title: Electrical status epilepticus in sleep: clinical presentation and pathophysiology.
abstract: Electrical 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.
authors.affiliation: Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Iván
authors.initials: I
authors.lastname: Sánchez Fernández
authors.forename: Tobias
authors.initials: T
authors.lastname: Loddenkemper
authors.forename: Jurriaan M
authors.initials: JM
authors.lastname: Peters
authors.forename: Sanjeev V
authors.initials: SV
authors.lastname: Kothare
mesh_terms: D001921:Brain / Q000503:physiopathology*; D058256:Brain Waves / Q000502:physiology*; D004569:Electroencephalography; D006801:Humans; D012640:Seizures / Q000503:physiopathology*; D012890:Sleep / Q000502:physiology*; D013226:Status Epilepticus / Q000503:physiopathology*