Abstracts

Rationale: Electrical status epilepticus during slow sleep (ESES) is a rare epileptic syndrome with a characteristic continuous spike-waves electroencephalographic pattern during slow sleep (CSWS). These patients can present with either obvious clinical seizures, or a developmental regression. Here we report seven cases of ESES diagnosed during the year 2009 as well as the response to different treatment strategies. Methods: During the year 2009 seven patients with previously controlled epilepsy (2-3 anti epileptic drugs), presented for recurrence of seizures (day and night in 5 patients) and for learning regression (in 2 patients). A 24 hour video EEG monitoring was performed in all these patients Results: All these patients showed an EEG pattern with CSWS and met the clinical criteria of ESES. The age ranged between 2 and 9 years. The patients presented either generalized or partial epilepsy. Three of these patients had symptomatic epilepsy (hypoxic ischemic encephalopathy, head trauma, dural sinus thrombosis with intraventricular hemorrhage), one with cryptogenic epilepsy, and three with idiopathic epilepsy. The first seizures were noted as early as 10 days of age in one patient, and as late as 9 years of age in the oldest patient. Two patients were treated with Hydrocortisone (5mg/kg/day) for 3 months, one patient was given intra venous Methylprednisolone (three courses of 30 mg/kg/day for 3/7 consecutive days) followed by a course of prednisone 2 mg/kg/day for one month. Three patients were given oral Prednisone (2mg/kg/day) for 3 months. Clinical improvement was noted in 4 patients, no clinical seizures were noted in the following three months, and three patients showed 50-80 % improvement of the EEG electrical activity. Conclusions: Electrical status epilepticus during slow sleep is one of the epilepsy syndromes, even though rare; yet, easy to diagnose, and should not be missed in front of a decompensation epilepsy, or an acute learning regression. The traditional anti epileptic drugs tend to fail, but these patients present a good response to corticoid therapy in most of the cases.