[P2-114] A Prospective Study of Ketogenic Diet in Dravet Syndrome
[Introduction] KD has been used as an alternative to antiepileptic drugs for patients with refractory epilepsy. In this prospective study we assess the efficacy of the KD in patients with DS.
[Methodology] Between December 1, 2013 and December 1, 2015, 20 patients were included prospectively in our department. They met diagnostic criteria of DS and had high frequency seizures after rational use of AEDs treatment. They were given 4:1 ketogenic ratio diet, which was introduced using a non-fasting protocol. Seizure frequency, especially the time over 5 minutes of generalized convulsions and the status epilepticus, cognitive improvement and adverse effects were recorded at 3, 6 months.
[Results] 20 patients all had SCN1A mutation, of which 13 were male. The mean (range) age at seizure onset was 6 (1–11) months, and the mean age of the participants was 27.5 (range: 1-141) months. Seizure types include both generalized seizures (generalized convulsions, atypical absence seizures, myoclonic seizures), focal seizures and status epilepticus (SE). Overall, >50% seizure improvement was seen in 17 at 3 months, 16 at 6 months. No SE occurred in our patients and the time of prolonged generalized convulsions decreased. Cognitive function of 16 patients was improved. AEDs numbers were reduced to 1.8 from 2.25 at 3–6 months after initiation of the diet.
[Conclusions] The KD is an efficacious therapy for DS. KD reduced emergency frequencies in DS patients, and decreased the risk of nerve injury of SE; KD improved cognition in patients with Dravet syndrome.
[Methodology] Between December 1, 2013 and December 1, 2015, 20 patients were included prospectively in our department. They met diagnostic criteria of DS and had high frequency seizures after rational use of AEDs treatment. They were given 4:1 ketogenic ratio diet, which was introduced using a non-fasting protocol. Seizure frequency, especially the time over 5 minutes of generalized convulsions and the status epilepticus, cognitive improvement and adverse effects were recorded at 3, 6 months.
[Results] 20 patients all had SCN1A mutation, of which 13 were male. The mean (range) age at seizure onset was 6 (1–11) months, and the mean age of the participants was 27.5 (range: 1-141) months. Seizure types include both generalized seizures (generalized convulsions, atypical absence seizures, myoclonic seizures), focal seizures and status epilepticus (SE). Overall, >50% seizure improvement was seen in 17 at 3 months, 16 at 6 months. No SE occurred in our patients and the time of prolonged generalized convulsions decreased. Cognitive function of 16 patients was improved. AEDs numbers were reduced to 1.8 from 2.25 at 3–6 months after initiation of the diet.
[Conclusions] The KD is an efficacious therapy for DS. KD reduced emergency frequencies in DS patients, and decreased the risk of nerve injury of SE; KD improved cognition in patients with Dravet syndrome.