Abstracts

Rationale: Dravet syndrome (DS) is one of the most intractable epilepsies. New antiepileptic drugs including stiripentol (STP) and topiramate (TPM) are useful for only half of patients. Ketogenic diet is also reported to work for DS. As Dravet syndrome often shows multi-seizure types, it's important to know which seizures types can respond to KD. Methods: Eight children (1-7y) with Dravet syndrome continued KD longer than 3 months in our institute. All patients had SCN1A gene abnormality. We retrospectively investigated frequency changes of status epilepticus (SE), convulsive seizures (CS), absence or myoclonic seizures (AM), and photo or pattern sensitivity (PP) before and on KD. We considered responders when seizure frequency decreased more than 50% for each seizure type.Results: (1) Responder rate was 3/5 for SE, 4/7 for CS, 3/6 for AM, and 3/3 for PP. Seizure reduction of any seizure types was observed in 5/8. (2) Five patients (including 2 with STP) are still on KD for 4m-3y. (3) Three (including 1 with STP) patients stopped KD due to lack of enough efficacy. One of them responded to STP thereafter.Conclusions: KD is useful choice for Dravet syndrome. KD can be effective for all seizure types.