Ketogenic diet for infantile spasms refractory to first-line treatments: An open prospective study

Pires, Maria Elisa Paiva; Iléa, Adina; Bourel-Ponchel, Emilie; Bellavoine, Vanina; Merdariu, Dana; Berquin, Patrick; Auvin, Stéphane

doi:10.1016/j.eplepsyres.2012.11.009

Cited by 64 publications

(68 citation statements)

References 41 publications

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“…This has been reported by several studies using the KD in infants [4,7,9]. This outcome requires further evaluation in the future.…”

Section: Evaluation and Discontinuationmentioning

confidence: 87%

“…This implies early, aggressive and optimal treatment is warranted. As a non-pharmacological treatment KD is currently used in infants with refractory epilepsy syndromes (see table 1) such as infantile spasms (West syndrome) resistant to first line medication [6][7][8][9][10], Ohtahara syndrome [11,12], epilepsy of infancy with migrating seizures [13] and resistant epilepsy with focal seizures awaiting epilepsy surgery.…”

Section: Epilepsy Syndromes In Infancy Where Kd Is Of Benefitmentioning

confidence: 99%

“…Raju et al (2011) showed in a randomized trial of 38 infants that a 2.5:1 ratio KD was as effective as 4:1 ratio KD but less side effects were seen [16]. Pires et al (2013) showed in a prospective trial of 17 infants receiving a KD during hospitalisation that a 3:1 (some 4:1) ratio was very effective and tolerated well. Diet initiation was performed by a non-fasting protocol with daily steps of increasing calories and ratio [7].…”

mentioning

confidence: 99%

“…Pires et al (2013) showed in a prospective trial of 17 infants receiving a KD during hospitalisation that a 3:1 (some 4:1) ratio was very effective and tolerated well. Diet initiation was performed by a non-fasting protocol with daily steps of increasing calories and ratio [7].…”

mentioning

confidence: 99%

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Ketogenic diet guidelines for infants with refractory epilepsy

Louw

Hurk

Neal

et al. 2016

European Journal of Paediatric Neurology

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“…This has been reported by several studies using the KD in infants [4,7,9]. This outcome requires further evaluation in the future.…”

Section: Evaluation and Discontinuationmentioning

confidence: 87%

Section: Epilepsy Syndromes In Infancy Where Kd Is Of Benefitmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Ketogenic diet guidelines for infants with refractory epilepsy

Louw

Hurk

Neal

et al. 2016

European Journal of Paediatric Neurology

Self Cite

151

150

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“…Ketogenic diet has been proposed as an alternative adjunctive therapy for IS to be considered after failure of corticosteroids and/or vigabatrin and has shown some efficacy in refractory IS (Hong, Turner, Hamdy & Kossoff 2010, Pires, Ilea, Bourel, Bellavoine, Merdariu, Berquin et al 2013). A smaller percentage of patients with IS may respond to other antiseizure drugs (valproate, topiramate, zonisamide) or vitamin B6 (Pellock, Hrachovy, Shinnar, Baram, Bettis, Dlugos et al 2010, Riikonen 2014).…”

Section: Introductionmentioning

confidence: 99%

Inflammation in Epileptic Encephalopathies

Shandra

Moshé

Galanopoulou

2017

Stress and Inflammation in Disorders

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West syndrome (WS) is an infantile epileptic encephalopathy (EE) that manifests with infantile spasms, hypsarrhythmia (in ~60% of infants) and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12–15%) or of unknown origin. The current treatment options include hormonal treatment [adrenocorticotropic hormone (ACTH) and high dose steroids], the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS. There is a need to identify new therapeutic targets and more effective treatments for WS. Theories about the role of inflammatory pathways in the pathogenesis and treatment of WS have emerged, being supported by both clinical and preclinical data from animal models of WS. Ongoing advances in genetics have revealed numerous genes involved in the pathogenesis of WS, including genes directly or indirectly involved in inflammation. Inflammatory pathways also interact with other signaling pathways implicated in WS, such as the neuroendocrine pathway. Furthermore, seizures may also activate pro-inflammatory pathways raising the possibility that inflammation can be a consequence of seizures and epileptogenic processes. With this targeted review we plan to discuss the evidence pro and against the following key questions. Does activation of inflammatory pathways in the brain cause epilepsy in WS and does it contribute to the associated comorbidities and progression? Can activation of certain inflammatory pathways be a compensatory or protective event? Are there interactions between inflammation and the neuroendocrine system that contribute to the pathogenesis of West syndrome? Does activation of brain inflammatory signaling pathways contribute to the transition of WS to Lennox-Gastaut syndrome? Are there any lead candidates or unexplored targets for future therapy development for WS targeting inflammation?

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Treatment of infantile spasms

Hussain

2018

Epilepsia Open

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SummaryThe treatment of infantile spasms is challenging, especially in the context of the following: (1) a severe phenotype with high morbidity and mortality; (2) the urgency of diagnosis and successful early response to therapy; and (3) the paucity of effective, safe, and well‐tolerated therapies. Even after initially successful treatment, relapse risk is substantial and the most effective therapies pose considerable risk with long‐term administration. In evaluating any treatment for infantile spasms, the key short‐term outcome measure is freedom from both epileptic spasms and hypsarrhythmia. In contrast, the most important long‐term outcomes are enduring seizure‐freedom and measures of intellectual performance in later childhood and adulthood. First‐line treatment options—namely hormonal therapy and vigabatrin—display moderate to high efficacy but also exhibit substantial side‐effect burdens. Data on efficacy and safety of each class of therapy, as well as the combination of these therapies, are reviewed in detail. Specific hormonal therapies (adrenocorticotropic hormone and various corticosteroids) are contrasted. Those etiologies that prompt specific therapies are reviewed briefly, as are an array of second‐line therapies supported by less‐compelling data. The ketogenic diet is discussed in greater detail, with a focus on the limitations of numerous available studies that generally suggest that it is efficacious. Special discussion is allocated to cannabidiol—the investigational therapy that has received the most attention, and which is already in use in the form of various artisanal cannabis extracts. Finally, a treatment algorithm reflecting the concepts and controversies discussed in this review is presented.

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Ketogenic diet for infantile spasms refractory to first-line treatments: An open prospective study

Cited by 64 publications

References 41 publications

Ketogenic diet guidelines for infants with refractory epilepsy

Ketogenic diet guidelines for infants with refractory epilepsy

Inflammation in Epileptic Encephalopathies

Treatment of infantile spasms

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