• Iván Sánchez Fernández, Tobias Loddenkemper, Jurriaan M Peters, and Sanjeev V Kothare.
• Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
• Pediatr. Neurol. 2012 Dec 1; 47 (6): 390-410.

AbstractElectrical status epilepticus in sleep involves an electroencephalographic pattern where interictal epileptiform activity is potentiated in the transition from wakefulness to sleep. Near-continuous spikes and waves that occupy a significant proportion of nonrapid eye movement sleep appear as a result of sleep-potentiated epileptiform activity. This electroencephalographic pattern appears in different electroclinical syndromes that present three common characteristics with different degrees of severity: seizures, sleep-potentiated epileptiform activity, and neuropsychologic regression. Continuous spikes and waves during sleep comprise the severest epileptic encephalopathy in the electroclinical spectrum. Landau-Kleffner syndrome presents with intermediate severity. Some "benign" pediatric focal epileptic syndromes represent the mildest end of this continuum. Based on published data, we provide a framework for clinical and electrical events. The underlying mechanisms leading to sleep potentiation of epileptiform activity in electrical status epilepticus in sleep are incompletely understood. A genetic basis or acquired early developmental insult may disrupt the normal maturation of neuronal networks. These factors may dynamically alter normal processes of brain development, leading to an age-related pattern of electroclinical expression of electrical status epilepticus in sleep.Copyright © 2012 Elsevier Inc. All rights reserved.

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