Syndrome of Electrical Status Epilepticus During Sleep: Epileptic Encephalopathy Related to Brain Development |
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| Institution: | 1. Neuromodulation Program, Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children''s Hospital, Boston, MA, USA;2. F.M. Kirby Neurobiology Center, Boston Children''s Hospital, Boston, MA, USA;3. Neuromodulation Program, Division of Pediatric Neurology, Department of Pediatrics, University of Iowa, Iowa City, IA, USA;4. Berenson-Allen Center for Noninvasive Brain Stimulation, Division of Cognitive Neurology, Department of Neurology, Harvard Medical School and Beth Israel Deaconess Medical Center, Boston, MA, USA;1. Serviço de Neurologia, Hospital Egas Moniz, Centro Hospitalar Lisbon Ocidental, Lisbon, Portugal;2. Neurology Department, Hospital Santa Maria, Centro Hospitalar Lisbon Norte, Lisbon, Portugal;3. Neurology Department, Hospital de Braga, Braga, Portugal;4. Neurology Department, Hospital de Cascais, Alcabideche, Portugal;5. Neurology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal;6. Neurology Department, Centro Hospitalar São João, Porto, Portugal;7. Neurology Department, Hospital Santo António dos Capuchos, Centro Hospitalar Lisbon Central, Lisbon, Portugal;8. Neurology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal;9. CEDOC, Nova Medical School, Universidade Nova de Lisbon, Lisbon, Portugal |
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| Abstract: | BackgroundEpileptic encephalopathy with electrical status epilepticus during sleep is an age-related and self-limited disorder. The present study analyzed the etiology, demographics, and pathogenesis of patients with electrical status epilepticus during sleep to provide information on the diagnosis and therapy of this syndrome.MethodsThe etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in patients admitted in Chinese People's Liberation Army General Hospital from 2009 to 2014 were retrospectively analyzed. Patients were classified into the genetic, structural-metabolic, and unknown groups according to the etiology. Demographics and clinical characteristics of all the patients were then analyzed and compared among groups.ResultsThe etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in 75 patients mainly included benign childhood epilepsy with centrotemporal spikes, Landau-Kleffner syndrome, polymicrogyria, and migration disorders. Age at onset of epilepsy did not show a specific pattern, but age at onset of epileptic encephalopathy with electrical status epilepticus during sleep was concentrated at age 6-9 years. The mean age at onset of epilepsy in the genetic group was significantly older than that in the structural-metabolic group (P < 0.05). Age at onset of epileptic encephalopathy with electrical status epilepticus during sleep did not significantly differ between the two groups.ConclusionsElectrical status epilepticus during sleep is an epileptic encephalopathy related to brain development and presents an age-dependent occurrence. |
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| Keywords: | electrical status epilepticus during sleep etiology children genetic structural-metabolic |
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