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Epilepsy Research and Treatment
Volume 2013 (2013), Article ID 583531, 12 pages
http://dx.doi.org/10.1155/2013/583531
Review Article

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

1Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Harvard Medical School, Boston Children’s Hospital, Boston, MA 02115, USA
2Department of Child Neurology, Hospital Sant Joan de Déu, Universidad de Barcelona, 08950 Barcelona, Spain
3Department of Neurology, Children’s Hospital Colorado, University of Colorado, Aurora, CO 80045, USA

Received 25 March 2013; Accepted 7 July 2013

Academic Editor: Elaine Wirrell

Copyright © 2013 Iván Sánchez Fernández et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized in most patients by (1) difficult to control seizures, (2) interictal epileptiform activity that becomes prominent during sleep leading to an electroencephalogram (EEG) pattern of electrical status epilepticus in sleep (ESES), and (3) neurocognitive regression. In this paper, we will summarize current epidemiological, clinical, and EEG knowledge on CSWS and will provide suggestions for treatment. CSWS typically presents with seizures around 2–4 years of age. Neurocognitive regression occurs around 5-6 years of age, and it is accompanied by subacute worsening of EEG abnormalities and seizures. At approximately 6–9 years of age, there is a gradual resolution of seizures and EEG abnormalities, but the neurocognitive deficits persist in most patients. The cause of CSWS is unknown, but early developmental lesions play a major role in approximately half of the patients, and genetic associations have recently been described. High-dose benzodiazepines and corticosteroids have been successfully used to treat clinical and electroencephalographic features. Corticosteroids are often reserved for refractory disease because of adverse events. Valproate, ethosuximide, levetiracetam, sulthiame, and lamotrigine have been also used with some success. Epilepsy surgery may be considered in a few selected patients.