Thursday, July 5, 2018

Ketogenic diet in Dravet syndrome

Ni Yan, Wang Xin-Hua, Zhang Lin-Mei, Chai Yi-Ming, Li Wen-Hui, Zhou Yuan-Feng, Zhou Shui-Zhen.  Prospective study of the efficacy of a ketogenic diet in 20 patients with Dravet syndrome. Seizure August 2018 Volume 60, Pages 144–148.


•Patients with Dravet syndrome were placed on a ketogenic diet (KD) for 6 months.
•Of the 20 patients, 17 exhibited ≥50% decreases in seizure frequency on the KD.
•On the KD, no generalised convulsions ≥5 min or status epilepticus was detected.
•The KD also improved patients’ cognitive function.
•The KD is a good treatment option for medically intractable epilepsy.


We evaluated the efficacy and tolerability of the ketogenic diet (KD) on generalised convulsions and status epilepticus (SE) in patients with Dravet syndrome (DS).

Patients with DS having ≥2 generalised convulsions/month despite drug treatment were included in this study and placed on a KD for 6 months. From 3 months before (baseline) to 6 months after KD initiation, caregivers recorded patients’ seizure activity, antiepileptic drug use, and adverse events. The KD efficacy was determined by examining the frequency and duration of seizures at 3 and 6 months vs. baseline. Responders were defined as individuals whose generalised convulsions decreased in frequency by ≥50% vs. baseline. Seizures lasting ≥5 min and SE were specifically evaluated. Patients’ cognition was also assessed at 3 and 6 months via questionnaire.

Twenty patients continued the KD for at least 3 months. Of the 17 responders identified at month 3, seizures decreased by 50–89% and 90–99% in nine and two patients, respectively; six patients were seizure free. The KD was ineffective in three patients, who discontinued the diet. By month 6, seizures decreased by 50–89% and 90–99% in six and one patient(s), respectively; 10 patients were seizure free. The frequency of other seizure types also improved. During all 6 months, neither generalised convulsions lasting ≥5 min nor SE was detected in the 17 responders. The KD also improved patients’ cognition.

The KD is a good treatment option for medically intractable epilepsy.

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1 comment:

  1. Wu Q, Wang H, Fan YY, Zhang JM, Liu XY, Fang XY, Yang FH, Cao QJ, Qi Y. Ketogenic diet effects on 52 children with pharmacoresistant epileptic encephalopathy: A clinical prospective study. Brain Behav. 2018 Apr 18;8(5):e00973.

    To evaluate the clinical impact of ketogenic diet (KD) on children with pharmacoresistant epileptic encephalopathy.

    In 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.

    At 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.

    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.