PURPOSE:The ketogenic diet (KD) has been used as a therapeutic alternative to antiepileptic drugs (AEDs) for refractory epilepsy. Severe myoclonic epilepsy in infants or Dravet syndrome (DS) is one of the most malignant epileptic syndromes. In this retrospective study, we evaluated the efficacy and tolerability of the KD in patients with diagnostic criteria of DS.

METHODS:Between March 1, 1990, and August 31, 2004, 52 patients who met diagnostic criteria for DS were enrolled in a study at our department. Twenty of them were placed on the KD with the Hopkins protocol and followed up for a minimum of 1 year.

RESULTS:Three of the 20 original children stayed on the diet for 12 months, four children for 2 years, four children for 3 years, and two children for 4 years. One year after initiating the diet, 13 (65%) of the initial patients remained on the diet. Two (15%) patients were seizure free, eight (61.7%) children had a 75-99% decrease in seizures, and the remaining three (23%) children had a 50-74% decrease in seizures. Thus 1 year after starting the diet, 10 (77%) children had achieved a>75% decrease in their seizures. Four patients have been off the diet for>2 years; one of them is seizure free, two have sporadic seizures, and one, who abandoned the diet after 2 years of adhering to it, relapsed. No differences in seizure control when compared with age, sex, or seizure type were found.

CONCLUSIONS:Considering the severity and intractability of seizures in patients with DS, the fact that 10 of the 13 children who remained on the diet had a significant reduction in number of seizures shows that the KD is at present an interesting therapeutic alternative. Even in patients in whom seizure reduction was not dramatic, quality of life improved, and in all of them, the number of AEDs was reduced to one or two. We consider that children with DS should be offered the KD immediately after three adequate trials of AEDs have failed.

PURPOSE:To evaluate the long-term efficacy/tolerability of the ketogenic diet (KD) in paediatric drug-resistant epilepsies.

METHODS:Data from children who were treated between 1999 and 2008 and had continuous follow-up of at least 6 months after initiation of the KD were analysed retrospectively. Response was defined as>or = 50% seizure reduction. Treatment effects on EEG, developmental outcome and the "outcome-predictive" value of various clinical factors were also assessed.

RESULTS:50 children (22 boys; mean age 4.5 years+/-3.55) were included. Mean follow-up was 3.93+/-2.95. 50% of the patients were responders, 48% of them became seizure free. 50% were non-responders, 20% of them deteriorated. In responders, EEG background activity improved significantly (p=0.014) and a significantly lower rate of epileptic discharges (p=0.009) was seen after 6 months. In addition, neurological examination findings demonstrated significant developmental progress (p=0.038). Favourable treatment outcome was associated with a shorter disease duration (p=0.025) and generalised tonic clonic seizures (p=0.059). No further significant outcome predictors were detected. However, response was 44% in patients with infantile spasms, 62.5% in those with Dravet syndrome and 50% in Lennox-Gastaut-syndrome. Side effects occurred in 28%, but discontinuation of the KD was not required in any case. They most often observed with concomitant topiramate (p=0.001) and valproate (p=0.046).

CONCLUSION:Despite the retrospective nature of the study and the inhomogeneous patient sample, we found good long-term effects of the KD on seizure frequency, EEG and neurological development.

To evaluate the efficacy and safety of ketogenic diet (KD) in patients with Dravet syndrome (DS).60 DS patients receiving treatment of KD for more than 12 weeks from 2009 to 2018 were analyzed retrospectively. Modified Johns Hopkins protocol was used to initiate KD. Seizure frequency, electroencephalogram (EEG), cognition, language, and motor function of the patients were assessed. Side effects were monitored and adjusted accordingly. SPSS 23.0 software was used for all statistical analysis.In total, 60 DS patients (34 boys, 26 girls) received treatment of KD for more than 12 weeks, and among them 41 (68.3%) patients remained on the diet for more than 24 weeks, 22 (36.7%) patients for more than 48 weeks. Seizures in 35 patients (58.3%) were reduced by over 50% at 12 weeks, and the KD effect was observed within 2 weeks in most of them. At 24 weeks, 61.1% (25/41) of the patients had a>50% seizure reduction. At 48 weeks, 77.3% (17/22) had an over 50% reduction in their seizure frequency. With the treatment of KD in the 60 DS patients, 10 patients had ever been seizure free for 12 months to 24 months (The median duration was 20 months). In 10 KD-effective patients, the background rhythm of their EEG showed obvious improvement, and interictal epileptic discharges decreased significantly. Cognitive function of 22 patients was improved. Language progressed in 14 patients. Motor function was improved in 13 patients. The efficacy of KD in DS patients did not correlate with the seizure onset age, the starting age of KD treatment, themutation and the numbers of antiepileptic drugs combined with KD treatment. The main adverse reactions of KD in the treatment process were gastrointestinal symptoms and metabolic disorders.KD treatment in DS patients has many advantages, including working rapidly, being effective in more than half of the DS patients and tolerable adverse reactions. Pharmoco-resistant DS patients are suggested to receive KD treatment.