Ketogenic Diet in the Treatment of Super-Refractory Status Epilepticus at a Pediatric Intensive Care Unit: A Single-Center Experience

Abstract

Background: To evaluate the use of the ketogenic diet (KD) for treatment of super-refractory status epilepticus (SRSE) at a pediatric intensive care unit (PICU).Design: A retrospective analysis of all pediatric patients treated for SRSE with the KD at our center was performed using patient data from our prospective longitudinal KD database.Setting: SRSE is defined as refractory SE that continues or recurs 24 h or more after initiation of anesthetic drugs. We describe the clinical and electroencephalographic (EEG) findings of all children treated with KD at our PICU. The KD was administered as add-on after failure of standard treatment. Response was defined as EEG seizure resolution (absence of seizures and suppression–burst ratio ≥50%).Patients: Eight consecutive SRSE patients (four females) treated with KD were included. Median age at onset of SRSE was 13.6 months (IQR 0.9–105), and median age at KD initiation was 13.7 months (IQR 1.9 months to 8.9 years). Etiology was known in 6/8 (75%): genetic in 4 (50%), structural in 1 (12.5%), and autoimmune/inflammatory in 1 (12.5%).Main Results: Time from onset of SRSE to initiation of KD was median 6 days (IQR 1.3–9). Time until clinically relevant ketosis (beta-hydroxybutyrate (BHB) >2 mmol/L in serum) was median 68.0 h (IQR 27.3–220.5). Higher ketosis was achieved when a higher proportion of enteral feeds was possible. Four (50%) patients responded to KD treatment within 7 days. During follow-up (median 4.2 months, IQR 1.6–12.3), 5/8 patients—three of them responders—died within 3–12 months after SRSE.Conclusions: In eight patients with SRSE due to severe etiologies including Alpers syndrome, we report an initial 50% response to KD. KD was used early in SRSE and sufficient levels of ketosis were reached early in most patients. Higher ketosis was achieved with combined enteral and parenteral feedings.

Immune Modulatory Effects of Ketogenic Diet in Different Disease Conditions

“…Patients on KD require special attention throughout surgical practices as various medical intervention procedures involve exposure of carbohydrates into the blood circulation. The epilepsy should be managed by excluding drugs such as propofol, a carbohydrate-comprising remedy to counteract post-surgical seizure incidents [159,160]. A disturbing effect of KDs on micronutrient levels was found in a case of a refractory epilepsy female patient who developed scurvy due to an insufficient level of vitamin C while on a KD [161][162][163].…”

Favorable response to classic ketogenic diet in a child with anti-GAD 65 antibody mediated super refractory status epilepticus

“…But most of these studies are adult based. A few studies have reported the effectiveness of K D for management of SRSE in paediatric patients due to various etiologies specifically FIRES [ [13] , [14] ]. In a case report with literature review by Prasoppokakorn et al, medium chain triglyceride based K D was reported to be beneficial in young adult patients with SRSE and AI encephalitis [ 15 ].…”

The use of ketogenic diets in children living with drug‐resistant epilepsy, glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency: A scoping review

“…Studies highlight that the need for a fasting KD initiation is not necessary for KD efficacy and it was proposed that fasting increases the risk of hypoglycaemia. 39,73,77,85,141,182 Studies also propose the use of KDs earlier on in the epileptic journey, 83,91,99,122,149,156,185,291,299 specifically in LGS, 233 TSC, 271 infants, 60,75,119,161,282,329 DS, 118,163 DOOSE, 144,209 CDKL5, 140 FIRES and SRSE, 36,160,169,256,280,283 and the metabolic conditions GLUT1DS 114,272,286 and PDHD. 247,296 Recommendations for treating new onset-refractory SE, including FIRES, includes the advice to escalate treatment from ASMs not only to immunotherapy, but also to KD, as soon as possible in the treatment course.…”