Summary: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.

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To analyze the electroclinical features and evolution of seven infants with benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE). Material and methods. Seven patients were examined at our department between February 2003 and February 2009, with onset of seizures between six and 13 months of age (mean, 10.2 months; median, 11 months). Patients with cryptogenic and symptomatic focal epilepsies were excluded. Sex, age, familial history, type of seizures and AED treatment were noted and EEG monitoring, MRI and CT scanning, and developmental and psychomotor evolution were investigated. Results. Patients included five males and two females. All patients suffered from seizures during wakefulness. Two of the patients (29%) did not have a recurrence. Five (71%) had sporadic seizures (ranging between two and five). One of the seven patients (14%) presented with seizures in clusters. During seizures, staring was observed in six (86%), motion arrest in five (71%), stiffening in five (71%), cyanosis in three (42%), automatisms in one (14%) and lateralizing signs in four (57%). Two patients (29%) had secondary generalisation. The duration of the seizures ranged between 30 seconds and five minutes. No status epilepticus was observed. The interictal EEG recording during sleep showed low-voltage unilateral or bilateral spikes located in the central and vertex regions, followed by slow waves in all patients. Outcome was excellent in all patients. Conclusion. We believe that BIMSE is a new syndrome rather than an early presentation of benign epilepsy of childhood with centrotemporal spikes, Panayiotopoulos syndrome, or a late presentation of benign focal infantile seizures.