Epileptic encephalopathy with CSWS: clinical case

Abstract

Background: Continuous spike-wave of sleep syndrome (CSWS), according to the ILAE, is characterized by epileptic seizures, neurocognitive deterioration, and specific EEG changes, mainly affects girls (40/60 ratio) between the age of 4 – 7 years. The aim of the study is the analysis of the clinical case of the CSWS in the context of scientific literature and prognosis evaluation, assuming correct application of the international protocol. Material and methods: Clinical case presentation. Results: A 10-year-old girl (18.09.2002) came in on 21.09.2012 with history of myoclonic seizures. Hypnogenic myoclonic jerks were described on the night video-EEG monitoring. The awake EEG pattern was unremarkable, but the sleep EEG pattern had characteristic signs of CSWS. The patient has no pathological history, the MRI was unremarkable. The therapy with clobazam 10 mg in the evening was initiated. Night video-EEG monitoring on 08.06.2013 was unremarkable. The patient is monitored for 9 years. On 27.07.2018, the dose was adjusted (5 mg). At the moment the patient is without neurocognitive decline, under treatment and continues the scheduled follow-up. From the epidemiological and symptomatic point of view, our case is homogeneous according to literature, located in the first standard deviation of the Gaussian curve. Considering the diagnosis, the recommended treatment achieved the goals: cessation of seizures, normalization of the EEG pattern, and the preservation of neurocognitive abilities. Conclusions: In the case of a non-structural etiology, early diagnosis and treatment initiation, provides favorable prognosis, with preservation of neurocognitive abilities and cessation of clinical and electrophysiological signs.