Ketogenic Diet Treatment for Status Epilepticus

Abstract

The ketogenic diet (KD) is a high fat normoproteic, low carbohydrate diet considered in recent years as an alternative treatment option for patients with refractory status epilepticus (RSE) or super-refractory status epilepticus (SRSE), both, in children and adults. The aim of current research is to review and compare the efficacy of KD in children and adults with RSE and SRSE regardless of underlying aetiology. We performed a retrospective PubMed search between June 1997 until 30 th of October 2017 and selected all articles in which KD was used for the treatment of children and adults with status epilepticus refractory to standard therapy. 120 patients (adults and children) with RSE and SRSE were identified. An important proportion of patients (92/120 ~ 77%, 64 children and 28 adults) responded to KD therapy. In conclusion, KD is proving to be a valuable therapeutic option in the intensive care units for the treatment of RSE and SRSE in children and adults, available data showing efficacy mostly but not limited to (febrile infection related to encephalopathy in school aged children) FIRES in children and encephalitis in adults. Specific protocols and training should be made available in intensive care units in order to implement and manage KD in children and adults with status epilepticus who do not respond to standard therapeutic approaches. RezumatDieta cetogenă (DC) este o dietă bogată în grăsimi, normoproteică şi cu un conținut sărac de carbohidrați, considerată în ultimii ani drept o opțiune de tratament pentru pacienții cu status epilepticus refractar (RSE) sau super-refractar (SRSE), atât la copii, cât și la adulți. Scopul cercetării actuale este de a analiza datele existente în literatură şi de a compara eficienţa DC la copii și adulți cu RSE și SRSE, indiferent de etiologie. S-a efectuat o cercetare retrospectivă a bazei de date PubMed din iunie 1997 pînă la 30 octombrie 2017, fiind selectate toate articolele în care DC a fost utilizată pentru tratamentul copiilor și adulților cu status epilepticus refractar la terapia standard. Au fost identificați 120 de pacienți (adulți și copii) cu RSE și SRSE. O proporție importantă de pacienți (92/120 ~ 77%, 64 copii și 28 adulți) au răspuns la terapia cu DC. În concluzie, DC se dovedește a fi o opțiune terapeutică valoroasă în unitățile de terapie intensivă pentru tratamentul statusului epileptic refractar şi superrefractar la copii și adulți, datele existente demonstrând eficienţa mai ales, dar nu exclusiv, în FIRES (febrile infection related encephalopathy in school-age children) la copii și encefalită la adulți. Protocoale specifice și instruire adecvată ar trebui să fie puse la dispoziție în unitățile de terapie intensivă pentru a implementa și administra DC la copiii și adulții cu status epilepticus care nu răspund la abordările terapeutice standard.

Chinese expert recommendations on ketogenic diet therapy for super-refractory status epilepticus

“…KD successfully achieved cessation of SE in 45 out of the 55 (82%) patients [24]. Types of SE reported included generalized, focal motor, myoclonic, and nonconvulsive in both children and adults [12,16]. Taken together, in these studies involving patients with different sex, ages, seizure types and diagnosis/etiology, most patients achieved cessation of SE after KD initiation.…”

“…The most common diagnosis/etiology of SRSE was febrile infection-related epilepsy syndrome (FIRES), reported in 72/126 (57%) children with known etiology [21]. Autoimmune encephalitis and viral encephalitis are also common etiologies in children [12]. Among the 147 children, 141 (96%) achieved ketosis, and 85 of the 141 (60%) children achieved resolution of SRSE after a mean of 6.3 ± 4.4 days of KD [21].…”

“…In the past, KD was usually initiated late in the course of SRSE. In previous reports, the interval between the onset of SE and initiation of KD varied widely from 1 to 420 days, with a mean interval of 24 days [12,13,16,18]. Risks may arise from the long lag from SE onset to KD initiation, as unremitting seizures, prolonged coma therapy, ventilatory support, and use of a number of anticonvulsants during this period may increase the risk of serious infection, critical multiorgan dysfunction, and irreversible brain damage.…”

“…KD is a high-fat, low-carbohydrate, and moderateprotein diet that can alter the primary cerebral energy metabolism from glucose to ketone bodies and involves multiple mechanisms of antiepileptic action and antiepileptogenic properties [9][10][11]. Although KD is typically suggested for chronic intractable epilepsy, recent studies have illustrated the safety and reasonable efficacy of KD in treating SRSE in both adults and children [12][13][14][15]. Treatment for SRSE is difficult due to the failure of first-and second-line therapies.…”

“…Treatment for SRSE is difficult due to the failure of first-and second-line therapies. Despite the insufficient evidence for KD use as an established treatment for SRSE, previous studies have repeatedly demonstrated the essential role of KD in cutting off continuous infusion of anesthetic agents, weaning from mechanical ventilation, or controlling seizures in more than half of patients [12,13,[15][16][17][18][19][20][21]. As recommended by the International Ketogenic Diet Study Group, KD is "particularly useful" for SRSE, with greater benefits (> 70%) than the average KD response (defined as > 50% seizure reduction) [9].…”