title: Epileptic encephalopathy with continuous spike-waves during sleep: the need for transition from childhood to adulthood medical care appears to be related to etiology.
abstract: Epileptic encephalopathy with continuous diffuse spike-waves during slow-wave sleep (ECSWS) presents clinically with infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic-clonic seizures. The unique electroencephalography (EEG) pattern found in ECSWS consists of continuous, diffuse, bilateral spike-waves during slow-wave sleep. Despite the eventual disappearance of clinical seizures and EEG abnormalities by adolescence, the prognosis is guarded in most cases because of neuropsychological and behavioral deficits. ECSWS has a heterogeneous etiology (genetic, structural, and unknown). Because epilepsy and electroencephalography (EEG) abnormalities in epileptic encephalopathy with continuous diffuse spike-waves during slow-wave sleep (ECSWS) are self-limited and age related, the need for ongoing medical care and transition to adult care might be questioned. For adolescents in whom etiology remains unknown (possibly genetic) and who experience the disappearance of seizures and EEG abnormalities, there is rarely need for long-term neurologic follow-up, because often a relatively normal cognitive and social evolution follows. However, the majority of patients with structural and possibly "genetic syndromic" etiologies will have persistent cognitive deficits and will need suitable socioeducative care. Therefore, the transition process in ECSWS will depend mainly on etiology and its related features (epileptic active phase duration, and cognitive and behavioral evolution) and revolve around neuropsychological and social support rather than medical and pharmacologic follow-up.
authors.affiliation: Pediatric Neurology, Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France; Referent Center for Rare Epilepsies, Associated, Strasbourg, France.
authors.forename: Anne
authors.initials: A
authors.lastname: de Saint-Martin
authors.forename: Gabrielle
authors.initials: G
authors.lastname: Rudolf
authors.forename: Caroline
authors.initials: C
authors.lastname: Seegmuller
authors.forename: Maria Paola
authors.initials: MP
authors.lastname: Valenti-Hirsch
authors.forename: Edouard
authors.initials: E
authors.lastname: Hirsch
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001921:Brain / Q000503:physiopathology*; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology* / Q000523:psychology; D006801:Humans; D012890:Sleep / Q000235:genetics / Q000502:physiology*; D060305:Transition to Adult Care; D055815:Young Adult
keywords: Epileptic encephalopathy with continuous diffuse spike-waves during slow-wave sleep; Prognosis; Transition