The penelope syndrome not always means bad prognosis

Abstract

Introduction Penelope spun during the day and undid it during the night so, which the brain makes during the day, is erased during the night by the epileptic activity. The epileptic syndromes with continuous spike-wave during slow sleep (CSWS) are a group of authentic epileptic encephalopathies and show a wide clinical spectrum with common characteristics that includes the increase and generalization of epileptifrom discharges in the EEG during sleep. In addition, patients have neuropsychological disturbances, including aphasia. The onset of these syndromes is around 4 or 5 years of age and stabilize or even improve around puberty. It is a rare syndrome that occurs exclusively in childhood, with a variable etiology. Prognosis is influenced by the age of onset and the persistence over the time of the CSWSS pattern, being worse in cases in which this abnormality appears at early ages and longer permanence. Materials and methods We report two cases of children with CSWS pattern but with different clinical presentations. Results The first one is a 6 years aged girl with a diagnosis of benign epilepsy of childhood, who changes her seizures pattern and starts to have delay in the school, showing a CSWS in the sleep EEG. She was diagnosed of atypical benign partial epilepsy of childhood, treated, with a good outcome (disappearance both seizures and CSWS pattern). This case would be the benign side of the CSWS encephalopathies spectrum. The second one is a case of a sudden acquired aphasia when the patient was 6 years old, who never suffered epileptic seizures, but shows a CSWS pattern in the EEG. He was diagnosed of Landau–Kleffner syndrome, trated with antiepileptics drugs and corticosteroids, with a benign clinical course (language recovering and EEG normalization). However this case would be at the more severe end of the spectrum. Conclusion The status epilepticus during slow sleep can cause serious neurocognitive development alterations of children affected. Early age of onset and persistence of CSWS pattern significantly influence the prognosis of these patients. Our cases would confirm this assumption, because the delayed onset and benign course, despite one of them was a case of Landau–Kleffner syndrome. In a child with sudden decrease in school performance or language difficulties would be entirely necessary to carry out a study of sleep EEG. Acknowledgements Dres. Paloma Quintana, Julia Saez and Felix Paradinas.