Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES)

Abstract

Summary Purpose: Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian refractory status epilepticus (SE) triggered by fever of unknown cause. SE may last more than 1 month, and this condition may evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed to report the effect of ketogenic diet (KD) in this condition. Methods: Over the last 12 years we collected data of nine patients with FIRES who received a 4:1 ratio of fat to combined protein and carbohydrate KD. They presented with SE refractory to conventional antiepileptic treatment. Results: In seven patients, KD was efficacious within 2–4 days (mean 2 days) following the onset of ketonuria and 4–6 days (mean 4.8 days) following the onset of the diet. In one responder, early disruption of the diet was followed by relapse of intractable SE, and the patient died. Epilepsy affected the other six responders within a few months. Discussion: KD may be an alternative therapy for refractory SE in FIRES and might be proposed in other types of refractory SE in childhood.

Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet

“…The 6 responders remained on the KD for a mean of 1 year (range, 6 months to 2 years). 7 Another report described 2 children treated with the KD in the acute phase with a 50% to 70% seizure reduction. 16 Detailed cognitive outcomes were not reported in either study.…”

“…5,6 One group suggested the ketogenic diet (KD) may be effective in the acute SE phase of FIRES, based on experience with 9 patients, but cognitive outcomes were not described. 7 The KD is a high-fat, adequate-protein, lowcarbohydrate diet that can reduce seizure burden via incompletely understood mechanisms. 8 We report 2 children with FIRES for whom the KD was initiated during the acute SE phase and was maintained during convalescence.…”

Epileptic Syndromes With Possible Immunological Mechanisms (Rasmussen Encephalitis, FIRES, West Syndrome, Landau-Kleffner Syndrome)

“…[46] İnfantil Spazmlar ve West Sendromu Giriş Karakteristik EEG paterni olan hipsaritmi, West sendromu klinik olarak tanımlandıktan yaklaşık 100 yıl sonra tespit edilebilmiştir. [54] İnsidansı 10 000 canlı doğumda 2-5 ve yaşa bağlı prevalansı 10 yaşındaki çocuklarda 1-2'dir.…”

“…[39] Awaya and Fukuyama'nın ilk olarak 1986'da tanımladığı olgulardan sonra dünyanın çeşitli yerlerinden çok sayıda FI-RES olgusu tanımlanmıştır. [40][41][42][43][44][45][46][47][48] Bu özgün sendrom, günümüze kadar, yeni-başlangıçlı dirençli status epileptikus (New-Onset Refractory Status Epilepticus (NORSE)), okul çağı çocuklarının yıkıcı epileptik ensefalopatisi (Devastating Epileptic Encephalopathy in School Aged Children (DESC)), dirençli, tekrarlayıcı parsiyel nöbetlerle seyreden akut ensefalit (Acute Encephalitis with Refractory Repetitive Partial Seizures (AERRPS)), ateşli enfeksiyonla ilişkili epilepsi sendromu (febrile infection-related epilepsy syndrome (FIRES)) ve okul çağı çocuklarının ateşle tetiklenen dirençli epileptik ensefalopatisi (Fever-Induced Refractory Epileptic Encephalopathy of School-Age Children (FIRES) olmak üzere çok çeşitli başlıklar altında tartışılmıştır. [39,42,44,47,49,50] Son yıllarda FIRES en çok kullanılan ve kabul gören tanım olmuştur.…”

The New Ketone Alphabet Soup: BHB, HCA, and HDAC

“…Neither study provides direct evidence in this regard, but based on emerging lines of research (1, 2, 7), both strongly indicate that BHB (and by extension, the KD) could influence the epileptic state. In support of this, the KD has recently been shown to affect epigenetic changes in epileptic brain (17) and is also proving useful in patients with neuroinflammation-related epilepsy (18). With respect to neuroprotection, there are indeed many other KD-related mechanisms that likely play a role (3), such as ketone-mediated inhibition of vesicular glutamate release (19).…”