Ketogenic diet effects on 52 children with pharmacoresistant epileptic encephalopathy: A clinical prospective study

Abstract

ObjectiveTo evaluate the clinical impact of ketogenic diet (KD) on children with pharmacoresistant epileptic encephalopathy.MethodsIn all, 52 children with pharmacoresistant epileptic encephalopathy that diagnosed in our hospital from July 2012 to June 2015 were selected, including West syndrome 38 cases, Lennox–Gastaut Syndrome 7 cases, Doose Syndrome 1 case, and Dravet syndrome 6 cases, and the effect, compliance, adverse reactions, electroencephalogram (EEG), and cognitive function were analyzed. Modified Johns Hopkins protocol was used to initiate KD, and Engel scale was used to evaluate the effect, and evaluated the effect of KD on the cognition, language, and motor function.ResultsAt 12 weeks of KD treatment, the patients achieved I, II, III, and IV grade effect were accounted for 26.9% (14/52 cases), 17.3% (9/52 cases), 11.5% (6/52 cases), and 44.2% (23/52 cases), respectively, according to Engel scale. KD has different effect on different epileptic syndromes, best effect on Doose syndromes of 100%, and better effect on West syndrome with the effect rate of 57.9%, and the total effect number was 22 cases. The reduction of epileptiform discharges in the awake state before KD treatment was correlated with the seizure time after 3 months of KD treatment (r = .330, p = .017). The cognitive function of 23 patients was improved, 12 patients had language improvement, and the motor function was improved in 10 patients. In all, 23 patients had adverse reactions, and all patients were tolerated and improved.Conclusion KD has certain effect on children with pharmacoresistant epileptic encephalopathy, and it can reduce interictal epileptic discharge frequency, and improve the background rhythm of EEG. The reduction of epileptiform discharges in awake state is in favor of the reduction of seizures frequency, thus increasing the efficacy, and improve the cognitive function, language, and motor function to varying degrees, combined with less adverse reaction, which is worthy of clinical application.

Epilepsy-Related Voltage-Gated Sodium Channelopathies: A Review

“…Antisense nucleotides (ASO) therapy to increase mRNA of SCN1A for NaV1.1 channel expression in normal levels is a promising strategy for genetic disorders involving haploinsufficiency (Hsiao et al, 2016;Stoke Therapeutics, 2018). Drug-resistant Dravet syndrome cases may thrive on alternative therapeutic strategies based on ketogenic diets (Nabbout et al, 2011;Wu et al, 2018). A recent study with 20 patients with medically intractable Dravet syndrome caused by missense, non-sense, insertion, deletions and splicing mutations presents efficacy during three months of treatment in 17 patients, decreasing seizure frequency in more than 50% (Yan et al, 2018).…”

Clinical characteristics and treatment outcomes of pediatric patients with postencephalitic epilepsy characterized by epileptic spasms

“…However, this result needs to be clarified by investigating the effects of this drug in a larger cohort. KD and VNS are common alternative therapies for DRE [11,12]. In our cohort, six children received KD, and five failed to respond.…”

The efficacy of non-fasting ketogenic diet protocol in the management of intractable epilepsy in pediatric patients: a single center study from Saudi Arabia

“…The efficacy results shown in the present study were similar to that of other published studies. 24 – 26 …”