Abstracts

Rationale: Continuous spike and wave discharges in the EEG during slow sleep, or electrical status epilepticus during slow sleep (ESES) is the result of secondary bilateral synchronies which may appear in the course of idiopathic or symptomatic focal epilepsies in childhood. We present the effect of add-on treatment with Sulthiame in 35 children with idiopathic or symptomatic focal epilepsies presenting ESES and refractory seizures. Methods: Between 1995 and 2006, 35 patients collected in two neuropediatric centers in Argentina met the following criteria of inclusion: 1) onset with focal seizures and focal EEG discharges; 2) further appearance of atypical absences, myoclonic, atonic, and/or secondary generalized seizures associated with ESES; 3) refractory to usual AEDs. All children were studied with MRI and repeated EEGs and neuropsychologic evaluations. Sulthiame, in doses ranging between 10 and 30 mg/kg/day, was added in patients receiving no more than two other AEDs. Most of the patients had already received Clobazam and/or Ethosuximide since onset of ESES. Results: Time of follow up ranged between two and 13 years. Age of onset of ESES ranged between 2 and 11 years. Patients are presented belonging to three categories: a) idiopathic (15 cases); b) symptomatic (17 cases); and c) cryptogenic or probably symptomatic (3 cases). In the group of idiopathic cases all the types of atypical evolutions of benign focal epilepsies in childhood were represented, namely Atypical benign partial epilepsy of childhood, Status of benign focal epilepsy in childhood, Landau Kleffner syndrome and Continuous spike and wave during slow sleep syndrome. Results in terms of seizures and EEG abnormalities: a) Idiopathic group: 13 cases became seizure free and had normalized EEGs, while 2 cases became seizure free without ESES, but with abnormal EEGs; b) Symptomatic group: 6 cases seizure free without ESES but with abnormal EEGs, 2 cases seizure free with persistence of ESES, 6 cases with persistence of seizures without ESES, 3 cases with no response; c) Cryptogenic or probably symptomatic group: one case with seizures but without ESES, two cases with no response. Conclusions: Favorable results in terms of seizure control and amelioration of EEG discharges were seen after adding Sulthiame in these patients. Excellent outcomes were more frequent in idiopathic cases. Ten of the 17 symptomatic cases had congenital hemiparesis with unilateral polymichrogyria, and also showed very good results with control of seizures and disappearance of ESES. According to our experience, Sulthiame is a very useful drug in the treatment of children with focal epilepsies associated with ESES.