title: Long-term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike-waves during slow-wave sleep.
abstract: OBJECTIVE
  To present the long-term follow-up of 10 adolescents and young adults with documented cognitive and behavioral regression as children due to nonlesional focal, mainly frontal, epilepsy with continuous spike-waves during slow wave sleep (CSWS).
  
  
  METHODS
  Past medical and electroencephalography (EEG) data were reviewed and neuropsychological tests exploring main cognitive functions were administered.
  
  
  RESULTS
  After a mean duration of follow-up of 15.6 years (range, 8-23 years), none of the 10 patients had recovered fully, but four regained borderline to normal intelligence and were almost independent. Patients with prolonged global intellectual regression had the worst outcome, whereas those with more specific and short-lived deficits recovered best. The marked behavioral disorders resolved in all but one patient. Executive functions were neither severely nor homogenously affected. Three patients with a frontal syndrome during the active phase (AP) disclosed only mild residual executive and social cognition deficits. The main cognitive gains occurred shortly after the AP, but qualitative improvements continued to occur. Long-term outcome correlated best with duration of CSWS.
  
  
  CONCLUSIONS
  Our findings emphasize that cognitive recovery after cessation of CSWS depends on the severity and duration of the initial regression. None of our patients had major executive and social cognition deficits with preserved intelligence, as reported in adults with early destructive lesions of the frontal lobes. Early recognition of epilepsy with CSWS and rapid introduction of effective therapy are crucial for a best possible outcome.
authors.affiliation: Department of Neurology, University Hospital of Strasbourg, Strasbourg, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Seegmüller
authors.forename: Thierry
authors.initials: T
authors.lastname: Deonna
authors.forename: Claire Mayor
authors.initials: CM
authors.lastname: Dubois
authors.forename: Maria-Paula
authors.initials: MP
authors.lastname: Valenti-Hirsch
authors.forename: Edouard
authors.initials: E
authors.lastname: Hirsch
authors.forename: Marie-Noëlle
authors.initials: MN
authors.lastname: Metz-Lutz
authors.forename: Anne
authors.initials: A
authors.lastname: de Saint Martin
authors.forename: Eliane
authors.initials: E
authors.lastname: Roulet-Perez
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001526:Behavioral Symptoms / Q000175:diagnosis / Q000209:etiology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000523:psychology; D005260:Female; D006801:Humans; D007360:Intelligence; D007859:Learning Disabilities / Q000175:diagnosis / Q000209:etiology; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D010551:Personality; D011569:Psychiatric Status Rating Scales; D012189:Retrospective Studies; D012890:Sleep / Q000502:physiology*; D012919:Social Behavior; D055815:Young Adult