj, Millichap; Jg, Millichap

doi:10.15844/pedneurbriefs-29-1-2

Cited by 8 publications

(4 citation statements)

References 6 publications

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“…39,40 There is evidence from 3 epilepsy centers for the benefits of KDT in tuberous sclerosis complex. [41][42][43] Additional indications include febrile infection-related epilepsy syndrome (FIRES), [44][45][46][47] formula-fed children, 48,49 Ohtahara syndrome, [50][51][52] superrefractory focal and myoclonic status epilepticus, 44,46,53,54 complex 1 mitochondrial disease, 51,55 and Angelman syndrome. 56,57 For FIRES and status epilepticus specifically, it is unknown if KDTs would have similar benefits when used earlier in the course of these conditions.…”

Section: Indications and Contraindicationsmentioning

confidence: 99%

“…Angelman syndrome 56,57 Complex 1 mitochondrial disorders 51,55 Dravet syndrome 35,36 Epilepsy with myoclonic-atonic seizures (Doose syndrome) 34,37,38 Glucose transporter protein 1 (Glut-1) deficiency syndrome (Glut1DS) 27,[29][30][31][32] Febrile infection-related epilepsy syndrome (FIRES) [44][45][46][47] Formula-fed (solely) children or infants 48,49 Infantile spasms 10,39,40 Ohtahara syndrome [50][51][52] Pyruvate dehydrogenase deficiency (PDHD) 28 Super-refractory status epilepticus 44,46,53,54 Tuberous sclerosis complex [41][42][43] Table 2. Several conditions (listed alphabetically) in which KDT has been reported as moderately beneficial (not better than the average dietary therapy response, or in limited single-center case reports)…”

Section: Indications and Contraindicationsmentioning

confidence: 99%

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Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Kossoff¹,

Zupec‐Kania²,

et al. 2018

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SummaryKetogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre‐KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow‐up, side events, and KDT discontinuation. It has been helpful in outlining a state‐of‐the‐art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organizers and authors of this guideline present a revised version with additional authors, in order to include recent research, especially regarding other dietary treatments, clarifying indications for use, side effects during initiation and ongoing use, value of supplements, and methods of KDT discontinuation. In addition, authors completed a survey of their institution's practices, which was compared to responses from the original consensus survey, to show trends in management over the last 10 years.

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Section: Indications and Contraindicationsmentioning

confidence: 99%

Section: Indications and Contraindicationsmentioning

confidence: 99%

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Kossoff¹,

Zupec‐Kania²,

et al. 2018

View full text Add to dashboard Cite

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“…These include ketosis; reduced glucose; elevated fatty acid levels; enhanced bioenergetic reserves; and others with anticonvulsant, anti-epileptogenic, and neuroprotective properties [8]. The KD must be considered precociously as treatment in specific epileptic syndromes and in some etiologies where it is known to be very effective [9,10], including Dravet syndrome [11,12], infantile spasms and West syndrome [13,14], Doose syndrome [15,16], tuberous sclerosis complex [17], and FIRES syndrome (febrile infection related epilepsy syndrome) [18,19]. Moreover, it is the treatment of choice in the deficiency of glucose type 1 transporter (GLUT-1) [20] and in pyruvate dehydrogenase deficiency (PDH) [21].…”

Section: Introductionmentioning

confidence: 99%

Safety and Effectiveness of the Prolonged Treatment of Children with a Ketogenic Diet

et al. 2020

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Background: The ketogenic diet (KD) is an effective treatment against drug-resistant epilepsy in children. The KD is a diet rich in fats that produces anticonvulsant and neuroprotective effects that reduces seizures and improves the cognitive state. Nevertheless, it can produce side effects that sometimes can be serious. Further, the effect on growth is quite controversial when used for an extended period of time. The aim of this paper was to assess the effectiveness, side effects, and repercussions in the development of children who have been treated with a KD for more than 2 years. Methods: Observational descriptive study of 26 pediatric patients on a KD, with data collection at baseline, at 3, 6, and 12 months, and then once a year. Number of seizures, type of seizures, anti-seizure drugs, anthropometry, side effects, and alterations in laboratory assessment were monitored. Results: In every assessment, about 60%–75% of the patients experienced a reduction in number of seizures of over 90%, and at least 50% experienced side effects, of which digestive issues, alteration in the lipid metabolism, and hypercalciuria were the most common. The KD significantly affected height after 2 years of treatment. Conclusions: The KD is an effective treatment for drug-resistant epilepsy. Its side effects, although common, are very mild; therefore, this constitutes a very safe treatment for children of all ages. More studies are needed to identify and prevent potential causes of growth retardation in children on the KD.

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“…The most promising form of treatment in FIRES is the use of the KD. 46 The therapeutic use of a KD, which is a high-fat, low protein, low carbohydrate diet, has been used in childhood epilepsy for almost a century. However, the first study on the effects of a KD in status epilepticus was published in 2003.…”

Section: Ketogenic Dietmentioning

confidence: 99%

Febrile Infection-Related Epilepsy Syndrome

et al. 2019

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Febrile infection-related epilepsy syndrome (FIRES) is a subset or variant of new-onset refractory status epilepticus in children. FIRES is characterized by the occurrence of a febrile episode between 24 hours and 2 weeks before the onset of refractory status epilepticus. A infectious cause is rarely identified in FIRES and an inflammatory or autoimmune etiology is implied. Seizures in FIRES are very difficult to control, and treatments include antiepileptic drugs, ketogenic diet, intravenous immunoglobulin, plasmapheresis, and corticosteroid therapy. The prognosis for patients with FIRES is poor, and most children are left with refractory epilepsy and cognitive impairment. The new consensus guidelines on the terminology of FIRES and recent interest in new treatment approaches have been welcome developments for clinicians who face the challenge of diagnosing and managing status epilepticus in a previously healthy child that occurs following a minor febrile episode. This review aims to provide clinicians with an update on the current hypotheses for the etiology, pathogenesis, clinical evaluation, management, and future directions in the diagnosis and treatment of FIRES.

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Ketogenic Diet as Preferred Treatment of FIRES

Abstract: Investigators from the University of Alabama, Birmingham, AL, and University of Michigan, Ann Arbor, MI, report 2 children who presented with FIRES and prolonged AED-resistant status epilepticus.

Cited by 8 publications

References 6 publications

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Safety and Effectiveness of the Prolonged Treatment of Children with a Ketogenic Diet

Febrile Infection-Related Epilepsy Syndrome

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